This document contains questions and answers about various topics related to cell biology and histology. It discusses different cell types (permanent, stable, labile cells), organelles like the endoplasmic reticulum and its functions, the cell cycle, vesicular trafficking proteins, microtubules, cilia, the plasma membrane, collagen synthesis and types, Ehlers-Danlos syndrome, and Osteogenesis Imperfecta. It also provides immunohistochemical stains for different cell and tissue types.
This document contains questions and answers about various topics related to cell biology and histology. It discusses different cell types (permanent, stable, labile cells), organelles like the endoplasmic reticulum and its functions, the cell cycle, vesicular trafficking proteins, microtubules, cilia, the plasma membrane, collagen synthesis and types, Ehlers-Danlos syndrome, and Osteogenesis Imperfecta. It also provides immunohistochemical stains for different cell and tissue types.
This document contains questions and answers about various topics related to cell biology and histology. It discusses different cell types (permanent, stable, labile cells), organelles like the endoplasmic reticulum and its functions, the cell cycle, vesicular trafficking proteins, microtubules, cilia, the plasma membrane, collagen synthesis and types, Ehlers-Danlos syndrome, and Osteogenesis Imperfecta. It also provides immunohistochemical stains for different cell and tissue types.
Q0002:Rough Endoplasmic Reticulum rich cells -Mucus-secreting goblet cells of small intestine;-Antibody- secreting plasma cells;(ie lost of secretory protein) Q0003:Cell cycle: Permanent cells Remain in G0. If they regenerate; they regenerate from stem cells. Q0004:Cell cycle: Stable cells Enter G1 from G0 when stimulated Q0005:Cell cycle: Labile cells Never go to G0; divide rapidly with a short G1 Q0006:Smooth Endoplasmic Reticulum: Functions -Steroid synthesis;-Detoxification of drugs and poisons Q0007:Permanent cells: cell types -Neurons;-Skeletal and cardiac muscle;-RBCs (regenerate from stem cells) Q0008:Stable cells: cell types -Hepatocytes;-Lymphocytes Q0009:Smooth Endoplasmic Reticulum rich cells -Liver hepatocytes;-Steroid hormone-producing cells of the adrenal cortex Q0010:Labile cells: cell types -Bone marrow;-Gut epithelium;-Skin;-Hair follicles;(think about chemotherapy side effects) Q0011:Rough Endoplasmic Reticulum: Functions -Synthesis of secretory (exported) proteins;-N-linked oligosaccharide addition to many proteins Q0012:Neurons: name of Rough Endoplasmic Reticulum and its function Nissl bodies sytnhesize enzymes (eg ChAT) and peptide neurotransmitters. Q0013:6 functions of Golgi apparatus 1. Distribution center;--Input: Proteins and lipids from endoplasmic reticulum;--Output: Plasma membrane; lysosomes; secretory vesicles;2. Modifies N-oligosaccharides on asparagine;3. Adds O-oligosaccharides to serine and threonine residues (on protein);4. Addition of mannose-6- phosphate to proteins designated for lysosome;5. Proteoglycan assembly from core proteins;6. Sulfation of sugars in proteoglycans and of selected tyrosine on proteins Q0014:I-cell disease: Presentation -coarse facial features;-clouded corneas;-restricted joint movement;-high plasma levels of lysosomal enzymes;-fatal in childhood Q0015:3 types of vesicular trafficking proteins COPI; COPII; and Clathrin Q0016:COPI: Function Retrograde Intracellular transport: cis-Golgi to RER Q0017:COPII: Function Anterograde Intracellular transport: RER to cis-Golgi Q0018:Clathrin: Function Extracellular/lysosomal transport;-lysosome: trans-Golgi to lysosome ;-trans-Golgi to plasma membrane to endosomes (for receptor mediated endocytosis) Q0019:Microtubules: Diameter 24 nm Q0020:Microtubules: Structure 13 dimers of alpha/beta tubulin per circumference (each dimer bound to GTP); repeated in helical configuration Q0021:Microtubules: Found in ? -Flagella;-Cilia;-Mitotic spindles;-Slow neuronal axoplasmic transport Q0022:Microtubules: Speed of growth and collapse Grows slowly and collapses quickly Q0023:Microtubules: Affected by which drugs? -Mebendazole/thiabendazole (antihelminthic);-Taxol (anti- breast cancer);-Griseofulvin (anti-fungal);- Vincristine/Vinblastine (anti-cancer);-Colchicine (anti-gout) Q0024:Microtubule polymerization defects: Found in what syndrome Chediak-Higashi syndrome Q0025:Chediak-Higashi syndrome Microtubule polymerization defect resulting in decreased phagocytosis Q0026:Cilia: Structure -9+2 arrangement of microtubule doublets;-The 9 peripheral doublets are linked by dynein atpase Q0027:Cilia: Mechanism The 9 peripheral doublets are linked by dynein atpase; which causes bending of cilium by differential sliding of doublets Q0028:Molecular motors and direction of transport in cell Dynein: retrograde (DIES back towards nucleus);Kinesin: anterograde (MOVES kinetically forward) Q0029:Kartagener's syndrome: Defect Dynein arm defect causing immotile cilia Q0030:What is phosphatidylcholine also known as? lecithin Q0031:Kartagener's syndrome: Presentation -Male infertility (immotile sperm);-Female infertility (immotile fallopian cilia);-Bronchiectasis and recurrent sinusitis (bacteria and particles not pushed out);-Situs inversus Q0032:What syndrome is situs inversus associated with? Kartagener's Q0033:Plasma membrane: composition -Cholesterol (~50%);-Phospholipids (~50%);-Sphingolipids;- Glycolipids;-Proteins Q0034:What is lecithin also known as? phosphatidylcholine Q0035:Plasma membrane: melting temperature association High cholesterol or long saturated fatty acid content means increased melting temperature Q0036:Where is phosphatidylcholine found? Major component of;1. RBC membranes;2. myelin;3. bile;4. surfactant (DPPC- dipalmitoyl phosphatidylcholine) Q0037:Drugs that inhibit the sodium-potassium pump -Ouabain;-Cardiac glycosides (digoxin; digitoxin) Q0038:Ouabain: Mechanism Inhibits Na-K atpase by binding to K site. Q0039:What process uses phosphatidylcholine? Esterification of cholesterol with LCAT (lecithin-cholesterol acyltransferase) Q0040:Cardiac glycosides: Mechanism Inhibits Na-K atpase; increasing cardiac contractility Q0041:True or False: Collagen is not the most abundant protein in the body? False; it is. (25% of all protein in the human body is collagen) Q0042:True or False: Collagen is the most abundant protein in the body? True. (25% of all protein in the human body is collagen) Q0043:Collagen types and their primary locations Be (So Totally) Cool; Read Books;1. (90% of all collagen) Bone; Skin; Tendon (bONE);2. Cartilage (carTWOlage);3. (Reticulin)-blood vessels;4. Basement membrane Q0044:Collagen Type I locations Be (So Totally) cool; read books;-Bone (bONE);-Skin;- Tendon;-dentin;-fascia;-cornea;-late wound repair Q0045:Collagen Type II locations be (so totally) COOL; read books;-Cartilage (carTWOlage);- vitreous body;-nucleus pulposus Q0046:Collagen Type III locations be (so totally) cool; READ books;(Reticulin);-blood vessels;- skin;-uterus;-fetal tissue;-granulation tissue Q0047:Collagen Type IV locations be (so totally) cool; read BOOKS;-basement membrane or basal lamina;(four under the floor) Q0048:Collagen synthesis: list of events and locations Intracellular;1. Synthesis (Rough Endoplasmic Reticulum);2. Hydroxylation (Endoplasmic Reticulum);3. Glycosylation (Golgi);4. Exocytosis;Extracellular;5. Proteolytic processing;6. Cross-linking Q0049:Collagen production step 1: Synthesis -In rough endoplasmic reticulum;-translation of collagen alpha chains (aka preprocollagen) Q0050:Composition and other name of collagen alpha-chains -Gly-X-Y (where X and Y are proline; hydroxyproline; or hydroxylysine);-aka preprocollagen Q0051:Composition and other name of preprocollagen -Gly-X-Y (where X and Y are proline; hydroxyproline; or hydroxylysine);-aka collagen alpha-chains Q0052:Collagen production step 2: Hydroxylation -In endoplasmic reticulum;-Hydroxylation of specific proline and lysine residues (using vitamin C) Q0053:Collagen production step for which vitamin C is required Hydroxylation of Gly-X-Y chains to form hydroxyproline or hydroxylysine Q0054:Collagen production step 3: Glycosylation -In Golgi apparatus;-Glycosylation of preprocollagen lysine residues and formation of procollagen (triple helix of three collagen alpha chains) Q0055:Composition of procollagen Triple helix of three collagen alpha chains (aka three preprocollagen chains) Q0056:Osteogenesis Imperfecta: Pathophysiology Cannot take glycosylated alpha-chains and form procollagen (triple helix) Q0057:Collagen production step 4: Exocytosis Exocytosis of procollagen Q0058:Collagen production step 5: Proteolytic processing Cleavage of terminal regions of procollagen; transforming it into insoluble tropocollagen Q0059:Composition of tropocollagen Procollagen (triple-helix) with terminal regions cleaved Q0060:Collagen production step 6: Cross-linking Reinforcement of many staggered tropocollagen molecules by covalent lysine-hydroxylysine cross-linkage to make collagen fibrils Q0061:Enzyme which performs collagen cross-linking (final step) Lysyl oxidase Q0062:Ehlers-Danlos syndrome: Pathophysiology Nonfunctioning lysyl oxidase resulting in lack of collagen fibrils (linked tropocollagen molecules) Q0063:Ehlers-Danlos syndrome: Presentation -Hyperextensible skin;-Tendency to bleed/easy bruising;- Hypermobile joints Q0064:Ehlers-Danlos syndrome: # of types 10 Q0065:Ehlers-Danlos syndrome: Inheritance Varies Q0066:Ehlers-Danlos syndrome: Most frequently affected collagen type and result Type III collagen resulting in blood vessel instability Q0067:Osteogenesis Imperfecta: Types -Abnormal collagen type I (bONE);--Most common;-- Autosomal-dominant;-Type II;--Fatal in utero or neonatal period Q0068:Osteogenesis Imperfecta: Presentation 1. Brittle bone disease: Multiple fractures with minimal trauma (often during birth);2. Blue sclearae (due to translucency of connective tissue over the choroid);3. Hearing loss (abnormal middle ear bones);4. Dental imperfections due to lack of dentition Q0069:Multiple fractures in a child: Differential Diagnosis -Child abuse;-Osteogenesis Imperfecta Q0070:Osteogenesis Imperfecta: Incidence 1:10;000 Q0071:Immunohistochemical stain for: Connective tissue Vimentin Q0072:Immunohistochemical stain for: Muscle Desmin Q0073:Immunohistochemical stain for: Epithelial cells Cytokeratin Q0074:Immunohistochemical stain for: Neuroglia GFAP (Glial fibrillary acid proteins) Q0075:Immunohistochemical stain for: Neurons Neurofilaments Q0076:Vimentin stains for: Connective tissue Q0077:Desmin stains for: Muscle Q0078:Cytokeratin stains for: Epithelial cells Q0079:GFAP (Glial fibrillary acid proteins) stains for: Neuroglia Q0080:Neurofilaments stain for: Neurons Q0081:Elastin: Description and Location Stretchy protein within lungs; large arteries; elastic ligaments Q0082:Elastin and Collagen: Peptide composition difference Both: Proline and lysine rich;Collagen: Hydroxylated forms;Elastin: Non-hydroxylated forms Q0083:Elastin: Structure Tropoelastin with fibrillin scaffolding (fibrillin defect in Marfan's syndrome) Q0084:Elastin: Conformations Relaxed and stretched Q0085:Elastase: function Break down elastin Q0086:Elastase inhibitor alpha-1-antitrypsin Q0087:Elastase excess: Found where Found in emphysema Q0088:Association of emphysema with elastase Excess elastase activity can cause emphysema Q0089:Apoptosis: Characteristics 1. Cell shrinkage;2. Chromatin condensation;3. Membrane blebbing;4. Formation of apoptotic bodies which are then phagocytosed Q0090:Apoptosis: Events which initiate it -Embryogenesis;-Hormone induction (menstruation);-Immune cell-mediated death;-Injurious stimuli (eg radiation; hypoxia);- Atrophy Q0091:Necrosis: Definition Enzymatic degradation of a cell resulting from exogenous injury Q0092:Necrosis: Characteristics 1. Enzymatic digestion;2. Protein denaturation;3. Release of intracellular components;4. Inflammatory process. Q0093:Necrosis: Types and where they are found 1. Coagulative (heart; liver; kidney);2. Liquefactive (brain);3. Caseous (Tuberculosis);4. Fat (Pancreas);5. Fibrinoid (blood vessels);6. Gangrenous (limbs; GI tract) Q0094:Reversible or irreversible cell injury: Cellular swelling Reversible Q0095:Reversible or irreversible cell injury: Nuclear chromatin clumping Reversible Q0096:Reversible or irreversible cell injury: Decreased ATP synthesis Reversible Q0097:Reversible or irreversible cell injury: Ribosomal detachment Reversible Q0098:Reversible or irreversible cell injury: Glycogen depletion Reversible Q0099:Reversible or irreversible cell injury: Plasma membrane damage Irreversible Q0100:Reversible or irreversible cell injury: Lysosomal rupture Irreversible Q0101:Reversible or irreversible cell injury: Calcium influx leading to oxidative phosphorylation Irreversible Q0102:Reversible or irreversible cell injury: Nuclear pyknosis Irreversible Q0103:Reversible or irreversible cell injury: Karyolysis Irreversible Q0104:Reversible or irreversible cell injury: Karyorrhexis Irreversible Q0105:Reversible or irreversible cell injury: Mitochondrial permeability Irreversible Q0106:Inflammation: Classic signs -Rubor (redness);-Dolor (pain);-Calor (heat);-Tumor (Swelling);-Functio lassa (Loss of function) Q0107:Characteristics of Inflammation: Fluid exudation 1. Increased vascular permeability;2. Vasodilation;3. Endothelial injury Q0108:Characteristics of Inflammation: Leukocyte activation 1. Emigration;2. Chemotaxis;3. Phagocytosis and killing Q0109:Characteristics of Inflammation: Fibrosis 1. Fibroblast emigration and proliferation;2. Deposition of extracellular material Q0110:Characteristics of Acute Inflammation Mediated by;1. Neutrophils;2. Eosinophils;3. Antibodies Q0111:Characteristics of Chronic Inflammation Mediated by mononuclear cells;-Characterized by persistant destruction and repair;-Granulomas: nodular collections of macrophages and giant cells Q0112:Characteristics of Inflammation: Resolution 1. Restoration of normal structure;2. Granulation tissue;3. Abscess;4. Fistula;5. Scarring Q0113:Granulation tissue: histologic characteristics -highly vascularized;-fibrotic Q0114:Abscess: histologic characteristics fibrosis surrounding pus Q0115:Fistula: characteristics abnormal communication Q0116:Scarring: histologic characteristics Collagen deposition resulting in altered structure and function Q0117:Steps in leukocyte emigration 1. Rolling;2. Tight binding;3. Diapedisis;4. Migration Q0118:Leukocyte emigration step characteristics: Rolling Binding between;-E-selectin on vascular endothelium;-Sialyl- LewisX on the leukocyte Q0119:Leukocyte emigration step characteristics: Tight binding binding between;-ICAM-1 on vascular endothelium ;-LFA-1 on leukocyte Q0120:Leukocyte emigration step characteristics: Diapedesis leukocyte travels between endothelial cells and exits blood vessel Q0121:Leukocyte emigration step characteristics: Migration Leukocyte travels through interstitium to the site of injury or infection guided by chemotactic signals Q0122:Chemotactic signals 1. Bacterial products;2. Complement;3. Chemokines Q0123:Free radical injury: Initiated by what? 1. Radiation exposure;2. Metabolism of drugs (phase I);3. Redox reactions;4. Nitric oxide;5. Transition metals;6. Leukocyte oxidative burst Q0124:Free radical injury: Mechanism -Membrane lipid peroxidation;-Protein modification;-DNA breakage Q0125:Free radical injury: Stopped by what? -Spontaneous decay;-Antioxidants;--Vitamin E;--Vitamin A;- Enzymes;--Catalase;--Superoxide dismutase;--Glutathione peroxidase Q0126:Major cause of injury after thrombolytic therapy Free-radical production induced by reperfusion after anoxia Q0127:Hyperplasia: definition reversible increase in number of cells Q0128:Metaplasia: definition Reversible substitution of one cell type for another. Q0129:Metaplasia: When found? Often secondary to irritation and/or environmental exposure (eg squamous metaplasia in trachea and bronchi of smokers) Q0130:Dysplasia: definition Reversible abnormal growth with loss of cellular orientation; shape and size in comparison to normal tissue maturation Q0131:Dysplasia: When found? In paraneoplastic syndromes Q0132:Anaplasia: definition abnormal cells lacking differentiation; like primitive cells of same tissue. Q0133:Anaplasia: When found? Undifferentiated malignancies Q0134:Neoplasia: Definition A clonal proliferation of cells that is uncontrolled and excessive Q0135:Difference between hyperplasia and dysplasia Can occur together;1. hyperplasia - increase in number;2. dysplasia - abnormal proliferation of cells with loss of size; shape; and orientation Q0136:Appearance of cancerous cells -High nuclear/cytoplasmic ratio;-Clumped chromatin Q0137:How does carcinoma invade a basement membrane? -Collagenases;-Hydrolases Q0138:Seed and soil theory of metastasis Seed: Tumor embolus;Soil: Target organ Q0139:Tumor grade: definition Degree of cellular differentiation based on histologic appearance of tumor Q0140:Tumor stage: definition Degree of localization/spread based on site and size of primary lesion; spread to regional lymph nodes; and presence of metastases Q0141:Tumor grade vs stage: Quick characterization grade: character of tumor itself;stage: spread of tumor in a specific patient (Stage=Spread) Q0142:Tumor grade vs stage: Which has more prognostic value? Stage Q0143:TNM staging system Tumor size;Node involvement;Metastases Q0144:Benign tumors of cell type: Blood cells Does not exist. The malignant blood cells are automatically metastasizing. Q0145:Benign tumors of cell type: Blood vessels Hemangioma Q0146:Benign tumors of cell type: Smooth muscle Leiomyoma Q0147:Benign tumors of cell type: Skeletal muscle Rhabdomyoma Q0148:Benign tumors of cell type: Bone Osteoma Q0149:Benign tumors of cell type: Fat Lipoma Q0150:Benign tumors of cell type: >1 cell type Mature teratoma (women) Q0151:Benign tumors of cell type: Epithelium -Adenoma;-Papilloma Q0152:Malignant tumor of cell type: Epithelium -Adenocarcinoma;-Papillary carcinoma Q0153:Malignant tumor of cell type: Blood cells -Leukemia;-Lymphoma Q0154:Malignant tumor of cell type: Blood vessels Angiosarcoma Q0155:Malignant tumor of cell type: Smooth muscle Leiomyosarcoma Q0156:Malignant tumor of cell type: Skeletal muscle Rhabdomyosarcoma Q0157:Malignant tumor of cell type: Bone Osteosarcoma Q0158:Malignant tumor of cell type: Fat Liposarcoma Q0159:Malignant tumor of cell type: >1 cell type Immature teratoma; Mature teratoma (men only) Q0160:Name the cell type: Adenoma Epithelium Q0161:Name the cell type: Papilloma Epithelium Q0162:Name the cell type: Adenocarcinoma Epithelium Q0163:Name the cell type: Papillary carcinoma Epithelium Q0164:Name the cell type: Leukemia Blood cells Q0165:Name the cell type: Lymphoma Blood cells Q0166:Name the cell type: Hemangioma Blood vessels Q0167:Name the cell type: Angiosarcoma Blood vessels Q0168:Name the cell type: Leiomyoma Smooth muscle Q0169:Name the cell type: Leiomyosarcoma Smooth muscle Q0170:Name the cell type: Rhabdomyoma Skeletal muscle Q0171:Name the cell type: Rhabdomyosarcoma Skeletal muscle Q0172:Name the cell type: Osteoma Bone Q0173:Name the cell type: Osteosarcoma Bone Q0174:Name the cell type: Lipoma Fat Q0175:Name the cell type: Liposarcoma Fat Q0176:Name the cell type: Teratoma >1 cell type Q0177:Neoplasm associated with: Down syndrome ALL (we ALL fall Down); AML Q0178:Neoplasm associated with: Xeroderma pigmentosum Melanoma and basal/squamous cell carcinomas of the skin Q0179:Neoplasm associated with: albinism Melanoma and basal/squamous cell carcinomas of the skin Q0180:Neoplasm associated with: Chronic atrophic gastritis Gastric adenocarcinoma Q0181:Neoplasm associated with: Pernicious anemia Gastric adenocarcinoma Q0182:Neoplasm associated with: Post surgical gastric remnants Gastric adenocarcinoma Q0183:Neoplasm associated with: Tuberous sclerosis (facial angiofibroma; seizures; mental retardation) Astrocytoma and cardiac rhabdomyoma Q0184:Neoplasm associated with: Actinic Keratosis Squamous Cell Carcinoma of skin Q0185:Neoplasm associated with: Barrett's esophagus Esophageal adenocarcinoma Q0186:Neoplasm associated with: Plummer-Vinson syndrome (atrophic glossitis; esophageal webs; anemia; all due to iron deficiency) Squamous Cell carcinoma of the esophagus Q0187:Neoplasm associated with: Cirrhosis (alcoholic; hepatitis B and C) Hepatocellular carcinoma Q0188:Neoplasm associated with: Ulcerative colitis Colonic adenocarcinoma Q0189:Neoplasm associated with: Paget's disease of bone Secondary osteosarcoma and fibrosarcoma Q0190:Neoplasm associated with: Immunodeficiency states Malignant lymphomas Q0191:Neoplasm associated with: AIDS Aggressive malignant lymphomas (non-Hodgkin's) and Kaposi's sarcoma Q0192:Neoplasm associated with: Autoimmune diseases Benign/malignant thymomas Q0193:Neoplasm associated with: Acanthosis nigricans (hyperpigmentation and epidermal thickening) Visceral malignancy (stomach; lung; breast; uterus) Q0194:Neoplasm associated with: Dysplastic nevus Malignant melanoma Q0195:Condition associated with: ALL Down syndrome (we ALL fall Down) Q0196:Condition associated with: AML Down syndrome Q0197:Condition associated with: Melanoma Xeroderma pigmentosum and albinism;If malignant; dysplastic nevus Q0198:Condition associated with: Basal cell carcinoma of skin Xeroderma pigmentosum and albinism Q0199:Condition associated with: Squamous cell carcinoma of skin Actinic keratosis; Xeroderma pigmentosum and albinism Q0200:Condition associated with: Gastric adenocarcinoma 1. Chronic atrophic gastritis;2. Pernicious anemia;3. Postsurgical gastric remnants Q0201:Condition associated with: Astrocytoma Tuberous sclerosis (facial angiofibroma; seizures; mental retardation) Q0202:Condition associated with: Cardiac rhabdomyoma Tuberous sclerosis (facial angiofibroma; seizures; mental retardation) Q0203:Condition associated with: Esophageal adenocarcinoma Barrett's esophagus Q0204:Condition associated with: Squamous cell carcinoma of esophagus Plummer-Vinson syndrome Q0205:Plummer-Vinson syndrome: Presentation -Atrophic glossitis;-Esophageal webs;-Anemia Q0206:Plummer-Vinson syndrome: Cause Iron deficiency Q0207:Condition associated with: Hepatocellular carcinoma Cirrhosis due to;1) alcohol;2) hepatitis B or C Q0208:Condition associated with: Colonic adenocarcinoma Ulcerative colitis Q0209:Condition associated with: Secondary osteosarcoma Paget's disease of bone Q0210:Condition associated with: Fibrosarcoma Paget's disease of bone Q0211:Condition associated with: Malignant lymphomas Immunodeficiency states Q0212:Condition associated with: aggressive malignant non- Hodgkin's lymphoma AIDS Q0213:Condition associated with: Kaposi's sarcoma AIDS Q0214:Condition associated with: Benign thymoma Autoimmune diseases (eg Hashimoto's thyroiditis; myasthenia gravis) Q0215:Condition associated with: Malignant thymoma Autoimmune diseases (eg Hashimoto's thyroiditis; myasthenia gravis) Q0216:Condition associated with: Visceral malignancy Acanthosis nigricans Q0217:Acanthosis nigricans: Presentation -Hyperpigmentation;-Epidermal thickening Q0218:Tumor associated with this gene: abl CML Q0219:Tumor associated with this gene: c-myc Burkitt's lymphoma Q0220:Tumor associated with this gene: bcl-2 Follicular and undifferentiated lymphomas (inhibits apoptosis) Q0221:Tumor associated with this gene: erb-B2 Breast; ovarian; and gastric carcinomas Q0222:Tumor associated with this gene: ras Colon carcinoma Q0223:Tumor associated with this gene: L-myc Lung tumor (L for Lung) Q0224:Tumor associated with this gene: N-myc Neuroblastoma (N for Neuroblastoma) Q0225:Tumor associated with this gene: ret Multiple endocrine neoplasia types II and III Q0226:Gene associated with this tumor: CML oncogene abl (philadelphia chromosome) Q0227:Gene associated with this tumor: Burkitt's lymphoma oncogene c-myc Q0228:Gene associated with this tumor: Follicular lymphoma oncogene bcl-2 (inhibits apoptosis) Q0229:Gene associated with this tumor: Undifferentiated lymphoma oncogene bcl-2 (inhibits apoptosis) Q0230:Gene associated with this tumor: Breast carcinoma oncogene erb-B2 Q0231:Gene associated with this tumor: Ovarian carcinoma oncogene erb-B2 Q0232:Gene associated with this tumor: Gastric carcinoma oncogene erb-B2 Q0233:Gene associated with this tumor: Colon carcinoma oncogene ras Q0234:Gene associated with this tumor: Lung tumor oncogene L-myc (L for Lung) Q0235:Gene associated with this tumor: Neuroblastoma oncogene N-myc (N for neuroblastoma) Q0236:Gene associated with this tumor: Multiple Endocrine Neoplasia type I oncogene MEN1 encoding menin on chromosome 11q Q0237:Gene associated with this tumor: Multiple Endocrine Neoplasia type II oncogene ret Q0238:Gene associated with this tumor: Multiple Endocrine Neoplasia type III oncogene ret Q0239:Chromosome and tumor associated with this gene (what type of gene): Rb Tumor suppressor gene on 13q: Retinoblastoma; osteosarcoma Q0240:Chromosome and tumor associated with this gene (what type of gene): BRCA1 Tumor suppressor gene on 17q: Breast and ovarian cancer Q0241:Chromosome and tumor associated with this gene (what type of gene): BRCA2 Tumor suppressor gene on 13q: Breast and ovarian cancer Q0242:Chromosome and tumor associated with this gene (what type of gene): p53 Tumor suppressor gene on 17p: Most human cancers and Li- Fraumeni Syndrome Q0243:Chromosome and tumor associated with this gene (what type of gene): p16 Tumor suppressor gene on 9p: Melanoma Q0244:Chromosome and tumor associated with this gene (what type of gene): APC Tumor suppressor gene on 5q: Colorectal cancer Q0245:Chromosome and tumor associated with this gene (what type of gene): WT1 Tumor suppressor gene on 11q: Wilm's tumor Q0246:Chromosome and tumor associated with this gene (what type of gene): NF1 Tumor suppressor gene on 17q: Neurofibromatosis type 1 Q0247:Chromosome and tumor associated with this gene (what type of gene): NF2 Tumor suppressor gene on 22q: Neurofibromatosis type 2 Q0248:Chromosome and tumor associated with this gene (what type of gene): DPC Tumor suppressor gene on 18q: Pancreatic cancer Q0249:Chromosome and tumor associated with this gene (what type of gene): DCC Tumor suppressor gene on 18q: Colon cancer Q0250:Gene and associated tumor on chromosome: 5q Tumor suppressor gene APC: Colorectal cancer Q0251:Gene and associated tumor on chromosome: 9p Tumor suppressor gene p16: melanoma Q0252:Gene and associated tumor on chromosome: 11q Tumor suppressor gene WT1: Wilm's tumor Q0253:Gene and associated tumor on chromosome: 13q Tumor suppressor genes Rb (Retinoblastoma; Osteosarcoma) and BRCA2 (Breast and Ovarian cancer) Q0254:Gene and associated tumor on chromosome: 17p Tumor suppressor gene p53: Most human cancers and Li- Fraumeni syndrome Q0255:Gene and associated tumor on chromosome: 17q Tumor suppressor genes BRCA1 (Breast and ovarian cancer) and NF1 (Neurofibromatosis type 1) Q0256:Gene and associated tumor on chromosome: 18q Tumor suppressor genes DPC (Pancreatic cancer) and DCC (Colon cancer) Q0257:Gene and associated tumor on chromosome: 22q Tumor suppressor gene NF2: Neurofibromatosis type 2 Q0258:Gene and matching chromosome associated with: Retinoblastoma Tumor suppressor gene Rb on chromosome 13q Q0259:Gene and matching chromosome associated with: Osteosarcoma Tumor suppressor gene Rb on chromosome 13q Q0260:Gene and matching chromosome associated with: Breast cancer Tumor suppressor genes BRCA1 (17q) and BRCA2 (13q) Q0261:Gene and matching chromosome associated with: Ovarian cancer Tumor suppressor genes BRCA1 (17q) and BRCA2 (13q) Q0262:Gene and matching chromosome associated with: Most human cancers Tumor suppressor gene p53 on chromosome 17p Q0263:Gene and matching chromosome associated with: Li- Fraumeni syndrome Tumor suppressor gene p53 on chromosome 17p Q0264:Gene and matching chromosome associated with: Melanoma Tumor suppressor gene p16 on chromosome 9p Q0265:Gene and matching chromosome associated with: Colorectal cancer Tumor suppressor gene APC on chromosome 5q Q0266:Gene and matching chromosome associated with: Wilms' tumor Tumor suppressor gene WT1 on chromosome 11q Q0267:Gene and matching chromosome associated with: Neurofibromatosis type 1 Tumor suppressor gene NF1 on chromosome 17q Q0268:Gene and matching chromosome associated with: Neurofibromatosis type 2 Tumor suppressor gene NF2 on chromosome 22q Q0269:Gene and matching chromosome associated with: Pancreatic cancer Tumor suppressor gene DPC on chromosome 18q Q0270:Gene and matching chromosome associated with: Colon cancer Tumor suppressor gene DCC on chromosome 18q Q0271:Oncogenes: gain or loss of function QUICK onco: gain Q0272:tumor suppressor genes: gain or loss of function QUICK tumor suppressor: loss Q0273:Oncogenes: one or two alleles need to be damaged QUICK One (gain of function) Q0274:Tumor suppressor genes: one or two alleles need to be damaged QUICK Two (loss of function) Q0275:Association for tumor marker: PSA Screening for Prostatic Carcinoma Q0276:Association for tumor marker: CEA -Carcinoembryonic antigen;-In ~70% of colorrectal and pancreatic cancers;-Also produced by gastric and breast carcinomas;-Very nonspecific Q0277:Association for tumor marker: alpha-fetoprotein -Hepatocellular carcinomas;-Nonseminomatous germ cell tumors of the testis (eg yolk sac tumor) Q0278:Association for tumor marker: beta-hCG -Hydatidiform moles;-Choriocarcinomas;-Gestational trophoblastic tumors Q0279:Association for tumor marker: CA-125 Ovarian malignant epithelial tumors Q0280:Association for tumor marker: S-100 -Melanoma;-Neural tumors;-Astrocytomas Q0281:Association for tumor marker: Alkaline phosphatase -Metastases to bone;-Obstructive biliary disease;-Paget's disease of bone Q0282:Association for tumor marker: Bombesin -Neuroblastoma;-Lung cancer;-Gastric cancer Q0283:Association for tumor marker: TRAP -Tartrate-resistance acid phosphatase;-Found in hairy cell leukemia Q0284:What type of cell is found in hairy cell leukemia? B-cells Q0285:Association for tumor marker: CA-19-9 Pancreatic adenocarcinoma Q0286:Tumor marker for: Prostatic carcinoma -PSA;-Prostatic acid phosphatase Q0287:Tumor marker for: Colorectal cancer CEA - Carcinoembryonic antigen;-Produced by 70% of colorectal cancers Q0288:Tumor marker for: Pancreatic cancer CEA - Carcinoembryonic antigen;-Produced by 70% of pancreatic cancers;CA-19-9;-Produced in pancreatic adenocarcinoma Q0289:Tumor marker for: Gastric carcinoma -CEA - Carcinoembryonic antigen;-Bombesin Q0290:Tumor marker for: Breast carcinoma CEA - Carcinoembryonic antigen Q0291:Tumor marker for: Hepatocellular carcinoma alpha-fetoprotein Q0292:Tumor marker for: Nonseminomatous germ cell tumors of the testis alpha-fetoprotein Q0293:Tumor marker for: Hydatidiform moles beta-hCG Q0294:Tumor marker for: Choriocarcinoma beta-hCG Q0295:Tumor marker for: Gestational Trophoblastic tumors beta-hCG Q0296:Tumor marker for: Malignant ovarian epithelial tumors CA-125 Q0297:Tumor marker for: Melanoma S-100 Q0298:Tumor marker for: Neural tumors General: S-100;Neuroblastoma: Bombesin Q0299:Tumor marker for: Astrocytomas S-100 Q0300:Tumor marker for: Metastases to bone Alkaline phosphatase Q0301:Tumor marker for: Obstructive biliary disease Alkaline phosphatase Q0302:Tumor marker for: Paget's disease of bone Alkaline phosphatase Q0303:Tumor marker for: Neuroblastoma Bombesin Q0304:Tumor marker for: Lung cancer Bombesin Q0305:Tumor marker for: Hairy cell leukemia TRAP (Tartrate-resistant acid phosphatase) Q0306:Tumor marker for: Pancreatic adenocarcinoma CA-19-9 Q0307:Cancer associated with: HTLV-1 HTLV=Human T-Lymphotropic Virus;Adult T-cell leukemia Q0308:Cancer associated with: HBV Hepatocellular carcinoma Q0309:Cancer associated with: HCV Hepatocellular carcinoma Q0310:Cancer associated with: EBV -Burkitt's lymphoma;-Nasopharyngeal carcinoma Q0311:Cancer associated with: HPV -Cervical carcinoma (with HPV 16;18);-Penile/anal carcinoma Q0312:Cancer associated with: HHV-8 HHV-8 = Kaposi's sarcoma-associated herpes virus;-Kaposi's sarcoma;-body cavity fluid B-cell lymphoma Q0313:Oncogenic virus associated with: Adult T-cell leukemia HTLV-1 (HTLV=Human T-Lymphotropic Virus) Q0314:Oncogenic virus associated with: Hepatocellular carcinoma -HBV ;-HCV Q0315:Oncogenic virus associated with: Burkitt's lymphoma EBV Q0316:Oncogenic virus associated with: Nasopharyngeal carcinoma EBV Q0317:Oncogenic virus associated with: Cervical carcinoma HPV 16 and 18 Q0318:Oncogenic virus associated with: Penile carcinoma HPV Q0319:Oncogenic virus associated with: Anal carcinoma HPV Q0320:Oncogenic virus associated with: Kaposi's sarcoma HHV-8 (aka Kaposi's sarcoma-associated herpesvirus) Q0321:Oncogenic virus associated with: body cavity fluid B- cell lymphoma HHV-8 (Human Herpesvirus 8) Q0322:Organ affected by: Aflatoxins Liver (hepatocellular carcinoma) Q0323:Organ affected by: Vinyl chloride Liver (angiosarcoma) Q0324:Organ affected by and effects of: CCl4 Liver;-centrilobular necrosis;-fatty change Q0325:Organ affected by: Nitrosamines -Esophagus;-Stomach Q0326:Organ affected by: Cigarette smoke -Larynx;-Lung Q0327:Organ affected by: Asbestos Lung;-Mesothelioma;-Bronchogenic carcinoma Q0328:Organ affected by: Arsenic Skin (squamous cell carcinoma) Q0329:Organ affected by: Naphthalene (aniline) dyes Bladder (transitional cell carcinoma) Q0330:Organ affected by: Alkylating agents Blood (leukemia) Q0331:Carcinogen associated with: Hepatocellular carcinoma Aflatoxins Q0332:Carcinogen associated with: Angiosarcoma Vinyl chloride Q0333:Carcinogen associated with: Centrilobular necrosis of liver CCl4 Q0334:Carcinogen associated with: Fatty change of liver CCl4 Q0335:Carcinogen associated with: Liver -Aflatoxins;-Vinyl chloride;-CCl4 Q0336:Carcinogen associated with: Esophagus Nitrosamines Q0337:Carcinogen associated with: Stomach Nitrosamines Q0338:Carcinogen associated with: Larynx Cigarette smoke Q0339:Carcinogen associated with: Lung -Cigarette smoke;-Asbestos Q0340:Carcinogen associated with: Mesothelioma of lung Asbestos Q0341:Carcinogen associated with: Bronchogenic carcinoma of lung Asbestos Q0342:Carcinogen associated with: Skin Arsenic (squamous cell carcinoma) Q0343:Carcinogen associated with: Bladder Naphthalene dyes (transitional cell carcinoma) Q0344:Carcinogen associated with: Blood Alkylating agents (leukemia) Q0345:Paraneoplastic effects: definition Symptoms not directly related to tumor or hormones of tumor tissue Q0346:Name/effect and cause of paraneoplastic syndrome associated with: Small cell lung carcinoma (with intracranial neoplasms);-Cause: ADH;-Effect: SIADH;(without intracranial neoplasms);- Cause:ACTH/ACTH-like peptide;-Effect: Cushing's;- Cause:Antibodies against presynaptic Ca2+ channels at neuromuscular junction;-Effect: Lambert-Eaton Syndrome (muscle weakness) Q0347:Name/effect and cause of paraneoplastic syndrome associated with: Squamous cell lung carcinoma Causes;-PTH-related peptide;-TGF-beta;-TNF-alpha;-IL- 1;Effect: Hypercalcemia Q0348:Name/effect and cause of paraneoplastic syndrome associated with: Renal cell carcinoma Cause: Erythropoietin;Effect: Polycythemia;Causes;-PTH- related peptide;-TGF-beta;-TNF-alpha;-IL-1;Effect: Hypercalcemia Q0349:Name/effect and cause of paraneoplastic syndrome associated with: Breast carcinoma Causes;-PTH-related peptide;-TGF-beta;-TNF-alpha;-IL- 1;Effect: Hypercalcemia Q0350:Name/effect and cause of paraneoplastic syndrome associated with: Multiple myeloma Causes;-PTH-related peptide;-TGF-beta;-TNF-alpha;-IL- 1;Effect: Hypercalcemia Q0351:Name/effect and cause of paraneoplastic syndrome associated with: Bone metastasis (lysed bone) (Though technically not a paraneoplastic syndrome);Causes;- PTH-related peptide;-TGF-beta;-TNF-alpha;-IL-1;Effect: Hypercalcemia Q0352:Name/effect and cause of paraneoplastic syndrome associated with: Hemangioblastoma Cause: Erythropoietin;Effect: Polycythemia Q0353:Name/effect and cause of paraneoplastic syndrome associated with: Hepatocellular carcinoma Cause: Erythropoietin;Effect: Polycythemia Q0354:Name/effect and cause of paraneoplastic syndrome associated with: Thymoma -Cause:Antibodies against presynaptic Ca2+ channels at neuromuscular junction;-Effect: Lambert-Eaton Syndrome (muscle weakness) Q0355:Name/effect and cause of paraneoplastic syndrome associated with: Leukemia Cause: Hypercalcemia due to excess nucleic acid turnover (ie; cytotoxic therapy);Effects;-Gout;-Urate nephropathy Q0356:Name/effect and cause of paraneoplastic syndrome associated with: Lymphoma Cause: Hypercalcemia due to excess nucleic acid turnover (ie; cytotoxic therapy);Effects;-Gout;-Urate nephropathy Q0357:Paraneoplastic syndrome and neoplasm associated with: ACTH Paraneoplastic syndrome: Cushing's syndrome;Neoplasm: Small cell lung carcinoma Q0358:Paraneoplastic syndrome and neoplasm associated with: ACTH-like peptide Paraneoplastic syndrome: Cushing's syndrome;Neoplasm: Small cell lung carcinoma Q0359:Paraneoplastic syndrome and neoplasm associated with: ADH Paraneoplastic syndrome: SIADH;Neoplasm: Small cell lung carcinoma with intracranial neoplasms Q0360:Paraneoplastic syndrome and neoplasm associated with: PTH-related peptide Paraneoplastic syndrome: Hypercalcemia;Neoplasms;- Squamous cell lung carcinoma;-Renal cell carcinoma;-Breast carcinoma;-Multiple myeloma;-Bone metastasis (lysed bone) (not really a paraneoplastic syndrome for this one) Q0361:Paraneoplastic syndrome and neoplasm associated with: TGF-beta Paraneoplastic syndrome: Hypercalcemia;Neoplasms;- Squamous cell lung carcinoma;-Renal cell carcinoma;-Breast carcinoma;-Multiple myeloma;-Bone metastasis (lysed bone) (not really a paraneoplastic syndrome for this one) Q0362:Paraneoplastic syndrome and neoplasm associated with: TNF-alpha Paraneoplastic syndrome: Hypercalcemia;Neoplasms;- Squamous cell lung carcinoma;-Renal cell carcinoma;-Breast carcinoma;-Multiple myeloma;-Bone metastasis (lysed bone) (not really a paraneoplastic syndrome for this one) Q0363:Paraneoplastic syndrome and neoplasm associated with: IL-1 Paraneoplastic syndrome: Hypercalcemia;Neoplasms;- Squamous cell lung carcinoma;-Renal cell carcinoma;-Breast carcinoma;-Multiple myeloma;-Bone metastasis (lysed bone) (not really a paraneoplastic syndrome for this one) Q0364:Paraneoplastic syndrome and neoplasm associated with: Erythropoietin Paraneoplastic syndrome: Polycythemia;Neoplasms;-Renal cell carcinoma;-hemangioblastoma;-hepatocellular carcinoma Q0365:Paraneoplastic syndrome and neoplasm associated with: Antibodies against Ca2+ channels Paraneoplastic syndrome: Lambert-Eaton syndrome (muscle weakness due to presynaptic channels being destroyed);Neoplasms;-Thymoma;-Small-cell lung cancer Q0366:Paraneoplastic syndrome and neoplasm associated with: Hyperuricemia due to excess nucleic acid turnover (ie cytotoxic therapy) Paraneoplastic syndromes;-Gout;-Urate nephropathy;Neoplasms;-Leukemia;-Lymphoma Q0367:For the following paraneoplastic syndrome; give the causes and associated neoplasms: Cushing's syndrome Cause: ACTH or ACTH-like peptide;Neoplasm: Small cell lung carcinoma Q0368:For the following paraneoplastic syndrome; give the causes and associated neoplasms: SIADH Cause: ADH;Neoplasms: Small cell lung carcinoma with intracranial neoplasms Q0369:For the following paraneoplastic syndrome; give the causes and associated neoplasms: Hypercalcemia Causes;-PTH-related peptide;-TGF-beta;-TNF-alpha;-IL- 1;Neoplasms;-Squamous cell lung carcinoma;-Renal cell carcinoma;-Breast carcinoma;-Multiple myeloma;-Bone metastasis (lysed bone: technically not a paraneoplastic cause) Q0370:For the following paraneoplastic syndrome; give the causes and associated neoplasms: Polycythemia Cause: Erythropoietin;Neoplasms;-Renal cell carcinoma;- Hemangioblastoma;-Hepatocellular carcinoma Q0371:For the following paraneoplastic syndrome; give the causes and associated neoplasms: Lambert-Eaton syndrome (muscle weakness) Cause: Antibodies against presynaptic Ca2+ channels at neuromuscular junction;Neoplasms;-Thymoma;-Small cell lung carcinoma Q0372:For the following paraneoplastic syndrome; give the causes and associated neoplasms: Gout Cause: Hyperuricemia due to excess nucleic acid turnover (ie cytotoxic therapy);Neoplasms;-Leukemias;-Lymphomas Q0373:For the following paraneoplastic syndrome; give the causes and associated neoplasms: Urate nephropathy Cause: Hyperuricemia due to excess nucleic acid turnover (ie cytotoxic therapy);Neoplasms;-Leukemias;-Lymphomas Q0374:Primary tumors that metastasize to the brain Lots of Bad Stuff Kills Glia;-Lung;-Breast;-Skin (melanoma);- Kidney (renal cell carcinoma);-GI Q0375:Primary tumors that metastasize to the liver Cancer Sometimes Penetrates Benign Liver;By decreasing frequency;-Colon;-Stomach;-Pancreas;-Breast;-Lung Q0376:Primary tumors that metastasize to bone Primary Tumors Like Killing Bone;-Prostate (blastic);- Thyroid/Testes;-Lung (Lytic);-Kidney;-Breast (Both lytic and blastic);Prostate and bone are most common Q0377:% of brain tumors from metastases 50% Q0378:% of liver tumors from metastases Unspecified; but there are many more metastases than primary liver tumors Q0379:% of bone tumors from metastases Unspecified; but there are many more metastases than primary bone tumors Q0380:Brain metastasis: Morphology -Multiple;-Well circumscribed;-At gray/white border Q0381:Incidence and mortality in men of the following cancer: Prostate Incidence: 32%;Mortality: 13% Q0382:Incidence in men of the following cancer: Lung Incidence: 16%;Mortality: 33% Q0383:Incidence in men of the following cancer: Colon and rectum Incidence: 12%;Mortality: Unspecified Q0384:Incidence and mortality in women of the following cancer: Breast Incidence: 32%;Mortality: 18% Q0385:Incidence and mortality in women of the following cancer: Lung Incidence: 13%;Mortality: 23% Q0386:Incidence and mortality in women of the following cancer: Colon and Rectum Incidence: 13%;Mortality: Unspecified Q0387:Type of necrosis found in: Kidney Coagulative Q0388:Type of necrosis found in: Brain Liquefactive Q0389:Type of necrosis found in: Tuberculosis Caseous Q0390:Type of necrosis found in: Pancreas Fat Q0391:Type of necrosis found in: Blood vessels Fibrinoid Q0392:Type of necrosis found in: Limbs Gangrenous Q0393:Type of necrosis found in: GI tract Gangrenous Q0394:receptor: alpha 1; G-protein class? Gq Q0395:receptor: alpha 2; G-protein class? Gi Q0396:receptor: beta 1; G-protein class? Gs Q0397:receptor: beta 2; G-protein class? Gs Q0398:receptor: M1; G-protein class? Gq Q0399:receptor: M2; G-protein class? Gi Q0400:receptor: M3; G-protein class? Gq Q0401:receptor: D1; G-protein class? Gs Q0402:receptor: D2; G-protein class? Gi Q0403:receptor: H1; G-protein class? Gq Q0404:receptor: H2; G-protein class? Gs Q0405:receptor: V1; G-protein class? Gq Q0406:receptor: V2; G-protein class? Gs Q0407:receptor: alpha 1; f(x)? increase vascular smooth muscle contraction Q0408:receptor: alpha 2; f(x)? decrease sympathetic outflow; decrease insulin release Q0409:receptor: beta 1; f(x)? increase HR; increase contractility; increase renin; increase lipolysis; increase aqueous humor formation Q0410:receptor: beta 2; f(x)? vasodilation; bronchodilation; increase glucagon release Q0411:receptor: M1; f(x)? CNS Q0412:receptor: M2; f(x)? decrease HR Q0413:receptor: M3; f(x)? increase exocrine gland secretions Q0414:receptor: D1; f(x)? relaxes renal vascular smooth muscle Q0415:receptor: D2; f(x)? modulates transmitter release; especially in brain Q0416:receptor: H1; f(x)? increase nasal and bronchial mucus production; contraction of bronchioles; pruritus; and pain Q0417:receptor: H2; f(x)? increase gastric acid secretion Q0418:receptor: V1; f(x)? increase vascular smooth muscle contraction Q0419:receptor: V2; f(x)? increase H2O permeability and reabsorption in the collecting tubules of the kidney Q0420:interphase Gi; S; G2;precedes mitosis Q0421:G1 (gap) phase 12 hrs. avg;cell growth: synthesis of carbs; proteins; lipids Q0422:G0 LONG G1 state (almost stops cycling!);ex: muscle; nerve cells Q0423:mitochondria + centrioles divide during ____ phase S phase;(both contain DNA) Q0424:cells perform their differentiated functions during ____ phase G1/G0 Q0425:during G1; cells are _n 2n (diploid) Q0426:during G2; cells are _n 4n (tetraploid) Q0427:S 6-8 hrs avg;ALLDNA synthesis + chr replication happens here;also RNA synthesis rate increases; cell prepares for mitosis Q0428:G2 3-4 hrs avg;resembles G1 except cell now TETRAPLOID (4n) Q0429:in mitosis; cell goes from _n to _n 4n to 2n Q0430:6 phases of mitosis preprophase;prophase;metaphase;early anaphase;late anaphase;telophase Q0431:preprophase chr condense (recognizable);centrioles (barrel-like) visible in cytoplasm Q0432:prophase 2 copies of each chromosome separated into single chromosome called CHROMATIDS;mitotic spindle forms;centrioles begin to separatre; microtubules assemble b/w them;near end; nuclear envelope starts to rupture Q0433:metaphase nuclear envelope + nucleolus disappear;spindle moves to where nucleus was;chr move to mid-spindle + attach to MT Q0434:early anaphase CHROMATIDS split longitudinally + migrate to cell poles;*note: after chromatids split; they are called chromosomes again Q0435:late anaphase chr aggregate at poles;CLEAVAGE FURROW begins to form (beginning of cytokinesis) Q0436:chromatids/chromosomes until ANAPHASE; each chromosome contains 2 sister chromatids;after ANAPHASE each chromatid = separate chromosome Q0437:during G2; each chromosome consists of _________ 2 sister chromatids; connected at centromere Q0438:telophase (cytokinesis) nucleolus forms;nuclear envelopes form around each set of daughter chromosomes;condensed chromatin expands again + begins to reappear;cytoplasm divides by deepening cleavage furrow until --> 2 daughter cells Q0439:cells entering meiosis are called ________ primary gametes (spermatocytes or oocytes);have same DNA content as cell entering mitosis Q0440:cells entering meiosis II are called ________ secondary gametocytes;contain 23 chromosomes (each consisting of 2 sister chromatids) Q0441:meiosis occurs in______ germ cells (sperm; ova);2 parts; cell division in each resembles mitosis; but no DNA replication during either Q0442:meiosis reduced chromosome # by ______ half Q0443:genetic recombination occurs in meiosis;exchange of chr segments;occurs b/w chr within tetrad;changes allelic linkages; does NOT change gene sequence Q0444:stages of Meiosis I prophase I;metaphase I;anaphase I;telophase I Q0445:stages of meiosis II prophase II;metaphase II;anaphase II;telophase II Q0446:during meiosis II; _______ separate sister chromatids (of the 23 chromosomes) Q0447:recombinbation occurs b/w chromatids within a tetrad Q0448:meiotic prophase occurs in _ steps 3 (A;B;C) Q0449:meiotic prophase A chromosomes condense (visible); homologous chromosomes pair (except X;Y chr; centromeres of homologues don't pair) Q0450:meiosis prophase step B homologous chromosome pairing complete;4 chromatids appear (= tetrad) Q0451:meiosis prophase step C RECOMBINATION/CROSS-OVER Q0452:recombination chromatid segments exchanged b/w 2 paired homologous chromosomes Q0453:chiasma pt of exchange during recombination; shaped like an X Q0454:cells resulting from meiosis II spermatids;OR;ova (plus polar body) Q0455:meiotic metaphase I paired chromosomes line up on mitotic spindle;2 chromosomes of each homologous pair attach to MT going to opposite poles of cell Q0456:meiotic anaphase I both chromatids migrate to same end of cell Q0457:meiotic telophase I each daughter gets 1 part of each chromosome pair;each gets total 23 chromosomes Q0458:2nd meiotic division similar to meiotic division except NO PRIOR DNA SYNTHESIS;23 chromosomes divide at centromere;each new daughter cell gets 23 chromatids (HAPLOID) Q0459:5 causes of tissue hypoxia ischemia; hypoxemia; ETC block; uncoupled ETC; AV shunts Q0460:Ultimate effects of tissue hypoxia No O2 to accept electrons in ETC; no production of ATP. Na/K pump fails and cell swells (reversible change). Ribosomes fall from RER. Disruption of cell membrane and mitochondria induces apoptosis. Q0461:Effects of low ATP in cell Increased glycolysis to support ATPase pump. Anaerobic glycolysis produces lactate with decreased intracellular pH which denatures proteins (coagulation necrosis); cell swelling; entry of calcium and apoptosis Q0462:Pathophysiology of cell injury in hypoxia ETC fails due to lack of oxygen; 2. No ATP production in ETC increases anaerobic glycolysis (high citrate and AMP activate PFK-1); 3. increased lactate decreases cell pH which denatures proteins and produces coagulation necrosis; 4. ATPase fails and cell swells with fall off of ribosomes from RER; 5. disruption of cell membrane with entry of Ca activates phspholipase (lipid peroxidation); complement activation; nuclear enzymes with pyknosis and destruction of mitochondria and apoptosis Q0463:What is methhemoglobin? Hemoglobin with oxidized (Fe3+) iron that cant bind O2. Decreases SaO2 and produces cyanosis. Caused by nitro/sulfa compounds. Rx.: methylene blue Q0464:Increased PACO2; decreased PaO2; decreased O2 content; decreased SaO2 Respiratory acidosis Q0465:Normal PaO2 and SaO2; decreased Hb Anemia Q0466:Normal Hb; PaO2; decreased SaO2; decreased O2 content CO poisoning or methhemoglobinemia Q0467:CO poisoning tissue hypoxia Decreased O2 content and SaO2; normal PaO2; left shift of dissociation curve and cytochrome oxidase inhibition all cause hypoxia. Produced by car exhaust; heaters; smoke inhalation; wood stoves. Rx.: 100% O2. First symptom: headache Q0468:Factors that left-shift O2 dissociation curve and decrease P50 Decreased 2;3BPG; CO; MetHb; HbF; hypothermia; alkalosis Q0469:Factors that right-shift O2 dissociation curve and increase P50 Increased 2;3BPG; fever; acidosis Q0470:Causes of hypoxia with normal O2 content Ischemia; cyanide poisoning; ETC uncouplers (alcohol; salicylates; dinitrophenol) Q0471:Free radical metabolism NADPH oxidase and spontaneous superoxide; Superoxide dismutase makes H2O2 from superoxide. Catalase breaks down H2O2. Gluthathione reductase and GSH peroxide breakdwon H2)2 using reduced GSH and NADPH from G6PDH in HMP shunt Q0472:Causes of free radical injury Aging process produces lipofuscin which peroxidates membrane; MPO system; O2 free radicals; ionizing radiation; acetaminophen (treat with acetylcyteine); CCl4 poisoning Q0473:Features of apoptosis Eosinophilic cytoplasm; pyknotic nucleus; no inflamatory infiltrate Q0474:Physiologic examples of apoptosis Thymus involution; Mullerian and Wolffian structure involution; gravid uterus Q0475:Pathologic examples of apoptosis Councilman bodies in viral hepatitis; psammoma bodies; cancer Q0476:Coagulation necrosis Denaturing and coagulation of proteins in cytoplasm (infarction). Pale Vs. hemorrhagic infarcts Q0477:Liquefactive necrosis Neutrophil destruction with hemolytic enzymes. Abesesses; wet gangrene; brain; pancreas Q0478:Caseous necrosis Combination of coagulation and liquefaction necrosis. Cheese- like material; casseating granulomas with macrophages Q0479:Fat necrosis Lipases on fatty tissue. Pancreas. Chalky-white appearance Q0480:Fibrinoid necrosis Histologically resembles fibrin. Eosinophilic mitral valve vegetations; immunocomplexes Q0481:Fatty liver change In alcoholics - liver stores excess tryglycerides because increased NADH produces glycerol 3P and increased acetate (acetyl CoA) increases FA synthesis. In kwashiorkor; no apolipoproteins for VLDL Q0482:Regulation of apoptosis Genes bcl-2 (inhibits apoptosis) prevents release of cytochrome C and binds protease activating factor (Apaf-1); p53 stimulates apoptosis. Mediated by caspases. Stimulated by cell injury; lack of hormones; Fas and TNF Q0483:Rb suppressor gene and Rb protein Located on chromosome 13. Produces unphosphorylated Rb protein which stops cell from entering S phase. Phosphorylation by cyclin D/cdk complex allows it to enter S phase. Mutation of Rb gene produces cancer Q0484:cdk/cyclin D complex When activated it phosphorylates Rb protein allowing cell to enter S phase Q0485:p53 suppressor gene Located on chromosome 17. Produces a protein that inactivates cyclin D/cdk complex preventing Rb protein phosphorylation which keeps cell in G1 Q0486:Name 5 characteristics of the metabolic syndrome central obesity;atherogenic lipid patterns;hypertension;insulin resistance or diabetes;pro-inflammatory state; ie C-reactive protein Q0487:Describe stable angina Chest pain precipitated by exertion; relieved by rest or vasodilators. Q0488:Describe unstable angina Chest pain that is prolonged or recurrent at rest. Q0489:Describe Prinzmetal angina Intermittent chest pain at rest; usually caused by vasospasm. Q0490:The two patterns of myocardial ischemic necrosis? Describe them. Transmural: entire ventricular wall from endo- to epicardium;Subendocardial: limited to interior one third of ventricular wall Q0491:Name 6 complications of myocardial infarction. Arrhythmia: common cause of death in first hours after MI;Heart failure: depends on MI size;Myocardial rupture: risk highest 4-7 days after MI;Papillary muscle rupture;Mural thrombosis: forms over infarct in heart; can embolize;Ventricular aneurysm Q0492:Timeline of GROSS morphological changes in acute myocardial infarction? < 12 hrs: no gross changes;24 hrs: swelling; pale or red brown infarct with surrounding hyperemia;7 days: yellow infarcted area with red border;10 days: red vascular connective tissue gradually replaces necrotic tissue;5 weeks: pallor of infarct due to fibrosis;3-6 months: gray-white scar Q0493:Timeline of MICROSCOPIC morphological changes in acute myocardial infarction? < 6hrs: none;12-24hrs: nuclei disappear; striations lost; neutrophils infiltrate; coagulative necrosis apparent;3 days: macrophages replace neutrophils; phagocytose debris;7 days: growth of fibroblasts and new vessels in the lesion;2-4 weeks: collagen and matrix synthesis;>5 weeks: progressive fibrosis replaces lesion Q0494:When do LDH; troponin; and CK-MB each peak in acute myocardial infarction? LDH peaks at 3 days and persists;Troponin peaks at 24 hours and persists;CK-MB peaks at 24 hours and tapers to zero. Q0495:Rheumatic fever: etiology Immunologic disease. Streptococcal antigens elicit an antibody response that is reactive to human heart and other tissues. Q0496:Rheumatic fever: name and characteristics of the classic histologic lesion? Aschoff body;Focal interstitial myocardial inflammation with collagen; fibrinoid material; multinucleated giant cells; and large myocytes. Q0497:Rheumatic fever: how long after what infection? What lab sign indicates recent infection? 1-4 weeks after Group A beta hemolytic streptococcus infection. Elevated anti-streptolysin (ASO) indicates recent infection. Q0498:Rheumatic fever: non-cardiac manifestations Fever; malaise; elevated ESR; arthralgias/arthritis/migratory polyarthritis; subcutaneous nodules; erythema marginatum; Sydenham chorea. Q0499:Rheumatic fever: cardiac manifestations Pancarditis (all 3 layers);Non-friable mitral and aortic vegetations; which after healing cause fibrosis; calcification; and deformation of the values. Chordae tendineae are also thickened and shortened. Q0500:Organisms causing acute vs subacute bacterial endocarditis Acute: staph aureus (also beta hemolytic strep and pneumococcus);Subacute: strep viridans (also enterococcus; HACEK organisms) Q0501:Complications of infective endocarditis? Distal embolization to brain or other tissues can lead to septic infarcts;Renal glomeruli involvement due to immune complex disease or septic emboli. Q0502:Suspect what if tricuspid valve is involved in infective endocarditis? IV drug use. 50% of cases of endocarditis in this population have tricuspid involvement. Q0503:Risk factors for infectious endocarditis? Congenital heart disease;Preexisting valvular heart disease;Artificial valve;IV drug use Q0504:What is nonbacterial thrombotic endocarditis associated with? What are its complications? Metastatic cancer and other wasting disorders;Valve deposits are sterile and made of fibrin; as a result emboli are sterile; not septic. Q0505:What is Libman-Sacks endocarditis? Occurs in SLE; is characterized by small fibrin vegetations forming on either or both sides of the valve leaflets. Q0506:What is endocarditis of the carcinoid syndrome? Caused by secretory products of carcinoid tumors such as serotonin and other vasoactive peptides and amines. They cause endocarditis resulting in thickened endocardial plaques; usually in the RIGHT of the heart because these substances are inactivated in the pulmonary circulation. Q0507:Most common valvular lesion? Mitral prolapse (7% of population) Q0508:Characteristic sound of mitral prolapse? Systolic murmur with midsystolic click. Q0509:Causes of mitral stenosis? Almost always rheumatic heart disease. Q0510:Causes of aortic stenosis? Age related calcific stenosis;Congenital bicuspid valve;Rheumatic valve Q0511:Causes of aortic regurgitation? Nondissecting aortic aneurysm;Rheumatic heart disease;Syphilitic aortitis with dilation of valve ring Q0512:Coarctation of aorta is more common in what syndrome? Turner's syndrome (monosomy X) Q0513:Congenital rubella syndrome includes what fetal defects? Cardiovascular (PDA and others);Microcephaly;Deafness;Cataracts;Growth retardation Q0514:What drug keeps PDA open? what drug closes it? Prostaglandin keeps it open;Indomethacin closes it. Q0515:Name three conditions associated with dilated cardiomyopathy. Alcoholism; thiamine deficiency; prior myocarditis. Q0516:What is a common cause of restrictive cardiomyopathy? Cardiac amyloidosis. Q0517:what are the morphological changes seen in hypertrophic cardiomyopathy? hypertrophy of all chamber walls especially the ventricular septum;Disoriented tangled and hypertrophied myocardial fibers;Left ventricular outflow obstruction. Q0518:What is the inheritance of hypertrophic cardiomyopathy? Usually autosomal dominant Q0519:what is the most common cause of myocarditis? Coxsackievirus Q0520:what is the most common tumor of the heart? Atrial myxoma Q0521:What is cor pulmonale? Name one common cause. Cor pulmonale is right ventricular hypertrophy or dilation secondary to lung disease or primary disease of pulmonary vasculature such as primary pulmonary hypertension;Emphysema is a common cause. Q0522:pansystolic murmur at LLSB radiating right towards midclavicular line that is medium pitched; has a blowing quality; increases on inspiration; S3; incr. JVP and "v" wave tricuspid regurg. The S3 sound is from the overdistended RV Q0523:harsh; shrill; midsystolic crescendo-decrescendo murmur; sometimes S4 aortic stenosis Q0524:soft; blowing; pansystolic murmur; S3; elevated left atrial pressures; mitral regurg Q0525:immediate diastolic murmur in a middle-aged guy who has always had some problems keeping up bicuspid aortic valve Q0526:asymptomatic adult with prominant RV impulse; midsystolic ejection murmuer heard in pulmonic area and along the LSB; fixed splitting of S2 classic for ASD Q0527:systolic ejection murmer on RSB that radiates to jugular aortic stenosis Q0528:opening snap and diastolic murmur mitral stenosis Q0529:incr. RV pressures --> RVH and PA dilation & cresc- decresc murmur if severe; no RA enlargement pulmonary stenosis Q0530:child with a harsh systolic murmer and increased oxygen saturation in the RV VSD Q0531:Mitral/tricuspid regurgitation holosystolic; high-pitched 'blowing murmur';mitral: loudest at apex and radiates towards AXILLA;tricuspid: loudest at tricuspid area and radiates to right sternal border Q0532:Aortic stenosis crescendo-decrescendo systolic ejection murmur following an ejection click!!;LV>>aortic pressure during systole;radiates to carotids/apex;'pulsus parvus et tardus' - pulses weak compared to heart sounds Q0533:VSD holosystolic; harsh-sounding murmur;loudest at tricuspid valve Q0534:Mitral prolapse late systolic murmur with midsystolic click (MC);most frequent valvular lesion;loudest at S2 Q0535:Aortic regurgitation immediate high-pitched 'blowing' diastolic murmur;wide pulse pressure when chronic Q0536:Mitral stenosis follows opening snap;secondary to rheumatic fever;delayed rumbling late diastolic murmur;LA>>>LV pressure during diastole;tricuspid stenosis differs because it gets louder with inspiration (more blood flows into RA upon inspiration) Q0537:PDA continuous machine-like murmur;loudest at time S2;(aorta --> pulmonary artery;the pressure difference is obscene and thus you have a continuous murmur) Q0538:Congenital heart defects associated with 22q11 syndrome truncus arteriosus; tetralogy of fallot Q0539:Down syndrome heart defects ASD; VSD; AV septal defect (endocardial cushion defect) Q0540:Congenital rubella!!! septal defects; PDA; pulmonary artery stenosis;(microcephaly; mental retardation; deafness; cataracts; growth retardation) Q0541:Turner's syndrome heart stuff coarctation of aorta Q0542:Marfan's syndrome heart stuff aortic insufficiency (late complication) Q0543:Offspring of diabetic mother transposition of great vessels Q0544:MC arteries affected by Monckenberg arteriosclerosis Radial and Ulnar arteries Q0545:Definition;Hyaline thickening of small arteries in Essential HTN and DM Arteriolosclerosis Q0546:Dx;"Onionskin" thickening of the arteriolar walls Hyperplastic Arteriolosclerosis;(Malignant HTN) Q0547:Definition;Dx specific to an aneurysm of the Ascending Aorta;where does it exert its effect?;what heart problem can it lead to? Syphillic (Leutic) Anneurysm;effects: Vaso Vasorum;leads to: Aortic valve incompetence Q0548:what may be confused w/ a MI if it wasn't for the normal serum enzymes? Dissecting Aneurysm Q0549:Definition;dilated small vessel surrounded by radiating fine channels and associated w/ hyperestrinism Spider Telangiectasia Q0550:Dx;Port-wine stain birthmark Hemangioma Q0551:Dx;Hemangioblastomas of the retina; cerebellum and medulla;What can it lead to?;what genetics? gene? Von Hippel-Lindau Dz;can lead to: Bilateral Renal Cell CA;genetics;Auto Dominant;deletion on VHL gene on chrom 3;(chrom 3 = 3 words in name VHL) Q0552:Malignant vascular tumor caused by Arsenic exposure Hemangiosarcoma Q0553:Dx;fever; weight loss; abd pain; HTN; cutaneous eruptions; arthralgia; vasculitis in arterioles and glomeruli of kidney;What is possible predisposing virus? Polyarteritis Nodosa;(P-ANCA);;virus: HBV Q0554:Dx;granulomatous vasculitis w/ eosinophilia and asthma; prominent in pulmonary vasculature Churg-Strauss syndrome Q0555:Dx;child w/ previous URI gets hemorrhagic urticaria of extensor surfaces; arthralgia; abd pain; melena Henoch-Schonlein purpura Q0556:Dx;necrotizing granulomatous vasculitis in lung and upper airway and necrotizing glomerulonephritis Wegener's Glanulomatosis;(C-ANCA) Q0557:Dx;unilateral HA; visual impairment; polymyalgia rheumatica (muscle pain); increased ESR Temporal arteritis;(Giant cell arteritis) Q0558:Dx;fever; arthritis; night sweats; myalgia; skin nodules; ocular disturbances; weak pulse in upper extremities; elevated ESR Takayasu's Arteritis Q0559:Dx;child w/ fever; congested conjuctiva; changes in lips/oral mucosa; lymphadenitis Kawasaki Dz Q0560:Dx;intermittent claudication; nodular phlebitis; cold sensitivity; heavy smoker Buerger's Dz;(Tx: quit smoking) Q0561:Dx;marked increase in diastolic BP; retinal hemorrhages; papilledema; "flea-bitten" kidney Malignant HTN Q0562:Definition;focal interstitial myocardial inflammation w/ fragmented collagen and fibrinoid material and some mulitnucleated giant cells; seen in Rheumatic fever Aschoff Body;(w/ Anitschkow's cells) Q0563:MC coronary artery for MI LAD Q0564:Definition;Autoimmune phenomenon resulting in fibrinous pericarditis post-MI Dressler's syndrome Q0565:what lab is elevated in Rheumatic Heart Dz? elevated ASO titer Q0566:Dx;fever; erythema marginatum; valvular damage; elevated ESR; polyarthritis; Sub-q nodules; chorea Rheumatic Heart Dz;(mitral = MC valve) Q0567:MCC of Subacute Endocarditis Strep Viridians Q0568:MCC of Acute Endocarditis Staph Aureus Q0569:Definiton;Endocarditis secondary to metastasis or renal failure Marantic endocarditis;(can result in peripheral emboli) Q0570:(3) congenital Right -> Left shunts 3 Ts;Tetralogy of Fallot;;Transposition of great vessels;;Truncus Arteriosus Q0571:Dx;Fixed S2 split ASD Q0572:Definition;uncorrected VSD; ASD; or PDA leads to progressive pulmonary HTN. With Inc pulm resistance; shunt reverses from L -> R to ;R -> L; causing late cyanosis; clubbing and polycythemia Eisenmenger's syndrome Q0573:which congenital heart defect is not compatable with life unless a shunt is present? Transposition of great vessels Q0574:Dx;notching of ribs; HTN in upper extremities and weak pulses in lower extremities Coarctation of Aorta Q0575:Dx;continuous "machine-like" murmur PDA Q0576:what is given to close a PDA? Indomethacin Q0577:what is given to keep a PDA open? Prostaglandins;[PROp it open] Q0578:Congenital defect with;Congenital Rubella;(2) Septal defect;;PDA Q0579:Congenital defect with;Marfan's syndrome Aortic insufficiency Q0580:Congenital defect with;offspring of Diabetic mother Transposition of Great vessels Q0581:Murmur;systolic High-pitched "blowing" Mitral Regurg Q0582:Murmur;Midsystolic Click Mitral Prolapse Q0583:Murmur;Wide pulse-pressure Aortic Regurg Q0584:MC heart tumor in children Rhabdomyoma;(w/ Tuberous Sclerosis) Q0585:which type of emboli can lead to DIC? Amniotic fluid embloi Q0586:Dx;low CO; equilibrium of pressures in all 4 chambers; JVD; pulsus paradoxus Cardiac tamponade Q0587:(4) causes of Serous Pericarditis SLE;;RA;;Infection;;Uremia Q0588:(3)* causes of Fibrinous pericarditis Fiber in your RUM;Rheumatic fever;;Uremia;;MI Q0589:(4) signs of Tetralogy of Fallot PROVe;Pulmonary stenosis;;RVH;;Overriding Aorta;;VSD Q0590:developmental cause of Tetralogy of Fallot? Anterosuperior displacement of Infundibular septum Q0591:Definition;passage of emboli from the venous circulation into the arterial circulation via a R -> L shunt Paradoxic Emboli Q0592:True of false: 50% is classified as secondary HTN (usually resulting from renal disease) false. (90% essential/primary; 10% secondary) Q0593:read the questioner's mind: HTN predisposes individuals to this disease (the one John Ritter died of) aortic dissection Q0594:Pathology changes associated with HTN hyaline thickening & atherosclerosis Q0595:This awful term refers to a stiffening of the arteries that invovles the media. Particularly likely to occur at the radial & ulnar arteries. Monckeberg arteriosclerosis Q0596:Atherosclerosis: True or false: atherosclerosis is a disease of small sized arteries false. affects elastic; large & medium muscular arteries. Q0597:Atherosclerosis: Earliest sign of atherosclerotic disease fatty streak Q0598:Atherosclerosis: most likely location abdominal aorta. (then coronary artery; popliteal artery; and carotid artery) Q0599:Type of angina resulting from coronary artery spasm Prinzmetal's variant Q0600:This coronary artery branch is most commonly implicated in myocardial infarction LAD (left anterior descending) Q0601:most common cause of sudden cardiac death (lethal) arrhythmia Q0602:Solid tissues like the heart; brain; kidney and spleen have only a single blood supply (not so good collaterals). Therefore infarcts are more likely to be --? pale Q0603:2 instances where red infarct is likely (1) reperfusion (2) loose tissues with good collaterals - like the lungs or intestine Q0604:Evolution of MI: Rank the following vessels from most to least commonly occluded: RCA; LAD; circumflex LAD>RCA>circumflex Q0605:Evolution of MI: Histologic changes on day 1 of an MI? pallor of infarcted area; coagulative necrosis Q0606:Evolution of MI: days 2-4? dilated vessels (hyperemia); neutrophil invasion; extensive coagulative necrosis Q0607:Evolution of MI: days 5-10? yellow-brown softening of infarcted region; macrophages present; granulation tissue begins to grow in Q0608:Evolution of MI: after 7 weeks? infarct is gray-white; scar complete Q0609:Diagnosis of MI True or false: ECG is not diagnostic during the first 6 hours following an MI False; it is the gold standard within this time period Q0610:Diagnosis of MI What is the test of choice within the first 24 hours? CK-MB Q0611:Diagnosis of MI This enzyme is elevated from 4 hours up to 10 days after an MI and is the most specific protein marker cardiac troponin I Q0612:Diagnosis of MI on ecg; transmural infarction causes ______ ST elevation; Q wave changes Q0613:MI complications: Most common (90% of patients) arryhthmias; esp. 2 days after infarct Q0614:MI complications: automimmune phenomen several weeks post-MI that results in fibrinous pericarditis Dressler's syndrome Q0615:MI complications: high risk of mortality cardiogenic shock (large infarcts) Q0616:MI complications: seen about a week after the infarction rupture of ventricular wall; septum; or papillary muscle Q0617:Cardiomyopathies Most common dilated (congestive) cardiomyopathy; heart looks like a ballon on X-ray Q0618:Cardiomyopathies True or False: substance abuse is a common cause of dilated cardiomyopathy True; cocaine and alcohol especially Q0619:Cardiomyopathies These two infectious diseases are associated with dilated myopathy coxsackievirus B and Chagas' disease Q0620:Cardiomyopathies True or false: hypertrophic cardiomyopathy causes systolic dysfunction False; dilated myopathy causes systolic dysfunction; hypertrophic causes diastolic Q0621:Cardiomyopathies Half of hypertrophic myopathies are inherited as an _________ trait (x-linked; dominant; etc.) autosomal dominant; major cause of sudden death in young athletes Q0622:Cardiomyopathies On echo in hypertrophic disease; the LV thickens and the chamber looks how? like a banana Q0623:Cardiomyopathies These "-osis" diseases are major causes of restrictive/obliterative cardiomyopathy sarcoidosis; amyloidosis; hemochromatosis; endocardial fibroelastosis; endomyocardial (Loffler's) fibrosis….also; scleroderma but it's not an -osis Q0624:Name two causes of holosystolic murmurs 1) VSD; 2) mitral regurg; and 3) tricuspid regurg Q0625:Widened pulse pressure seen with this diastolic murmur aortic regurg Q0626:Describe the murmur associated with the most common valvular lesion Mitral prolapse; late systolic murmur following mid-systolic click Q0627:True or false: aortic stenosis causes a decrescendo- crescendo murmur following an ejection click False; ejection click is followed by a crescendo-decrescendo systolic murmur Q0628:cause of a continuous murmur loudest at time of S2? patent ductus artieriosis Q0629:opening snap followed by late diastolic rumbling? mitral stenosis Q0630:most common heart tumor? metastasis Q0631:primary cardiac tumor in 1) adults and 2) children adults=myxoma (almost always in left atrium); children=rhabdomyoma Q0632:fun gross pathologic term for changes in liver with CHF? nutmeg Q0633:what are "heart failure cells"? hemosiderin-laden macrophages in lung Q0634:dyspnea on exertion; pulmonary edema; and paroxysmal nocturnal dyspnea are symptoms of? left heart failure Q0635:patient says "I have to sleep upright." the clinical term for this is? orthopnea Q0636:most pulmonary emboli arise from? DVT Q0637:True or false: Amniotic fluid can lead to DIC TRUE Q0638:what are the component of virchow's triad? stasis; hypercoagulability; endothelial damage Q0639:what is pulsus paradoxus? greater than 10 mmHg drop in systolic on inspiration Q0640:what is electrical alternans? characteristic of tamponade on ECG in which QRS complex height varies beat-to-beat Q0641:midsystolic cresendo-decrescendo murmur aortic stenosis Q0642:high-pitched; blowing murmur at the left sternal border aortic regurgitation Q0643:low pitched; mid dyastolic rumble aortic regurgitation Q0644:midsystolic murmur at the base aortic regurgitation Q0645:late mid-diastolic rumble mitral stenosis Q0646:high pitched holosystolic murmur at apex to axilla mitral regurgitation Q0647:What valve is usually involved in endocarditis? Mitral Q0648:What valve indicated drug use if it is involved? Tricuspid Q0649:Can endocarditis be non-bacterial? Yes. It can be secondary to metastasis; renal failure (maranctic or thrombotic); fungal Q0650:What type of endocarditis does s. Aureus cause? rapid onset; high virulence; tends to occur secondary to infection elsewhere Q0651:What do the vegetations look like Large. Q0652:What type of endocarditis does s. viridians cause? subacute. Tends to have smaller vegetations. Q0653:What predisposes you to s.viridins endocarditis? tends to occur on previously damaged valves; so rheumatic fever. It is commonly seen after dental work. Q0654:What are the 8 sings of endocarditis? JR=NO FAME Janeway lesions; Roth's spots; Nail-bed hemorrhages; Osler's nodes; Fever; Anemia; Murmur (new); Emboli Q0655:What do Janeway Lesions look like? multiple small flat erythematous lesions on palms and soles Q0656:What are roth spots? round white spots on the retina surrounded by hemorrhage. Q0657:What are osler's nodes? Tender raised lesions on the fingers and toes. Q0658:What is the etiology of these olser's nodes; roth spots; and janeway lesions? Bacterial vegetations flipping off the heart valve and lodging in the periphery. Q0659:What type of bacteria causes rheumatic fever? Group A beta-hemolytic strep Q0660:when does it occur? Children 5-15 years; four weeks after a bacterial infection Q0661:Is the bacteria responsible for the symptoms? No. RF is an autoimmune reaction of a cross-reactive protein that is found in the initial bacterial infection. Q0662:What are the non cardiac clinical signs of rheumatic fever? FEVERSS - Fever; Erythema marginatum; valve damage; Elevated ESR; Red-hot joints (migratory polyarthritis); Subcutaneous nodules; and St. Vitus dance (chorea) Q0663:What hear valves are effected? Mitral (most frequent); Aortic; Tricuspid (5%) - high pressure valves mainly. Q0664:What is an Aschoff body? classic histological sign of RF; found in the myocardium; contained fibrinoid material; fragmented collages; surrounded by giant cells. Q0665:What are the cardiac signs of RF? Verrucious vegetations on the valve; pancarditis; possible pericardial effusions and myocarditis (most common cause of death) Q0666:Name four causes of serous pericarditis. RAIL - Rheumatic Arthritis; Infection; Lupus; and Uremia Q0667:What is serous pericarditis? straw colored; protein rich exudates - non-purulent; and acute Q0668:What are three causes of fibirnous exudates? MI; Rheumatic fever; and Uremia Q0669:Cloudy pericardial exudates indicated what? Bacterial infection Q0670:What are two causes of Hemorrhagic pericarditis? malignancy and TB Q0671:What is hemorrhagic pericarditis? Bloody and inflammatory exudates Q0672:What are the clinical signs of pericardial exudates? pericardial pain; friction rub; decreased heart sounds; ST elevation throughout; and pulses paradoxes (like cardiac tamponade) Q0673:What are the long term sequela of pericarditis? chronic adhesive or constrictive pericarditis Q0674:What is constiricit pericadritis Fibrous scarring in the pericardium obliterates the space and constrict the right side of the heart (because it is less able to withstand the pressure) Q0675:What types of pericaditis lead to constrictive pericarditis TB and pyrogenic staph infections Q0676:Syphilitic Heart Disease: What part of the heart does syphilis damage? The vaso vasorum of the aorta Q0677:Syphilitic Heart Disease: What does this lead to? Dilation of the aorta and valve ring Q0678:Syphilitic Heart Disease: What clinical results does this have? it can cause an aortic aneurysm or valvular incompetence Q0679:Syphilitic Heart Disease: What parts of the aorta are effected ascending and arch Q0680:Syphilitic Heart Disease: What is the appearance or the aorta? Called a "tree-bark" appearance. Q0681:Lab findings in PAN p-anca. HBsAg+ in 30%; anemia; leukocytosis Q0682:Microscopic features of PAN Segmental necrotizing vasculitis in three stages: fibrinoid necrosis with neutrophils; fibroblast proliferation; nodular fibrosis with loss of internal elastic lamina Q0683:Lab findings in PAN p-anca. HBsAg+ in 30%; anemia; leukocytosis Q0684:Microscopic features of PAN Segmental necrotizing vasculitis in three stages: fibrinoid necrosis with neutrophils; fibroblast proliferation; nodular fibrosis with loss of internal elastic lamina Q0685:Clinical features of PAN Affects all organs except lungs. Fever; hematuria/renal failure/hypertension; abdominal pain/GI bleeding; myalgia/arthralgia Q0686:Clinical features of Wegner granulomatosis Bilateral pneumonitis with nodular and cavitary infiltrates; chronic sinusitis; nasopharyngeal ulcerations; renal disease Q0687:Microscopic features of Wegner granulomatosis Necrotizing vasculitis of small vessels (granulomas); necrotizing granulomas of respiratory tract; focal necrotizing glomerulonephritis Q0688:Lab findings in Wegner granulomatosis c-anca Q0689:Treatment of Wegner granulomatosis cyclophosphimide Q0690:Clinical features of temporal arteritis Throbbing unilateral headache; visual disturbances; jaw claudication Q0691:Microscopic features of temporal arteritis Segmental granulomatous vasculitis with multinucleated giant cells and fragmentation of the internal elastic lamina with intimal fibrosis and luminal thickening Q0692:Diagnosis; lab findings and treatment of temporal arteritis Dx.: biopsy of temporal artery. Lab: increased ESR. Rx.: steroids Q0693:Clinical features of Takayasu asteritis Loss of pulse in upper extremities; visual disturbances; neurologic abnormalities Q0694:Microscopic features of Takayasu arteritis Granulomatous vasculitis with massive intimal fibrosis; thickening of the aortic arch and narrowing of the major arterial branches Q0695:Clinical features of Buerger's disease Severe pain in affected extremity; thrombophlebitis; Raynaud phenomenon; ulceration and gangrene. Associated with heavy cigarette smoking Q0696:Microscopic features of Buerger's disease Recurrent neutrophilic vasculitis with microabseses; segmental thrombosis and vascular insuficiency Q0697:Clinical features of Kawasaki disease Affects children < 4. Acute febrile illness; conjuctivitis; maculopapular rash; lymphadenopathy; coronary aneurysms in 70% of cases Q0698:Microscopic features of Kawasaki disease Segmental necrotizing vasculitis with coronary aneurysms Q0699:Diseases that feature Raynaud phenomenon SLE; CREST; Buerger; atherosclerosis Q0700:Raynaud diseasse Small artery vasospasm resulting in blanching cyanosis of fingers and toes precipitated by cold temperature and emotions Q0701:Henoch-Schonlein purpura IgA-C3 immunocomplexes; IgA nephropathy (Berger disease); palpable purpura on buttocks Q0702:Major risk factors for atherosclerosis Hyperlipidemia; hypertension; smoking; diabetes Q0703:Most common sites for atherosclerosis Abdominal aorta followed by coronary arteries Q0704:Complications of atherosclerosis Ischemic heart disease; abdominal aortic aneurysm; peripheral vascular disease (pain; pulselessness; paresthesia; claudication); TIA (vertebral basilar oclussion); renovascular hypertension (high renin). Q0705:Pathophysiology of essential hypertension Retention of sodium and water with increase in stroke volume (systolic pressure). Sodium in smooth muscle opens up calcium channels with vasoconstriction of arterioles (increased diastolic pressure). Low renin hypertension. Q0706:Complications of hypertension Concentric ventricular hypertrophy; AMI; hyaline arteriosclerosis; nephrosclerosis and CRF; intracranial bleeds; athersoclerosis Q0707:Renovascular hypertension Atherosclerosis of renal artery orifice in males or fibromuscular hyperplasia in women. Severe hypertension; epigastric bruit. High renin hypertension. Screen with captopril. Q0708:Captopril screening test for renovascular hypertension In renovascular hypertension there's decreased RPF and high levels of renin and angiotensin II. With captopril (ACE inhibitor); there's loss of negative feedback on renin and exagerated high levels of renin post-stimulation. The test has the potential for renal failure if bilateral renal artery stenosis is present as AII is responsible for maintaining renal blood flow. Q0709:Ahterosclerotic aneurysms MC site is abdominal aorta below renal arteries (no vasa vasorum). Pulsitile mass with pain and abdominal bruit Q0710:Syphilitic aneurysm Obliterative endarteritis of vasa vasorum with ischemia and atrophy of ascending aorta; aortic insuficiency; airway encroachment and laryngeal nerve involvment (brassy cough) Q0711:Associated diseases of dissecting aortic aneurysm Marfan; Ehlers-Danlos; copper deficiency (no lysyl oxidase) Q0712:Signs and symptoms of dissecting aortic aneurysm Acute retrosternal severe chest pain; aortic insuficiency and cardiac tamponade Q0713:Phlebothrombosis Vs. Thrombophlebitis Phlebothrombosis is venous thrombosis of deep veins without inflamation or infection. Thrombophlebitis is venous thrombosis of superficial veins due to inflamation and infection Q0714:Signs; symptoms; diagnosis and complications of DVT Leg swelling; warmth; erythema. Increased venous pressure from deep to superficial veins (which drain in deep veins) produces varicosities in superficial system. Complications are thromboembolism; thrombophlebitis. Dx.: Doppler Q0715:Signs; symptoms and causes of thrombophlebitis Palpable cord; pain; induration; warmth; erythema. MCC is superficial varicose veins; phlebothrombosis; catherthers; drug abuse Q0716:Clinical features of varicose veins Edema; thrombosis; stasis dermatitis; ulcerations Q0717:Clinical features of superior vena cava syndrome Compression of SVN by primary lung cancer. Blue discoloration of the face; arms and shoulders; dizziness; convulsions; visual disturbances; distended jugular veins Q0718:Clinical features of Kaposi sarcoma Malignant endothelial cell tumor caused by HHV-8. Multiple red-purple patches; plaques or nodules. Spindle shaped cells Q0719:What will aspiration of a foreign body result in the lung? Obstruction atelectasis Q0720:Presence of fluid; air or tumor in the pleural space results in what type of atelectasis? Compression atelectasis Q0721:Contraction atelectasis is due to what cause? Fibrosis of the lung Q0722:Causes of patchy atelectasis Lack of surfactant (hyaline membrane disease of newborn or ARDS) Q0723:Clinical features of typical pneumonia Sudden onset; high fever; productive cough; tachypnea; pleuritic chest pain; consolidation on x-ray Q0724:Clinical features of atypical pneumonia Insidious onset; low fever; no cough; no consolidation Q0725:Differential diagnosis of rusty sputum Strep pneumonia; CHF; mitral stenosis; Goodpasture syndrome Q0726:Features of sarcoidosis "GRAIN": gammaglubilinemia; rheumathoid arthritis; ACE increase; interstitial fibrosis; non-casseating granuloma; bilateral lymphadenopathy Q0727:Causes of restrictive pulmonary disease Kyphoscoliosis; obesity; pneumoconiosis; ARDS; pulmonary fibrosis; sarcoidosis Q0728:Causes of obstructive pulmonary disease Asthma; emphysema; chronic brnchitis; bronchiectasis Q0729:Lung volumes in obstructive pattern Increased TLC; FRC and RV. Decreased FEV1; FVC; FEV1/FVC Q0730:Lung volumes in restrictive pattern Decreased; TLC; FEV1; FVC; FRC; RV. Increased or normal FEV1/FVC Q0731:Diagnosis criteria for chronic bronchitis Persistent cough and copius sputum production for at least 3 months in 2 consecutive years Q0732:Clinical features of chronic bronchitis Cough; sputum production; dyspnea; infections; hypoxia; cyanosis; weight gain. "Blue bloater" Q0733:Microscopic findings in chronic bronchitis Hypertrophy of bronchial mucous glands; globlet cell hyperplasia; mucus hypersecretion; bronchial metaplasia Q0734:Complications of chronic bronchitis Recurrent infections; cor pulmonale; lung cancer Q0735:Definition of emphysema destruction of alveolar septa resulting in enlarged air spaces and loss of elastic recoil Q0736:Etiology of emphysema Protease/antiprotease imbalance. Proteases are made by macrophages and neutrophils. Antiproteases are alpha-1- antitrypsin; alpha-1-macroglubulin and secretory leukoprotease inhibitor Q0737:Features of centriacinar emphysema Proximal brnchioles involved; distal brnchioles spared; most common (95%); associated with smoking; worst in apical segments of upper lobes Q0738:Features of panacinar emphysema Entire acinus invololved; alpha-1-antitrypsin deficincy; worse in bases of lower lobes Q0739:Clinical features of emphysema Progressive dyspnea; pursing of lips and accesory muscles; barrel chest; weight loss; "Pink puffer"' Q0740:Clinical features of asthma Wheezing; severe dyspnea; coughing Q0741:Microscopic features of asthma Charcot-leyden crystals; mucous plugs; goblet cell hyperplasia and hypertrophy; eosinophils; edema; hypertrophy of smooth muscle; thick basement membranes Q0742:Clinical features of bronchiectasis cough; fever; malodorous purulent sputum; dyspnea; dilated bronchi extending out to pleura on x-ray Q0743:Etiology of bronchiectasis Bronchial obstruction; necrotizing pneumonia; cystic fibrosis; Kartagener syndrome Q0744:Definition of acute respiratory distress syndrome damage of alveolar epithelium and capillaries resulting in respiratory failure that is unresponsive to O2 treatment Q0745:Causes of ARDS shock; sepsis; trauma; gastric aspiration; radiation; O2 toxicity; drugs; infections Q0746:Clinical features of ARDS dyspnea; tachypnea; hypoxemia; cyanosis; use of accesory respiratory muscles. Bilateral lung opacity on x-ray Q0747:Microscopic features of ARDS interstitial and alveolar edema; interstitial inflamation; loss of type I pneumocytes; hyaline membrane formation Q0748:RDS of newborn Deficiency of surfactant in prematures (<28 weeks) and sons of diabetic mothers. Dyspnea; tachypnea; nasal flaring and cyanosis. Lecithin:sphyngomyelin < 2. Rx.: surfactant and dexamethasone Q0749:Causes of pulmonary edema left heart failure; mitral stenosis; fluid overload; nephrotic syndrome; liver disease Q0750:Microscopic features of pulmonary edema Intra-alveolar fluid; engorged capillaries; hemosiderin-ladden macrophages Q0751:Risk factors and genetics of bronchogenic CA Cigarette smoking; pneumoconiosis; pollution. Oncogenes: L- myc (SCC); K-ras (adeno). Suppressor genes: p53 and Rb Q0752:Clinical features of bronchogenic CA Cough; sputum production; weight loss; anorexia; fatigue; dyspnea; hemoptysis; chest pain. Obstruction may produce emphysema; atelectasis; bronchiectasis or pneumonia Q0753:Lung adenocarcinoma Most common - 35%. More common in women. Peripheral gray mass; scarring and mucin-producing glands Q0754:Squamous cell carconima 2nd most common - 30%. More common in males; related to smoking. Centrally located. Invasive squamous cells with desmosomes and keratin production; PTH production Q0755:Small cell carcinoma 20%. More common in males; associated to smoking. Central location. Basophilic neurosecretory granules and paraneoplastic syndromes (ACTH; ADH) Q0756:Pancoast tumor Apical tumor causing Horner syndrome (ptosis; miosis; anhidrosis; enopthalmos) Q0757:Superior vena cava syndrome Obstruction; distended head and neck veins; plethora; facial edema Q0758:Effects of lung masses within the thorax structures Pancoast tumor; superior vena cava syndrome; esopahgeal obstruction; recurrent laryngeal nerve hoarseness; Eaton- Lambert syndrome Q0759:Sites of metastasis of lung cancer Adrenals (>50%); liver; brain; bone Q0760:Eaton-lambert syndrome auto-antibodies against presynaptic Ca channels in neuromuscular junction Q0761:Metastasis to the lung Breast (most common) Q0762:Clara Cells found in bronchioles;non-ciliated columnar with secretory granules;secrete part of surfactant; degrade toxins; act as reserve cells Q0763:Goblet Cells found from trachea to terminal bronchioles;secrete mucus Q0764:Pseudocolumar Cells ciliated cells found from nose to respiratory bronchioles Q0765:When are lungs mature? L:S ratio > 2.0 Q0766:Where does aspirant go? when standing; posterobasal segment of R. lower lobe;when supine; superior segment of R. lower lobe Q0767:Relation of artery to bronchus? RALS;on the right; pulmonary artery is anterior to bronchus;on left; pulmonary artery is superior to bronchus Q0768:At what levels do structures pass through diaphragm? T8=IVC;T10=oesophagus;T12=aorta; thoracic duct; azygous Q0769:Causes of neonatal RDS? prematurity;maternal DM with high insulin (insulin decreases surfactant synthesis);C-section (denies baby of the of stress/cortisol which increases surfactant synthesis) Q0770:Causes of Right Shift in O2-HgB curve? CADET face right;CO2;Acid/altitude;DPG;Exercise;Temperature Q0771:CO poisoning left shit in O2-HgB curve; decreases O2 binding capacity Q0772:Innervation of lungs (sensory) parietal pleura = phrenic (c3;c4;c5) refers to neck/shoulders and by intercostals;visceral pleura = vagus Q0773:when do you hear fremitus? lobar pneumonia Q0774:Paraneoplastic syndromes associated with lung cancer? squamous cell = PTHrP;small cell = ACTH; ADH; Eaton Lambert Q0775:Locations of Lung Cancers central = squamous; small S's;peripheral = adenocarcinoma Q0776:Relation of Lung Cancer with smoking all related; but adenocarcinoma the least related;S's Q0777:What's the difference between small and non-small cell carcinomas? for small cells; surgery is useless Q0778:Horner's Symptoms ptosis (droopy eyelid);miosis (pupil constriction);anhidrosis (lack of sweat) Q0779:COPD = also known as OLD: obstructive lung disease: why is it called obstructive? obstruction of AIR FLOW → air trapping in lungs Q0780:COPD = also known as OLD: obstructive lung disease: what is the major PFT finding? FEV1 / FVC ration is decreased (hallmark finding) Q0781:COPD = also known as OLD: obstructive lung disease: name the 4 types of COPD 1) Chronic Bronchitis (Blue Bloater) 2) emphysema (pink puffer) 3) asthma 4) bronchietasis Q0782:COPD = also known as OLD: obstructive lung disease: what is the definition of Chronic Bronchitis productive cough for >3 consecutive months in two or more years. Q0783:COPD = also known as OLD: obstructive lung disease: what do you expect on lung histology? hypertrophy of mucus-secreting glands in the bronchioles (Reid index of >50%) Q0784:COPD = also known as OLD: obstructive lung disease: leading cause is smoking: what are the physical findings for Chronic Bronchitis? (name 3) 1)cyanosis 2) wheezing 3) crackles Q0785:what is the pathophysiological mechanism of EMPHYSEMA? destruction of fibrous septa/alveolar walls → enlargement of air space and decreased total surface area for gas exchange Q0786:if the cause is smoking; what kind of emphysema would you see on histo slide? centri-acinar emphysema Q0787:what else can cause emphysema: (also may work synergistically with smoking): What kind of findings do you see in lung histo and name another organ affected? alpha 1-antitrypsin deficiency → panacinar emphysema + liver cirrhosis Q0788:what causes the emphysema in this disorder? increased elastase activity to damage lung tissue. Q0789:name 4 findings of emphysema (in general) 1) dyspnea; 2) decreased breath sounds 3) tachycardia 4) decreased I/E ratio Q0790:What is mechanism of asthma BRONCHIAL hypersensitivity/hyperresponsiveness → REVERSIBLE bronchoconstriction Q0791:name 3 common asthma triggers 1) viral URI 2) allergens 3) stress!! Q0792:name 7 asthma findings 1) cough 2) wheezing 3) dyspnea 4) hypoxemia 5)decreased I/E ratio 6) tachypnea 7) pulsus paradoxus Q0793:BRONCHIECTASIS: what is its mechanism?? chronic necrotizing infection of BRONCHI → dilated airways; purulent sputum; recurrent infections; hemoptysis (see Robbins for a good discussion of this) Q0794:what disorders is bronchietasis commonly associated with? 1) bronchial obstruction 2) cystic fibrosis 3) poor ciliary motility Q0795:What are classic PFT findings for RLD? decreased VC decreased TLC ; FEV1/FVC ration > 80% Q0796:Name the two MAJOR types of RLD 1) poor breathing mechanics (EXTRA-pulmonary) 2) Interstitial lung diseases Q0797:Name 3 lung volumes that are increased in COPD increased TLC; increased FRC; increased RV Q0798:What 2 values are BOTH reduced in COPD and RLD? 1) FEV1 and 2) FVC (think FEV1/FVC ratio) NOTE! in COPD; FEV1 is more dramatically reduced and thus the FEV1/FVC ratio is decreased Q0799:what is the main pathology resulting from asbestosis? DIFFUSE; interstitial fibrosis caused by inhaled asbestos Fibers. Q0800:What cancers are increased in pts with asbestosis? 1) pleural mesothelioma 2) bronchogenic carcinoma (BC) Q0801:Major finding in lung w/ asbestosis? Ferruginous bodies: asbestos fibers coated with hemosiderin also 2) ivory white pleural plaques Q0802:Neonatal respiratory distress syndrome: What is the main cause surfactant deficiency --> to increased surface tension --> alveolar collapse Q0803:Neonatal respiratory distress syndrome: surfactant is made by which cells? After when? type 2 pneumocytes after 35th gestational week Q0804:Neonatal respiratory distress syndrome: what do you measure? Where do you get this fluid? lecithin-to-sphingomyelin ratio in the amniotic fluid = measure of lung maturation <1.5 in neonatal distress syndrome Q0805:Neonatal respiratory distress syndrome: what is surfactant made of (chemical name) dipalmitoyl phosph-tidyl-choline (DP-PTC) Q0806:Neonatal respiratory distress syndrome: treatment for poor maturation of lungs 1) before birth = maternal steroids 2) after= artificial surfactant Q0807:Karta-gener's syndrome: what is this? immotile cilia due to dynein arm defect Q0808:Karta-gener's syndrome: results in what in female and male? (4 things) 1) sterility (in male sperm also immotile) 2)bronchietasis 3)recurrent sinusitis (bacteria & particles not pushed out) 4) associated with situs inversus (e.g. dextro-cardia) Q0809:name the 3 main classes of cancers that affect parts of the lung 1) bronchogenic carcinoma (with different subtypes) 2) carcinoid tumor 3) metastasis Q0810:list the 5 types of major bronchogenic carcinomas CENTRAL 1) squamous cell ca 2)small cell ca; PERIPHERAL 3) adenocarcinoma 4) bronchoalveolar ca 5) large cell ca Q0811:mnemonic: what is meant by SPHERE of symptoms? S= superior vena cava syndrome; P= pancoast tumor; H= Horner's syndrome; E= Endocrine (paraneoplastic); Recurrent laryngeal / hoarseness; E = Effusions (pleural OR pericardial) Q0812:What can a CARCINOID tumor cause? Carcinoid Syndrome = flushing; diarrhea; wheezing; and salivation Q0813:Metastases to lung is very common; LUNG cancer also prone to metastasize to what other parts? 1) brain (epilepsy) 2) bone (fracture) 3)liver (jaundice + hepatomegaly) Q0814:What is Pancoast's tumor? it's a carcinoma of the apex of lung Q0815:what may Pancoast's tumor affect? may affect CSP= cervical sympathetic plexus causing Horner's syndrome Q0816:what is Horner's syndrome? P.A.M. is Horny = Ptosis; Anhydrosis; Miosis Q0817:1st AID breaks it down into TYPE/ ORGANISM/ CHARACTERISTICS = cover parts of this table and fill in the blanks 0 Q0818:what causes acute epiglottitis; which may be life- threatening in young children? H. influenzae Q0819:MC nasal and laryngeal tumor Squamous cell CA Q0820:Dx;Decreased FEV1/FVC ratio COPD Q0821:Dx;Charcot-Leyden crystals; Curschmann spirals; bronchial smooth muscle hypertrophy Asthma Q0822:Definition;Productive cough occurring at least 3 consecutive months over at least 2 years;what change occurs w/ cells? Chronic Bronchitis;;(Inc mucous due to hyperplasia of mucous-secreting glands) Q0823:Definition;Dilation of air spaces w/ destruction of alveolar walls and lack of elastic recoil Emphysema Q0824:Type of emphysema dealing with smoking Centriacinar Q0825:Type of emphysema dealing with A-1-A deficiency;what gene is difficient in most severe? Panacinar;(piZZ - homozygous) Q0826:Type of emphysema dealing with blebbing or subpleural bullae Paraseptal Q0827:Definition;perminant abnormal bronchial dilation caused by chronic infection w/ inflammation and necrosis of the bronchial wall Bronchiestasis Q0828:Dx;dilated airways; copious purulent sputum; hemoptysis and recurrent pulmonary infections leading to lung abscess (obstruction) Bronchiectasis Q0829:Definition;Diffuse alveolar damage w/ resultant increase in alveolar permeability; causing a leak of protein-rich fluid from the alveoli and formation of intra-alveolar hyaline membrane Adult Respiratory Distress Syndrome (ARDS) Q0830:Definition;Group of environmental diseases caused by inhalation of inorganic dust particles Pneumoconioses Q0831:Dx;inhalation of carbon dust; marked by carbon- carrying macrophages Anthracosis Q0832:Dx;progressive massive fibrosis w/ necrotic black fluid and dark macules around bronchioles Coal Miners Pneumoconiosis Q0833:Dx;diffuse interstitial fibrosis mainly in the lower lobes w/ ferruginous bodies and dense fibrocalcific plaques of parietal pleura;what can it lead to? Asbestosis;leads to: Mesothelioma Q0834:Dx;bilateral hilar lymphadenopathy; hypercalcemia; noncaseating granulomas Sarcoidosis Q0835:Dx;localized proliferation of histiocytes that have characteristic inclusions resembling tennis rackets (birbeck granules) Eosinophilic granulomas Q0836:Definition;Immune complex Dz w/ progressive fibrosis of the alveolar wall Idiopathic Pulmonary fibrosis Q0837:MCC of Pulmonary HTN COPD Q0838:MCC of interstitial pneumonia;(2) Mycoplama Pneumoniae;;Viruses Q0839:What virus produces a Giant cell pneumonia? Measles Q0840:What pneumonia is seen in people working with cattle or sheep? Rickettsial pneumonia;(Q-fever) Q0841:Dx;Gohn complex Primary TB Q0842:Pulmonary infection;Abscess and sinus tract formation w/ exudate containing sulfur granules Actinomycosis Q0843:Tx of Neonatal Respiratory Distress syndrome Give mother steroids before birth;(or artificial surfactant for infant) Q0844:MCC of Lobar pneumonia Pneumococcus Q0845:Definition;Carcinoma in the apex of the lung that may affect cervical sympathetic plexus Pancoast tumor Q0846:what are the (2) lung cancers that are central?;what do they have a causitive link to? Squamous cell;Small cell (Oat cell);;both: Smoking! Q0847:Dx;cough; hemoptysis; bronchial wheezing; pneumonic "coin" lesion on x-ray Lung Cancer Q0848:Describe the (3) peripheral Lung Cancers Adenocarcinoma;MC non-smoking; K-RAS oncogene;Bronchoalveolar;Tall columnar cells w/ along alveoli;Large cell;Undifferentiated Q0849:Dx;lung tumor of major bronchi that may also cause flushing; diarrhea; wheezing and salivation Carcinoid tumor Q0850:(5)* complications of Lung tumors SPHERE;Superior vena cava syndrome;Pancoast tumor;Horner's syndrome;Endocrine (paraneoplastic);Recurrent Laryngeal Sx (hoarsemess);Effusions (pleural or pericardial) Q0851:Causes of Respiratory sounds;Stridor Inspiratory sound from narrowing extrathoracic area ;(above glottis) Q0852:Causes of Respiratory sounds;Wheezing Narrowing of the intrathoracic airway during expiration Q0853:Causes of Respiratory sounds;Crackles Alveoli popping open ;due to;fluid (pneumonia; HF; ARDS) ;or fibrosis Q0854:Causes of Respiratory sounds;Dullness to Percussion fluid; pus; tumor or blood absorbing the sound b/t alveoli and chest wall Q0855:Causes of Respiratory sounds;Hyperresonance Air under the chest wall;(Pneumothorax) Q0856:Causes of Respiratory sounds;Fremitus / Egophany Consolidation;(Pneumonia) Q0857:Dx;Increased Cold Agglutinin Mycoplasma pneumonia Q0858:Dx;laryngeal edema; steeple sign; unusual cough Parainfluenza;(Croup) Q0859:Dx;inhaling quartz or talc Silicosis Q0860:what is the second MCC of lung CA? Radon gas Q0861:Dx;Tracheal deviation towards side of lesion;(one main one possible) Atelectasis;(Bronchial obstruction) Q0862:Dx;Tracheal deviation away from the side of lesion;(one main one possible) Pneumothorax;(Pleural Effusion) Q0863:Dx;bronchiectasis; recurrent sinusitis; infertility; situs inversus;defect? Kartagener's syndrome;(Dynein arm defect) Q0864:What is the most common cause of typical community acquired pneumonia? Streptococcus pneumoniae Q0865:What is the most common cause of atypical pnuemonia? Mycoplasma Pneumoniae Q0866:Interstitial pneumonia is; atypical Q0867:bronchopneumonia is; typical Q0868:signs of consolidation; in terms of percussion; egophany; tactile fremitus; whispered pectorliloquy dullness to percussion;egophany +;increased tactile fremitus;whipsered pectoriliquy + Q0869:Consolidation indicates atypical or typical pneumonia? Typical. Q0870:What is the most common source of nosocomial pneumonia infection? Respirators. Q0871:What pathogen is usu responsible for respirator contracted pneumo? Pseudomonas aeruginosa Q0872:What three pathogens are associated with nosocomial pneumo? Pseudomonas aeruginosa; Escheria coli; Staph aureus. Q0873:Most common cause of the common cold. Rhinovirus Q0874:What type of influenzavirus is most often involved? A Q0875:2nd most common cause of atypical pneumonia Chlamydia pneumoniae Q0876:Case: newborn: afebrile; staccato cough; conjunctivitis; wheezing. Pathogen? Chlamydia trachomatis Q0877:Water loving bacteria that causes green sputum Pseudomonas aeruginosa (respirators!) Q0878:Most common cause of pneumonia in alcoholics. Then another cause. Klebsiella pneumoniae or just S. pneumoniae. Q0879:How do you differentiate between the two common causes of pneumo in alcoholics? Klebsiella pneumoniae causes thick mucoid sputum; where as S pneumo does not. Q0880:What bacterial cause of pneumo is associated with water coolers and produce mists? Legionella pneumophila Q0881:Case: Pneumo with hyponatremia. Pathogen and pathophys? Legionella can cause tubulointerstitial disease with JG destruction learing to hyporeninemic hypoaldosteronism with hyponatremia; hyperk; and met acid. Q0882:What is the most common opportunistic fungal infection and where is it found in the community? Cryptococcus neoformans; found in pigeon poop. Q0883:What fungal infection is associated with Ohio/central Mississippi River valley? What 3 animals carry it? Histoplasma capsulatum. Bats; starlings; chickens. Thus assoc with cave explorers; spelunkers; and chicken farmers. Q0884:What systemic fungi is associated with the central and southeast US? Blastomyces dermatidis. Q0885:SW "Valley fever;" increases after earth quakes (in dust). Coccidioides immitis. Q0886:Cave explorer in the Arizona desert with nonproductive cough. Coccidioides; not Histoplasma. SW! dry! Q0887:I say pigeons; you say; Cryptococcus neoformans! Q0888:What yeast will be phagocytosed by macrophages? Histoplasma Q0889:A nasal polyp in a kid is most likely caused by; Cystic fibrosis. Q0890:A nasal polyp in an adult is most likely caused by; Allergies. Q0891:A woman with chronic pain has asthma. What else? A nasal polyp caused by NSAID use. Block cyclooxygenase; leave lipoxygenase path open. Leukotrienes C-D-E are increased; causing bronchoconstriction. Q0892:What is a medically significant A-a gradient? > 30 Q0893:What causes an increased A-a gradient? Hypoxemia of pulmonary origin. Q0894:Calculate and A-a gradient in a pt breathing .3 O2 who has PCO2 of 80 mmHg and PaO2 of 40 mmHg. PAO2 = 0.3 (713) - 80/0.8 = 114 mmHg;A-a = 114-40 = 74 mm Hg; which is clinically significant Q0895:Name 3 causes of hypoxemia with a normal A-a gradient. 1. Depression of medullary resp centers;- barbituates; brain injury;2. Upper Airway obstruction;-food block; epiglottitis; croup;3. Muscles of respiration dysfunction;-paralyzed diaphragm Q0896:Name 3 general causes of hypoxemia with an increased A-a gradient 1. Ventilation defect;2. Perfusion defect;3. Diffusion defect Q0897:Example of a ventilation defect Airway collapse in ARDS - impaired O2 deliver to alveoli Q0898:Example of perfusion defect Pulmonary emobolus blocks blood flow to alveoli Q0899:2 Examples of Diffusion defects Interstitial Fibrosis;Pulmonary Edema;O2 can't diffuse alveoli->capillary Q0900:What is the most common cause of fever 24-36 hours after surgery? Resorption atelectasis;Ex: mucus plug obstructs airway; alveoli collapse. Q0901:On Tension pneumothorax; what side does the trachea deviate to? Contralateral - pushed by air compressing the lung. Q0902:With resorption atelectasis; what side does the trachea deviate to? Ipsilateral - goes where there is space. Q0903:What cell synthesizes surfactant? Type II pneumocytes. Q0904:What increases surfactant synthesis? (2) Decreases? (2) Increases: cortisol and thyroxine;Decreases: insulin (maternal diabetes); prematurity; c-section (less cortisol) Q0905:How does surfactant work? Prevents alveolar collapse on expiration by reducing surface tension. Q0906:Is Infant RDS a ventilation; perfusion; or diffusion defect? Ventilation; alveoli collapse. Q0907:infant RDS treatment PEEP Q0908:Free radicals from O2 thrx in infant RDS can cause;(2) blindness;bronchopulmonary dysplasia Q0909:What causes hypoglycemia in the newborn? Excess insulin from response to fetal hyperglycemia in maternal diabetes. Q0910:What causes hyaline membranes? proteins leaking out of damages pulmonary vessels Q0911:What causes ARDS? Pathophys. Pulmonary edema from acute alveolar capillary damage. Q0912:What causes capillary damage in ARDS? Acute damage ->macrophages release cytokines-> chemotactic to netrophils -> neuts transmigrate through pulm capillaries - > leave holes -> protein leakage -> hyaline membranes Q0913:Gram + diplocci on sputum stain = ;How do you treat? Strep Pneumo. Treat with Penicillin G. Q0914:How do you prevent Pneumocystis in AIDS pts? Rx TMP/SMX (trimethoprim-sulfamethxazole) when CD4 is <200. Q0915:What 3 things can Aspergillus cause? 1. Aspergilloma: fungus ball in old TB site; hemoptysis;2. Allergic reaction;3. Vessel invasion with hemorrhagic infarction and necrotic bronchopnuemo Q0916:Where do most PE's come from? Clot in femoral vein; NOT lower leg DVT. Q0917:Where will an aspiration mass go if you are sitting? supine? right sided? Which is most common? Sitting = R lower lobe; posterobasal;Supine = R lower lobe; superior seg;*most common;Right side= R upper lobe; superior seg Q0918:What 2 diagnostic tests are used to diagnose PE? Gold standard? 1. VQ scan - V normal; Q off;2. Gold = pulm angiogram Q0919:How do you prevent Pneumocystis in AIDS pts? Rx TMP/SMX (trimethoprim-sulfamethxazole) when CD4 is <200. Q0920:What 3 things can Aspergillus cause? 1. Aspergilloma: fungus ball in old TB site; hemoptysis;2. Allergic reaction;3. Vessel invasion with hemorrhagic infarction and necrotic bronchopnuemo Q0921:Where do most PE's come from? Clot in femoral vein; NOT lower leg DVT. Q0922:Where will an aspiration mass go if you are sitting? supine? right sided? Which is most common? Sitting = R lower lobe; posterobasal;Supine = R lower lobe; superior seg;*most common;Right side= R upper lobe; superior seg Q0923:What 2 imaging diagnostic tests are used to diagnose PE? Gold standard? 1. VQ scan - V normal; Q off;2. Gold = pulm angiogram;NOT spiral CT;D-dimers also useful. Neg rules out PE. Q0924:Interstitial Fibrosis;Restrictive or Obstructive?;FEV1;FVC;FEV1/FVC;TLC Restrictive; all V decreased;FVC; TLC down;FEV1/FVC increased - FEV1 up bc of increased elasticity Q0925:What is Caplan syndrome? Rheumatoid nodules in lung + restrictive disease like Coal WP or Asbestos disease. Q0926:What does Coal WP do to TB risk? Cancer risk? Does not affect TB or cancer risk. Q0927:What does silicosis do to cancer and TB risk? Increases risk of cancer and TB Q0928:What do lung opacities contain in silicosis? Collagen and quartz. HARD as rocks; as they are made of rocks. Quartz is super fibrogenic Q0929:What is the most common lesion related to asbestos exposure? What is the most common disease process? 1. Benign plueral plaques;2. Primary bronchogenic carcinoma;- Mesothelioma takes longer to show up; thus less common than primary cancer Q0930:What 2 complications are common in all 3 pneumoconiosis? (silicosis; coalwp; asbetosis) Cor pulmonale;Caplan syndrome Q0931:What do roofing and working in shipyards expose you to? Asbetos Q0932:What do foundries (casting metal); sandblasting; and mines expose you to? Quartz / Silicosis Q0933:What is the most common sxs of Sarcoidosis? Dyspnea Q0934:What does sarcoidosis do to the lung? causes granulomas in interstitium and nodes; contain multinucleated giant cells Q0935:Name 3 other manifestations of sarcoidosis (outside the lung) 1. Nodular skin lesions;2. Eye lesions;3. Granulomatous hepatitis;4. Enlarged lacrimal glands;5. increased ACE;6. Hypercalcemia Q0936:What is the likely cause of a pleural effusion in a young woman? SLE - Lupus;example of serositis Q0937:Farmer's Lung is caused by; Moldy hay = saccharopolyspora rectivirgula / thermophilic actinomyces;Type III hypersensitivity - immune complex mediated;Type IV with Chronic exposure -> granuloma Q0938:In Emphysema; compliance___ and elasticity___. Compliance increases (inhalation) ;Elasticity decreases (exhalation) Q0939:What's the difference between a transudate and an exudate? Transudate: ultrafiltrate of plasma. ex: heart failure;Exudate: protein-rich; cell-rich fluid;ex: pneumonia; infarction; metastasis Q0940:Pleural fluid protein / serum protein = 0.6;Transudate or exudate? over 0.5 = exudate ;(more protein in fluid than plasma) Q0941:Pleural fluid LDH/serum LDH = 0.7;Transudate or exudate? over 0.6 = Exudate Q0942:What lung cancer can cause Horner's syndrome and why? Pancoast tumor of superior sulcus - usu primary sq cell cancer in extreme apex. Can destroy superior cervical ganglion and cause horners (eye lid lag; miosis - pinpoint pupil; anhydrosis - no sweating) Q0943:What is the most common type of lung cancer? Metastatic more common than primary;Primary; most common is Adenocarcinoma. Q0944:Are cancers caused by smoking usually peripheral or central? Central;However; after filters were installed in cigarettes; the big carcinogens got filtered out. Now; peripheral cancers caused by the small carcinogens are increasing. Q0945:Which two cancers have the strongest association to smoking? 1. Squamous cell;2. Small cell Q0946:Is adenocarcinoma related to smoking? Where in the lungs is it usually found. Adenocarcinoma is not usually associated with smoking; usually found peripherally. Q0947:What is the most important first step in evaluating a "coin lesion" on a chest x-rays. Compare size from previous x-rays. Q0948:What three cancers are most often associated with lung mets? 1. Primary Breast;2. Colon; Renal Cell Q0949:What is the most common symptom of primary lung cancer? What about metastatic? Cough for primary; dyspnea for metestatic. (Still have dyspnea in primary.) Q0950:Where does primary lung cancer metastisize to? 1. Hilar lymph nodes;2. Adrenal gland;3. Liver (50%); brain (20%); bone Q0951:Name 5 issues associated with CF that are not pulmonary. 1. Malabsorption - pancreatic exocrine def;2. Type 1 diabetes - chronic pancreatitis;3. Male infertility;4. Meconium ileus in bebes;5. 2ary Biliary cirrhosis - bile duct obstruction by thick secretions Q0952:What is the most common respiratory pathogen in CF? pseudamonas aeruginosa Q0953:What is the most common cause of death in CF? Respiratory infection. Q0954:Most common cause of bronchiectasis in US? INternational? US - CF;Int - TB Q0955:What condition is associated with cupfuls of sputum production? Bronchiectasis; due to dilation of bronchi and bronchioles that get filled with pus. Ew. Q0956:Extrinsic asthma is associated with what type of hypersensitivity rxn? Type 1 - IgE dependent activation of Mast cells. Q0957:All these things are involved in asthma pathology. Tell me the story of how: Eotaxin; IL4; T cells; eosinophils; mast cells; major basic protein; IL5; histamine; cationic protein; LTC-D-E; Acetylcholine 1. Inhaled allergen sensitizes by stimulating helper T cells (CD4Th2) that release IL 4 and IL 5;IL 4 - IgE production;IL 5 - production and activation of eosinophils;2. Antigens crosslink IgE on mast cells; release histamine + mediators -> bronchoconstriction; mucus; leukocytes;3. Late phase (4-8 hrs later);Eotaxin - chemotactic for eosinophils;Eosinophils release major basic protein and cationic protein; which damage epithelial cells and produce airway constriction;LTC-D-E cause prolonged bronchoconstriction;Acetylcholine causes airway muscle contraction. Q0958:How are IL4 and IL5 involved in extrinsic asthma? Released by Tcells;IL4 stimulates isotope switching to IgE production;IL5 stimulated production and activation of eosinophils. Q0959:Name two causes of emphysema 1. Smoking;2. alpha-1-antitrypin def (AAT) Q0960:What causes airway collapse in emphysema? Loss of elastic tissue - loss of radial traction. Q0961:What causes panacinar emphysema? Centriacinar? Panacinar = AAT def;Centriacinar = smoking Q0962:Curschmann spirals and Charcot-Leyden crystals are histologic evidence of what disease? Asthma. Q0963:What lung cancer is associated with hypercalcemia? Squamous Cell Q0964:What lung cancer is associated with Cushings? Small Cell Q0965:(3) branches of the celiac trunk Left Gastric A;Splenic A;Common Hepatic A Q0966:(2) branches of the Common Hepatic Artery that supply the stomach Right Gastric A;Gastroduodenal A Q0967:trace the Gastroduodenal Artery to the spleen GDA -> R. Gastroepiploic A ->;L. Gastoepoploic A -> spleen;(Gastroepiploic AA supply blood to Greater curvature of stomach - can also be called Gastro-omenal AA) Q0968:Name the Veins that you would see varices in w/ portal HTN;(3 sets) Esophageal;Left gastric -> Azygos;External hemorrhoids;Superior -> Inferior rectal;Caput Medusae;Paraumbilical -> Inferior Epigastric Q0969:what does the Submucosa portion of the GI contain and control? Submucosa;meiSSner's plexus;control;Secretions; blood flow and absorption Q0970:what does the Muscularis portion of the GI contain and control? Muscularis;Myenteric Auerbach's plexus;control: Motility Q0971:what is the immunoglobulin of the gut? IgA Q0972:Definiton;Secrete alkaline mucous to neutralize acid contents entering the duodenum from the stomach Brunner's glands Q0973:what is the name of the structure where the hindgut meets the ectoderm in development?;why is it significant? Pectinate line;Above line: Internal hemorrhoids;Below line: External Q0974:Innervation; blood supply and pain level to Internal Hemorrhoids Nerves: Visceral innervation;Blood: Superior rectal A;Pain: none Q0975:Innervation; blood supply and pain level to;External Hemorrhoids Nerves: Somatic innervation;Blood: Inferior rectal (Pudendal branch);pain: Painful Q0976:what are the objects from lateral to medial in the Femoral Triangle? NAVEL;Nerve;Artery;Vein;(Empty);Lymphatics Q0977:what are the top; medial and lateral edges of the Femoral triangle? Top: Inguinal ligament;Medial: Adductor Longus m;Lateral: Sartorius m Q0978:What is Hasselbach's triangle made of? Lateral: Inferior Epigastric A;Medial: Rectus Abdominis;Bottom: Inguinal ligament Q0979:best landmark to determine of the inguinal hernia is Direct or Indirect Inferior Epigastric artery;[MDs don't LIe];Medial to artery = Direct;Lateral to artery = Indirect Q0980:why is the Indirect hernia more common in infants? failure of Processus Vaginalis to close Q0981:what is the primary cause of Achalasia?;secondary? Primary;Failure of LES to relax due to loss of Auerbach's plexus;Secondary;Chagas Dz Q0982:Dx;"Olive" mass in epigastric region and projectile vomiting at 2 weeks of age Pyloric stenosis Q0983:Dx;Infecton of GI causing PAS-positive macros in intestinal lamina propria and mesenteric nodes Whipple's Dz;(T. Whippelii) Q0984:Definition;epithelial tumor arising from precursor cells of the enamel origin Ameloblastoma Q0985:MC Salivary gland tumor Pleomorphic Adenoma Q0986:Dx;newborn w/ copious salivation assoc w/ choking; coughing and cyanosis on attempts at eating Tracheoesophageal fistula Q0987:Definiton;Acute gastritis in assoc w/ severe burns Curling Ulcer Q0988:Definiton;Acute gastritis in assoc w/ brain injury Cushing ulcer Q0989:Definition;focal damage to the gastric mucosa w acute inflammation; necrosis and hemorrhage Acute (erosive) Gastritis Q0990:Dx;Autoimmune dz; pernicious Anemia; Achlorhydria;where in stomach? Chronic (nonerosive) Gastritis Type A (3 As);(Fundal) Q0991:MC form of Chronic Gastritis;where in stomach? Type B;H. Pylori-assoc Gastritis;[B = Bug];(Antrum) Q0992:Dx;Pain greater w/ meals;Ulcer cause? Gastric;[Gastric = Greater w/ meals];Cause;low mucosal protection vs. acid;(H.pylori and NSAIDs) Q0993:Dx;Pain Decreases w/ meals;Ulcer cause? Duodenal ulcer;[Duodenal = Dec w/ meal];Cause: H.pylori Q0994:MC type of stomach cancer;(2) Extra-GI sites of involvement Adenocarcinoma;extra sites;Virchow node;;Krukenberg tumor - signet-ring cells in bilateral Ovary Q0995:Definition;Stomach cancer that is diffusely infiltrative (thickened; rigid appearance) Linitis Plastica Q0996:Definition;extreme enlargement of gastric rugae and possible loss of plasma proteins from altered mucosa Menetrier Dz;(giant hypertrophic gastritis);[MENE large Rugae] Q0997:Dx;colon inflammation w/ crypt abscesses and pseudopolyps Ulcerative colitis Q0998:Definition;twisting of portion of bowel around its mesentary Volvulus Q0999:remnant of Embryonic Vitelline duct Meckel's Diverticulum Q1000:Dx;inc serotonin; cutaneous flushing; diarrhea; bronchospasm; valvular lesions of right heart; RLQ mass Carcinoid tumor;(MC place = Appendix) Q1001:Definition;dilation of the colon due to the absence of ganglion cells;underlying cause? Hirschsprungs Dz;(colon SPRUNG open);cause: failure of neural crest cells to migrate Q1002:Dx;trisomy 21 patient w/ chronic constipation early in life Hirschsprungs Dz Q1003:MC place for ischemic bowel due to being poorly vascularized regions;(2) Splenic flexure;Rectosigmoid junction Q1004:Dx;Unexplained lower bowel bleeding Angiodysplasia Q1005:Dx;inflammation of the colon w/ flask-shaped ulcers Amebic colitis;(E. Histolytica infection) Q1006:Dx;barrium swallow has "apple core" lesion in lower GI Colorectal cancer Q1007:which polyp is benign?;which is most malignant? Benign: Tubular Adenoma;;most malignant: Villous Adenoma Q1008:Dx;polyps of the colon and small intestines and melanotic accumulations in the mouth; lips; hands and genitals Peutz-Jegher syndrome Q1009:Dx;adenomatous polyps w/ osteomas and soft tissue tumors Gardner syndrome Q1010:Dx;adenomatous polyps w/ tumors of the CNS Turcot syndrome Q1011:Causes of Micronodular Cirrhosis;(3) Metabolic insult;Wilsons Dz;;Alcohol;;Hemochromatosis Q1012:what type of shunt may releive portal HTN?;connecting what vessels? Portacaval shunt;between Splenic vein and Lt Renal vein Q1013:Another name for AST in lab SGOT Q1014:Dx;swollen; necrotic hepatocytes; PMN infiltrates; intracytoplasmic eosinophilic inclusions; fatty change; sclerosis around central vein Alcoholic Hepatitis Q1015:Best tx for staph food poisoning? Bed rest Q1016:Bacterial infection from raw oyster? Cholera Q1017:What is the antibx of choice for severe cholera? Tetracycline Q1018:Tightly knotted ball of nematodes (15 to 35 cm in length); worms have tapered ends without hooks;species? Ascaris lumbricoides Q1019:Which Vibrio cholera serogroup is the most common cause of cholera epidemics? 1 Q1020:Celiac disease is associated with which HLAs? Predisposes to which neoplasms? HLA-B8 and DQW2; predisposes to T-cell lymphoma; and GI and breast cancer Q1021:The retropharyngeal space is located between which two layers of fascia? Buccopharyngeal layer of deep cervical fascia and the prevertebral layer of deep cervical fascia Q1022:Metoclopramide is used to treat;? can antagonize which receptors? Prokinetic agent used to treat GERD. Can antagonize dopamine receptors. Q1023:Both pancreatic buds form from evaginations from which part of the duodenum? Second part Q1024:Which antibiotic can cause a disulfiram-like reaction when taken with alcohol? Metronidazole Q1025:Which vessel gives off the short gastric arteries? Splenic artery Q1026:Internal hemorrhoids are produced by varicosities of which vein? Superior rectal vein Q1027:Misoprostol is what type of drug? Prophylaxis to prevent;? Prostaglandin analog to prevent NSAID-induced ulcers Q1028:Which muscle elevates the lower jaw during chewing? Moves the lower jaw forward? Backward? Medial pterygoid. Lateral pterygoid. Temporalis Q1029:Which salivary gland has the greatest % of mucus secreting cells? Serous? Sublingual. Parotid. Q1030:Which H2-receptor antagonist is a potent hepatic enzyme inhibitor? Cimetidine Q1031:What type of mucosa is normal for the distal esophagus? Non-keratinized; stratified; squamous epithelium Q1032:What ligament separates the greater peritoneal sac from the right portion of the lesser peritoneal sac? Gastrohepatic ligament Q1033:What type of heterotopia can occur in the small intestine without causing bleeding? Pancreatic heterotopia Q1034:The left renal vein drains into;? IVC Q1035:The splenorenal ligament contains which vessels? The splenic artery and vein Q1036:Mesalamine is indicated for the treatment of;? Ulcerative colitis. Q1037:Pseudomembranous colitis produces what kind of diarrhea? Greenish; foul-smelling watery diarrhea. Q1038:The right gastroepiploic artery is a branch of;? Gastroduodenal artery Q1039:Which anesthetic can cause idiosyncratic hepatic failure? Halothane Q1040:Elevated AST and ALT reveal what process in which tissue? Hepatocellular death Q1041:CCK is secreted in response to;? The presence of amino acids and fatty acids in the duodenum Q1042:Ondansetron selectively antagonizes which receptors? 5HT3 receptors Q1043:Lipoprotein lipase is located where? External surface of endothelium of tissues with triglyceride needs such as muscle and lactating breast. Q1044:What type of stones can be seen in liver fluke infestation? Bilirubin stones Q1045:The free edge of the lesser omentum contains which three important structures? Common bile duct; hepatic artery; and the portal vein Q1046:What agents are absolutely contraindicated in patients with bowel obstruction? Prokinetics; such as metoclopramide Q1047:Gastroschisis? Caused by? Abdominal viscera protrude from the abdominal cavity at birth. Caused by incomplete fusion of the lateral body folds Q1048:Motilin is released by;? Small intestine Q1049:How does secretin affect the rate of stomach emptying? Decreases the rate of stomach emptying Q1050:Inferior rectal nerve is a branch of;? Pudendal nerve of the sacral plexus Q1051:Sepsis; hypotensions; and neutrophilia in an infant who has been started on oral foods;? Necrotizing enterocolitis. Q1052:Which narcotic is least likely to cause spasm of the sphincter of Oddi? Why? Meperidine; due to its anti-muscarinic properties. Q1053:Superior pancreaticoduodenal artery gets it's blood supply from? Inferior? Celiac trunk by means of the common hepatic artery. SMA. Q1054:Which tx for GERD could cause torsades de pointes when given with erythromycin? Cisapride. Q1055:Disulfiram? a drug used to support the treatment of chronic alcoholism by producing an acute sensitivity to alcohol. Q1056:This parasite can cause mild diarrhea; or more aggressive dysentery; peritonitis; or liver abscess formation; Entamoeba histolytica Q1057:Microabscesses in mesenteric lymph nodes is associated with which bug? Yersinia enteritis Q1058:Enlarged and ulcerated Peyer's patches are associated with which infection? Salmonella typhii Q1059:Cholera enterotoxin activates which enzyme? Adenylate cyclase Q1060:Megaloblastic anemia can be caused by which parasite? Diphyllobothrium latum Q1061:Kid vomits up a cylindrical white worm 30 cm long;How was this organism acquired? Ingestion of eggs from human feces (Ascaris lumbricoides) Q1062:Which hepatitis virus is a major cause of epidemics but does not cause significant mortality in pregnant women? Hepatitis A Q1063:A patient with dumping syndrome should eat small; frequent meals that are high in;? Fat Q1064:What deficiency could cause a decrease in serum calcium and phosphate? Vitamin D Q1065:Mini epidemic of pediatric diarrhea;? Yersinia Q1066:Traveler's diarrhea - empiric tx? Ciprofloxacin Q1067:Undercooked chicken - bloody diarrhea; abdominal pain; and fever;? Campylobacter jejuni Q1068:Liver abscesses - what parasite;? Entamoeba histolytica Q1069:OOcysts 5-7 microns in diameter;organism? Where does it's sexual phase occur? Crytosporidiosis - invades the brush border of the intestinal epithelium Q1070:An obstruction in the common bile duct would affect the urine how? Increased urine bilirubin - tea-colored urine Q1071:Oval eggs flattened along one side? Causes anal pruritis? Enterobius vermicularis (pinworm) Q1072:Hep B infection causes what in the majority of cases? Subclinical disease followed by recovery Q1073:Darkly colored faceted stones with irregular shapes are associated with which infestation? Opisthorchis sinensis (oriental liver fluke) Q1074:Rotavirus symptoms in kids? Watery non-bloody diarrhea; often coupled with nausea and vomiting Q1075:Norwalk agent usually affects kids older than;? 2 Q1076:A stomach lesion on the lesser curvature with piled up ulcer edges suggests;? Gastric carcinoma Q1077:What are predisposing factors for gastric carcinoma? Pernicious anemia; chronic gastritis; achlorhydria; bacterial overgrowth; and neoplastic polyps Q1078:Achlorhydria? Absence of HCl in the stomach Q1079:Type A gastritis - increased or decreased likelihood of developing gastric carcinoma? Increased Q1080:Symptoms of fever; chills; hypotension; and fluid- filled blisters progressing to muscle necrosis;? Vibrio vulnificus Q1081:Nonmotile bug; cuases dysentery? Shigella Q1082:Plague bug? Yersinia pestis Q1083:Hypertrophic pyloric stenosis usually presents when? Several weeks of age Q1084:The parasympathetic preganglionic innervation to the internal anal sphincter is provided by which nerve? Pelvic nerve Q1085:Courvoisier sign? Painless distention of the gallbladder Q1086:Which bug produces exotoxin A? Staph aureus Q1087:Which apolipoprotein molecule activates lipoprotein lipase? CII Q1088:Supparative cholangitis can occur as a complication of infestation by;? Roundworm (ascaris lumbricoides) and the liver flukes Clonorchis sinensis and Fasciola hepatica Q1089:Which damaging agents could cause development of hepatocellular carcinoma in the setting of cirrhosis? HBV; alcohol; hemochromatosis Q1090:Loss of villi and intraepithelial lymphocytic infiltration? Celiac sprue Q1091:Granular; spherical; thin-walled cysts measuring 10-20 um in diameter;trichrome stains show up to four nuclei in most of the cysts;? Entamoeba histolytica Q1092:scleral icterus typically reflects which fraction of biliribuin that binds tissues more avidly;? Uncojugated bilirubin Q1093:Which nodes drain the greater curvature of the stomach? The bladder and male internal genitalia? The distal stomach; pancreas and duodenum? Gastroepiploic nodes. Internal iliac nodes. Subpyloric nodes. Q1094:What are the most important predictors of a carcinoid's metastatic potential? Location and size Q1095:Acute hemorrhagic pancreatitis causes what changes to the pancreas? Autodigestion; chalky-white 'fat necrosis;' hemorrhagic; edematous Q1096:Name two polyposis syndromes that do not preispose for colon cancer? Juvenile polyposis syndrome and Peutz-Jeghers syndrome Q1097:Glands and sawtooth crypts with a proliferation of goblet and columnar epithelial cells - what type of polyp? Hyperplastic polyp Q1098:What is a pancreatic pseudocyst? Complication of pancreatitis - lined by granulation tissue and collagen; containing pancreatic juices and lysed blood. Q1099:Acetaminophen toxicity would cause elevation in which enzymes? AST and ALT Q1100:This is formed by herniation of the mucosa at a point of weakness at the junction of the pharynx and esophagus in the posterior hypopharyngeal wall…? Zenker’s diverticulum Q1101:What is the triad of Plumner-Vinson syndrome? Esophageal webs; atrophic glossitis; and iron-deficiency anemia Q1102:Markedly enlarged rugal folds can be seen in several conditions; including…? Infiltrative cancer; lymphoma; hypersecretory gastropathy; and Menetrier disease Q1103:What is Menetrier disease? Men at rear – protein losing enteropathy – marked hyperplasia of mucus-secreting cells. Q1104:Tuberculosis GI involvement? Mass lesions and strictures Q1105:Oral squamous cell carcinoma is most often located;? Which HPV is associated with almost half of all oral cancers? Floor of the mouth. HPV16 Q1106:Acute gastritis can be seen as a complication of which other conditions? Alcohol use; NSAIDs; smoking; shock; steroid use; and uremia Q1107:Which autoimmune disease is associated with atrophic gastritis? Pernicious anemia Q1108:Tumor composed of uniform round cells arranged in trabeculae; with a 'salt-and-pepper' chromatin pattern Carcinoid Q1109:Cardiac failure; megaesophagus in Brazilian man;? Chagas Q1110:One of the most common etiologic agents of infective esophagitis;? Herpes simplex Q1111:What parts of the liver are Zones 1; 2; and 3; associated with;? Zone 1 - periportal;Zone 2 - intermediate;Zone 3 - Central vein Q1112:Which liver zone contains the P450 oxidase enzyme system? Zone 3 Q1113:Disease characterized by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts; producing alternating strictures and dilatation;? Primary sclerosing cholangitis Q1114:Usual cause of intestinal amebiasis; with small nuclei with distinctive tiny central karyosomes;? Entamoeba histolytica Q1115:Most likely cause of sudden gastrointestinal obstruction? Intussusception Q1116:On biopsy; this protozoan appears crescent-shaped;? Giardia lamblia Q1117:Tumors from the penis; vagina; and anal canal drain to which lymph nodes? Medial side of the horizontal chain of the superficial group of inguinal lymph nodes Q1118:Eosinophilic cytoplasmic inclusions;? Mallory bodies Q1119:Predisposing condition to angiosarcoma in the liver? Vinyl chloride exposure (used in the rubber industry) Q1120:Liver steatosis would progress to what if the noxious stimulus is removed? Complete regression Q1121:Pancreatic calcifications constitute strong radiologic evidence of;? Chronic pancreatitis Q1122:T or F? Pernicious anemia is an autoimmune disease associated with atrophic gastritis True Q1123:Celiac sprue is located;? Proximal small bowel Q1124:Polycythemia vera woman develops progressive severe ascites and tender hepatomegaly;? Budd-Chiari Q1125:Definition;intracytoplasmic eosinophilic inclusions;what are they seen in? Mallory bodies;in: Alcoholic hepatitis Q1126:Dx;high unconjugated bilirubin w/o hemolysis; decreased UDP-glucuronyl transferase; stress induced; no sx Gilbert's syndrome Q1127:Dx;high unconjugated bilirubin w/o hemolysis; absent UDP-glucuronyl transferase; jaundice; kernicterus; CNS damage Crigler-Najjar syndrome Q1128:Dx;Increased Conjugated bilirubin; brown to black discoloration of liver Dubin-Johnson syndrome Q1129:Dx;hypergammaglobinemia and anti-smooth muscle Ab Autoimmune Hepatitis Q1130:Dx;severe obstructive jaundice; itching; hypercholesterolemia; xanthomas Primary Biliary cirrhosis;(Anti-mitochondrial Ab) Q1131:Definition;extrahepatic biliary obstruction causing increased pressure in intrahepatic ducts leading to injury and fibrosis; Inc Alk phos; Inc conjugated bilirubin Secondary Biliary Cirrhosis Q1132:Dx;tender hepatomegaly; ascites; polycythemia; hypoglycemia;what is elevated in serum? Hepatocellular CA;(elevated alpha-fetoprotein) Q1133:Definition;Inadequate hepatic copper excretion and failure of copper to enter circulation; leading to copper accumulation in organs Wilson's Dz;(Auto Resessive) Q1134:Dx;asterixis; parkinsonian sx; cirrhosis; low ceruloplasmin; dementia Wilson's Dz;ABCD;Asterixis;Basal ganglia sx;Copper accumulation/low Ceru;Dementia Q1135:Dx;occlusion of IVC or hepatic veins w/ centrilobular congestion and necrosis; assoc w/ polycythemia; abd CA; or complication of pregnancy Budd-Chiari syndrome Q1136:Hyperbilirubinemia type; urine bilirubin and urine urobilinogen changes for;Hepatocellular jaundice Conjugated/Unconjugated;Inc Urine Bilirubin;nml/Dec Urobilinogen Q1137:Hyperbilirubinemia type; urine bilirubin and urine urobilinogen changes for;Obstructive jaundice Conjugated;Inc Urine Bilirubin;Dec Urobilinogen Q1138:Hyperbilirubinemia type; urine bilirubin and urine urobilinogen changes for;Hemolytic jaundice Unconjugated;Absent urine Bilirubin;Inc Urobilinogen Q1139:Dx;hepatoencephalopathy in young children w/ virus who are given aspirin Reye's syndrome Q1140:what is chronic calcifying pancreatitis assoc with? alcoholism Q1141:what is chronic obstructive pancreatitis assoc with? Gallstones Q1142:Cause of Cholangiocarcinoma Liver fluke;(C.sinensis) Q1143:Dx;"Strawberry gallbladder" Cholesterolosis Q1144:what causes the GB to become enlarged and distended? Tumors (not stones) Q1145:(3) changes in the pancreas w/ Acute Pancreatitis Hemorrhagic Fat Necrosis;Calcium soaps (leading to HypoC);Pseudocysts Q1146:Dx;abdominal pain radiating to back; migratory thrombophlebitis; obstructive jaundice w/ palpable GB Pancreatic Adenocarcinoma Q1147:Dx;malabsorption problem w/ positive D-xylose test;what CA can it lead to? Celiac Sprue;;leads to: T-cell lymphoma Q1148:Diverticuli are blind pouches found in the: alimentary tract Q1149:Diverticuli are lined by: Mucosa; muscularis; and serosa Q1150:The 4 GI tract locations of diverticuli are: esophagus; stomach; duodenum and colon Q1151:Why are most diverticuli termed false? They lack or have an attenuated muscularis mucosa. Q1152:Percent of older patients likely to get diverticulosis (many diverticula): 50% of patients >60 Q1153:The reasons for increasing number of diverticula with age are: Increased intraluminal pressure and focal weaknes in the colonic wall. Q1154:What type of diet is diverticulosis associated with? low-fiber Q1155:Sx of diverticulosis? usually asymptomatic; sometimes vague discomfort. Q1156:Diverticulitis commonly presents with pain where? LLQ Q1157:Complications of diverticulitis include: perforation; peritonitis; abcess formation; bowel stenosis Q1158:What is intussuception? telecoping of 1 bowel segment into distal segment. Q1159:Complication of intussusception is: compromised blood supply. Q1160:What is volvulus? Twisting of portion of the bowel around its mesentery. Q1161:What volvulus lead to? obstruction. Q1162:What is the most common type of stomach cancer? adenocarcinoma Q1163:Is this cancer aggresive? Yes; it has early aggressive local spread and node/liver mets Q1164:Stomach Cancer is associated with what 3 etiologic factors? dietary nitrosamines; achlorhydria; chronic gastritis Q1165:What is stomach cancer termed when it is diffusely infiltrative with thickened and rigid appearance? Linitis plastica Q1166:What does Virchow's node signify? involvement of supraclavicular node by stomach mets Q1167:What is Krukenberg's tumor? bilateral stomach cancer mets to the ovary Q1168:What are characteristics of Krukenberg's tumor? Abundant mucus; "signet ring" cells Q1169:What is Hirschprung's diease? congential megacolon Q1170:What is missing? enteric nerve plexus (both Auerbach's and Meissner's). Seen on biopsy Q1171:What is the cause of this disease? failure of neural crest migration. Q1172:How does this disease present? Chronic constipation early in life. Q1173:Which part of the colon is dilated? That part proximal to the aganglionic segment - aganglionic portion is constricted. Q1174:The risk factors for colorectal cancer are: colorectal villous adenoma; IBD; low-fiber diet; age; FAP; HNPCC; personal and family hx. of colon cancer. Q1175:What is Peutz-Jeghers; and does it lead to colorectal cancer? It is a benign polyposis syndrome which is not a risk factor. Q1176:Who schould be screened for colorectal cancer and how? People over age 50; screen with stool occult blood test. Q1177:Cirrho in Greek means: tawny yellow Q1178:Cirrhosis is: diffuse fibrosis of the liver with destruction of norml architecture; nodular regeneration. Q1179:Cause of micronodular cirrhosis (nodules <3mm; uniform in size) is: metabolic insult Q1180:Causes of macronodular cirrhosis (nodules >3mm; varied in size) are? Significant liver injury leading to hepatic necrosis (post- infectious; drug-induced hepatitis) Q1181:Cirrhosis is assocated with what type of cancer? hepatocellular carcinoma Q1182:The effects of portal hypertension are: esophageal varices (hemetemesis; melena); peptic ulceration (melena); splenomegaly; caput medusae; ascites; hemmorhoids; and testicular atrophy Q1183:Portal hypertension may be relieved by what method? Portacaval shunt between splenic vein ad left renal vein. Q1184:What are the effects of liver cell failure? Coma; scleral icterus; fetor hepaticus (breath smells like a freshly opened corpse); spider nevi; gynecomastia; jaundice; loss of sexual hair; asterixis; bleeding tendency; anemia; ankle edema Q1185:Hepatocytes in alcoholic hepatitis are: swollen and necrotic Q1186:Other histologic changes seen in aloholic hepatitis are: neutrophil infiltration; mallory bodies (hyaline); fatty change; and sclerosis areound central vein Q1187:SGOT (AST):SGPT (ALT) ratio in alcoholic hepatitis is: usually more than 1.5 (A Scotch and Tonic: AST elevated) Q1188:What is Budd-Chiari Syndrome? Occlusion of the IVC or hepatic veins with centrilobular congestion and necrosis; leading to congestive liver disease Q1189:The features of congestive liver disease are: hepatomegaly; ascites; abdominal pain; and eventual liver failure Q1190:Budd-Chiari Syndrome is associated with what 3 conditions: polycythemia vera; pregnancy; hepatocellular carcinoma Q1191:Wilson's disease is? Copper accumulation; especially in liver; brain and cornea Q1192:It is due to what? failure of copper to enter circulation in the form of ceruloplasmin Q1193:What is another name for Wilson's Disease? Hepatolenticular degeneration Q1194:What are the symptoms of Wilson's Disease? (A;B;C- 6;D) Asterixis; basal ganglia degeneration (parkinsonian symptoms); Ceruloplasmin decrease; cirrhosis; corneal deposits (kayser-fleischer rings); copper accumulation; carcinoma (hepatocellular); choreiform movements; Dementia Q1195:Somatotropic adenoma would affect glucose tolerance how? Impaired glucose tolerance Q1196:Describe how coma could result from complications of DM II? Osmotic diuresis + dehydration = severe hyperosmolarity; Osmotic loss of water from neurons can produce coma Q1197:Even small amounts of insulin are sufficient to prevent;? Ketosis Q1198:Beta hydroxybutyrate is a;? Ketone body Q1199:An XY fetus with a mutated dysfunctional androgen receptor molecule would develop how? male sexual development is impaired and female external genitalia are formed;Because the androgen receptor in hypothalamic and pituitary tissue is also defective; normal testosterone suppression of pituitary gonadotrophs is absent Q1200:How does the pituitary affect the sensitivity of peripheral tissues to the action of insulin? GH - anti-insulin effects on liver and muscle. ACTH causes increased cortisol = decreased insulin sensitivity in peripheral tissues. TSH also tends to increase blood glucose levels Q1201:ANP is released when? When the atria are distended - suppresses the renin- angiotensin-aldosterone system Q1202:Graves is caused by;? Increased production of thyroid-stimulating immunoglobulins Q1203:T or F? Hyperthyroidism and hypothyroidism are always associated with increased and decreased radioactive iodine uptake; respectively. False Q1204:The sweating and palpitations of hypoglycemia are caused by;? Epinephrin Q1205:Link the following: dissecting osteitis and chronic renal failure Hyperparathyroidism results from decreased serum calcium and increased serum phosphate (sequelae of renal failure). This may lead to osteomalacia and osteitis fibrosa cystica; which is classically associated with dissecting osteitis. Q1206:Thyrotoxic crisis is most commonly seen in which disease? Inadequately treated Graves' disease Q1207:What are the HLA associations of DM I ? DR3 and DR4 Q1208:What are the two most dangerous hormonal deficiencies? Glucocorticoids and thyroid hormone Q1209:Why does mild renal failure cause hyperkalemia? Deficient renin production by the damaged kidney causes decreased secretion of aldosterone Q1210:What types of infections are more frequently considered with Addison's? Tuberculosis; fungal infections Q1211:How to clinically distinguish primary from secondary adrenocortical insufficiency? Secondary is not associated with skin hyperpigmentation (which results from increased production of ACTH precursor; which also stimulates melanocytes) Q1212:Nonenzymatic glycosylation is seen in which disease? Diabetes mellitus Q1213:Euthyroid sick syndrome? After systemic illnesses or stresses; there can be a decrease in 5'-monodeiodinase activity in peripheral tissues. Causes a decreased conversion of T4 to T3 Q1214:Describe how a short-term vs long-term severe Mg deficiency affects PTH? Short-term - increased PTH;Long-term; severe - decreased PTH Q1215:Which zones of the adrenal cortex does Addison's affect? All three Q1216:Early manifestations of cretinism? Difficulty feeding; somnolence; failure to thrive; and constipation Q1217:Chronic thyoiditis is aka.? Hashimoto's thyroiditis Q1218:How would a decrease in GH affect IGF-1 release? Decrease Q1219:What is testicular feminization? Androgen insensitivity due to an androgen receptor defect Q1220:PTH acts on which biochemical pathway? Increased intracellular cAMP Q1221:Somatostatin would inhibit the release of what from the pituitary? GH and TSH Q1222:Antimicrosomal antibodies are seen in which thyroid condition? Hashimoto thyroiditis Q1223:ACTH affects which zones of the adrenal cortex? Inner two - fasciculata and reticularis Q1224:When is gynecomastia normal/common? Adolescent boys during puberty Q1225:hCG is in the same hormone family as;? TSH; FSH; and LH Q1226:T or F? In a patient with DM II; you would expect deactivation of fatty acid synthase False - fatty acid synthase is rapidly induced in the liver by high glucose and insulin levels Q1227:What is Chvostek's sign? Facial muscle contraction on tapping in front of the ear;Detects hypocalcemia Q1228:Tx for acute hypocalcemia? Calcium gluconate and cholecalciferol Q1229:Which metabolite helps to raise serum calcium by increasing proximal intestinal absorption of calcium? Cholecalciferol (Vit D3; 1;25-OH2-D3) Q1230:T or F? Hypercalcemia can 'hyperstabilize' excitable tissue membranes and reduce normal responsiveness True Q1231:Laron dwarfism? Congenital absence of growth hormone receptors;detected by an absence of GH binding protein; which is identical to the extracellular portion of the GH receptor Q1232:T or F? Plasma levels of proinsulin are increased in patients with insulinomas? True Q1233:Prolactin has close structural homology with;? GH Q1234:Child who develops weight loss despite increased food intake? Diabetes mellitus Q1235:Sheehan's syndrome? Hypopituitarism due to ischemic damage to the pituitary resulting from excessive hemorrhage during parturition Q1236:T or F? Somatotropes are basophils. False - they are acidophils Q1237:Which drugs are antidiabetic agents that increase insulin sensitivity through a variety of mechanisms that result in decreased hepatic gluconeogenesis and increased insulin- dependent muscle glucose uptake? Thiazolidinediones Q1238:Hyperthyroid patient with mononuclear cell infilitrate in thyroid with multinucleated giant cells; follicular disruption; and loss of colloid;? Subacute thyroiditis (aka granulomatous giant cell; de Quervain thyroiditis) Q1239:Which syndrome is associated with coarctation of the aorta? Turner syndrome Q1240:Elevated anion gap; decreased arterial blood pH; and elevated blood lactate is a rare complication of which drug? Metformin Q1241:This disease is characterized among other things by Hurthle cells (oncocytes) and associated with various thymic disorders Hashimoto thyroiditis Q1242:Mucosal neuromas in a patient with a marfanoid syndrome is a marker for;? MEN III Q1243:Pituitary apoplexy is what? may result after;? Life-threatening infarction of the pituitary gland. May result after obstetric hemorrhage: Sheehan syndrome Q1244:How would clonidine affect pancreatic insulin secretion? Clonidine; an alpha2-receptor agonist; would inhibit pancreatic insulin secretion Q1245:T or F? Glucagon; gastrin; secretin; and CCK all stimulate insulin secretion True Q1246:Which category of drugs could blunt or prevent the premonitory signs and symptoms of acute episodes of hypoglycemia in a diabetic patient? Beta adrenergic blockers - such as propnalol Q1247:Solid balls of neoplastic follicular cells in a thyroid nodule biopsy? Papillary carcinoma Q1248:Riedel thyroiditis? Rocklike stroma; fibrous reaction that may involve adjacent structures Q1249:Which pancreatic tumor could cause necrotizing skin eruptions on the legs? Glucagonoma - alpha cell tumor Q1250:How does propylthioracil work? Inhibiting the peripheral conversion of T4 to T3 Q1251:Which oncogene are MEN II and III associated with? Ret oncogene Q1252:Thyroid gland is enclosed by which fascia? Pretracheal fascia Q1253:GIP - how does it affect insulin secretion? Increases beta cell release of insulin Q1254:Nortriptyline - what is it; and how could it cause galactorrhea? A TCA; inhibits dopamine pathways; stimulating prolactin; which causes galactorrhea Q1255:What is the most reliable indicator of metastatic potential of a pheochromocytoma? The actual presence of distant metastases Q1256:How does adult polycystic kidney disease affect aldosterone? Pressure exerted by the cysts can compromise glomerular blood flow; stimulating increased renin and thus increased aldosterone Q1257:What is VMA and what is it used for? Vanillylmandelic acid; a norepinephrine metabolite. Urine levels of this can diagnose pheochromocytoma Q1258:Which one drains directly into the IVC: left renal vein or right renal vein? Right renal vein Q1259:Which thyroid condition frequently develops after a viral infection? Subacute granulomatous (de Quervain's thyroiditis) Q1260:Most common pituitary tumor? % of all pituitary tumors? Prolactinoma with hyperprolactinemia makes up 30% of pituitary tumors. Q1261:Characteristics of prolactinoma? Most common pituitary tumor;Chromophobic staining;In women; leads to amenorrhea; galactorrhea;Undergoes dystrophic calcification;Underlying cause for 1/4 cases of amenorrhea Q1262:Treatment for prolactinoma? Most commonly bromocriptine: a dopamine receptor agonist that causes the lesion to shrink;Surgery Q1263:What hypothalamic factors control release of the following pituitary hormones: TSH; PRL; ACTH; GH; FSH; LH?;Is the controlling factor stimulatory or inhibitory for each? TSH: stimulated by TRH; inhibited by somatostatin;Prolactin: inhibited by dopamine;ACTH: stimulated by CRH;GH: stimulated by GHRH; inhibited by somatostatin;FSH: stimulated by GnRH;LH: stimulated by GnRH Q1264:Characteristics of somatotropic adenoma? Second most common pituitary tumor;Acidophilic staining;Causes release of somatomedins by liver (IGF-I; somatomedin C);Causes gigantism if during childhood; acromegaly during adulthood;Causes local compression in sela turcica. Q1265:Characteristics of corticotropic adenoma? Hypercorticism (Cushing disease);Basophilic staining;Weight gain; moon facies; thin/atrophied skin; hirsuitism; HTN; hyperglycemia due to insulin resistance Q1266:Difference between Cushing disease and Cushing syndrome? "Syndrome" is hypercorticism regardless of cause; "disease" is in the case of corticotropic adenoma of the pituitary. Q1267:What is pituitary cachexia (Simmonds disease)? What are the 2 most common causes? Generalized panhypopituitarism leading to marked wasting;Pituitary tumors and Sheehan's syndrome (postpartum pituitary necrosis) are the most common causes. Q1268:What is Sheehan's syndrome? Ischemic necrosis of the pituitary during child birth; due to hypoperfusion. The pituitary enlarges during pregancy but its blood supply does not; hence it is suspectible to peripartum blood loss. Q1269:Symptoms of growth hormone deficiency? In children: growth retardation;In adults: increased insulin sensitivity with hypoglycemia; decreased muscle strength; and anemia. Q1270:Symptoms of gonadotropin deficiency? In children: retarded sexual maturation;In adult men: loss of libido/muscular mass; impotence; decreased body hair;In adult women: amenorrhea; vaginal atrophy Q1271:Result of TSH deficiency? Secondary hypothyroidism. Q1272:How to distinguish between deficiency of ACTH and primary adrenal failure (Addison disease)? In Addison disease B-melanocyte stimulating hormone is still high leading to hyperpigmentation of skin. Not true in ACTH deficency; where B-MSH is also low. Q1273:Function of oxytocin? Induces uterine contraction during labor; ejection of milk from mammary alveoli Q1274:Most common cause of SIADH? Small cell carcinoma of lung;Other tumors can also produce ectopic ADH. Q1275:Causes of ADH deficency (diabetes insipidus)? Tumors;Trauma;Inflammatory processes;Lipid storage disorders;etc. Q1276:What is empty sella syndrome? Pituitary hypofunction due to destruction of all or part of the pituitary. Q1277:What is Nelson syndrome? Large pituitary adenomas that develop after bilateral adrenalectomy: due to a loss of feedback inhibition on preexisting pituitary microadenomas. Q1278:What hormones are released by the neurohypophysis? Is it anterior or posterior? Oxytocin; ADH;Posterior Q1279:What hormones are released by the adenohypophysis? Is it anterior or posterior? TSH; prolactin; ACTH; GH; FSH; LH;Anterior Q1280:What is a craniopharyngioma? Benign childhood tumor from remnants of Rathke pouch;Often cystic or calcified;Not a true pituitary tumor; but can have mass effects that cause pituitary hypofunction. Q1281:What are the symptoms of a nonsecreting pituitary adenoma or other mass lesion in the sella? Hypopituitarism; headache; visual disturbances (bilateral hemianopsia due to optic chiasm pressure) and palsies (due to cranial nerve compression) Q1282:What is the most common congenital anomaly of the thyroid? other anomalies? thyroglossal duct cyst; it does not lead to alterations in thyroid function;Ectopic thyroid tissue may also be found anywhere along the course of the thyroglossal duct. (If removing these; be sure they are not the ONLY thyroid tissue!) Q1283:what are the causes of goiter? physiologic enlargement during puberty and pregnancy;iodine deficiency;Hashimoto thyroiditis;goitrogens: substances that suppress synthesis of thyroid hormones;dyshormonogenesis: failure of hormone synthesis due to enzyme defects Q1284:what is the difference between a hot nodule and cold nodule in the thyroid? most nodules are hypoplastic and do not take up radioactive iodine (cold);Some nodules are hyperplastic and actively produce thyroid hormone; and take up radioactive iodine (hot);Nonfunctional (cold) nodules are more likely to be malignant compared to functional (hot) nodules. Q1285:what are the causes and characteristics of myxedema? Therapy for hyperthyroidism; Hashimoto thyroiditis; idiopathic; iodine deficiency;Clinical characteristics include cold intolerance; weight gain; low voice; mental slowness; menorrhagia; constipation; dry skin; hair loss; puffiness of the face Q1286:what are the causes and characteristics of cretinism? Iodine deficiency; thyroid enzyme deficiency; poor thyroid development; failure of fetal thyroid dissent; trans placental transfer of antithyroid antibodies from a mother with autoimmune thyroid disease;Characteristics include severe mental retardation; impaired physical growth and dwarfism; a large tongue; and a protuberant abdomen Q1287:what are the symptoms of hyperthyroidism (thyrotoxicosis)? Restlessness; irritability; fatigability; tremor; heat intolerance and sweating; tachycardia; muscle wasting and weight loss; fine hair; diarrhea; menstrual abnormalities; and most importantly greatly increased T4. Q1288:what is Graves' disease? What is its mechanism? Susceptible individuals? Hyperthyroidism caused by a diffuse toxic goiter. It occurs more frequently in women than in men; and the incidence is increased in HLA-DR3 and HLA-B8 positive individuals. Exophthalmos is characteristic and unique;Autoimmune disease; with thyroid stimulating immunoglobulin (TSI) and thyroid growth immunoglobulin (TGI) causing hormone production and hyperplasia. Q1289:what are the three types of thyroiditis? Hashimoto thyroiditis;subacute granulomatous thyroiditis;Riedel thyroiditis Q1290:What is Hashimoto thyroiditis?;Who is susceptible? Autoimmune disorder; common cause of hypothyroidism. Histologically; massive infiltrates of lymphocytes with germinal center formation and Hurtle cells. Autoantibodies are present; including anti-thyroglobulin; antithyroid peroxidase; anti-TSH receptor; and anti-iodine receptor;Associated with increased incidence of pernicious anemia; diabetes mellitus; and Sjogren's syndrome. Associated with HLA-DR5 and HLA-B5. Q1291:What is subacute granulomatous thyroiditis? What appears to be a common cause? focal destruction of thyroid tissue and granulomatous inflammation caused by a variety of viral infections; including mumps and Coxsackie virus;Follows a self-limiting course of several weeks duration consisting of a flulike illness; pain and tenderness of the thyroid; and a transient hyperthyroidism. Q1292:what is Riedel thyroiditis? Idiopathic replacement of the thyroid by fibrous tissue; can mimic carcinoma. Q1293:What are the characteristics of papillary carcinoma of the thyroid? Common causes? the most common thyroid cancer;Histologically: papillary projection into gland-like spaces; empty "orphan Annie" nuclei; calcified spheres;excellent prognosis; even when the adjacent lymph nodes are involved;can be caused by long-term radiotherapy to the neck; or RET-PTC fusion gene Q1294:List the four types of malignant thyroid tumors. Which has the best prognosis and which has the worst? papillary carcinoma - best;follicular carcinoma;medullary carcinoma;undifferentiated carcinoma -worst Q1295:what are the characteristics of medullary carcinoma of the thyroid? Originates from C-cells in the thyroid; produces calcitonin;histologically: sheets of tumor cells in amyloid stroma;associated with multiple endocrine neoplasia II; III Q1296:What are the causes of primary hyperparathyroidism? What are the laboratory findings associated with it? What are the clinical manifestations? Most common cause is parathyroid adenoma. Primary parathyroid hyperplasia and carcinoma are less likely. PTH- like hormone can be produced by bronchogenic squamous cell carcinoma or renal cell carcinoma;Lab findings include hypercalcemia/hypercalciuria; decreased serum phosphorus/increased urinary phosphorus; increased serum alk.phos; and increased serum PTH;Clinical symptoms include cystic bone changes; "metastatic calcification"; renal calculi; peptic ulcer. Q1297:What are the causes of secondary hyperparathyroidism? What are the lab values and clinical characteristics? Commonly caused by hypocalcemia in chronic renal disease. Vitamin D conversion by the kidney is impaired; decreasing intestinal absorption of calcium;Lab values include decreased serum calcium; increased serum phosphorus; increased serum alkaline phosphatase; increased serum PTH. Clinical symptoms include osteoclastic bone disease and metastatic calcification;(Personal note: many apparent paradoxes in this disease) Q1298:What causes hypoparathyroidism? What are the symptoms? The most common cause is accidental surgical excision during thyroidectomy. Sometimes associated with congenital thymic hypoplasia (DiGeorge syndrome);Resulting in severe hypocalcemia; manifested as increased neuromuscular excitability and tetany. Q1299:What is pseudo-hypoparathyroidism? Multi-hormone resistance including PTH; and the pituitary hormones TSH; LH; and FSH;Clinical characteristics include unresponsiveness of the kidney to PTH; shortened fourth and fifth metacarpal and metatarsals; short stature; and other skeletal abnormalities. Q1300:What are the causes of Cushing's syndrome? What is the difference between Cushing's syndrome and Cushing's disease? Exogenous corticosteroid medication;Hyper production of ACTH by pituitary adenoma;Adrenal cortical adenoma or carcinoma;Ectopic production of ACTH (by small cell carcinoma of the lung);Cushing's syndrome results from increased circulating cortisol from any source; Cushing's disease refers to only hyper production of ACTH by pituitary adenoma. Q1301:What is primary hyperaldosteronism? Conn syndrome; usually due to an aldosterone producing adrenocortical adenoma or carcinoma;Causes hypertension; sodium and water retention; and hypokalemia;Decreased serum renin is present due to negative feedback from hypertension. Q1302:What is secondary hyperaldosteronism? Caused by stimulation of the renin angiotensin system. Serum renin is increased in contrast to primary hyperaldosteronism;Usually secondary to renal ischemia; renal tumors; and edema (cirrhosis; nephrotic syndrome; cardiac failure). Q1303:What is adrenal virilism? Congenital enzyme defects that lead to diminished cortisol production and compensatory increased ACTH. This leads to adrenal hyperplasia and androgenic steroid production;Deficiencies include 21-hydroxylase deficiency and 11-hydroxylase deficiency;Clinical characteristics include precocious puberty in males and virilism in females. Q1304:What is Addison disease? Symptoms and labs? Primary adrenocortical deficiency; usually due to idiopathic adrenal atrophy. Can also be caused by tuberculosis; metastatic tumor; and infection. Characteristics include hypotension; skin pigmentation; low serum sodium; chloride; glucose; bicarbonate and increased potassium. Q1305:What is Waterhouse-Friedrichsen syndrome? Catastrophic adrenal insufficiency and vascular collapse due to hemorrhagic necrosis of the adrenal cortex. Often associated with DIC and characteristically due to meningococcal meningitis (Neisseria meningitis). Q1306:What is a pheochromocytoma? Adrenal medulla tumor of chromaffin cells. Causes paroxysmal hypertension due to hyperproduction of catecholamines. Increased urinary excretion of catecholamines and metabolites (metanephrine; normetanephrine; vanillylmandelic acid). Can also cause hyperglycemia. Q1307:What is a neuroblastoma? A highly malignant catecholamine producing tumor occurring in early childhood. Urinary catecholamines and metabolites are seen as in pheochromocytoma. Hypertension is common. Originates in the adrenal medulla and presents as a large abdominal mass. Q1308:Epidemiology and etiological factors of type 1 diabetes mellitus? Usually early in life; less common than type 2 disease. The disease is due to failure of insulin synthesis by beta cells of the pancreatic islets. Cause may be due to genetic predisposition or autoimmune inflammation of the islets. Incidences significantly higher in individuals with a mutation in HLA DQ; and in HLA DR3/HLA DR4 positive individuals. Q1309:Clinical characteristics of type 1 diabetes? Without insulin replacement: carbohydrate intolerance with hyperglycemia leading to polyuria; polydipsia; weight loss; ketoacidosis; coma; and death. Ketoacidosis results from increased metabolism of fat leading to production of "ketone bodies". Q1310:What are etiologies of Cushing’s syndrome? Is ACTH always elevated? increased cortisol due to: 1. Cushing’s disease (primary pituitary adenoma) increased ACTH 2. Primary adrenal (hyperplasia/neoplasia) decreased ACTH 3. Ectopic ACTH production (ie- small cell lung ca) increased ACTH 4. Iatrogenic; decreased ACTH Q1311:Describe the clinical picture of Cushings. HTN; wt gain; moon facies; truncal obesity; buffalo hump; hyperglycemia (insulin resistance); skin change (thinning; striae); osteoporosis; immune suppression Q1312:T/F Conn’s syndrome is secondary hyperaldosteronism. False. Conn’s syndrome is primary hyperaldosteronism; caused by an aldosterone-secreting tumor. Results in HTN; hypokalemia; metabolic alkalosis; low plasma renin. Q1313:Which hyperaldosteronism is associated with high plasma renin? Secondary hyperaldosteronism. It is due to renal artery stenosis; chronic renal failure; CHF; cirrhosis; or nephrotic syndrome. Kidney perception of low intravascular volume results in an overactive renin-angiotensin sysem. Q1314:What is the tx for hyperaldosteronism? Spironolactone; a diuretic that works by acting as a aldosterone antagonist. Q1315:What characterizes addison’s disease? (which hormones are elevated or deficient)? Is it associated with HTN or hypotension? Primary deficiency of aldosterone and cortisol due to adrenal atrophy; causing hypotension and skin hyperpigmentation. Adrenal atrophy; absence of hormone production; involves all 3 cortical divisions. Q1316:T/F In primary insufficiency decreased pituitary ACTH production is characterized by skin hyperpigmentation. False: increased ACTH causes MSH activity & hyperpigmentation Q1317:T/F Neuroblastoma is the most common tumor of adrenal medulla in adults. False. Pheochromocytoma is the most common tumor of adrenal medulla in adults. It is derived from chromaffin cells (arise from neural crest). It is associated with neurofibromatosis MEN types II and III. Q1318:Where does neuroblastoma occur? Neuroblastoma is the most common tumor of adrenal medulla in children. It can occur anywhere along the sympathetic chain. Q1319:Pheochromocytoma: secrete combination of two molecules epinephrine and norepinephrine Q1320:Pheochromocytoma: epidemiology (rule of 10's) 10% malignant; 10% bilateral; 10% extra-adrenal; 10% calcify; 10% kids; 10% familial Q1321:Pheochromocytoma: symptoms - 5 P's (elevated blood) Pressure; Pain (headache); Perspiration; Palpitations; Pallor/diaphoresis --> relapsing and remittant Q1322:Pheochromocytoma: elevations in two lab values urinary VMA and serum catecholamines Q1323:Pheochromocytoma: association with two other endocrine diseases MEN II and III Q1324:Pheochromocytoma: treatment alpha-antagonists Q1325:Multiple Endocrine Neoplasia type I (Wermer's syndrome) - 3 P's Pancreas; Pituitary; and Parathyroid tumors Q1326:Multiple Endocrine Neoplasia type II (Sipple's syndrome) medullary carcinoma of thyroid; pheochromocytoma; parathyroid tumor; or adenoma Q1327:Multiple Endocrine Neoplasia type III medullary carcinoma of thyroid; pheochromocytoma; oral/intestinal ganglioneuromatosis Q1328:Hypothyroidism or hyperthyroidism myxedema is prominent in which one hypothyroidism Q1329:Hypothyroidism or hyperthyroidism chest pain; palpitations; arrhythmias hyperthyroidism Q1330:TSH is (increased/decreased) in primary hyperthyroidism? In primary hypothyroidism? hyper - decreased; hypo - increased Q1331:Graves' disease involves autoantibodies with what mechanism of action? stimulation of TSH receptors Q1332:three symptoms of Graves' ophthalmopathy; pretibial myxedema; diffuse goiter Q1333:Graves' is a type __ hypersensitivity type II Q1334:Hashimoto's thyroiditis: thyroid is (enlarged/not enlarged) and (tender/nontender) enlarged; nontender Q1335:Hashimoto's thyroiditis: autoimmune antibodies directed against --- microsomes Q1336:Hashimoto's thyroiditis: histology shows (type of cell) infiltrate lymphocytes (with germinal centers) Q1337:Subacute thyroiditis (de Quervain's): self-limited (hyper/hypo)thyroidism following --- hypothyroidism following flu-like illness Q1338:Subacute thyroiditis (de Quervain's): symptoms include jaw pain; tender thyroid gland; early hyperthyroidism Q1339:Thyroid cancer: most common; good prognosis; "ground glass" nuclei; psammoma bodies papillary carcinoma Q1340:Thyroid cancer: poor prognosis; uniform follicles follicular carcinoma Q1341:Thyroid cancer: calcitonin producing (C cells); sheets of cells medullary carcinoma (MEN II and III) Q1342:Thyroid cancer: older patients; horrible prognosis undifferentiated/anaplastic Q1343:Cretinism: caused by a lack of dietary --- or defect in - -- formation iodine (endemic); T4 (sporadic) Q1344:Cretinism: symptoms include pot-belly; paleness; puffy face; protuberant tongue; protruding umbilicus Q1345:Acromegaly: caused by excess --- in adults growth hormone Q1346:Acromegaly: symptoms include large furrowed tongue; deep voice; large hands and feet; coarse facial features Q1347:Acromegaly: in children; leads to --- gigantism Q1348:Metyrapone stimulation test Metyrapone blocks 11OHase which stimulates ACTH and 11-deoxycortisol and decreases cortisol. If ACTH increases and 11-deoxycortisol decreases the problem is adrenal insuficiency. If both increase; the problem is pituitary insuficiency. If 11-deoxycortisol or ACTH dont change look for adrenal or ectopic Cushing's. Q1349:High dose dexamethasone suppression test Dexamethasone is a cortisol analog and should suppress ACTH and cortisol. If it does suppress cortisol its pituitary Cushing's. If it doenst; its adrenal or ectopic Cushing's Q1350:Clinical features of hyperthyroidism Tachychardia; palpitations; atrial fibrillation; systolic hypertension; nervousness; diaphoresis; tremors; diarrhea; weight loss. High free T4 and decreased TSH (if primary) or increased TSH (if secondary) Q1351:Graves disease IgG autoantibodies stimulate TSH receptors. Signs and symptoms of hyperthyroidism plus goiter; exophthalmus and pretibial myxedema (due to glycosamynoglycan deposition) Q1352:Effect of oral contraceptives and anbolic steroids on binding proteins Contraceptives increase binding proteins and increase toal levels of the hormone with normal TSH or ACTH. Anabolics do the opposite. Increased total T4 or total cortisol with normal TSH or ACTH indicates contraceptives. Decreased total T4 or cortisol with normal TSH or ACTH indicates anabolic steroids. Q1353:Clinical features of hypothyroidism Fatigue; cold sensitivity; decreased cardiac output; myxedema; constipation; anovulatory cycles. Low free T4 with high TSH (primary) or low TSH (secondary) Q1354:Cretinism Congenital hypothyroidism. Mental retardation; failure to thrive; stunted bone growth. Q1355:Hashimoto thyroiditis Anti-microsomal antibodies against thyroid gland produces hypothyroidism. Lymphocytic inflamation with germinal centers Q1356:Subacute thyroiditis Preceded by viral illness. Granulomatous inflamation Q1357:Reidel thyroiditis Destruction of the thyroid gland by dense fibrosis. Irregular hard thyroid that is adherent to trachea (dyspnea) and esopahgus (dysphagia) Q1358:Thyroid adenoma Painless solitary cold nodule that may be functional Q1359:Papillary thyroid carcinoma 80% of malignant thyroid tumors. Papillary pattern; psammoma bodies; clear "orphan Annie" nuclei. Lymphatic spread to cervical nodes is common Q1360:Follicular thyroid carcinoma 15% of malignant thyroid tumors. Hematogenous metastasis to bone or lungs Q1361:Medullary thyroid carcinoma 5% of malignant thyroid tumors. Arises from C cells and produce calcitonin. May be associated with MEN II Q1362:Anaplastic thyroid carcionoma Firm enlarging mass that metastasizes to trachea and esophagus and causes dyspnea and dysphagia Q1363:Primary hyperparathyroidism Excess PTH with hypercalcemia caused by parathyroid adenoma (80%); hyperplasia (15%) or paraneoplastic syndromes (lung SCC and renal cell carcinoma). High serum Ca+ and PTH; kidney stones; osteoporosis; short QT interval. Q1364:Secondary hyperparathyroidism Caused by chronic renal failure (no phosphate excretion increases serum phosphate; decreasing Ca+ and increasing PTH); chronic renal failure also causes deficiency of alpha-1- hydroxylase and vitamin D. Vitamin D deficiency and malabsorption Q1365:Functions of PTH Increase reabsorption of Ca+ in distal tubule; decreases phosphate reabsorption in proximal tubule; increases alpha-1- hydroxylase in proximal tubule; binds to PTH receptor on osteoblast releasing IL-1 (osteoclast activating factor) to activate osteoclast resorption Q1366:Hypoparathyroidism Due to surgical removal of glands or DiGeorge syndrome. Hypocalcemia with low PTH; tetany; prolonged QT interval Q1367:Prolactinoma Hyperprolactinemia produces galactorrhea; amenorrhea (tumor mass inhibits GnRH) and infertility Q1368:GH producing adenoma High GH and somatomedin C (IGF-1) produce gigantism (in children; tall stature with long extremities) or acromegaly (in adults; prominent jaw; flat forehead; enlarged hands and feet; diabetes and visceromegaly) Q1369:Sheehan syndrome Ischemic necrosis of pituitary secondary to post partum hypotension results in panhypopituitarism Q1370:Diabetes insipidus Hypotonic polyuria; polydipsia; hypernatremia; dehydration. Central DI is due to lack of ADH. Nephrogenic DI is due lack of renal response to ADH. Q1371:SIADH Excesive ADH. Oliguria; water retention; hyponatremia; cerebral edema. Due to paraneoplastic syndrome (lung SCC) or trauma Q1372:Clinical features of Cushing's syndrome Thin extremeties (protein catabolism); truncal obesity and buffalo hump (hyperglycemia increases insulin with fat deposition); purple stria (low collagen in vessels); hyperlipidemia (hormone-sensitive lipase); hirsutism; hypertension and hypokalemic alkalosis (high aldosterone). Q1373:Differentiation of Cushing's syndrome High ACTH with dexamethasone suppression --> pituitary. High ACTH without dexamethasone suppression --> ectopic (lung SCC). Low ACTH --> adrenal. Low ACTH with low cortisol and adrenal atrophy --> steroid therapy (MCC). High ACTH produces skin pigmentation in pituitary and ectopic. Q1374:Conn syndrome Adrenocortical adenoma causes hypertension; hypernatremia; hypokalemia; metabolic alkalosis; tetany. High aldosterone and low renin Q1375:Waterhouse-Friderichsen syndrome Bilateral hemorrhagic infarction of the adrenals associated with menigococcemia. DIC; hypotension; shock. Q1376:Addison disease Autoimmune destruction of adrenal cortex due to abrupt withdrawal of corticosteroids; miliary TB or menincoccemia. Weakness; hyperpigmentation of skin (high ACTH); hypotension; hypoglycemia; poor response to stress Q1377:Pheochromocytoma Catecholamine-producing benign tumor of the adrenal medulla. Severe headaches; tachycardia; palpitationss; diaphoresis; anxiety; hypertension. Associated with MEN II. Elevated urinary vanillylmandelic acid (VMA) and catecholamines. Q1378:MEN I Tumors of the pituitary (non-functioning); parathyroids (hypercalcemia); and pancreas gastrinoma (zollinger-ellison) Q1379:MEN II Medullary carcinoma of thyroid; pheochromocytoma; parathyroid hyperplasia or adenoma Q1380:Pathophysiology of diabetic ketoacidosis Hyperglycemia (due to increased glycogenolysis and gluconeogenesis). Ketone bodies (low insulin and high cortisol/epinephrine activate hormone-sensitive lipase; B- oxidation and ketogenesis). Osmotic diuresis and volume depletion with loss of potassium. Dilutional hyponatremia due to osmotic effect of hyperglycemia. Low insulin fails to activate lipoprotein lipase leading to hypertriglyceridemia. Q1381:Pathophysiology of diabetic vascular disease Non-enzymatic glycosylation and arteriosclerosis produces ischemic injury and diabetic foot. Accelerated atherosclerosis; abdominal aortic aneurysms and MI. Q1382:Pathophysiology of diabetic ocular disease Cataracts due to conversion of glucose into sorbitol by aldose reductase in the lens. Retinopathy with microaneurysms (non-proliferative) and neovascularization (proliferative) Q1383:Pathophysiology of diabetic neuropathy Destruction of Schwann cells with decreased peripheral sensitivity liads to pressure ulcers on bottom of diabetic foot Q1384:(4)* functions of T3 4 Bs;Brain maturation;Bone growth;Beta-adrenergic effects;BMR increase Q1385:what is the cause of increased cortisol w/ low ACTH ;(aside from Cushings)? Chronic steroid use Q1386:(2) steps of Dexamethasone test to determine cause of Increased cortisol;(3 causes) 1. Give LOW dose;(result: Lower cortisol = Healthy);(result: Higher cortisol = step 2);2. Give HIGH dose;(result: Lower cortisol = ACTH-producing tumor);(result: Higher cortisol = Cortisone producing tumor) Q1387:Dx;Low cortisol; High ACTH Primary Hypocortisol;(Addisons) Q1388:Dx;Low cortisol; Low ACTH Secondary Hypocortisol;(no ACTH; no skin pigmentation) Q1389:Dx;High cortisol; Low ACTH;(2) Primary Hypercortisol;(Cushings; chronic steroids) Q1390:Dx;High cortisol; High ACTH Secondary Hypercortisol;(Tumor) Q1391:Dx;HTN; hypoK; metabolic alkalosis; low plasma renin Primary Hyperaldosteronism;(Conn's syndrome) Q1392:Dx;HTN; hypoK; metabolic alkalosis; high plasma renin Secondary Hyperaldosteronism;;(CRF; CHF; renal artery stenosis; cirrhosis; anything that stimulates venous pooling) Q1393:Dx;low aldosterone; low cortisol; hypotension; skin pigmentation; Adrenal Atrophy; Absence of hormones; All 3 cortical divisions affected Addison's Dz;(Primary Hypoaldosteronism and Hypocortisol) Q1394:MC tumor of the adrenal medulla in children Neuroblastoma Q1395:Dx;elevated Pressure; HA Pain; Perspiration; Palpitations; Pallor Pheochromocytoma Q1396:Dx;kidney stones; stomach ulcers; pituitary tumor MEN I (Wermer's syndrome);3P = Pancreas; Pituitary; Parathyroid Q1397:Dx;medullary CA of thyroid; pheochromocytoma; parathyroid tumor MEN II - Sipple syndrome Q1398:Dx;medullary CA of thyroid; pheochromocytoma; mucosal neuromas MEN III Q1399:Dx;Inc ESR; jaw pain; very tender thyroid; hypothyroidism following flu-like illness Subacute Thyroiditis;(de Quervain's Thyroiditis) Q1400:MC thyroid anomaly Thyroglossal duct cyst Q1401:Dx;Increased TSH at thyroid Goiter Q1402:MCC of hypothyroidism Iodine deficiency Q1403:Dx;child w/ potbelly; pale; puffy-faced; impaired growth; protruding umbilicus and tongue; mental retardation;what is deficient? Cretinism;(Iodine deficiency) Q1404:Dx;hyperthyroidism; nodular goiter; no exophthalamos Plummer Dz Q1405:Dx;hypothyroidism w/ massive infiltrates of lymphocytes in germinal center Hashimoto thyroiditis Q1406:Dx;previous radiation of neck; "ground-glass" nuclei in thyroid; psammoma bodies Papillary carcinoma of thyroid;(MC type of thyroid CA) Q1407:Dx;Increased Calcium; Decreased Phosphorus Primary Hyperparathyroidism;(Inc PTH) Q1408:Dx;Decreased Calcium; Decreased Phosphorus Secondary Hyperparathyroidism;(Dec Vit-D) Q1409:Dx;Decreased Calcium; Increased Phosphorus Primary Hypoparathyroidism;(Dec PTH) Q1410:Dx;Increased Calcium; Increased Phosphorus Secondary Hypoparathyroidism;(Inc Vit-D) Q1411:Dx;stones; bones (pain) and groans (constipation) Primary Hyperparathyroidism Q1412:Definition;cystic bone spaces filled w/ brown fibrous tissue as a result of the osteoclastic reabsorption in primary hyperparathyroidism;(2 names) Osteitis Fibrosa Cystica;(von Recklinghausen's syndrome) Q1413:Definition;bone lesions due to secondary hypoparathyroidism due to renal Dz Renal Osteodystrophy Q1414:(2) Physical exam signs of low calcium tapping facial nerve -> contraction of facial muscles;(Chvostek's sign);occlusion of Brachial artery w/ BP cuff -> carpal spasm;(Trousseau's sign) Q1415:Dx;Decreased Calcium; Increased Phosphorus; Increased PTH; shortened 4th/5th digits; short stature;what protein is deficient? Pseudohypoparathyroidism;(Dec Guanine Nucleotide BP) Q1416:Dx;kussmaul respirations; hyperthermia; N/V; Abd pain; psychosis; dehydration; fruity breath Diabetic Ketoacidosis Q1417:Dx;adrenal insufficiency due to hemorrhagic necrosis of adrenal cortex; assoc c/ DIC; due to Meningococcemia Waterhouse-Friderichsen syndrome Q1418:Dx;hyperinsulinemia and hypoglycenia; CNS dysfunction; reversal of CSN symptoms w/ glucose administration Insulinoma Q1419:Dx;hypresecretion of HCl in stomach; recurrent peptic ulcer Dz; hypergastrinemia Zollinger-Ellison syndrome Q1420:Dx;rare tumor w/ watery diarrhea; hypokalemia; achlorhydria VIPoma;(Inc VIP) Q1421:Dx;Adrenal hyperplasia; High BP; Low Cortisol; High Androgens 11-Hydroxylase deficiency Q1422:Dx;Adrenal hyperplasia; Low BP; Low Cortisol; High Androgens 21-Hydroxylase deficiency Q1423:Dx;Adrenal hyperplasia; High BP; Low Cortisol; Low Androgens 17-Hydroxylase deficiency Q1424:Dx;Increase in Dilute Urine; Hyposmolar serum Primary Polydipsia Q1425:Dx;Increase in Dilute Urine; Hyperosmolar serum Diabetes Insipidus;(ADH deficiency = Neurogenic);(ADH receptor issue = Nephrogenic) Q1426:Dx;Increase in concentrated Urine; Hyposmolar serum SIADH Q1427:what receptors promote insulin secretion?;inhibit? promote: Beta receptors;inhibit: Alpha receptors Q1428:what stimulates both insulin and glucagon? Amino Acids Q1429:what hormone is needed for GH to function correctly? Thyroid hormones Q1430:Definition;causes the increased synthesis of cartilage in epiphyseal plates of long bones; is a good measure of GH secretion Insulin-like Growth Factor-1;(IGF-1: a somatomedian) Q1431:how is GH similar to glucagon?;insulin? glucagon: Inc blood glucose and fat;Insulin: Inc uptake of AA into cells Q1432:Calcium change w/ Acidosis Increased Q1433:Name bone calcium and phosphate Hydroxyapatite Q1434:blood cells which contain;heparin &;histamine basophil;mast cell Q1435:type of hypersensitivity reaction?;mast cell type I Q1436:fnx of cromolyn sodium prevents mast cell degranulation;(tx asthma) Q1437:major basic protein? in eosinophils for defending against protozoans Q1438:acute inflammatory response cell neutrophil Q1439:40-75% of WBC neutrophil Q1440:hypersegmented polys; what diseases B12 and folate deficiency Q1441:cell type?;kidney shaped nucleus;frosted glass cytoplasm monocyte Q1442:cell type?;CD19 & CD20 B cell Q1443:cell type?;CD3 T cell Q1444:gamma interferon affect on macrophage activation Q1445:what are the common causes of chronic blood loss? In the United States: menorrhagia; gastrointestinal bleeding;Worldwide: hook worm disease (necator americanus) Q1446:What groups are at risk for dietary deficiency of iron? Premature infants: human milk is low in iron; and newborn iron is depleted within six months. Pregnant women may also require iron supplements;The elderly are also at risk of dietary deficiency. Q1447:List the common symptoms of anemia as well as the symptoms of severe anemia. Common: pallor; fatigue; dyspnea on exertion;Severe: angina pectoris; glossitis; gastritis; koilonychia; and Plummer-Vinson syndrome. Q1448:What are the iron study patterns in iron deficiency anemia? Serum iron is low;TIBC is elevated;Serum ferritin is low. Q1449:How do you distinguish iron deficiency anemia from beta thalassemia minor? In beta thalassemia minor; the alpha-2 hemoglobin is increased. Q1450:What are the three peripheral blood findings in macrocytic anemias? What is seen in the bone marrow? pancytopenia; global macrocytosis; hypersegmented neutrophils;the bone marrow shows megaloblastic hyperplasia. Q1451:Name eight causes of vitamin B12 deficiency megaloblastic anemia. pernicious anemia; total gastric resection; disorders of the distal Ilium; a strict vegetarian diet; intestinal malabsorption syndromes; blind loop syndrome; broad-spectrum antibiotic therapy; Diphyllobothrium latum infestation (fish tapeworm) Q1452:How do you distinguish folate deficiency from vitamin B12 deficiency? There are no neurologic abnormalities in folate deficiency. Q1453:Name six causes of folate deficiency anemia. dietary deprivation in alcoholics or dieters;Pregnancy;phenytoin or oral contraceptive therapy;folate acid antagonist chemotherapy;relative deficiency in hemolytic anemia;intestinal malfunction due to sprue or Giardia Q1454:what are the iron study patterns in the anemia of chronic disease? Serum iron is low;TIBC is low;Serum ferritin is elevated. Q1455:what are some causes of aplastic anemia? Toxic exposure;dysfunction of cytotoxic T cells;radiation exposure;chemicals such as benzene;therapeutic drugs such as chloramphenicol; sulfonamides; gold salts; chlorpromazine; antimalarial drugs; and alkylating agents;Viral infection by parvovirus or hepatitis C. Q1456:Which antibodies mediate warm antibody autoimmune hemolytic anemia versus cold agglutinin disease? IgG: warm;IgM: cold Q1457:cold agglutinin disease is a complication of what diseases? infectious mononucleosis and mycoplasma pneumonia;it may be a chronic complication of lymphoid neoplasms. Q1458:What preventative measure can be taken to prevent erythroblastosis fetalis? administer anti-D IgG antiserum to D negative mothers at the time of delivery of a D positive child. this causes fetal red cells to be removed from maternal circulation. Q1459:What are the possible causes of erythroblastosis fetalis? Maternal alloimmunization to fetal Rh antigens;ABO incompatibility. Q1460:What is the pathophysiology of paroxysmal nocturnal hemoglobinuria? Defect in the PIG-A gene causes impaired synthesis of the GPI anchor; which fixes CD55; CD59; and CD8 proteins to the cell surface. These proteins protect red cells from compliment mediated lysis. Q1461:What are the possible molecular defects in hereditary spherocytosis? What is its inheritance? Spectrin; ankyrin; protein 4.1 or other RBC skeletal proteins;Autosomal dominant. Q1462:What is the pathophysiology of G6PD deficency? What is its inheritance? Lack of G6PD reduces the body's ability to deal with oxidative stress. G6PD is an X-linked disorder. Q1463:What are sources of oxidative stress that can cause an episode of hemolysis in G6PD? drugs such as primaquine (anti malarial); sulfonamides; other oxidant drugs;fava beans;infection Q1464:How can you differentiate the presentation of pyruvate kinase deficiency from G6PD deficiency? In contrast to G6PD deficiency anemia; pyruvate kinase deficiency anemia is chronic and sustained. Q1465:What is the inheritance of pyruvate kinase deficiency anemia? autosomal recessive. Q1466:What percentage of African-Americans carry the hemoglobin S gene? What advantages does the gene confer? Approximately 7%;The hemoglobin S gene confers resistance to malarial infection (Plasmodium falciparum). Q1467:Specifically; what is the genetic defect in hemoglobin S disorders? A point mutation on chromosome 11 in codon six of the beta globin gene results in a substitution of valine for glutamic acid. Q1468:What are the six symptoms of sickle cell anemia? severe hemolytic anemia;chronic leg ulcers;vaso-occlusive crises ;auto splenectomy;aplastic crises (parvovirus);infectious complications (salmonella osteomyelitis) Q1469:What are six clinical and laboratory signs of beta thalassemia? severe anemia;severe splenomegaly;distortion of skull facial bones and long bones;microcytosis; hypochromia; target cells;increased Hemoglobin F;generalized hemosiderosis Q1470:What is sickle-cell thalassemia? Co-inheritance of hemoglobin S and thalassemia of the beta globin gene. Clinically similar to sickle cell anemia but less severe. Q1471:What are possible causes of mechanical disruption leading to hemolysis? What is seen on the peripheral smear? aortic valve prosthetics;disseminated intravascular coagulation;thrombotic thrombocytopenic purpura;smear shows schistocytes or helmet cells Q1472:What is the spectrum of clinical abnormalities in alpha thalassemia according to the number of deletions? one deletion: no abnormalities;two to three deletions: mild to moderate thalassemic symptoms;four deletions: hydrops fetalis Q1473:What is hepcidin? A peptide hormone that decreases both intestinal iron absorption and the release of iron from macrophages. In anemia of chronic disease; proinflammatory cytokines induce hepatic synthesis of hepcidin. This causes serum iron to be low despite normal or even elevated iron stores (ferritin). Q1474:Anemia of Pregnancy not really anemia. inc plasma volume => dec Hct; RBC; Hg Q1475:Causes of anemia 1) decreased production: hematopoietic cell damage (rad; drugs; inf) deficiency in factors (iron for heme; vit b12 + folate for DNA). 2) Inc loss: external blood loss or hemolysis Q1476:Acute Posthemorrhagic Anemia initially; no dec in Hct; RBC; Hg b/c parallel loss in volume. Reactive increase in platelet count. Then; hemodilution => detect anemia. Q1477:Iron deficiency anemia Causes: chronic blood loss; dietary deficiency; inc requirement. Sx: pallor; fatigue; DOE. Angina if CAD. Severe: glossitis; gastritis; koilonychia; Plummer-Vinson syndrome; pica. Lab: 1) dec Hct 2) smear: hypochromic; microcytic 3) dec serum Fe; inc TIBC 4) dec body iron stores (dec hemosiderin in bone marrow; dec serum ferritin) Q1478:Chronic blood loss Iron deficiency anemia (major cause). Menorrhagia; GI bleed (carcinoma; hookworm). Q1479:Dietary iron deficiency Iron deficiency anemia. Infants (milk is low in iron). Rarely elderly. Q1480:Increased iron requirement Pregnancy. Also; infants and preadolescents can outgrow stores. Q1481:Koilonychia spooning of the nails. A/w severe iron defiency Q1482:Plummer-Vinson syndrome partially obstructing upper esophageal webs a/w sever iron deficiency Q1483:Pica appetite for substances not fit as food or no nutritional value. A/w severe iron deficiency Q1484:DDx (hypochromic; microcytic) Anemia of chronic disease (low TIBC); ?-thalassemia (inc HgA2); Sideroblastic anemia Q1485:Megaloblastic anemia Def B12 or folate => dec DNA synth => delay divison => nuclear-cytoplasmic asynchrony (loose chromatin) => dec production + ineffective hematopoiesis. Megaloblasts (erythroid precursors) in marrow. Labs: 1) smear: pancytopenia; oval macrocyotsis (MCV > 110); hypersegmented PMN. 2) marrow: megaloblastic hyperplasia. 3) B12 (homocysteine; methyl malonic acid); folate levels. Folate tx will rev B12 anemia but not neuro sx Q1486:Pernicious anemia Autoimmune gastritis: Abs vs. IF (and parietal cells) => 1) B12 def 2) achlorhydria 3) inc risk gastric carcinoma. Sx: insidous onset; lemon yellow skin; stomatitis; glossitis; *posterolateral degeneration* (demyelination) =>ataxia; hyperreflexia; impaired vib/proprioception. No neuro w/ folate def. Lab: 1) smear: pancytopenia; macrocytosis; hypersegmented PMN. marrow: megaloblastic hyperplasia. 2) Anti-IF Ab (Anti-parietal cell less specific) 3) Schilling test Q1487:Schilling test Give B12 alone and B12+IF. 1) NL abs of b12 => dietary deficiency. 2) Abs corrected w/ IF => pernicious anemia 3) Abs not correct w/ If => malabsoprtion (Crohn's; blind-loop syndrome; giant tapeworm). Q1488:DDx B12 megaloblastic anemia Pernicious anemia; total gastric resection (no IF); ileum disorder (B12-IF abs); intestinal malabsoprtion syn; blind loop syn (bacterial overgrowth); broad spectrum abio (bacterial overgrowth); strict vegetarian; *diphyllobothrium latum* Q1489:Diphyllobothrium latum giant fish tapeworm. freshwater fish. b12 defiency (megaloblastic anemia) Q1490:Folate deficiency No neuro. Causes: dietary def (alcoholics; fad dieters); pregnancy; *phenytoin* (dec folate abs); *OC* (dec folate abs); folate antagonistic chemo; inc demand (hemolytic anemia); malabs (sprue; *Giardia lamblia*) Q1491:Giardia lamblia flagellated protozoa. ADEK + folate def (megaloblastic anemia). Campers injest cyst from mnt stream => trophozoite => coats SI => dec fat abs => stinky; greasy diarrhea. Homosexual males. Tx: metro ("flagyl"). Q1492:Anemia of chronic disease A/w chronic infection; chronic immune disorders (RA); neoplasms; renal dz. Problem releasing iron from storage. IL1; TNF; IFN-g => dec EPO + inc hepcidin. 1) low serum Fe 2) *dec TIBC* (vs. iron def) 3) high storage in macrophages 4) high serum ferritin (reflects high stores). (Normochromic/normocytic or hypochromic/microcytic. Renal dz => macrocytic.) Q1493:Aplastic anemia pancytopenia; hypocellular marrow. Autoimmunity (CD8 T); radiation; benzene; *chloramphenicol (rev or irrev)*; sulfonamides; gold salts; chlorpromazine; alkylating agents; parvovirus; hep C (?). Q1494:Myelophthisic anemia Replacement of marrow by neoplasm or fibrosis => leukoerythroblastosis Q1495:Leukoerythroblastosis smear: small #s of nucleated RBC + immature granulocytic precursors Q1496:Hemolytic anemia 1) Inc RBC destruction: maximal conjugation => unconjugated bilirubinemia (indirect) (acholruic jaundice); pigmented gallstones; inc urine urobilinogen; hemosiderosis (tx: deferoxamine). if intravascular => hemoglobinemia; hemoglobinuria; no haptoglobin. 2) compensatory inc RBS production: marrow erythroid hyperplasia; reticulocytosis/polychromatophilia (inc MCV) Q1497:Warm antibody autoimmune hemolytic anemia Most common immune hemolytic anemia. IgG. 2° to SLE; Hodgkin or non-Hodkin lymphoma. Usual hemolytic anemia fx + spherocytosis (loss of membrane) + positive direct Coombs test. Q1498:Cold agglutinin disease Anti-i IgM. Below 30°. 2° to infectious mononucleosis (EBV) or Mycoplasma pneumoniae. Q1499:Chronic cold agglutinin disease Anti-i IgM. A/w lymphoid neoplasms; Raynaud's. Chronic hemolytic anemia exacerbated by cold weather. Sometimes: hemoglobinemia; hemoglobinuria. Q1500:Hemolytic disease of the newborn Erythroblastosis fetalis. Maternal ab vs. fetal RBC. Maternal alloimmunization (Rh blood group: mom=d; fetus=D). ABO incompatibility. => kernicterus (unconjugated bilirubin in CNS esp basal ganglia); hydrops fetalis (heart failure w/ generalized edema). Prevent: Anti-D IgG to mom at delivery/termination to prevent alloimmunization. Q1501:Paroxysmal nocturnal hemoglobinuria Acquired: somatic mutation in PIG-A => impaired GPI achnor synthesis. No CD55; CD59; C8 bp on RBC; granulocytes; platelets => complement-mediated lysis => pancytopenia; venous thrombosis; intravascular hemolytic anemia. Dx: CD59 neg RBC on flow. Old dx: Ham test. Q1502:Hereditary spherocytosis AD. Most common inherited intracorpuscular hemolytic anemia in whites. Spherocytes; splenomegaly (sequestered); *MCHC*; osmotic fragility; spectrin deficiency. Causes inc mut in: spectrin; ankyrin; protein 4.1; etc. Q1503:Hereditary elliptocyosis AD. Hemolysis; splenomegaly. Often no anemia. Q1504:G6PD deficiency XR. Most common enz def hemolytic anemia. Blacks + Mediterraneans. Acute; self-limited episodes of intravascular (Hg-emia; -uria) <= oxidative stress: inf; drugs (primaquine; sulfonamides; etc.) fava beans. => Heinz bodies (precipitated Hg) => Bite cells. R to p. falciparum infection. Q1505:Pyruvate kinase deficiency AR. 2nd most common enz def hemolytic anemia. Nonspherocytosis hemolytic anemia. Chronic/sustained (vs. G6PD def) Q1506:Hemoglobin S Blacks (R to falciparum infection). Pt mut codon 6 Glu=>Val (lose MstII site: prenatal dx by amnotic or chorionic villus sample). Polymerize @ low O2 tension => sickle => obstruct microvasc. Q1507:Duffy Fy a- b- phenotype. R to p. falciparum infection. Q1508:Sickle cell anemia Homozygous HbS. HbS polmerize @ low O2 tension => sickle => hemolytic anemia; chronic leg ulcers; painful crises (infection or dehydration); lung + spleen infarct (autosplenectomy => inf w/ encapsulated bact). Parvovirus => aplastic crises. Salmonella osteomyelitis. Q1509:Sickle cell trait Heterozygous HbS. No clinical consequence. Q1510:Hemoglobin C Blacks. homozygous: mild hemolytic anemia; splenomegaly; target cells; (intraerythrocyte xtals). HbSC ~ HbS. Q1511:Hemoglobin E Souteast Asia. Urban US. Clinical: similar to HbC. Q1512:HbA; S; C on PAGE HbA- (aspartate). HbS (valine). HbC+ (lysine). Q1513:? Thalassemia Major Mediterranean and US. Dec Hb synth + alpha chains aggregate (short life) + ineffective erythropoiesis + relative folate deficiency => anemia. Chronic hemolysis + transfusion => hemosiderosis (Tx: deferoxamine). Splenomegaly. Marrow expansion => skull; facial bones; long bone distortion. Smear: micro; hypo; target cells; anisocytosis; poikilocytosis. Test: inc HbF (alpha2 gamma2) Q1514:? Thalassemia Minor Heterozygous. Mild hypochromic microcytic anemia. Inc HbA2 (alpha2 delta2) (vs. iron def; ACD). Q1515:Sickle cell thalassemia ~ sickle cell anemia (less severe). Q1516:Alpha thalassemia Ch 16. Southeast asia. 3a = silent carrier. 2a = a-thal trait. 1a = HbH dz. 0a = hydrops fetalis (Hb Barts: O2 affinity too high; no delivery). Q1517:Mechnical hemolytic anemas prosthetic aortic valve or microangiopathic hemolytic anemia (DIC; thrombotic thrombocytopenic purpura): partial occlusion of vessels. Schistocytes; helmet cells. Q1518:Heterophile+ infectious mononucleosis EBV invades B lymphocytes via CD21 receptors with atypical CD8 response; lymphocytosis and paracortex hyperplasia Fever; sore throat (gray-white membrane on tonsils) and tender lymphadenopathy Q1519:Heterophile- infectious mononucleosis Cytomegalovirus Q1520:Paul-Bunnell monospot test reaction IgM (heterophile) antibodies against EBV react with sheep red blood cells - postivie monospot test Q1521:Characteristics of acute lymphadenopathy Tender focal lymphadenopathy = bacterial. Generalized tender lymphadenopathy = viral Q1522:Characteristics of chronic lymphadenopathy Non-tender follicular hyperplasia (rheumathoid arthritis; toxoplasmosis; leukemia). Non-tender paracortical hyperplasia (viruses; drugs; SLE; leukemia). Q1523:Leukemoid reaction Vs. leukemia Leukemoid reaction lacks blast and has elevated leukocyte alkaline phosphatase (LAP) (TB; whooping cough). Chronic myelogenous leukemia has low LAP. Q1524:General signs and symptoms of leukemia Normo anemia; thrombocytopenia; leukocytosis or leukopenia; blast cells (>30%=acute); generalized non-tender lymphadenopathy; hepatosplenomegaly; bone pain and fever Q1525:Pre-B ALL Age < 15. Tdt+; CALLA+; cytoplasmic mu+ Q1526:Mature B ALL Age < 15. Surface Igs present Q1527:B cell CLL Age > 60. 95% of CLL cases. Differentiated cells are CD19+; CD20+; CD23+; CALLA- Q1528:T cell CLL Age > 60. Mature T cell markers and hypogammaglubulinemia. Lymphocytosis and neutropenia Q1529:Adult T cell leukemia Caused by HTLV-1 retrovirus. Leukemia sypmtoms and signs wih lytic bone lessions and hypercalcemia (osteoclast activating factor) Q1530:AML 15-60 years. Myeloblast proliferation. Auer rods are pathognomonic of myeloblasts. T(15;17). Abnormal retinoic acid receptor. Rx.: retinoic acid Q1531:CML 15-60 years. Pluripotent cell proliferation. Philadelphia chromosome t(9;22). All cells increased with low LAP Q1532:PRV Increased erythroid precursors; hematocrit and viscocity. Decreased EPO. Normal SaO2. Increased basophils with histamine release (pruritus; gastric ulcers); plethora and cyanosis. Q1533:Follicular B-cell lymphoma MC lymphoma. B lymphocytes. t(14;18); Chr 14 has immunoglobulin heavy chain genes; chr 18 has bcl-2 gene (normally inhibits apoptosis). Q1534:Burkitt's lymphoma MC lymphoma in children. Starry-sky. t(8;14). African affects mandible; american affects abdomen Q1535:Mycosis fungoides CD4 T-cells. Generalized prutitic erythematous rash. PAS+ Q1536:Histiocytosis X In children. Histiocytes are CD1+ Q1537:Hodgkin lymphoma Reed-Sternberg cells are CD15+; CD30+. Fever; night sweats; weight loss; localized lymphadenopathy Q1538:Multiple myeloma Neoplasm of plasma cells. Anemia; bone pain; pathologic fractures; hypercalcemia; renal failure; light-chain amyloids (Bence-Jones protein). Q1539:t(15;17) AML translocation Q1540:t(9;22) CML philadelphia chromosome translocation. Forms a protein with tyrosine kinase activity Q1541:t(14;18) Follicular B-cell lymphoma translocation Q1542:t(8;14) Burkitt's lymphoma translocation Q1543:Lymphomas: Hodgkin's Versus NHL: Which one presents with Reed-Sternberg cells? Hodgkin's Q1544:Lymphomas: Hodgkin's Versus NHL: Which one is associated with HIV and immunosuppression? NHL Q1545:Lymphomas: Hodgkin's Versus NHL: Which one involves multiple; peripheral nodes; with common extranodal involvement? NHL Q1546:Lymphomas: Hodgkin's Versus NHL: Which one is localized to a single group of nodes; with contiguous spread and rare extranodal involvement? HL Q1547:Lymphomas: Hodgkin's Versus NHL: Which one presents with constitutional signs/symptoms: low-grade fever; night sweats; weight loss? HL (NHL has few signs/symptoms) Q1548:Lymphomas: Hodgkin's Versus NHL: Which one presents with mediastinal lymphadenopathy? HL Q1549:Lymphomas: Hodgkin's Versus NHL: Which one involves mostly the B cells (except lymphoblastic origin)? NHL Q1550:Lymphomas: Hodgkin's Versus NHL: Which one has hypergammaglobulinemia? neither. Multiple Myeloma has hypergammaglobulinemia; where the excess B cells are in the resting state. Q1551:Lymphomas: Hodgkin's Versus NHL: Which one has a 50% association with EBV? HL Q1552:Lymphomas: Hodgkin's Versus NHL: Which one has bimodal age distribution? HL (NHL has peak incidence at 20-40 years old) Q1553:Lymphomas: Hodgkin's Versus NHL: Which one has more common male presentation? HL Q1554:Hodgkin's What factors denote a good prognosis? Increased lymphocytes; decreased RS cells. Q1555:Hodgkin's Which HL type has the best prognosis? Nodular sclerosing (65-75%); which has least RS cells and lots of lymphocytes. Lymphocyte-predominant LH also has excellent prognosis. Q1556:Hodgkin's Which HL type is the most common? Nodular sclerosing; affecting women more than men and primarily young adults. Q1557:Hodgkin's What is the prognosis of mixed cellular HL? Intermediate. There are lots of lymphocytes but also lots of RS cells. Q1558:Hodgkin's Which HL type has the worst prognosis? Lymphocyte-depleted; which affects older males with disseminated disease. Q1559:Hodgkin's Which HL type has the most RS cells? Mixed cellular. Q1560:Which NHL type has only B cells? Small Lymphocytic lymphoma; follicular lymphoma; Burkitt's Q1561:Which NHL type has a mix of B cells and T cells? Diffuse large cell ; occurring mostly in elderly but sometimes in children. Q1562:Which NHL type has only T cells? Lymphoblastic Lymphoma; which has immature T cells. It is a very aggressive T-cell lymphoma. Q1563:Which type is associated with a t(8;14) c-myc gene mutation? Burkitt's Lymphoma; occurring mostly in children. Has a high-grade "starry sky" appearance. Q1564:Which type is associated with a t(14;18) mutation and overexpression of bcl-2? Follicular lymphoma; which is difficult to cure but has an indolent course. Q1565:Which type is associated with EBV infection and is endemic in africa? Burkitt's Q1566:Which is the most common childhood NHL? Lymphoblastic Lymphoma; which also presents with ALL and a mediastinal mass. Q1567:Which is the most common adult version of NHL? Follicular lymphoma. Q1568:Which NHL types occur in adults? Small lymphocytic lymphoma; follicular lymphoma. Q1569:Which NHL types occur in children? Lymphoblastic lymphoma; Burkitt's lymphoma. Q1570:Which NHL has a distribution of 80% adults and 20% children? Diffuse large cell lymphoma; which ALSO has an 80% B cells 20% T cell distribution. Q1571:Which low-grade NHL type presents like CLL? Small Lymphocytic lymphoma. Q1572:Leukemias: Peripheral and bone marrow characteristics Increased circulating leukocytes; bone marrow infiltrates of leukemic cells Q1573:Leukemias: Consequences of marrrow failure Anemia (dec. RBC's); infections (dec. WBC's); hemorrhage (dec. platelets) Q1574:Leukemias: Common organs of infiltration Liver; spleen; lymph nodes Q1575:Leukemias: Characteristics of acute leukemias Blasts predominate; children or elderly; short or drastic course Q1576:Leukemias: ALL characteristics (4) Lympholasts (pre-B or pre-T); children; most responsive to therapy; associated with Down's Syndrome Q1577:Leukemias: AML characteristics (3) Myeloblasts; adults; auer rods Q1578:Leukemias: Characteristics of chronic leukemias More mature cells; midlife age range; longer; less devastating course Q1579:Leukemias: CLL characteristics - cells Lymphocytes; non-Ab producing B cells; increased smudge cells on peripheral blood smear Q1580:Leukemias: CLL - population older adults Q1581:Leukemias: CLL - presentation and course lymphadenopathy; hepatosplenomegaly; few sx; indolent course Q1582:Leukemias: CLL is similar to? very similar to SLL (small lymphocytic lymphoma) Q1583:Leukemias: CLL is associated with? warm antibody autoimmune hemolytic anemia Q1584:Leukemias: CML cell characteristics Myeloid stem cells; increased neutrophils and metamyelocytes Q1585:Leukemias: CML translocation? Ph Chromosome; t(9;22); bcr-abl Q1586:Leukemias: CML acute complications? blast crisis (AML) Q1587:what chromosomal translocation? CML Ph chromosome; t(9;22); bcr-abl Q1588:what chromosomal translocation? Burkitt's lymphoma t(8;14); c-myc activation Q1589:what chromosomal translocation? Follicular lymphomas t(14;18); bcl-2 activation Q1590:what chromosomal translocation? AML- M3 type t(15;17); responsive to all-trans retinoic acid (ATRA) Q1591:what chromosomal translocation? Ewing's sarcoma t(11;22) Q1592:what chromosomal translocation? Mantle cell lymphoma t(11;14) Q1593:What are the chronic leukemias associated with T- lymphoblasts? Sezary Syndrome; CLL-T (both L2) Q1594:What are the acute leukemias associated with T- lymphoblasts? ALL-T (L2); ALL-null (L1); ALL-common(L1) Q1595:What are the chronic leukemias associated with B- lymphoblasts? CLL-B (L3) Q1596:What are the acute leukemias associated with B- lymphoblasts? ALL-B (L3) Q1597:What are the chronic leukemias associated with monoblasts? Chonic monocytic (M5); chronic myelomonocytic (M4) Q1598:What are the acute leukemias associated with monoblasts? Acute monocytic (M5); acute myelomonocytic (M5) Q1599:What are the chronic leukemias associated with myeloblasts? CML (M1; 2 and 3); Polycythemia rubra vera (M1); myelofibrosis (M1) Q1600:What are the acute leukemias associated with myeloblasts? AML (M2 and M1); Promyelocytic (M1) Q1601:What are the chronic leukemias associated with eos- myeloblasts? Eosinophilic (rare) Q1602:What are the chronic leukemias associated with normoblasts? Chronic erythroid (M6; rare) Q1603:What are the acute leukemias associated with normoblasts? acute erythroleukemia (M6) Q1604:What are the chronic leukemias associated with megakaryoblasts? Idiopathic thrombocytopenia (M7) Q1605:What are the acute leukemias associated wwith megakaryoblasts? acute megakaryocytic leukemias (M7) Q1606:What type of cell proliferates in MM; and what is its histologic appearance Monoclonal plasma cell; fried egg appearance Q1607:Where does MM arise? bone marrow Q1608:The 2 most common ab's; in order; are: IgG (55%); IgA (25%) Q1609:Common multiple myeloma symptoms are: lytic bone lesions and hypercalcemia; renal insifficiency; increased suscpetibility to infection; anemia Q1610:This disease is associated with: primary amyloidosis Q1611:Ig light chains are also called: Bence Jones proteins Q1612:3 key diagnostic features: lytic bone lesions on x-ray; M-spike on serum protein electrophoresis; Bence-Jones proteins in urine Q1613:Red blood cell appearance on peripheral smear: Rouleaux formation (poker chips) Q1614:What 2 differences are seen in Waldenstrom's macroglobulinemia? M-spike is IgM; no lytic lesions Q1615:Target cells increased RBC membrane. Hemoglobinopathies; thalassemia; liver disease. Q1616:Acanthocytes Irregular spicules on surface. Abetalipoproteinemia Q1617:Spherocytes Decreased RBC membrane. No central area of pallor. Spherocytosis Q1618:Schistocytes RBC fragments. Microangiopathic hemolytic anemia; trauma Q1619:Bite cells RBC with removed bits of cytoplasm. G6PDH deficiency. Q1620:Sickle cells Sickle cell anemia Q1621:Howell-Jolly bodies Remnants of nuclear chromatin. Severe anemias or patients without spleen Q1622:Ring sideroblasts Trapped iron in mitochondria. Prussian-blue stain. Sideroblastic anemia Q1623:Heinz bodies Denatured Hb. G6PDH deficiency Q1624:Basophilic stipling RNA remnants. Lead poisoning Q1625:Hypersegmented neutrophil Megaloblastic anemia Q1626:EPO stimuli Low SaO2 (hypoxemia; anemia < 7gm/Dl; left shifted O2 curve Q1627:Reticulocytes Immature RBC with no nucleous and bluish color in peripheral blood indicate effective erithropoiesis. Require 24 hours to become mature. Q1628:Reticulocyte normal and corrected count Normal reticulocyte count is 1.5%. Corrected count is Hct/45 * reticulocyte count. >3% --> marrow responds well. <3% marrow is not well. If polychromasia (shift cells) divide corrected count by two because shift cells take double the time to mature Q1629:Signs of anemia Palpitations; dizziness; angina; pallor; weakness Q1630:Hypochromic RBCs Increased central pallor Q1631:MCV < 80 Iron deficiency; thalassemia; AOD; Sideroblastic Q1632:MCV 80-100; low reticulocyte count Marrow failure; aplastic anemia; leukemia; renal failure; AOD Q1633:MCV 80-100; high reticulocyte count Sickle cell; G6PDH deficiency; spherocytosis; AIHA; PNH Q1634:MCV > 100 Folate or B12 deficiency Q1635:Causes of iron deficiency anemia Ulcers; menstrual bleeding; left colon cancer; elderly and poor children; malabsorption; gastrectomy; hookworm; Plummer- Vinson Q1636:Low serum iron; % saturation and serum ferritin with high TIBC Iron deficiency anemia Q1637:Low serum iron; TIBC and % saturation with high serum ferritin AOCD Q1638:High serum iron; serum ferritin and % saturation with low TIBC Sideroblastic anemia Q1639:AOCD Iron is trapped in bone marrow macrophages due to high levels of IL-1 and lactoferrin. High ferritin and low TIBC. Q1640:HbA alpha 2beta 2 Q1641:HbF alpha 2gamma 2 Q1642:Hb Barts gamma 4 Q1643:HbH beta 4 Q1644:alpha -thalassemia Carrier has one alpha gene deletion; asymptomatic. ALPHA - Thal trait has two deletions. HbH disease three deletions with high HbH and Heinz bodies. Hydrops fetalis; four deletions; lethal; high Hb Barts Q1645:beta -thalassemia Minor; asymptomatic; 8% HbA2 and 5% HbF. Major - develop symptoms 6 months after birth as HbF declines; jaundice; bilirubin gallstones; secondary hemochromatosis due to life-long transfusions; CHF; crecut skull x-ray; target cells. 90% HbF and HbA2 Q1646:HbA2 alpha 2δ2 Q1647:Lead poisoning anemia Sideroblastic anemia. Lead denatures ferrochelatase; ALA dehydrse and ribonuclease (coarse basophilic stipling). Ringed sideroblasts and basophilic stipling. Lead colic; peripheral neuropahty; cerebral edema; learning disabilities; bone in epiphysis on x-rays. high serum Pb; high urine δ-ALA; high serum iron; ferritin and %saturation with low TIBC. Risk fators: Pb paint; battery factory; pottery painter. Q1648:Iron overload anemia Sideroblastic anemia with ringed sideroblasts. Alcoholism (MCC); pyridoxine deficiency (required by ALA synthase); isoniazid treatment. High serum iron; % saturation; ferritin and decreased TIBC. Q1649:Factors that induce and prevent sickling Deoxygenation of Hb/right shifting dissociation curve (acidosis); increasing HbS concentration (dehydration); low O2 tension (altitude and renal medulla). HbF left shifts dissociation curve and prevent sickling (hydroxeurea Rx) Q1650:Pathophysiology of sickle cell disease Valine subsitutes glutamic acid in position 6 of beta Hb chain causing sickling and thrombi that occlude vessels (painful crisis); hand-foot swelling; autosplenectomy with Howell- Jolly bodies and increased risk of infections by encapsulated orgainsms; Salmonella osteomyelitis; parvovirus B19 aplastic crisis. Q1651:Pathophysiology of G6PDH deficiency Mutation causes defective protein folding with low G6PDH activity and low levels od reduced gluthathione needed to neutralize ROS. Oxidative stress; oxidative drugs (primaquine; sulfonamides; anti-TB); bacterial infections and fava beans cause red cell damage and hemolysis with Heinz body formation (seen with methylene blue or crystal violet stains) Q1652:Pathophysiology of spherocytosis Spectrin defect with decrease in RBC membrane leads to circular RBCs which are removed by macrophages in the spleen (extravascular hemolysis). Triad of anemia; splenomegaly and jaundice with risk of bilirubinate gallstones. Increased osmotic fragility test. Q1653:Pathophysiology of AIHA IgG autoantibodies against Rh antigens on RBC with macrophage removal in spleen cause splenomegaly. Differentiate from hereditary spherocytosis with positive direct Coombs test Q1654:Pathophysiology of PNH Low levels of decay accelerating factor (DAF) are not able to normally inhibit C3 convertase with increased sensitivity of cells to complement lysis. Slow breathing at night (retains CO2) and exercise produce acidosis which activates the complement system with pancytopenia and increased risk of aplastic anemia; leukemia and venous thrombosis Q1655:Direct Coomb's test Detects IgG or C3 on surface of RBCs. Positive in AIHA; negative in hereditary spherocytosis. Q1656:Indirect Coomb's test Detects autoantibodies in the serum. Often positive in AIHA Q1657:Pathophysiology of microangiopathic hemolytic anemia RBCs are damaged by calcium in stenotic valves (aortic stenosis MCC); fibrin clots in DIC and platelet plugs in ITP and HUS. Presence of schistocytes. Q1658:Sites for reabsorption of iron; folate and B12 Iron: duodenum (Bilroth II; vitamin c deficiency and malabsorption syndromes produce deficiency). Folate: jejunum (contraceptives and alcohol decrease absorption). B12: terminal ileum (pernicious anemia; Crohn's and terminal ileum resection decrease absorption) Q1659:Pathophysiology of megaloblastic anemia Methyl THF is needed to make methylcobalamine to convert homocysteine into methione by methylTHF-homocysteine methyl transferase (requires cobalamine). Methylene THF is required by thymidilate synthetase to make nucleic acids. B12 is needed by methylmalonyl CoA mutase to make succinyl CoA.Tetrahydrofolate is made by dihydrofolate reductase (blocked by methotrexate and trimethropin). Deficiency of folate or B12 produces megaloblastic anemia with hypersegmented neutrophils (no nucleic acid synthesis); homocystinuria and methylmalonic aciduria. Q1660:Causes of folate deficiency Alcoholism (not beer); pregnancy; methotrexate; trimetrhoprim; phentoyn; birth control pills; celiac disease; leukemia Q1661:Causes of B12 deficiency Pernicious anemia; pure vegan diet; Crohn's disease; chronic pancreatitis (cant cleave R factor from saliva which protects B12); D. latum Q1662:Schilling's test Non-radioactive intramuscular B12 to saturate transcobalamin followed by radioactivee oral B12. No radioactive B12 detected in 24h urine confirms B12 absorption deficiency. Correct with intrinsic factor (pernicious anemia); pancreatic enzymes (chronic pancreatitis) or antibiotics (bacterial overgrowth) Q1663:describe structure of RBC biconcave;anucleate ;large surface:volume for easy gas exchange Q1664:how do RBCs derive energy glucose;90% anaerobic resp;10% HMP shunt Q1665:survival time of RBC 120 days Q1666:what does membrane of RBC contain chloride-bicarb antiprot ;allows RBC to transport CO2 from periphery to lungs for elimination Q1667:WBC differential from highest to lowest: Neutrophils Like Making Things Better;Neutrophils;Lymphocytes;Monocytes;Eosinophils;Ba sophils Q1668:which WBC are granulocytes BEN ;basophils;eosinophils;neutrophils Q1669:which WBC are mononuclear cells monocytes;lymphocytes Q1670:what are the steps in forming a granulocyte pluripotent hematopoietic cell;myeloid stem cell;promyelocyte;myelocyte;metamyelocyte;stab cell;granulocyte Q1671:what are the precursors to monocyte pluripotent hematopoietic stem cell;myeloid stem cell;monoblast ;monoctye Q1672:what are the precursors to lymphocytes pluripotent hematopoietic cell ;lymphoid stem cell;lyphoblasts;B/T cell Q1673:what are the steps to RBC formation pluripotent hematopoietic stem cell;proerythroblast;reticulocyte;RBC Q1674:what are hte steps to platelet formation pluripotent hematopoietic stem cell;myeloid stem cell;megakaryoblast;megakaryocyte;platelet Q1675:what are granulocytes; platelets; and monocytes all derived from (what is common precursor) myeloid stem cell Q1676:what is contained in the granules of basophils heparin ;histamine;LTD4;other vasoactive amines Q1677:when is basophilic stippling seen thalassemia ;anemia of chronic dz ;iron deficiency anemia;lead poisoning Q1678:what do eosinophils react to? helminths and protazoa ;phagocytose ag-ab complexes Q1679:causes of eosinophilai NAACP ;neoplastic;asthma;allergies;collagen vascular dz;parasites Q1680:what is contained in the PMNL granules hydrolytic enzymes;lysozyme;MPO ;lactoferrin Q1681:where do B cells go after they are mature? migrate to peripheral lymphoid tissue (follices of LN; white pulp of spleen; unencapsulated lymphoid tissue) Q1682:when does B cell --> plasma cell when it encounters ag Q1683:appearance of plasma cell off center nucleus ;abundant RER;well developed golgi Q1684:causes of DIC STOP Making New Thrombi;Sepsis (gram -);Trauma;OB complications;Pancreatitis (acute) ;Malignancy;Neoplasm;Transfusion Q1685:causes of extravascular hemolytic anemia odd shapes of RBC (spherocytes; target cells; schistocytes; etc) ;autoimmune anemia Q1686:causes of intravascular hemolytic anemia destruction via complement and lysis;paroxysmal nocturnal hemoglobinuria;microangiopathic anemia Q1687:microangiopathic anemia seen when theres is an occlusion of a small BV; which leads to mechanical disruption of RBC (seen in DIC; TTP/HUS; SLE; malignancy) Q1688:jaundice in the hemolytic anemias not seen in intravascular hemolysis normally; b/c the macrophages eat all of the destroyed Hb Q1689:what is haptoglobin ;levels during hemolysis a "suicide protein" that carries Hb to the spleen to get broken down ;levels are low Q1690:treatment for spherocytosis splenectomy Q1691:process behind paroxysmal nocturnal hemoglobinuria acidosis normally develops during sleep; which predisposes cells to destruction via complement; but this is normally inhibited by DAF (which degrades complement); if not enough DAF --> lysis of RBC InTRAVASCULARLY! Q1692:how to dx G6PD deficiency during acute crisis? look at blood smear and look for Heinz bodies;when crisis is over; look for enzyme deficiency Q1693:how to dx autoimmune hemolytic anemia direct coomb's test (or indirect) Q1694:some kids can have extremely elevated wbc counts (>50;000) and this is not malignancy; what is it called? leukemoid reaction Q1695:why do howell-jolly bodies appear in pts w scd? if pts had working spleens; they would have been able to remove these abnormal cells Q1696:what should all SC pts be vaccinated against s. pneumonia Q1697:#1 cause of osteomyelitis in SCD;#2 cause? salmonella;s. aureus Q1698:why is hydroxyurea effective in treating SCD? it creates an incresae in HbF; which binds to O2 tighter than adult Hb. Q1699:what would you do with an african american person who presents with microscopic hematuria; and is asymptomatic and has a normal CBC? test for SCD!!;there may be sickling in the renal medulla and peritubular capillary (so they might be a carrier of the trait) Q1700:what does antithrombin III do?;what activates it? breaks down factors IX; X; XI ;heparin Q1701:factor V leiden? factor 5 is resistant to breakdown by APC ;leads to venous clots Q1702:protein c;what does it require breaks down factors V and VIII ;requires vit K and protein s Q1703:protein s required as a cofactor for APC Q1704:tPA activates plasmin; which breaks down fibrin Q1705:difference between multiple myeloma and waldenstrom's macroglobulinemia MM: IgG or IgA in high amounts (lytic bone lesions; renal insuff from increased Ig light chains excretion - Bence Jones proteins) ;W<: IgM in large amts (--> hyperviscosicty; no lytic bone lesions) ;both have M spike Q1706:which form of hodgkin's lymphoma has the worst prognosis lymphocyte depleted Q1707:translocation (9;22) Philadelphia chromosome;CML Q1708:translocation (8;14) Burkitt's lymphoma (c-myc activation) Q1709:translocation (14;18) follicular lymphoma (bcl2 expression) Q1710:translocation (15;17) M3 - AML Q1711:translocation (11;22) ewing sarcoma Q1712:translocation (11;14) mantle cell lymphoma Q1713:when is WAIHA seen chronic anemia seen with;SLE ;CLL ;some drugs Q1714:when is CAIHA seen infectious mononucleosis;m. pneumonia Q1715:which NHLs are seen in children burkitt's lymphoma;diffuse large cell;lymphoblastic lymphoma Q1716:smudge cells cll Q1717:philadelphia chromosome cml Q1718:auer rods aml Q1719:how to differentiate cml from leukemoid reaction cml has low alk-phos levels Q1720:what is a result of treating aml cells can release auer rods --> DIC Q1721:What are they general characteristics of Neoplasia? Uncontrolled disorderly proliferation of cells resulting in a benign or malignant tumor or neoplasm. Q1722:What is dysplasia? 1) Reversible change;2) Often precedes malignancy;3) Morphologically manifests by disorderly amturation and spatial arrangement of cells marked variability in nuclear size and shape and increased often abnormal mitosis4) Exemplified by dysplasia of squamous epithelium of the cervix which is often a precursor of malignancy. Q1723:What are neoplasms? 1) Resemblance to tissue of origin is close the neoplasm is termed WELL DIFFERENTIATED; if little resemblance to teh tissue of origin is seen it is POORLY DIFFERENTIATED2) Neoplasms grow at the expense of finction and vitality of normal tissue without benefit to the host and are largely independent of host control mechanism. Q1724:What is Carcinoma? Malignant tumor of epithelial origin Q1725:What is Squamous cell carcinoma? 1) Originates from stratified squamous epithelium of for example the skin mouth esophagus and vagina as well as from areas of squamous metaplasia as in teh bronchi or the squamocolumnar junction of the uterine cervix2) marked by the production of keratin. Q1726:What is transitional cell carcinoma? Arises from the transitional cell epithelium of the urinary tract. Q1727:What is adenocarcinoma? Carcinoma of the glandular epithelium and includes amlignant tumors of the GI mucosa endometrium and pancreas2) Often associated with desmoplasia tumor-induced proliferation of non-neoplastic fibrous CT particularly in adenocarcinoma of the breast pancreas and prostate. Q1728:Wht is sarcoma? 1) malignant tumor of mesenchymal origin2) Often used with a prefi that denotes the tissue of origin of the timor as in osteosarcomaa rhabdomyocarcoma leiomyosarcoma and liposarcoma. Q1729:What are eponymically named tumors? 1) Burkitt lymphoma;2) Hodgkin disease;3) Wilms tumor Q1730:What is a teratoma? 1) neoplasm derived from all three germ cell layers which may contain structures such as skin bone cartilage teeth and intestinal epithelium2) May be either malignant or benign;3) Usually arises in the ovaries or testes. Q1731:Describe Benign Tumors. 1) Usually well differentiated and closely resemble teh tissue of origin;2) Do not metastasize and grow slowly. They can be harmful if their growth compresses adjacent tissues. For example benign intracranial tumors can be more lethal than some malignant skin tumors3) Tend to become encapsulated4) Denoted by the suffix -oma as in lipoma and fibroma5) Don't confuse this with some malignant neoplams as hepatoma melanoma lymphoma and mesotheliuma as well as several non-neoplastic swelings including granuloma and hematoma. Q1732:What is a papilloma? 1) Papilloma is a benign neoplasm most often arrising form surface epithelium such as squamous epithelium of the skin larynx or tongue2) Consists of delicate finger-like epithelial processes overlyig a core of connective tissue stroma that contains blood vessels3) May also develop from transitional epithelium of the urinary bladder ureter or renal pelvis. Q1733:What is an adenoma? Benign neoplasm of glandular epithelium that occurs in several variants like papillary cystadenoma and fibroadenoma. Q1734:What is papillary cystadenoma? Characterized by adenomatous papillary processes that extend into cystic spaces as in cystadenoma of the ovary. Q1735:What is a fibroadenoma? Marked by proliferation of CT surrounding neoplastic glandular epithelium; for example fibroadenoma of the breast. Q1736:What are benign tumors of mesenchymal origin? 1) Most often named by the tissue of origin; for example leiomyoma rhabdomoma lipoma fibroma and chondroma2) Include the most common neoplasm of women the uterine leiomyoma or fibroid tumor. Q1737:What is Choristoma? Small non-neoplastic area of normal tissue misplaced within another organ sucha as pancreatic tissue within the wall of the stomach. Q1738:What is a Hamartoma? Non-neoplastic disorganized tumor-like overgrowth of cell types that are regularly found within the affected organ; hemangioma an irregular accumulatoin of blood vessels is an example. Q1739:What is monoclonality? 1) Denotes origin from a single precursor cell;2) Characteristic of most neoplasms; in contrast polyclonal proliferations are almost always non-neoplastic3) Assessed by a variety of approaches. Q1740:What do Glucose-6-phosphate dehydrogenase isoenzyme studies do? 1) Offering compelling evidence for monoclonality of tumors; because of X inactivation in early embryonic life tissues of females heterozygous for G6PD isoenzymes consist of a mosaic of cell types with random cells expressing one or the other of the two isoenzymes2) monoclonal tumors express only one of the isoenzymes;3) Polyclonal cellular proliferations exhibit both isoenzymes. Q1741:How are immunoglobulins involved as indicators of monoclonality in malignancies of B cell origin? 1) Produced by B cell malignant tumors and are demonstrable as cytoplasmic or surface immunoglobulin or in the case of multiple myeloma are secreted and are demonstrable in the serum2) Monoclonal the resultant mixture of immunoglobulin molecules will exhibit either kappa or lambda chain specificity but not both a characteristic finding in neoplastic B cell proliferations3) B cell or plasma cell proliferations are polyclonal they result in the production of heterogeneous immunoglobulin molecules some of which express kappa specificity and others that express lambda specificity. Q1742:What is immunoglobulin gene rearrangement in regards to being an indicator of monoclonality in malignancies of B cell origin? 1) Characteristic of B cell maturation. The number of possible combinations achieved by rearrangement is almost countless; it can be assumed that each normal B cell is marked by a unique rearrangement pattern. Neoplastic proliferation results in large numbers of cells all demonstrating the same pattern of immunoglobulin gene rearrangement denoting their common origin form a single cell3) Assessed by molecular diagnostic techniques;4) Because immunoglobulin heavy chain rearrangement is limited to B cells this approach also demonstrates teh B cell origin of a tumor. Q1743:How are surface antigens idicators of monoclonality in malignancies of T cell origin? 1) demonstrable as T cells mature; they may be characteristic of either the stage of maturation or functional subclass. Cellular proliferations in which large numbers of T cells share surface markers in common are suggestive of monoclonality2) In addition to many others include the CD4 antigen marking T helper cells and the CD8 antigen marking T suppressor and cytotoxic cells. Q1744:What is T cell receptor gene arrangement and how is it involved as an indicator of monoclonality in malignancies of T cell origin? 1) Analogous to immunoglobulin gene rearrangement and is used in a similar manner to demonstrate both the T cell origin of a tuor and its monoclonality. Q1745:What is invasion of a tumor cell? 1) Aggressive infiltration of adjacent tissues by a malignant tumor2) Often extends into lymphatics and blood vessels with the formation of tumor emboli that may be carried to distal sites. not all tumor emboli results in metastatic tumor implants and the presence of tumor cells withing blood vessels or lymphatics indicates only the penetration of basement membranes and is not synonymous with metastasis. Q1746:What are the six steps of metastasis? 1) Growth and vascularization of the primary tumor;2) Invasiveness and penetration of basement membranes into lymphatics or blood vessels3) Transport and survival of tumor cells in the circulation4) Arrest of tumor emboli in the target tissue and passage again across basement membranes;5) Overcoming of target tissue defense mechanisms;6) Development of successful metastatic implants. Q1747:What are the preferential routes of metastasis? 1) Vary with specific neoplasms;2) Carconomas tent to metastasize via lymphatic spread3) Sarcomas tend to invade blood vessels early resulting in widespread blood-borne dissemination4) Notable exceptions include renal cell and hepatocellular carcinoma which are market by early venous invasion and hematogenous dissemination. Q1748:What are the target organs of metastasis? 1) Most commonly the liver lungs brain adrenal glands lymph nodes and bone marrow2) Rarely include skeletal muscle or the spleen. Q1749:What is tumor progression in regards to metastasis? 1) Characterized by the accumulation of successive cytogenetic or molecular abnormalities2) Exemplified by the progression of changes from normal colonic epithelium to adenoma to carcinoma to metastasis with parallel changes in APC K-ras DCC p53 and possibly other genes3) Individual neoplastic cells within a tumor may have varying metastatic potential. Q1750:What is Cachexia and wasting? 1) Origin is complex; it is characterized by weakness weight loss anorexia anemia infection and hyprmetabolism2) May be mediated in part by cachectin (TNF-alpha) a product of macrophages that promotes catabolism of fatty tissue. Q1751:What are the endocrine abnormalities of malignance? 1) Caused by tumors of endocrine gland origin which may actively elaborate hormones leading to a variety of syndromes2) Pituitary abnormalities;3) Adrenocortical abnormalities;4) Ovarian abnormalities;5) Trophoblastic tissue abnormalities Q1752:What are pituitary abnormalities of malignancy? 1) Prolactinoma leading to amenorrhea infertility and some times galactorrhea2) Somatotropic (acidophilic) adenoma leading to gigantism in children and acromegaly in adults3) Corticotropic (most often basophilic) adenoma leading to Cushing disease (adrenal hypercorticism of pituitary origin) Q1753:What are the adrenocortical abnormalities of malignancy? Include adrenogenital syndrome Conn syndrome and Cushing syndrome of adrenal origin resulting from adrenal cortical tumors. Q1754:What are ovarian abnormalities of malignancy? 1) Granulosa-theca cell tumor leading to hyperestrinism;2) Sertoli-Leydig cell tumor leading to excess androgen production. Q1755:What are trophoblastic tissue abnormalities of malignancy? Include hyperproduction of human chorionic gonadotropin from hydatiform mole or choriocarcinoma. Q1756:List 6 endocrinopathies. 1) Cushing syndrome;2) Inappropriate secretion of ADH;3) Hypercalcemia;4) Hypoglycemia;5) Polycythemia;6) Hyperthyroidism Q1757:What is Cushig syndrome in regards to paraneoplastic syndrome? Caused by production of ACTH-like substances by small cell carcinoma of the lung. Q1758:What is Inappropriate secretion of ADH in regards to paraneoplastic syndrome? Comes form a variety of tumors most commonly small cell carcinoma of the lung. Q1759:What is hypercalcemia as a paraneoplastic syndrome? Caused by metastatic disease in bone secretion of a substance similar to parathormone by squamous cell bronchogenic carcinoma or secretoin of a substance similar to osteoclast activating factor by the malignant plasma cells of multiple myeloma Q1760:What is Hypoglycemia in regards to paraneoplastic syndrome? Caused by secretion of insulin-like substances by hepatocellular carcinomas mesotheliomas and some sarcomas Q1761:What is Polycythemia in regards to paraneoplastic syndrome? Caused by elaboration of erythropoietin by renal tumors and other neoplams. Q1762:What is hyperthyroidism in regards to paraneoplastic syndrome? Caused by production of substances like thyroid-stimulating hormone by hydatidiform moles choriocarciomas and some lung tumors Q1763:What are neorologic abnormalities of paraneoplastic syndromes? 1) May occur in the absence of metastatic disease;2) Include degenerative cerebral changes with dementia cerebellar changes with resultant gait dysfunction and peripheral neuropathies Q1764:What are skin lesions related to paraneoplastic syndromes? 1) May be associated with visceral malignancies;2) Include acanthosis nigricans and dermatomyositis. Q1765:What coagulation abnormalities are associated with paraneoplastic syndromes? 1) Include migratory thrombophlebitis associated with carcinoma of the pancreas and other visceral malignancies (Trousseau phenomenon) and disseminated intravascular coagulation associated with various neoplasms. Q1766:What are oncofetal antigens? 1) Proteins normally expressed only in fetal or embryonic life; their expression by neoplastic cells is considered a manifestation of dediffrentiation;2) The undifferentiated neoplastic cells tend to resemble their embryonic counterparts3) Include carcinoembryonic antigen (CEA) which is associated with colon cancer and other cancers and preneoplastic processes and alpha-fetoprotein (AFP) which is associated with hepatocellular carcinoma and many germ cell tumors. AFP is also iincreased in fetal anencephaly and other neural tube defects. Q1767:What are direct-reacting carcinogens? Do not need to be chemically altered to act. Q1768:What are indirect-reacting carcinogens? Require metabolic conversion form procarcinogens to active ultimate carcinogensFor example a mucosal glucuronidase in the urinary bladder converts to beta-napthylamine glucuronide to the carcinogen beta-naphthylamine. Q1769:What are the stages of chemical carcinogenesis? Initiation and Promotion Q1770:What is Initiation? The first critical carcinogenic event and it is usually a reaction between a carcinogen adn DNA. Two or more agents may act together as cocarcinogens Q1771:What is promotion? Induced by a stimulator of cell proliferation and enhances the carcinogenic process. A promoter not a corcinogenic in itself enhances other agents' carcinogenicityFor example phorbol esters react with membrane receptors stimulating cell replication. This may enhance clonal selection resulting in cells with increasingly deleterious DNA changes. Q1772:How does exposure to UV radiation contribute to carcinogenesis? 1) In the form of sunlight is clearly related to the frequency of skin cancers such as squamous cell and basal cell carcinomas and melanomas2) Thought to act by inducing dimer formation between neighboring thymine pairs in DNA. In most cases such dimers are successfully repaired by enzymatically mediated mechanisms. That skin cancer may be induced by such dimer formation is suggested by the greatly increased incidence of skin tumors seen in Xeroderma pigmentosum an autosomal recessive disorder characterized by failure of DNA excision repair mechanisms. Q1773:What is ionizing radiation and how is it carcinogenic? 1) Classic cause of cancer exemplified by the increased incidence of cancers in those exposed to radiation2) skin cancer and myeloid leukemias in radiologists3) Lung cancer in uranium miners;4) Thyroid cancer in patients who have received head and neck radiation therapy;5) Acute and chronic myeloid (but not lymphoid) leukemias in survivors of atomic blasts6) Osteosarcoma in radium watch-dial workers. Q1774:How do DNA viruses contribute to carcinogenesis? 1) Integrate viral DNA into host genomes perhaps resultig in host cell expression of viral mRNA coding for specific proteins;2) Include haman papillomavirus EVB hepatitis B virus as prominent suspects that play a role in human carcinogenesis. Q1775:How do retroviruses contribute to carcinogenesis? 1) Marked by transcription of viral genomic RNA sequences into DNA by action of viral reverse transcriptase2) In the case of retroviruses that are tumorigenic in experimental animals are frequently characterized by substitutions of genomic sequences known as viral oncogenes. Q1776:What are viral oncogenes? 1) named with a three-seter abreviation preceded by v for viral;2) exhibit homology for DNA sequences of man and other eukaryotic species; these eukaryotic DNA sequences are called proto-oncogenes or cellular oncogenes and are identified with the same three-letter abbreviations preceded by c for cellular. Q1777:What are the characteristics of Ras and G proteins? 1) Located at the plasma membrane and have GTP binding and GTPase activities. GTPase hydrolytically converts active ras-GTP to ras-GDP2) Inactivated by ras-GTPase mediated by GTPase-activating protein (GAP);3) GTP activation of ras can stimulate or depress adenylate cyclase activity altering intracellular cAMP levels thus affecting cellular behavior. Q1778:Describe the mutation of the ras gene. 1) Usually occurs at codon 12;2) Results in an aberrant p21 protein product with intact GTP binding but with a loss of GTPase activity. Mutant ras proteins can be activated by GTP binding but cannot be inactivated by GTPase activity3) ras is mutated in 25%-30% of malignancies. Q1779:What is growth factor or GF receptor activity in regard to oncogenesis? alterations in expression or structural changes in oncogene products may result in inappropriate activvation of receptor proteins or their oncogenic analogs thus mimicking the actions of growth factors2) On stimulation with the appropriate growth factor receptor proteins often demonstrate tyrosine kinase activity of their cytoplasmic domains3) Significant homologies occur between several oncogenes and the genes for cellular growth factors and their receptorsa) v-sis and the gene for beta chain of PDGF;b) v-erb and the gene for EGF receptor;c) v-fms and the gene for CSF-1 receptor;d) c-neu and the gene for EGF receptor Q1780:What are nuclear proteins in regard to oncogenesis? Some oncogene products including the protein products of myc fos and myb are confined to the cell nucleus. Q1781:What is promoter insertion in regard to oncogenes? 1) Insertion of retroviral promoter or enhancer sequences into the host genome can lead to increased expression of a nearby oncogene2) This mechanism is similar to the promoter- induced hyperexpression associated with translocations characteristic of several human leukemias and lymphomas. Q1782:What are point mutations in regard to oncogenes? Exemplified by a single nucleotide changes in codon 12 of the ras family of genes associated with a number of human tumors. Q1783:What are chromosomal translocations in regard to oncogenes? Frequent association with malignancy seen in these genetic rearrangements has been clarified by demonstrating that important genes are situated at the sites of chromosomal breaks Q1784:What is 8;14 translocation? Burkitt lymphoma;c-myc proto-oncogene on chromosome 8 is translocated to a site adjacent to the imunoglobulin heavy chain locus on chromosome 14. Major regulatory sequences within the immunoglobulin gene are thought to increase the expression of c-myc Q1785:What is 14;18 translocation? Follicular lymphoma;Immunoglobulin heavy chain locus on chromosome 14 si transposed to a site adjacent to bcl-2 an oncogene on chromosome 18. This results in enhanced expression of bcl-2 thus inhibiting apoptosis. Q1786:What is 9;22 translocation? Chronic myeloid leukemia (CML);1) c-abl proto-oncogene on chromosome 9 is transposed to a site adjacent to bcr an oncogene on chromosome 222) The union of bcr adn abl results in a hybrid or chimeric bcr-abl fusion gene that codes for a protein with increased tyrosine kinase activity3) Altered chromosome carrying this hybrid gene the Philadelphia chromosome can be demonstrated by cytogenetic techniques in hematopoietic cells of patients with CML. Q1787:What is 15;17 translocation? Acute proyelocytic leukemia (FAB M3 AML);1) The translocation involves the PML gene on chromosome 15 and the retinoic acid receptor (RAR) alpha gene on chromosome 172) Therapy wiht the retinoic acid analogue all-trans retinoic acid can result in maturation of these leukemic cells and clinical remission. Q1788:Describe gene amplification. 1) Reduplication of the gene with multiple resultant genomic DNA copies and can sometimes result in a thousand or more copies of the amplified gene2) Extensive amplification can result in small free chromosome-like bodies called double minute chromosomes or in band-like structures within chromosomes called homogeneously staining regions (HSRs) which are both demonstrable cytogenetically Q1789:Name two neoplasms associated with gene amplification. Neuroblastoma and some Breast Cancers. Q1790:What is amplified in neuroblastoma? N-myc; correlates inversely with the degree of differentiation of the neuroblastoma cells. Q1791:What is amplified in some breast cancers? HER-2/neu oncogene; such amplification is associated with poor prognosis. Q1792:What are cancer suppressor genes (anti-oncogenes)? In contrast to oncogene mechanisms cancer suppressor genes promote cellular proliferation when the gene is inactivated (most often by deletion). A single residual copy of the anti- oncogene suppresses tuor formation but homozygous inactivation promotes the expressoin of the neoplastic phenotype. Q1793:Describe the anti-oncogene process using retinoblastoma. 1) An intraocular childhood tumor caused by inactivation of the Rb gene. The two hit hypothesis of Knudson holds that two mutagenic events are requird to induce alterations on both chromosomes2) In the familial forms of retinoblastoma the gene on one chromosome in teh germline is inactivated or deleted and the gene on the other chromosome is affected by a somatic mutation3) In sporadic nonfamilial cases of retinoblastoma both deletions occur as somatic mutations. Q1794:What is the p53 tumor suppressor gene? 1) Mutated in over 50% of all malignant tumors2) Has been called teh "guardian of the genome";3) In the seting of DNA damage causes cell cycle arrest in G1 providing time for DNA repair4) If repair is successful cells re-enter the cell cycle5) If not successful p53 product causes cell death by apoptosis6) Familial loss causes the Li-Fraumeni syndrome which is characterized by a wide variety of tumors: breast soft tissue sarcomas brain tumors and leukemias. Q1795:What are WT-1 and WT-2 tumor suppressor genes? 1) Are located on chromosome 11;2) Inactivation or deletion of either is associated with Wilms timor the most common renal neoplasm of children. Q1796:What is the APC tumor suppressor gene? Inactivation is common in familial polyposis coli and adenocarcinoma of the coon as well as a few other tumors; gastric and esophageal. Q1797:What is the BRCA-1 tumor suppressor gene? Inactivation is associated with familial propensity to breast and ovarian carcinomas. Q1798:What is the BRCA-2 tumor suppressor gene? Inactivation is associated with breast cancer. Q1799:What is von Recklinghausen neurofibromatosis type 1 and NF-1? 1) Characterized by multiple benign neurofibromas cafe au lait spots iris hamrtomas and an increased risk of developing fibrosarcomas2) Caused by mutations in the NF-1 tumor suppressor gene (which functions as a GAP protein that inactivates ras) Q1800:What is multiple endocrine neoplasia type II? 1) Familial occurence of the combination of medullary thyroid carcinoma bilateral pheochromocytomas and hyperparathyroidism due to hyperplasia or tumor2) Caused by mutations of teh ret proto-oncogene that are transmitted i the germline. Thus demonstration of a ret mutation in a patient with medullary thyroid carcinoma would indicate the need for surveillance for the development of pheochromocytoma or hyperparathyroidism. Q1801:What is hereditary nonpolyposis colon cancer (HNPCC or Lynch syndrome)? 1) Caused by an inherited mutation in certain DNA repair genes resulting in genomic instability2) Predisposes to mutations in other genes more diretly related to transformation. Q1802:What is Xeroderma pigmentosum? 1) An autosomal recessive disorder;2) Manifest by an increased incidence of skin cancers (basal cell carcinoma squamous cell carcinoma malignant melanoma) caused by hypersensitivity to ultraviolet light3) Involves defects in genes that function in nucleotide excision repair which is required for repair of ultraviolet-induced pyrimidine (often thymine) dimers (cross-linked pyrimidine residues). Q1803:Describe the grading of cancer. Histopathologic evaluation of the lesion based on teh degree of cellular differentiation. Q1804:Describe the staging of cancer. 1) Clinical assessment of the degree of localization or spread of the tumor2) Generally correlates better with prognosis than does histopathologic grading. However both approaches are useful3) Exemplified by teh generalized TNM system which evaluates the size and the extent of the tumor (T) lymph node involvement (N) and metastasis (M)4) Sometimes oriented toward specific tumors as exemplified by teh Dukes system for colorectal carcinoma and teh Ann Arbor system for Hodgkin disease adn non-Hodgkin lymphomas. Q1805:down syndrome associated neoplasm Acute Lymphoblastic Leukemia;we ALL go DOWN together Q1806:xeroderma pigmentosum assoc neoplasm squamus cell and basal cell carcinoma of skin Q1807:chronic atrophic gastritis pernicious anemia and postsurgical gastic remnants assoc neoplasia gastric adenocarcinoma Q1808:tuberculosis scerlosis (facial angiofibroma seizures mental retardation) assoc neoplasms astrocytoma and cardiac rhabdomyoma Q1809:actinic keratosis assoc neoplasm squamous cell carcinoma of skin Q1810:barret's esophagus assoc neoplasm esophageal adenocarcinoma Q1811:plummer-vinson syndrome (atrophic glossitis esophageal webs anemia all due to iron deficiency) assoc neoplasms squamous cell carcinoma of esophagus Q1812:cirrhosis assoc neoplasm hepatocellular carcinoma Q1813:ulcerative colitis assoc neoplasm colonic adenocarcinoma Q1814:paget's disease of bone assoc neoplasm 2ndary osteosarcoma and fibrosarcoma Q1815:aids associated neoplasom aggressive malignant lymphomas (non hodgkins) and kaposi's sarcoma Q1816:acanthosis nigricans (hyperpigmentation and epidermal thickening) assoc neoplasm visceral malignancy (stomach lung breast uterus) Q1817:dysplastic nevus assoc neoplasm malignant melanoma Q1818:tumor associated w/ oncogenes gain of function;1 c- myc;2 bcl-2;3 erb-B2;4 ras 1 burkitt's lymphoma;2 follicular and undifferentiated lymphomas (inhibits apoptosis);3 breast ovarian and gastric carcinomas;4 colon carcinoma Q1819:tymor and chromosome associated w/ homozygous loss of fx of tumor suppressor genes1 Rb;2 BRCA1 and 2;3 p53 1 13q retinoblastoma osteosarcoma;2 17q 13q Breast and ovarian cancer;3 17p most human cancers li-fraumeni syndrome Q1820:tumors assiciated w/ tumor markers1 PSA;2 CEA;3 AFP;4 beta-hCG;5 CA-125;6 S-100;7 alkaline phosphatase 1 prostatic carcinoma;2 carcinoembryonic antigen. produced by 70% colorectal and pancreatic cancers also by gastric and brast carcinomas;3 normally made by fetus. hepatocellular carcinomas. nonseminomatous germ cell tumors of the testis (i.e. ylk sac tumor);4 hydatiform moles choriiocarcinomas and gestational trophoblastic tumors;5 ovarian malignant epithelial tumors;6 melanoma neural tumors astrocytomas;7 metastases to bone obstructive biliary disease paget's disease of bone Q1821:tumors associated with Oncogenic viruses1 HTLV-1;2 HBV HCV;3 EBV;4 HPV;5 HHV-8 1 adult t cell leukemia;2 hepatocellular carcinoma;3 burkitt's lymphoma nasopharyngeal carcinoma;4 cervical carcinoma (16 18) penile/anal carcinoma;5 kaposi's sarcoma Q1822:chemical carcinogens and affected organs1 aflatoxins vinyl chloride;2 nitrosamines;3 asbestos;4 arsenic;5 CCl4;6 Napthalene dyes 1 Liver;2 esophagus stomach;3 lung (mesothelioma and bronchogenic carcinoma);4 skin (squamus cell);5 liver (centrilobular necrosis fatty change);6 bladder (transitional cell carcinoma) Q1823:Definition;when the resemblance to the tissue of origin is close Well-differentiated Q1824:(4) signs of Malignant Cancer Hyperchromatism;;Anaplasia (poor differentiation);;Inc Nuclear/cytoplasmic ratio;;prominent Nucleoli Q1825:the (3) types of CA from Epithelial origin Epithelial = "Carcinoma";Squamous Cell CA;;Adenocarcinoma;;Transitional Cell CA Q1826:Definition;reversible pre-neoplastic growth with loss of cellular orientation shape and size in comparison to normal tissue Dysplasia Q1827:Definition;a clonal proliferation of cells that is uncontrolled and excessive Neoplasia Q1828:Order of the "-plasias" in severity;(5) normal cells -> Hyperplasia ->;Metaplasia or Dysplasia - >;Anaplasia -> Neoplasia (Carcinoma in situ) ->;Metastasis Q1829:what cancer type is often associated w/ Desmoplasia (proliferation of fibrous tissue)?;name (3) main sites Adenocarcinoma;sitesBreast;Pancreas;Prostate Q1830:Definition;Neoplasm derived from all (3) germ layers;where is it usually seen? Teratoma;;MC in ovaries and testis Q1831:Definition;benign neoplasm often arising from surface or transitional epithelium;what does it look like? Papilloma;;(finger-like projections) Q1832:Difference b/t Choristoma and Hamartoma Chroistomanormal tissus misplaced w/i another organ;Hamartomabenign tumor-like overgrowth of cells regularly found w/i the infected organ Q1833:Definition;denotes origin from a single precursor cell;what is opposite? Monoclonal;(neoplastic);oppositePolyclonal;(non-neoplastic) Q1834:what type of metastatic tumors are via Lymphatic spread?;spread in blood? Carcinoma = Lymphatic;;Sarcoma = Blood Q1835:Pituitary tumor Dx;amenorrhea infertility Prolactinoma;(sometimes galactorrhea) Q1836:Pituitary tumor Dx;gigantism in children and acromegaly in adults Somatotropic (Acidophilic) adenoma Q1837:Pituitary tumor Dx;causes Cushings disease ;(secondary adrenal hypercorticism) Corticotropic (Basophilic) adenoma Q1838:Paraneoplastic effect;Inc ACTH -> Cushing's syndrome Small cell CA of lung Q1839:Paraneoplastic effect;Inc ADH -> SIADH;(2) Small cell CA of lung;;intracranial neoplasms Q1840:Paraneoplastic effect;PTH-related peptide -> Hypercalcemia;(5)* Some Really Breaks My Bones;Squamous cell CA of lung;;Renal cell CA;;Breast CA;;Multiple Myeloma;;Bone metastasis Q1841:Paraneoplastic effect;TSH -> Hyperthyroidism;(2) Hydatiform moles;;Choriocarcinoma Q1842:Paraneoplastic effect;EPO -> Polycythemia;(2) Renal cell CA;;Hemangioblastoma Q1843:Paraneoplastic effect;Hyperuricemia -> Gout;(2) Leukemias;;Lymphomas Q1844:Order of primary tumors that metastasize toBrain;(5)* Lots of Bad Stuff Kills Glia;Lung;Breast;Skin (melanoma);Kidney;GI Q1845:Order of primary tumors that metastasize toLiver;(5)* Cancer Sometimes Penetrates Benign Liver;Colon;Stomach;Pancreas;Breast;Lung;(from bottom -> up) Q1846:Order of primary tumors that metastasize toBone;(5)* Bone Problems Likely to Kill;Breast;Prostate;Lung;Thyroid / Testis;Kidney Q1847:Tumor marker seen in 70% of colorectal and pancreatic cancers CEA Q1848:Malignancy w/ Chemical Carcinogen;Vinyl Chloride Angiosarcoma of Liver Q1849:Malignancy w/ Chemical Carcinogen;Cigarette smoke;(2) CA of Lung;CA of Larynx Q1850:Malignancy w/ Chemical Carcinogen;Alkylating agents Acute Leukemia Q1851:Malignancy w/ Chemical Carcinogen;Asbestos;(3) Mesothelioma;;Lung bronchogenic CA;;GI cancers Q1852:Malignancy w/ Chemical Carcinogen;Smoked fish w/ Nitrosamines;(2) Adenocarcinoma of stomach;;Esophageal CA Q1853:Malignancy w/ Chemical Carcinogen;Alcohol;(2) Mouth CA;;Esophageal CA Q1854:Malignancy w/ Chemical Carcinogen;Arsenic Squamous cell CA Q1855:Malignancy w/ Chemical Carcinogen;High-fat diet Breast CA Q1856:Malignancy w/ Chemical Carcinogen;Naphthalene (Aniline) dyes aromatic amines Transitional CA of bladder Q1857:Malignancy w/ Chemical Carcinogen;Benzene Acute Leukemia Q1858:Malignancy w/ Chemical Carcinogen;Diethylstilbestrol (DES) Clear cell CA of vagina Q1859:Malignancy w/ Chemical Carcinogen;Nickel Chromium Uranium Lung CA Q1860:Dx;atrophic glossitis esophageal webs anemia low iron;what CA does it lead to? Plummer-Vinson syndrome;CASquamous cell CA of Esophagus Q1861:Dx;facial angiofibromas seizures mental retardation;what CA does it lead to?;(2) Tuberous Sclerosis;CAAstrocytoma;Cardiac Rhabdomyoma Q1862:what is a common skin presentation seen in malignancies of the stomach lung breast and uterus? Acanthosis Nigracans Q1863:Oncogene assoc tumor;abl CML Q1864:Oncogene assoc tumor;c-myc Burkitt's lymphoma Q1865:Oncogene assoc tumor;bcl-2 Follicular lymphoma Q1866:Oncogene assoc tumor;erb-B2;(3)* BOG;Breast;;Ovarian;;Gastric CA Q1867:Oncogene assoc tumor;ras Colon CA Q1868:Oncogene assoc tumor;L-myc Lung CA;[L = Lung] Q1869:Oncogene assoc tumor;N-myc Neuroblastoma;[N = Neuro] Q1870:Oncogene assoc tumor;ret;(2) MEN types 2 & 3 Q1871:Tumor assoc w/ Supressor gene;Rb;(2);Chromosome? Retinoblastoma;;Osteosarcoma;chrom: 13q Q1872:Tumor assoc w/ Supressor gene;BRCA1 and 2;(2);Chromosome? Breast CA;Ovarian CA;chrom: 17q 13q Q1873:Tumor assoc w/ Supressor gene;p53;Chromosome? Most cancers;(Li-Fraumeni syndrome);chrom: 17p Q1874:Tumor assoc w/ Supressor gene;p16;Chromosome? Melanoma;;chrom: 9p Q1875:Tumor assoc w/ Supressor gene;APC;Chromosome? Colorectal CA;;chrom: 5q;(5 letters in "polyp") Q1876:Tumor assoc w/ Supressor gene;WT1;Chromosome? Wilms Tumor;;chrom: 11q Q1877:Tumor assoc w/ Supressor gene;NF1;Chromosome? Neurofibromatosis type 1;(Von Recklinghausen);chrom: 17q;(17 letters in Von Recklinghausen) Q1878:Tumor assoc w/ Supressor gene;NF2;Chromosome? Neurofibromatosis type 2;;chrom: 22q;(type 2 = 22) Q1879:Tumor assoc w/ Supressor gene;DPC;Chromosome? Pancreatic CA = PC;;chrom: 18q Q1880:Tumor assoc w/ Supressor gene;DCC;Chromosome? Colon CA = CC;;chrom: 18q Q1881:Tumor marker;alpha-fetoprotein;(2) Hepatocellular CA;;Germ cell tumor of testis;(yolk sac tumors) Q1882:Tumor marker;beta-hCG;(3) HCG;Hydatidiform moles;;Choriocarcinoma;;Gestational Trophoblastic tumor Q1883:Tumor marker;CA-125;(2) Ovarian CA;;malignant Epithelial tumors Q1884:Tumor marker;S-100;(3)* MAN;Melanoma;;Astrocytoma;;Neural tumors Q1885:Tumor marker;Alkaline phosphatase;(3)* MOP;Metastasis to Bone;;Obstructive Biliary Dz;;Paget's Dz of bone Q1886:Tumor marker;Bombesin;(3) Neuroblastoma;;Lung CA;;Gastric CA Q1887:(4)* tumors w/ Psammoma Bodies PSaMMoma;Papillary Thyroid;Serous Papillary Cystadenocarcinoma of Ovary;;Meningioma;;Mesothelioma Q1888:Virus assoc tumors;HTLV-1 Adult T-cell Leukemia Q1889:Virus assoc tumors;HBV HVC Hepatocellular CA Q1890:Virus assoc tumors;EBV;(2) Burkitt's lymphoma;;Nasopharyngeal CA Q1891:Virus assoc tumors;HPV;(3) CAP it;Cervical CA (16 18);;Anal CA;;Penile CA Q1892:Virus assoc tumors;HHV-8 Karposi's sarcoma Q1893:where is person most likely to have cancer? Skin;(skin has highest incidence but unable to quantify) Q1894:top (3) MCC of CA in male and female MaleProstate;Lung;Colorectal;FemaleBreast;Lung;Colorectal Q1895:top (2) MCC of death from CA;in male and female MaleLung;Prostate;FemaleLung;Breast Q1896:What is osteogenesis imperfecta A group of heritable diseases characterized by abnormal type I collagen Q1897:How many types of osteogenesis imperfecta are there 4 (types I to IV) Q1898:What is the usual clinical presentation of osteogenesis imperfecta Multiple fractures; often with minimal trauma Q1899:Besides bone; what else is affected in osteogenesis imperfecta Teeth; skin; eyes Q1900:What are the characteristic eye findings in osteogenesis imperfecta Blue sclerae Q1901:What are the radiographic findings in osteogenesis imperfecta Thin and osteopenic bones; often with many foci of fracture callus Q1902:What is osteopetrosis An inherited disorder characterized by abnormally dense bone Q1903:What causes osteopetrosis Failure of osteoclastic cells by an unknown mechanism Q1904:What are 2 other names for osteopetrosis Marble bone disease;Alber-Schonberg disease Q1905:Why the name “marble bone” disease for osteopetrosis Bones look short and block-like; and are radiodense; like marble Q1906:What is the common clinical presentation of osteopetrosis Multiple fractures Q1907:Why are multiple fractures common in osteopetrosis Although bone is hyperdense; it is intrinsically disorganized. Consequently; it is weaker Q1908:What are 2 common conditions associated with osteopetrosis Anemia due to decreased marrow space;Blindness; deafness; and other cranial nerve involvement due to narrowing of neural foramina Q1909:What are the 2 genetic variants of osteopetrosis AR and AD Q1910:What variant of osteopetrosis is most severe The AR variant is fatal in infancy Q1911:What is osteoporosis A decrease in bone mass Q1912:What causes osteoporosis Impaired synthesis or increased resorption of bone matrix Q1913:Name 5 states with which osteoporosis is associated Postmenopause;Physical inactivity;Hypercorticism;Hyperthyroidism;Calcium deficiency Q1914:Describe the pathophysiology associated with osteoporosis of the elderly A continuous loss of bone occurs at the trabecular and cortical layers due to increased resorption Q1915:What commonly prescribed drug induces osteopenia Steroids Q1916:What commonly results from osteopenia Fractures Q1917:What are the calcium and phosphorus levels in the blood in patients with osteoporosis Normal Q1918:What is seen radiographically in patients with osteoporosis Diffuse radiolucency of bone Q1919:What is the treatment for osteoporosis No cure. Calcium supplements; exercise; and estrogen therapy (in some patients) help reduce the risk; however Q1920:What is the effect of PTH on bone It stimulates the active phase of bone remodeling Q1921:What are the two main causes of hyperparathyroidism Parathyroid hyperplasia and parathyroid adenoma Q1922:What are the two clinical features of hyperparathyroidism Bone pain and hypercalcemia Q1923:What are the significant laboratory values in hyperparathyroidism Calcium >11.5 mg/dl; Phosphorus <2.0 mg/dl Q1924:What is seen on bone histologic examination in hyperparathyroidism An increased number of osteoclasts Q1925:After bone is resorbed; what replaces it Fibrous tissue Q1926:What abnormality is often seen in the fibrous tissue of resorbed bone Hemosiderin pigment Q1927:What are the fibrous tissue lesions seen in resorbed bone called Brown tumors Q1928:How is hyperparathyroidism treated By removal of the parathyroid lesion Q1929:How does hypoparathyroidism affect bone It decreases the turnover rate Q1930:What is the most common reason for hypoparathyroidism Surgical removal of parathyroid glands Q1931:What are the clinical signs of hypoparathyroidism Signs of hypocalcemia; including soft tissue ossification and calcification; abnormal dentition; and otoscleorosis Q1932:What is seen on bone histologic examination in hypothyroidism Active osteoblasts and lack of osteoclasts Q1933:What is the treatment for hypoparathyroidism Administration of PTH or vitamin D Q1934:What is osteomalacia A bone abnormality caused by defective calcification of osteoid matrix Q1935:What causes osteomalacia Vitamin D deficiency Q1936:In what age group does osteomalacia typically occur Adults Q1937:What can osteomalacia mimic radiographically Osteoporosis Q1938:How is osteomalacia diagnosed By bone biopsy Q1939:What is the treatment for osteomalacia Correct vitamin D deficiency Q1940:What is osteomalacia called when secondary to renal disease Renal osteodystrophy Q1941:Define rickets Bone abnormality caused by defective calcification of osteoid matrix and increased thickness of epiphysial growth plates Q1942:What causes rickets Vitamin D deficiency Q1943:Describe the difference between rickets and osteomalacia Osteomalacia occurs in adults; rickets in children. Because bone growth is not complete in patients with rickets; skeletal deformities are common Q1944:What are six clinical manifestations of rickets Craniotabes—thickening and softening of occipital and parietal bones;Late closing of fontanelles;Rachitic rosary— costochondral swelling;Harrison groove—depression of insertion site of diaphragm into rib cage;Pigeon breast— protrusion of sternum;Short stature caused by spinal deformity Q1945:What is the treatment for rickets Correction of vitamin D deficiency Q1946:Define scurvy Bone abnormality characterized by impaired osteoid matrix formation Q1947:What causes scurvy Vitamin C deficiency Q1948:How does vitamin C deficiency lead to impaired bone formation Failure of praline and lysine hydroxylation required for collagen synthesis Q1949:Name three clinical characteristics of scurvy Subperiosteal hemorrhage;Osteoporosis;Epiphysial cartilage not replaced by osteoid Q1950:Why does subperiosteal hemorrhage occur with scurvy Because of increased capillary fragility Q1951:What is seen on bone histologic examination in scurvy Decreased trabecular bone mass and abnormal osteoblasts Q1952:What is the treatment for scurvy Correction of vitamin C deficiency Q1953:What is pyogenic osteomyelitis Infection of the medullary and cortical portions of the bone; including the periosteum Q1954:What bones are commonly affected by pyogenic osteomyelitis in children Long bones Q1955:What bones are commonly affected by pyogenic osteomyelitis in adults Vertebrae Q1956:What is the usual causative organism of pyogenic osteomyelitis in children Staph aureus Q1957:What are the usual causative organisms (2) of pyogenic osteomyelitis in newborns Group B beta -hemolytic strep;E coli Q1958:What is the usual causative organism of pyogenic osteomyelitis in sickle cell anemia patients Salmonella organisms Q1959:What is the usual causative organism of pyogenic osteomyelitis in IV drug abusers Pseudomonas organisms Q1960:How do the causative bacteria of pyogenic osteomyelitis spread in the body Hematogenously Q1961:In adults; what is the usual cause of pyogenic osteomyelitis Complications from surgery and compound fractures Q1962:What portion of the bone is most commonly involved initially in pyogenic osteomyelitis Metaphysis Q1963:Name 3 reasons for persistent pyogenic osteomyelitis Necrotic bone acting as a locus for persistent infection;Pyogenic exudate compressing vascular supply of bone;Inflammation in relatively avascular areas of bone Q1964:Name 2 clinical symptoms of pyogenic osteomyelitis Fever; local bone pain Q1965:What are significant laboratory test values in pyogenic osteomyelitis Marked leukocytosis; fever; and increased sedimentation rate Q1966:What is a localized bone infection surrounded by granulation tissue called Brodie abscess Q1967:How is a Brodie abscess treated Drain or debride the abscess;Administer antibiotics Q1968:How frequently do flare-ups occur with chronic osteomyelitis It varies; with intervals of months to years Q1969:What is tuberculous osteomyelitis Bone infection due to spread of tuberculous organisms Q1970:How are tuberculous organisms spread Hematogenously Q1971:What is tuberculous osteomyelitis with spinal involvement called Pott disease Q1972:What bones does tuberculous osteomyelitis affect Spine;Hip;Long bones;Bones of the hands and feet Q1973:What happens to bone affected by tuberculous osteomyelitis Progressive destruction; with little ossification Q1974:What is histiocytosis X A group of disorders affecting other organs systems in addition to bone. It is characterized by proliferation of histiocyte cells Q1975:Histiocyte cells are similar to what epidermal cells Langerhans cells Q1976:What are characteristic markers of histiocytic cells Birbeck granules Q1977:What do Birbeck granules look like Tennis rackets Q1978:Name 3 variants of histiocytosis X Eosinophilic granuloma;Hand-Schuller-Christian disease;Letterer-Siwe disease Q1979:What is characteristic of eosinophilic granuloma Histiocytic proliferation with inflammatory cells; including many eosinophils Q1980:What is the clinical presentation of eosinophilic granuloma Solitary bone lesion Q1981:Does extraskeletal involvement occur in eosinophilic granuloma Yes; commonly in the lung Q1982:What is the prognosis in eosinophilic granuloma Best of all variants of histiocytosis X. Lesions sometimes heal without treatment Q1983:What is characteristic of Hand-Schuller-Christian disease Histiocyte proliferation with inflammatory cells Q1984:What is affected in Hand-Schuller-Christian disease Bone;Liver;Spleen;Other tissues Q1985:What population is affected by Hand-Schuller- Christian disease Children less than 5 years old Q1986:List the classic triad of Hand-Schuller-Christian disease Skull lesions;Diabetes insipidus;Exophthalamos Q1987:What is the prognosis in Hand-Schuller-Christian disease Better than Letterer-Siwe; worse than eosinophilic granuloma Q1988:What is characteristic of Letterer-Siwe disease Widespread histiocyte proliferation Q1989:What population is affected by Letterer-Siwe disease Infants Q1990:What are 5 clinical findings in Letterer-Siwe disease Hepatosplenomegaly;Lymphadenopathy;Pancytopenia;Pulm onary involvement;Recurrent infections Q1991:What is the course of Letterer-Siwe disease Aggressive and fatal Q1992:What is another name for unicameral bone cyst Solitary bone cyst Q1993:What is the cause of unicameral bone cyst Unknown Q1994:What population is affected by unicameral bone cysts Young males Q1995:What portion of bone is affected by unicameral bone cysts Distal ends of long bones Q1996:Name 3 clinical signs of unicameral bone cysts Pain;Soft tissue swelling;Occasional fractures Q1997:What is seen on radiography of unicameral bone cysts Radiolucent area with smooth; thin cortex Q1998:What is the appearance of unicameral bone cysts on gross pathology Multiloculated cavity Q1999:What is the treatment for unicameral bone cysts Curettage with insertion of bone chips Q2000:What is the prognosis with unicameral bone cysts Excellent; with few recurrences Q2001:What population is affected by aneurysmal bone cysts Females in 2nd to 3rd decade of life Q2002:What portion of bone is usually affected with aneurysmal bone cysts Metaphysis of long bones;Vertebrae Q2003:Name the 2 clinical signs of aneurysmal bone cysts Pain;Soft tissue swelling Q2004:What is seen on radiography of aneurysmal bone cysts Circumscribed zone of rarefaction; with extension into soft tissues Q2005:What is the size range of aneurysmal bone cysts Up to 20 cm Q2006:What is the gross pathology of aneurysmal bone cysts Bone is greatly distorted with irregular outlines. It appears spongy; with cystic spaces of various sizes Q2007:Give 2 histologic differential diagnoses of aneurysmal bone cysts Giant cell tumor of bone;Telangiectatic osteosarcoma Q2008:How are aneurysmal bone cysts treated Removal of entire lesion with insertion of bone chips Q2009:What is the prognosis with aneurysmal bone cysts Recurrences occur 20% to 30% of the time Q2010:Fibrous dysplasia most commonly affects what bones Ribs; femur; tibia; maxilla Q2011:Is fibrous dysplasia monostotic or polyostotic 80% monostotic; 20% polyostotic Q2012:Polyostotic lesions are part of what syndrome Albright syndrome Q2013:What bone complications occur in fibrous dysplasia Deformity secondary to repeated fractures Q2014:Describe the radiographic appearance of fibrous bone dysplasia Well-defined zones of rarefaction surrounded by narrow rims of sclerotic bone Q2015:Describe the major histologic feature of fibrous dysplasia Proliferation of fibroblasts; which produce a dense collagenous matrix Q2016:What is the treatment for monostotic bone lesions Curettage or local resection Q2017:What is the treatment for polyostotic bone lesions Conservation (nonsurgical); because lesions stop growing after puberty Q2018:What is another name for osteochondroma Exostosis Q2019:Define osteochondroma Bony growth covered by a cartilaginous cap Q2020:What is osteochondroma’s claim to fame Most common benign tumor of bone Q2021:Where does osteochondroma originate In the metaphysis Q2022:What are the 2 most frequent locations for osteochondroma Distal femur;Proximal tibia Q2023:What population is most commonly affected by osteochondroma Males under 25 years of age Q2024:Does osteochondroma undergo transformation to a malignant tumor Rarely Q2025:Describe the clinical symptoms of osteochondroma Pain and compression of adjacent structures Q2026:What is the prognosis of osteochondroma Excellent. Resection is usually curative Q2027:What is giant cell tumor Benign tumor characterized by multinucleated giant cells and fibrous stroma Q2028:Where does giant cell tumor originate Epiphysis of long bones Q2029:What are the 2 most frequent locations of giant cell tumor Distal femur;Proximal tibia Q2030:How does giant cell tumor appear radiographically Soap bubble appearance Q2031:What population is most commonly affected by giant cell tumor Females 20 to 40 years old Q2032:What is the course of giant cell tumor Although benign; it is locally aggressive Q2033:What is the prognosis of giant cell tumor Frequently recurs after local curettage Q2034:What is enchondroma Benign intramedullary cartilaginous neoplasm Q2035:Where does enchondroma most frequently occur Hands and feet Q2036:What population is most commonly affected by enchondroma All age groups Q2037:What is osteoma Benign tumor of mature bone Q2038:What are the 2 most frequent locations of an osteoma Skull;Facial bones Q2039:What population is most commonly affected by osteoma Males of any age Q2040:Osteoma occurring as multiple lesions; with intestinal polyps and soft tissue tumors; is known by what name Gardner syndrome Q2041:What are the clinical features of osteoma It is asymptomatic; unless drainage of paranasal sinus is blocked Q2042:What is the prognosis of osteoma Excellent. Resection is curative Q2043:What is osteoid osteoma Neoplastic proliferation of osteoid and fibrous tissue Q2044:What are the most frequent locations of osteoid osteoma Ends of diaphysis of femur or tibia Q2045:What population is most commonly affected by osteoid osteoma Males less than 25 year old Q2046:What are the clinical features of osteoid osteoma Increasing pain; worse at night; relieved by aspirin Q2047:How does osteoid osteoma appear radiographically Central radiolucent area surrounded by sclerotic bone Q2048:What is the central radiolucent area in an osteoid osteoma called Nidus Q2049:What is the nidus of osteoid osteoma; microscopically Osteoblasts; calcification; and multinucleate giant cells Q2050:What is the prognosis for osteoid osteoma Excellent. Resection of nidus and sclerotic bone is curative Q2051:Name the 2 most frequent locations of osteoblastoma Vertebrae and long bones Q2052:What population is most commonly affected by osteoblastoma Males under 30 Q2053:What are the clinical features of osteoblastoma Usually none Q2054:Radiographically; how does osteoblastoma appear Well-circumscribed lesion surrounded by sclerotic bone Q2055:What treatment for osteoblastoma allows the best prognosis Results are excellent when the lesion is removed by curettage Q2056:Give another name for osteosarcoma Osteogenic sarcoma Q2057:State osteosarcoma’s claim to fame Osteosarcoma is the most common primary malignant tumor of bone Q2058:Define osteosarcoma Malignant osteoid and bone-producing neoplasm Q2059:What causes osteosarcoma The cause is unknown Q2060:Name the 2 most frequent locations of osteosarcoma Distal femur and proximal tibia Q2061:What population is most commonly affected by osteosarcoma Males 10-20 years old Q2062:What are the clinical features of osteosarcoma Pain; swelling; and pathologic fractures Q2063:What are the significant laboratory values of osteosarcoma A 2-3 fold increase in alkaline phosphatase levels Q2064:Radiographically; elevation of periosteum is called what Codman triangle Q2065:How does osteosarcoma spread Hematogenously Q2066:Name the 4 factors predisposing to osteosarcoma Paget disease;Ionizing radiation;Bone infarcts;Familial retinoblastoma Q2067:How does osteosarcoma appear on gross pathology Large necrotic and hemorrhagic mass Q2068:What is the microscopic appearance of osteosarcoma Malignant stroma containing osteoid and bone Q2069:How is osteosarcoma treated Surgical amputation of affected limb; and adjunctive chemotherapy Q2070:What is the prognosis for osteosarcoma Poor; 5 year survival rate is 5% to 20% Q2071:What is chondrosarcoma Malignant cartilaginous neoplasm Q2072:Name the 4 most frequent locations of chondrosarcoma Proximal femur; proximal humerus; pelvis; spine Q2073:What population is most commonly affected by chondrosarcoma Males 30-60 years old Q2074:Name three clinical features of chondrosarcoma Pain; swelling; and presence of mass for several years Q2075:Radiographically; how does chondrosarcoma appear Cortical destruction with occasional medullary involvement Q2076:From what two preexisting cartilaginous tumors can chondrosarcoma arise Multiple familial osteochondromatosis;Multiple enchondromatosis Q2077:How does chondrosarcoma appear on gross pathology Lobulated white or gray mass; with mucoid material and calcification Q2078:What is chondrosarcoma’s microscopic appearance Poorly developed cartilage cells with anaplastic cells Q2079:What is the treatment for chondrosarcoma Total resection; if possible Q2080:What is the prognosis for chondrosarcoma Chondrosarcoma is slow growing; but has a high tendency to recur; 10 year survival rate is 50-60% Q2081:What is Ewing sarcoma Undifferentiated round cell malignant tumor Q2082:In what 4 areas does Ewing sarcoma occur most often Long bones; pelvis; scapula; ribs Q2083:What population is most commonly affected with Ewing sarcoma Males less than 15 years Q2084:What are the clinical features of Ewing sarcoma Pain; swelling; and presence of mass for several years Q2085:How does Ewing sarcoma appear radiographically Destructive appearance Q2086:What does subperiosteal reactive new bone resemble Onion skin Q2087:The early phase of Ewing sarcoma mimics what other disease Acute osteomyelitis Q2088:What genetic defect is present in Ewing sarcoma 11;22 translocation Q2089:Where does Ewing sarcoma arise Undifferentiated mesenchymal cells of the medullary cavity Q2090:How does Ewing sarcoma appear on gross pathology Hemorrhagic and necrotic destruction of medullary cavity Q2091:Microscopically; what is seen with Ewing sarcoma Undifferentiated small round cells in sheets or cords Q2092:How is Ewing sarcoma treated Amputation of limb; possibly chemotherapy Q2093:What is the prognosis with Ewing sarcoma Poor. Malignant course with early metastases; the 5 year survival rate is 0-12% Q2094:Give another name for osteitis deformans Paget disease of the bone Q2095:Define osteitis deformans Bone disease characterized by abnormal bony architecture with increases in osteoblastic and osteoclastic activity; and a “ high turnover rate” Q2096:Name the five most common locations of osteitis deformans Spine; pelvis; skull; femur; and tibia Q2097:What population is most commonly affected with osteitis deformans Elderly persons Q2098:What causes osteitis deformans Cause is unknown; an infectious nature is postulated Q2099:Describe the clinical features of osteitis deformans Pain; fracture; and skeletal deformities; deafness when skull is involved; short stature when spine is involved Q2100:Is osteitis deformans monostotic or polyostotic Both Q2101:Microscopically; how does osteitis deformans appear Marked medullary fibrosis; disorganization of normal trabecular pattern Q2102:What is the treatment for osteitis deformans; and why? Calcitonin or one of the diphosphonates. They decrease resorption; and thus decrease the high turnover rate Q2103:What is avascular necrosis Necrosis of bone; usually the femoral head; caused by infarction Q2104:Give 3 possible causes of avascular necrosis Emboli;Decompression syndrome (the bends);Sickle cell anemia Q2105:Radiologically; what is seen with avascular necrosis Reparative foci replacing necrotic bone Q2106:With what other conditions is avascular necrosis commonly associated Alcoholism;Corticosteroid treatment;Hyperuricemia;SLE;Trauma Q2107:What is the treatment for avascular necrosis Hemiarthroplasty Q2108:When avascular necrosis occurs in the femoral head of children; what is it called Legg-Calve’-Perthes disease Q2109:What disease is characterized by pain in weight- bearing joints; is worse after use; has crepitation with motion; no signs of inflammation; and is seen in the middle-aged population Osteoarthritis; aka degenerative joint disease Q2110:What are signs of osteoarthritis on x-ray Joint space narrowing; osteophytes Q2111:What are Herberden’s nodules Palpable DIP joints with osteophytes Q2112:What are Bouchard’s nodules Palpable PIP joints with osteophytes Q2113:What are some treatments of osteoarthritis NSAIDs and weight reduction to reduce strain on joints Q2114:A 4 year old boy presents with arthralgias; soft hyperextensible skin; corneal and scleral abnormalities; joint laxity; and easy bruising. Diagnosis? Ehlers-Danlos syndrome Q2115:A 32 year old female presents to your office complaining of morning stiffness for greater than 1 hour; pain in joints bilaterally; with fatigue and hand deformations over time. Diagnosis? Rheumatoid arthritis Q2116:Which joints are most commonly involved in RA Wrists; PIP; and metacarpophalangeal Q2117:What are some common findings with RA Fever;Malaise;Pericarditis;Pleural effusions;Uveitis;Subcutaneous nodules Q2118:Which lab test should you order when you suspect RA Rheumatoid factor Q2119:What is rheumatoid factor Immunoglobin M antibody to fragment crystallizable (Fc) portion of IgG Q2120:What is the name for the chronic inflammation of cartilage found in RA Pannus Q2121:What disease would be expected in a young woman that suffers with RA-like symptoms (polyarthritis); leukopenia; leg ulcers; and splenomegaly? Felty’s syndrome Q2122:What disease is similar to RA (bilateral joint pain; fever) but is seen in children; along with rash and hepatosplenomegaly Still’s disease (juvenile RA);*Kids can’t sit Still when their joints hurt Q2123:What is different about pediatric RA It is often RF negative Q2124:A 5 year old child presents with complaints in two joints. Which disease is this Pauciarticular juvenile RA Q2125:What is a child with pauciarticular RA at risk for Iritis (do slit-lamp test) Q2126:A 43 year old male presents to your clinic with a history of falling on his knee two days ago. Since then; the knee has become red; swollen; and warm. Diagnosis? Septic joint Q2127:How can you diagnose septic joint Arthrocentesis with high white blood cells/ neutrophil count and Gram’s stain Q2128:What is the most common causative organism of septic joint Staphylococcus aureus Q2129:What are some common organisms that are found uniquely in the joints of infants and young children Group B streptococci;Haemophilus influenzae Q2130:What are some common organisms associated with implantable devices and prosthetics Staph aureus;Staphylococcus epidermidis;Gram-negative bacilli Q2131:A 23 year old male; sexually active college student arrives at your clinic complaining of a swollen red knee and urethritis. What organism is the most likely Neisseria gonorrhoeae Q2132:How is the diagnosis made in a patient with gonorrhea Urethral swab Q2133:How do you treat gonorrhea Ceftriaxone Q2134:Which organism should be suspected in a patient with diabetes and osteomyelitis Pseudomonas Q2135:Which organism should be suspected in a sickle cell patient with osteomyelitis Salmonella Q2136:A 65 year old white male presents to clinic with a 2 hour history of sudden onset of extreme pain in his great toe. What disease should be suspected in this patient Gout Q2137:What is podagra Gout of the metatarsophalangeal joint of the big toe Q2138:What other findings should be looked for in a patient with gout Tophi; subcutaneous deposits of uric acid crystals Q2139:How do tophi appear on x-ray “Punched out” lesions Q2140:What lab tests help to diagnose gout Uric acid level;Joint fluid aspiration of needle-shaped crystals with negative birefringence Q2141:What are contributing factors for gout Thiazide diuretics;Lesch-Nyhan syndrome;Diets with high protein and alcohol Q2142:What is the treatment for gout Acute—Colchicine; NSAIDs;Maintenance—Allopurinol Q2143:What diseases are associated with pseudogout Hyperparathyroidism;Hemochromatosis Q2144:A 12 year old presents with migratory polyarthritis; rash; fever; and general malaise. She recalls having a sore throat about 3 weeks ago but did not get treatment. Diagnosis? Acute rheumatic fever Q2145:What are the Jones criteria of rheumatic fever Fever;Erythema marginatum;Verrucous valvular vegetations;Erythrocyte sedimentation rate increase;aRthritis;Subcutaneous nodules;Chorea (Sydenham’ s);Preceded by Streptococcus infection;*FEVERS and Chorea preceded by a Streptococcus infection Q2146:What is the distinctive inflammatory heart lesion associated with rheumatic fever Aschoff’s bodies Q2147:What is the causative organism of the sore throat in rheumatic fever Group A beta -hemolytic streptococci Q2148:What is the actual cause of rheumatic fever Hypersensitivity—cross reaction antibodies against M protein of Streptococci Q2149:Which titers are elevated with rheumatic fever Antistreptolysin O titer; ESR Q2150:Which hematologic disease is associated with avascular necrosis of the femoral head Sickle cell anemia Q2151:A patient presents with urethritis; conjunctivitis; arthritis; and happens to be HLA-B27 positive. Diagnosis? Reiter disease;*Can’t pee; can’t see; can’t kick with your knee Q2152:Previous exposure to which bacteria can precipitate Reiter disease Chlamydiae;Shigella;Salmonella;Campylobacter;Yersinia species Q2153:A 35 year old white male presents with diffuse red/purple plaques with silver scale on extensor surfaces and scalp. What disease is suspected Psoriasis Q2154:What other symptoms are common with psoriasis Nail pitting;Psoriatic arthritis with sausage digits Q2155:What is the most common form of psoriatic arthritis Asymmetric arthritis in fingers or toes Q2156:What is the phenomenon that describes the development of a psoriatic plaque in an area of previous trauma Koebner phenomenon Q2157:What is the name of the sign that occurs when a small amount of scale is removed from a psoriatic plaque; leaving small bleeding points behind Auspitz sign Q2158:What can precipitate an outbreak of psoriasis Infection;Stress;Sunburn;Drugs— beta -blockers; lithium; and antimalarials Q2159:What is used to treat psoriasis Psoralen ultraviolet A (PUVA);Retinoids;Methotrexate;Cyclosporin Q2160:What HLA type is associated with psoriatic arthritis HLA-B27 Q2161:A 28 year old African-American female presents to the clinic with new onset of fatigue; weight loss; joint pain; and Raynaud’s phenomenon. On exam; she is found to have a malar rash. Diagnosis? SLE Q2162:What are the official criteria for the diagnosis of SLE Oral ulcers;Renal disorder;Photosensitivity;Hematologic (anemias; cytopenias);Arthritis (nonerosive synovitis);Neurologic (seizures; psychosis);Serositis;Malar rash;Antinuclear antibody;Immunologic (anti-DNA; anti- Smith; false positive rapid plasma reagin/Venereal Disease research Laboratory [RPR/VDRL]);Discoid rash;*The ORPHAN’S MAID has Lupus;*Need 4/11 criteria for diagnosis Q2163:In which sex and race is SLE most common and severe Black females Q2164:What cardiac lesion is associated with SLE in the adult and consists of nonbacterial verrucous valvular vegetations Libman-Sacks endocarditis Q2165:What are some causes for chest pain in a patient with SLE Pleuritis;Pericarditis Q2166:A 30 year old white male presents with new onset of SLE-like symptoms. Which drugs can cause these Hydrazine;INH;Procainamide;Phenytoin;*You won’t be HIPP with drug-induced Lupus Q2167:Which antibody system is associated with drug- induced Lupus Antihistone Q2168:How are the manifestations of SLE in the kidney classified Type I—appears normal by light microscopy;Type II— mesangial lupus glomerulonephritis;Type III—focal proliferative glomerulonephritis;Type IV—diffuse proliferative glomerulonephritis;Type V—membranous glomerulonephritis Q2169:What are wire-loop lesions in the kidney and what do they represent Thickening of the capillary wall found in diffuse proliferative glomerulonephritis; indicate a poor prognosis with SLE Q2170:Which neoplasm is associated with SLE and myasthenia gravis Thymoma Q2171:Which antibody is sensitive but not specific for the diagnosis of SLE Antinuclear antibody Q2172:Which two antibodies are very specific for SLE Anti-Smith;Antidouble-stranded DNA Q2173:A black female with SLE delivered an infant with bradycardia; which is later found to have AV-block. What autoantibody could have caused this congenital heart block Anti-Rho antibodies which cross the placenta Q2174:Which two HLA types is SLE linked to HLA-DR2 and HLA-DR3 Q2175:An 18 year old female patient presents with a 5 week history of arthritis; fever; 15lb weight loss; and diarrhea. What diseases should be considered Inflammatory bowel disease—Crohn’s and ulcerative colitis Q2176:On physical exam; a patient is found to have a right lower quadrant abdominal mass and positive fecal occult blood. Radiographic studies show a thickened terminal ileum; skip lesions; and areas of stricture. Diagnosis? Crohn’s disease Q2177:What are systemic symptoms of Crohn’s disease Aphthous ulcers;Erythema nodosum;Uveitis Q2178:What is the cause of Crohn’s disease Idiopathic;Associated with HLA-B27 Q2179:What findings would be expected on gross examination of a biopsy taken for a small bowel with Crohn’s disease Sharp demarcation of diseased bowel (skip lesions); Linear ulcers; Cobblestone mucosa; Sinus tract/fistula formation; Creeping fat around the bowel surface; *The old Crohn skips down the cobblestone tract Q2180:What findings would be expected histologically in Crohn’s disease Transmural inflammation of bowel wall; Noncaseating granulomas; Mucosal damage; Ulceration Q2181:What are treatment options for Crohn’s disease Sulfasalazine; Corticosteroids; Bowel rest; Surgery (fistulas; obstruction); B12 supplements for malabsorption Q2182:A 22 year old white female presents with a 2 week history of migratory polyarthritis; 15 lb weight loss; and grossly bloody mucoid diarrhea. Diagnosis? Ulcerative colitis Q2183:What features are characteristics of ulcerative colitis Continuous involvement beginning at rectum; Microabscesses; Pseudopolyps; Superficial ulceration Q2184:A patient with established ulcerative colitis presents to the ER with high fever and symptoms of shock and abdominal distention. What must be suspected Toxic megacolon Q2185:What systemic illnesses do patients with ulcerative colitis exhibit Toxic megacolon; Colon cancer; Primary sclerosing cholangitis; Pyoderma gangrenosum; Uveitis; Erythema nodosum Q2186:What are the major differences that separate UC from Crohn’s No skip lesions; no granulomas; no fistulas/sinuses in ulcerative colitis Q2187:A 45 year old white female presents with a 3 month history of diarrhea; fatty white stools; and a rash that was diagnosed as dermatitis herpetiformis. What is her probable diagnosis Celiac sprue Q2188:What causes celiac sprue Antigliaden antibody (IgA antiendosomal) Q2189:What pathology would be seen in biopsy of celiac sprue Blunting of villi; Lymphocytes in the lamina propria Q2190:What dietary recommendations should celiac sprue patients follow Avoid all foods containing gliadin (gluten); including wheat and beer Q2191:A 33 year old white male complains of joint pain. He mentions that he has noticed an increase in pigmentation along with frequent urination and a strange tendency to set off metal detectors. Diagnosis? Hemochromatosis Q2192:What are the symptoms for hemochromatosis Bronze diabetes; Congestive heart failure; Micronodular cirrhosis Q2193:What type of cancer are patients with hemochromatosis at risk of Hepatocellular cancer Q2194:What is the cause of the symptoms of hemochromatosis Increased iron deposition Q2195:Besides increased iron supply; what are other important lab findings in hemochromatosis Increased ferritin;Increased transferrin;Positive Prussin blue stain Q2196:What is the inheritance pattern of primary hemochromatosis Autosomal recessive Q2197:What is the cause of hemochromatosis as a secondary disorder Chronic blood transfusions Q2198:What are treatment options for hemochromatosis Phlembotomy; Deferoxamine Q2199:An 18 year old football player complains of joint pain; bruising; and somewhat limited range of motion. He reports that he has always been a free bleeder. Diagnosis? Hemarthroses associated with hemophilia Q2200:A 15 year old boy scout complains of a 2 week history of flu-like illness and joint pain which started in his left knee; and now is in his right knee. It all began after a camping trip in Connecticut. Diagnosis? Lyme disease Q2201:What causes Lyme disease Borrelia burgdorferi; which is transmitted by Ixodes tick bites Q2202:A boy scout with Lyme disease recalls a strange bull’s eye rash that appeared and then disappeared before he could get an appointment. What is the rash called Erythema chronicum migrans Q2203:What cardiac complications are possible with Lyme disease First-degree AV-block Q2204:What are the treatments for Lyme disease Tetracycline; Doxycycline Q2205:A 34 year old abstinent Asian patient presents with a several year history of arthritis; recurrent genital and oral ulcers; and a painful rash over the pretibial areas. Diagnosis? Behcet’s disease Q2206:An 18 year old man presents with migratory arthritis; currently in his ankle; a rash; and pain with urination. A Gram ’s stain of urethral discharge shows gram-negative cocci. Diagnosis? Gonococcal arthritis Q2207:A 24 year old man recently diagnosed and treated for chlamydia complains of persistent joint pain with tender Achilles’ tendons and eye irritation. Diagnosis? Reiter’s reactive arthritis Q2208:A 28 year old male presents with a 2 week history of joint pain; fever; malaise; as well as a new rash all over his body including his palms and soles. Diagnosis? Secondary (disseminated) syphilis Q2209:A patient with syphilis describes two papular lesions on his penis that are gray-white. What are these lesions Condylomata lata of the secondary stage of syphilis Q2210:A patient with syphilis admits that initially he had a red; firm; painless sore on his penis 6 weeks ago; but it disappeared. What is the lesion called Chancre—the primary stage of syphilis Q2211:What is the best way to diagnose syphilis in the primary stage Darkfield exam Q2212:Which serologic test detects syphilis the earliest; is the most specific; and stays positive even after treatment Fluorescent treponemal antibody absorption test (FTA-ABS) Q2213:Which serologic tests are used for presumptive diagnosis of syphilis VDRL and RPR Q2214:What are treatment options for syphilis in primary and secondary stages Penicillin G—intramuscular Q2215:What is the classic reaction hours after treatment for syphilis that involves shaking chills; sore throat; myalgia; and malaise Jarisch-Herxheimer reaction Q2216:If a patient with secondary syphilis is not treated; what are some outcomes in the following years One third of patients get tertiary syphilis (1-40 years after infection); Two thirds have no further symptoms Q2217:What are some of the findings in tertiary syphilis Iritis (Argyll Robertson pupil); Aortitis; Neurosyphilis (tabes dorsalis; paresis); Gummas Q2218:A 20 year old black male presents with a 1 month history of worsening back pain that is worse in the morning and improves with exercise. What is the suspected diagnosis Ankylosing spondylitis Q2219:What are some associated findings with ankylosing spondylitis Positive family history; Uveitis; Anemia Q2220:What clinical test for ankylosing spondylitis should be performed in the office Schober test—decreased angle of anterior flexion of the back; eliciting pain Q2221:What is the genetic association of ankylosing spondylitis AR; HLA-B27 Q2222:What is classic sign of ankylosing spondylitis on radiographs Bamboo spine Q2223:A 45 year old male smoker complains of cold sensitivity and pain in his fingers. Some fingers have signs of gangrene. What is he suffering from Buerger’s disease (smoking and thromboangiitis obliterans) Q2224:What are the signs and symptoms of Buerger’s disease Intermittent claudication of small vessels; Raynaud’s phenomenon; Nodular phlebitis Q2225:What HLA types are increased in patients with Buerger’s disease HLA-A9 and HLA-B5 Q2226:What is the treatment for Buerger’s disease Stop smoking Q2227:A 22 year old Asian female presents to your office with arthritis; fevers; night sweats; change in vision; and skin nodules. On physical exam; she is found to have weak and uneven pulses in the upper extremities. What disease should be ruled out Takayasu’s arteritis (pulselessness disease) Q2228:What vessels does Takayasu's arteritis affect Medium and large arteries Q2229:What lab test is usually abnormal in Takayasu’s arteritis ESR (elevated) Q2230:What imaging test should be done to confirm Takayasu’s arteritis Angiogram Q2231:In Takayasusu’s arteritis; what would an angiogram reveal Thickening (granulomatous inflammation) of the aortic arch and proximal vessels Q2232:What are some complications of Takayasu’s arteritis Pulmonary hypertension; Stroke Q2233:A 4 year old Japanese female presents to the ER with a 5 day history of fever above 102; arthritis; bright red lips; swollen hands and feet; and swollen lymph nodes. Diagnosis Kawasaki disease (mucocutaneous lymph node syndrome) Q2234:What are the criteria for a diagnosis of Kawasaki disease Fever >5 days; Lymphadenopathy; Bilateral conjunctival injection; Mucosal changes (fissuring; injection; strawberry tongue; erythema); Extremitiy changes (edema; erythema); Rash (truncal; may be desquamative); Arthritis (may be present) Q2235:What are the major complications of Kawasaki disease Myocardial infarction and coronary artery aneurysms Q2236:What autoantibodies are found in Kawasaki disease Antiendothelial antibodies Q2237:Which vessels does Kawasaki disease typically affect Large; medium; and small arteries Q2238:What should be done if Kawasaki disease is suspected Treat with aspirin and IV immunoglobuliins Q2239:Why is Kawasaki disease one of the only indications for using aspirin in children Reye’s syndrome limits the use of aspirin in children; except in the treatment for Kawasaki disease Q2240:A 7 year old boy presents with arthritis; lower extremity palpable purpura; abdominal pain; and blood in the stool and urine. Diagnosis? Henoch-Schonlein purpura Q2241:What are two common histories with Henoch- Schonlein purpura Post-streptococcal infection or upper respiratory infection; History of insect bites Q2242:What types of immune complexes are found in tissue biopsy of Henoch-Schonlein purpura IgA dominant Q2243:Which vessels are mainly affected in Henoch- Schonlein purpura Small vessels (capillaries; venules; and arterioles) Q2244:A young man presents with arthritis; asthma; allergy; weight loss; fever; and vasculitis. What disease is suspected Churg-Strauss syndrome Q2245:What are some laboratory findings of Churg-Strauss syndrome Blood eosinophilia; Increased IgE Q2246:Which vessels are affected in Churg-Strauss syndrome Small and medium sized vessels Q2247:Which autoantibody groups are associated with Churg-Strauss Antineutrophil cytoplasmic antibody (ANCA); Antimyeloperoxidase Q2248:A 65 year old female presents with increasing headache; vision changes; scalp pain; and jaw pain. She also complains of a few previous months of aching joints and muscles. What should immediately be suspected Temporal (giant cell) arteritis Q2249:What should be done immediately when temporal (giant cell) arteritis is suspected Start high-dose steroids Q2250:What is the major complication of temporal arteritis that prompts administration of steroids Blindness Q2251:Which lab test is elevated in temporal arteritis ESR Q2252:What is the diagnostic test that confirms temporal arteritis Temporal artery biopsy Q2253:What would a biopsy of temporal arteritis show Granulomatous arteritis Q2254:What other disease is strongly associated with temporal arteritis Polymyalgia rheumatica Q2255:A 42 year old male presents with chronic sinusitis; hemoptysis; necrotizing granulomas of the nose and palate; and a previous diagnosis of crescentic glomerulitis. Diagnosis? Wegener’s granulomatosis Q2256:Which vessels are usually affected in Wegener’s granulomatosis Medium and small vessels of the respiratory tract and kidneys Q2257:Which antibody is found in Wegener’s Cytoplasmic-ANCA (c-ANCA); predominantly antiproteinase 3 Q2258:What deformity is associated with Wegener’s Saddle nose deformity Q2259:Besides Wegener’s; what is the other disease that involves both the respiratory tract (hemoptysis) and kidney (renal failure) Goodpasture’s syndrome Q2260:What is the antibody associated with Goodpasture’s syndrome Antiglomerular basement membrane Q2261:What does immunoflorescence of affected tissue in Goodpasture’s syndrome show Linear deposits of IgG and C3 in the glomerular basement membrane Q2262:A 40 year old black female present with increasing shortness of breath; polyarthritis; change in vision; fevers; and malaise. On chest x-ray; there is bilateral hilar lymphadenopathy. What is the most likely diagnosis Sarcoidosis Q2263:What electrolyte abnormality is common with sarcoidosis Hypercalcemia Q2264:On biopsy of affected tissue; what is the classic finding in sarcoidosis Noncaseating granulomas Q2265:What skin findings are associated with sarcoidosis Erythema nodosum; Lupus pernio Q2266:A 26 year old white male presents with malaise; fever; weight loss; hypertension; ab pain; and melena. He has a history of hepatitis B and drug use. What disease is suspected Polyarteritis nodosa (PAN) Q2267:How is a diagnosis of polyarteritis made Tissue biopsy showing transmural necrotizing arteritis of medium-sized arteries Q2268:What is the treatment for polyarteritis nodosa Steroids and cyclophosphamide Q2269:What is the disease that is a variation of polyarteritis nodosa; which affects smaller arterioles; capillaries; and venules rather than the larger vessels Microscopic polyangiitis (leukocytoclastic vasculitis) Q2270:What clinical symptoms do patients with microscopic polyangiitis have Hemoptysis; Hematuria; Abdominal pain/blood in stool; Skin findings (purpura) Q2271:Which antibody is microscopic polyangiitis most closely associated with p-ANCA Q2272:A 50 year old man presents with fever; arthralgias; and palpable purpura on the lower extremities after starting several new medications. What is the most likely diagnosis Hypersensitivity angiitis Q2273:How is the diagnosis of hypersensitivity angiitis made Skin biopsy showing infiltration of dermal capillaries Q2274:A 55 year old white female presents with polyarthritis; dysphagia and reflux esophagitis; pulmonary fibrosis; and hypertension. On exam; her face appears tight and masklike and she has swelling of the hands and thickening of the skin. What is the most likely diagnosis Scleroderma (systemic scleroderma) Q2275:What is the autoantibody that is most closely associated with scleroderma Anti-DNA topoiosomerase I (anti-Scl-70) Q2276:What is the more limited version of scleroderma CREST syndrome Q2277:What are the characteristics of CREST syndrome Calcinosis (subcutaneous); Raynaud phenomenon; Esophageal dysfunction; Sclerodactyly; Telangiectasia Q2278:What antibodies are most closely associated with CREST syndrome Anticentromere antibody Q2279:A 50 year old white female presents with very dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca). She reports that she has several dental caries filled recently. What is the most likely syndrome Sjogren’s disease Q2280:What are some GI symptoms commonly associated with Sjogren’s Constipation and pancreatic insufficiency due to gland destruction Q2281:How is Sjogren’s disease diagnosed Lip biopsy; Schirmer’s test (showing decreased lacrimation) Q2282:What autoantibodies are associated with Sjogren’s disease Antinuclear antibody (ANA) (nonspecific) and antinucleoprotein antibodies (SS-A[Ro] and SS-B) Q2283:What are patients with Sjogren’s at risk for developing Lymphoma Q2284:A 23 year old white female presents with a 6 month history of weight loss; muscle weakness; palpitations; diarrhea; and fine tremor. Tachycardia and exophthalmos are present. What do you suspect Graves’ disease (diffuse toxic goiter) Q2285:What autoantibody causes Graves disease Thyroid-stimulating hormone (TSH)-receptor antibody Q2286:A 23 year old white female develops limb weakness; ptosis; diplopia; and difficulty chewing. Weakness improves after rest. Sensation and reflexes remain intact and normal. What disease is suspected Myasthenia gravis Q2287:How is the diagnosis of myasthenia gravis confirmed Edrophonium challenge temporarily improves muscle strength Q2288:What is edrophonium Anticholinesterase inhibitor Q2289:What causes myasthenia gravis Antiacetylcholine-receptor antibody Q2290:What HLA type is associated with myasthenia gravis HLA-DR3 Q2291:What diseases are associated with myasthenia gravis Thymoma; Thyrotoxicosis Q2292:What treatments are available for myasthenia gravis Anticholinesterase drugs; Corticosteroids; Thymectomy; Plasmapheresis Q2293:A 55 year old white woman presents with 1 month history of proximal muscle weakness and pain; increasing fatigue; and malaise. What disease should be ruled out Polymyositis Q2294:What lab finding support the diagnosis of polymyositis Increased ESR; Increased CPK; Increased aldolase; Increase LDH; Antinuclear may be positive; Abnormal electromyography Q2295:What is the only specific test that provides a definitive diagnosis of polymyositis Muscle biopsy showing lymphoid inflammation Q2296:What disease is characterized by symptoms and lab values similar to polymyositis; but also has a lilac edematous rash on the eyelids Dermatomyositis Q2297:What is the name of the rash on the eyelids found in dermatomyositis Heliotrope rash Q2298:What are patients with dermatomyositis and polymyositis at an increased risk of developing Ovarian cancer Q2299:What is the treatment for dermatomyositis and polymyositis High-dose steroids Q2300:A 55 year old white female complains of 3 months of neck stiffness; pelvic and pectoral girdle weakness; and pain; fatigue; and malaise. What is the most likely diagnosis Polymyalgia rheumatica Q2301:What lab values would be abnormal in polymyalgia rheumatica Elevated ESR Q2302:With what disease is polymyalgia rheumatica associated Temporal arteritis Q2303:A 45 year old white woman presents with a 2 month history of decreased sleep and several (>11) very tender points on her anterior and posterior torso and neck that produce extreme pain with palpation. She has a history of anxiety disorder and depression. Diagnosis? Fibromyalgia Q2304:What lab values should be evaluated in fibromyalgia CBC—normal; ESR—normal Q2305:How should a patient with fibromyalgia be treated NSAIDs and antidepressants Q2306:Dx;autosomal dominant trait leading to the failure of longitudinal bone growth; causing short limbs Achondroplasia Q2307:Dx;Pannus formation in joints Rheumatoid Arthritis Q2308:Dx;increased bone resorption due to decreased estrogen levels Osteoporosis (type 1);(type 2 is men and women >70) Q2309:(2) common fractures due to Osteoporosis Vertebral crush fractures;;Collies distal Radius Fx Q2310:Definition;failure of normal bone resorption leading to thick; dense bones due to abnormal function of Osteoclasts; no labs elevated; "marble bone Dz" OsteoPETrosis Q2311:Definition;increase in oseteoblastic and osteoclastic activity; all labs normal except great increase in Alk Phos Padget Dz of the Bone Q2312:Dx;bone replaced w/ fibroblasts and collagen (PFD) in unilateral bones; precocious puberty; unilateral pigmented skin Albright's syndrome Q2313:Definition;pain and stiffness in shoulders and hips; often w/ fever and weight loss in pt >50yo;what is it assoc w/? Polymyalgia rheumatica;;assoc w: Temporal (giant cell) arteritis Q2314:what joint problem can be caused by G6PD deficiency? Gout Q2315:Dx;painful MTP joint; formations on external ear or achilles tendon;test? Gout;(Tophus formations are on ear and achilles);Test: joint aspiration;(Needle-shaped Negatively birefringement) Q2316:Dx;calcium crystals in the knee;test? Pseudogout;Test: joint aspiration;(Basophilic; rhomboid; Positively Birefringement) Q2317:Dx;Gammaglobinemia; RA; ACE increase; Interstitial lung fibrosis; Noncaseating granulomas Sarcoidosis;"GRAIN" Q2318:(2) types of Seronegative spondyloarthropathies ;(arthritis w/o rheumatoid factor) Ankylosing Spondylitis;Reiter's syndrome Q2319:Dx;chronic inflammatory dz of spine and sacroiliac joints; uveitis and aortic regurg Ankylosing Spondylitis Q2320:Dx;Urethritis; conjunctivitis and anterior uveitis and arthritis Reiter's syndrome;(Can't see; can't pee; can't climb a tree) Q2321:Dx;an acquired loss of melanocytes in descrete areas of skin that appear as depigmented white patches Vitiligo Q2322:Definition;epidermal hyperplasia and hyperpigmentation most often in flexural areas and a marker of visceral malignancy Acanthosis Nigricans Q2323:Dx;port-wine stain on the face; ipsilateral glaucoma; vascular lesions of ocular tissue; extensive hemangiomatous involvement of meninges Sturge-Weber syndrome Q2324:Dx;pruitic eruption; commonly on the flexor surfaces;(2 names) Atopic Dermatitis;(Eczema) Q2325:Dx;Epidermal hyperplasia w/ parakeratotic scaling especially on knees and elbows;what layer of skin is increased?;Decreased? Psoriasis;;Inc Spinosum;Dec Granulosum Q2326:Definition;Pruritic papules and vesicles associated w/ Celiac sprue Dermatitis Herpetiformis Q2327:Dx;Pruitic; purple; polygonal papules; infiltrate of lymphocytes at the dermalepidermal junction Lichen Planus;[Lymphocytes = Lichen] Q2328:Definition;assoc w/ infections; drugs; CA and autoimmune Dz; presents w/multiple types of lesions including macules; papules; vesicles and Target lesions Erythema Multiforme Q2329:Dx;erythema multiforme; high fever; bulla formation and necrosis; ulceration of skin; high mortality rate Stevens-Johnson syndrome Q2330:Dx;common benign flat pigmented squamous epithelial proliferation of keratin-filled (horn) cysts that look pasted-on Seborrheic Keratosis Q2331:Definition;subepidermal blistering w/ characteristic inflammatory infiltrate of eosinophils in the surrounding dermis;auto Ab against epidermal BM Bullous Pemphigoid Q2332:Dx;Blistering of oral mucosa with extensive skin involvement following; breakdown of cell-to-cell junctions w/ sparing of basement layer; auto Ab against epidermal cell surface Pemphigus Vulgaris Q2333:Dx;skin cancer of face and hands w/ keratin "pearls" Squamous cell CA Q2334:Dx;skin cancer in sun-exposed areas of body; pearly papules w/ "palisading" nuclei Basal cell CA Q2335:Dx;skin CA that can metastasize;what characteristic correlates to increased risk of metastasis? Melanoma;;(Inc Depth of tumor = Inc Mets) Q2336:what is common w/ all primary metatastic bone tumors? they are all "Sarcomas" Q2337:Dx;epiphyseal tumor at end of long bone (usu at knee) w/ spindle-shaped cells and "soap bubble" or "double bubble" Giant cell Tumor;;(MC female bone tumor) Q2338:Dx;bone growth covered by a cap of cartilage; usu in men < 25yo Osteochondroma Q2339:Dx;benign neoplasm of intermedullary bone usu in fingers and toes and w/o pain Endochondroma Q2340:Dx;replacement of intermedullary bone w/ fibrous tissue; "ground-glass" appearance on x-ray; "Chinese character" w/o osteoblasts Fibrous Displasia Q2341:Definition;;Giant cell-like bone tumor of metaphysis or vertebrae; pain and swelling; hemorrhagic cysts w/ giant cells Aneurysmal Bone Cyst Q2342:Dx;males < 25yo w/ central "nidus" of osteoid surrounded by a shell; painful (wake from sleep); responsive to aspirin Osteoid Osteoma Q2343:Dx;boys < 15yo w/ tumor of pelvis or shaft of long bones; small round blue cell tumor; PAS+; "Onionskin" look to bone;what translocation? Ewing Sarcoma;[eWINGS and Onion rings];t(11;22) Q2344:Dx;bone tumor w/ osteoid spindle cells; usu the knee; painful and aggressive; Inc Alk Phos; elevation of periosteum ;(Codman's triangle) Osteosarcoma;(MC primary bone malignancy) Q2345:Dx;malignant cartilaginous tumor in the central skeleton; painful and slow-growing; bluish-white appearance w/ sectioning Chondrosarcoma Q2346:Dx;weakness in proximal muscles of extremities; later compensatory hypertrophy of distal site (calf) and eventual pseudohypertrophy; Inc serum CK; death from weak resp muscles;genetics? Duchenne Muscular Dystrophy;(frameshift deletion of Dystrophin) Q2347:Definition;similar to DMD; but less severe; segmental deletions in Dystrophin Becker muscular dystrophy Q2348:Dx;Inability to relax muscles once contracted; weakness; cataracts and testicular atrophy Myotonic Dystrophy;(CTG repeats - Auto Dominant) Q2349:Dx;weakness of extraocular and facial muscles; muscle weakness w/ use and recovery w/ rest; difficult chewing; swallowing; resp failure; more common in women Myasthenia Gravis;(Ab to ACh receptor) Q2350:Definition;paraneoplastic syndrome w/ clinical manifestations similar to those of myasthenia gravis Lambert-Eaton syndrome;(MC w/ Small cell CA of Lung) Q2351:Dx;pigeon breast; string-of-beads in costochondrial junction; depression along line of insertion of diaphragm to rib cage (Harrison groove); High-output Cardiac failure Rickets;(Vit D deficiency) Q2352:Achondroplasia? mutation of fibroblast growth factor receptor 3 Q2353:What happens with the mutation of FGFR3? results in the reduction of the proliferation of chondrocytes on growth plate Q2354:Morphology of Achondroplasia? zones of proliferation and hypertrophy are narrowed and disorganized and contain clusters of large chondrocytes instead of well-formed columns Q2355:Osteogenesis Imperfecta? deficiency in synthesis of type 1 collagen Q2356:deficiency in synthesis of type 1 collagen? incorporation of defective alpha2 chains that cause instability and degradation of the triple helix Q2357:Osteogenesis Imperfecta Type II fatal in utero or during the perinatal period;fetus is still within the womb Q2358:Findings for Osteogeness Imperfecta? extreme skeletal fragility;blue sclerae caused by a decrease in collagen content; exposing the coridal veins;hearing loss related to both a sensorineural deficit and impeded conduction owing to abnormalities in the bones of the middle and inner ear;dental imperfections due to a deficiency of dentin; Q2359:Mucopolysaccharidoses caused by deficiencies in the enzymes Q2360:Deficient enzymes in Mucopolysaccharidoses that degrade dermatan sulfate; heparan sulfate; and keratan sulfate Q2361:Mucopolysaccharidoses abnormalities in hyaline cartilage Q2362:Signs of Mucopolysaccharidoses patients are frequently of short stature and have chest wall abnormalities and malformed bones Q2363:Hunter's syndrome associated with increased tissue stores and excretion of dermatan sulfate and heparan sulfate Q2364:Senile Osteoporosis continuous loss of bone at both the trabecular and cortical layers; which become thinner by internal resorption Q2365:Steroid-induced Osteoporosis catabolic effect of corticosteroids may affect trabecular bone; producing a decrease in bone formation Q2366:Effects of Steroids on Caclium absorption steroids also decrease intestinal absorption and renal resorption of calcium; so that less calcium reaches the bone Q2367:Post-menopausal osteoporosis most common after the fifth decade of life;decreased remodeling and decreased amount of bone deposited by osteoblasts;b/c lack of estrogen Q2368:Immobilization osteoporosis confinement of bed results in a loss of 30% of initial bone volume;lack of exercise seems to be the cause; Q2369:anorexia nervosa and osteoporosis anorexia nervosa leads to osteoporosis in women b/c of loss of menstrual period;reduction in bone mass with normal bone mineralization Q2370:Osteopetrosis Marble bone disease; Albers-Schonberg disease Q2371:Osteopetrosis osteoclast dysfunction;stonelike quality of the bones; however; the bones are abnormally brittle and fracture like a piece of chalk Q2372:Osteopetrosis xray findings Erlenmeyer flask Q2373:Deficiency seen in Osteopetrosis carbonic anhydrase II deficiency Q2374:Morphology of Osteopetrosis osteoclasts lack the usual ruffled borders and show decreased functioning Q2375:Clinical features of Osteopetrosis fractures; anemia; hydrocephaly are often seen; Q2376:Paget Disease (Osteitis Deformans) haphazard arrangement of cement lines Q2377:Paget Disease (Osteitis Deformans) can often lead to bell's palsy if impinging on the CN VII Q2378:Paget Disease (Osteitis Deformans) predilection for the skull; pelvis; tibia; and femur Q2379:Paget Disease (Osteitis Deformans) Lab findings serum alkaline phosphatase level is markedly elevated/increased levels of urinary hydroxyproline; Q2380:X ray findings ofr Pagets bone lysis and reformation are characteristic; Q2381:Pathology of Pagets "mosaic" pattern of osteoclasts;so termed b/c of cement like material forms narrow boundaries Q2382:Cancer associated with Pagets Disease often associated with Osteosarcoma Q2383:Treatment for Pagets administration of calcitonin; which decreases the resorption Q2384:Signs and Symptoms of Pagets bone pain; fractures; and deformities;deafness when skull is affected;height distortion due to vertebral compression Q2385:Osteonecrosis (AvascularNecrosis)Differential Diagnosis 1) mechanical vascular interruption (fracture);2) corticosteroids;3)thrombosis and embolism (nitrogen bubbles in dysbarism)4) vessel injury (vasculitis; radiation therapy)5) increased intraosseous pressure with vascular compression;6) venous hypertension Q2386:Osteonecrosis course chronic pain that is initially associated only with activity but then becomes progressively more constant until finally it is present at rest Q2387:MCC bilateral segmental osteonecrosis or avascular necrosis (AVN) of the femoral head systemic steroid therapy Q2388:Osteomyelitis inflammation of the medullary and cortical portions of bone; including the periosteum Q2389:Osteomyelitis bones affected; child vs adult children; the long bones are most often affected; in adults; the vertebrae Q2390:Osteomyelitis Pathogenesis 1) Most commonly; the hematogenous spread of bacteria from a distant focus of sepsis;2) Invasion of bone from adjacent septic arthritis or soft tissue abscesses;3) Penetrating trauma;4) Complication of fractures;5) Complications of surgery Q2391:Osteomyelitis increased risk pts with sickle cell disease; hemodialysis patients; patients with bone or joint prostheses; and intravenous drug abusers Q2392:Pyogenic Osteomyelitis caused by Staphylococcus aureus is responsible for 80% to 90% of the cases Q2393:What are in utero manifestations of complete/bilateral renal agenesis? How common is this? Oligohydramnios occurs because the renal system is not excreting fluids swallowed by the fetus;This results in the oligohydramnios sequence: multiple fetal abnormalities including hypoplastic lung; defects in extremities; etc. Q2394:What are the clinical manifestations of the nephrotic syndrome? Massive proteinuria: >4 g per day;Hypoalbuminemia: <3 g per deciliter;Generalized Edema;Hyperlipidemia and Hypercholesterolemia: due to increased hepatic lipoprotein synthesis Q2395:What is Minimal Change Disease? Prototypical nephrotic syndrome. Most often in young children but can occur in older individuals;Light microscopy shows normal glomeruli;Electron microscopy is normal except for effacement of epithelial foot processes;Responds well to steroid therapy;Pathogenesis may be related to mutations in nephrin. Q2396:What is focal segmental glomerulosclerosis? Like the name: focal; involving only a subset of glomeruli; segmental; involving only a portion of the glomerular tuft;Symptoms include nephrotic syndrome or non-nephrotic proteinuria. Like minimal change disease; there is a loss of foot processes. There is also sclerosis -- collapse of the basement membranes; increase in matrix; and hyalinosis of the capillary wall with possible obstruction;Responds poorly to steroids. Q2397:What is membranous glomerulonephritis? An immune complex disease of unknown etiology that causes the nephrotic syndrome and azotemia;Light microscopy shows thickened capillary walls due to a five to 10 fold thickening of the basement membrane. Epimembranous (subepithelial) deposits with a spike and dome appearance. Granular pattern on immunofluorescence. Q2398:What is diabetic nephropathy? Dramatic thickening of the entire glomerular basement membrane; seen by electron microscopy;Other changes include an increase in mesangial matrix in both a diffuse and nodular (Kimmelstiel-Wilson nodules) pattern. Q2399:What is renal amyloidosis? Presents with nephrotic syndrome; due to subendothelial and mesangial amyloid deposits;Often caused by chronic inflammatory diseases such as rheumatoid arthritis or plasma cell disorders such as multiple myeloma. Q2400:What are the five patterns of lupus nephropathy? Type 1: no observable renal involvement;Type 2: mesangial cell and matrix proliferation; with slight proteinuria and minimal hematuria;Type 3: focal proliferative -- not all glomeruli involved; but some severely damaged;Type 4: diffuse proliferative -- all glomeruli involved with inflammation; thromboses; mesangial proliferation; and scarring. Also see wire loop abnormalities; endothelial cell proliferation; and subendothelial immune complex deposition;Type 5: membranous form -- same as primary membranous glomerulonephritis Q2401:What are the characteristics of the nephritic syndrome? Oliguria; azotemia; hypertension; hematuria with red cell casts. Patients often report having "smoky brown urine". Q2402:What is poststreptococcal glomerulonephritis? Prototype of the nephritic syndrome. Immune complex disease due to GAS infection. 95% show complete recovery; but a small minority develops rapidly progressive glomerulonephritis;Causes an inflammatory reaction with renal surface petechiae; enlarged; hypercellular glomeruli; normal basement membrane thickness; subepithelial bumps; and "lumpy bumpy" immunofluorescence. Q2403:What is rapidly progressive (crescentic) glomerulonephritis (RPGN)? Nephritic syndrome that progresses to renal failure within weeks or months. Crescents are formed by fibrin deposition in Bowman space and proliferation of epithelial cells;Type I: anti-GBM disease;Type II: poststreptococcal (50%);Type III: pauci-immune; associated with ANCAs Q2404:What is Goodpasture's syndrome? Nephritic syndrome; pneumonitis with hemoptysis; and RPGN crescentic glomerulonephritis due to anti-glomerular basement membrane antibodies;Fluorescent antibody staining demonstrates a linear pattern. Q2405:What is focal glomerulonephritis? Focal and segmental inflammatory changes (contrast to sclerotic changes in focal segmental glomerulosclerosis);Due to immune complexes; often secondary to SLE; subacute bacterial endocarditis; polyarteritis nodosa; Goodpasture's syndrome; Wegener granulomatosis; and IgA nephropathy. Can also be idiopathic. Q2406:What is Alport syndrome? Hereditary nephritis associated with nerve deafness; and ocular disorders (lens dislocation; cataracts);Due to a mutation of the Alpha-5 chain of type IV collagen; often causes end- stage renal disease by 30 years of age;Microscopically; irregular glomerular basement membrane thickening/thinning with foci of splitting of the lamina densa. Q2407:ureter crosses anterior to the origin of what artery to enter the pelvis? external iliac Q2408:ovoid; PAS-positive hyaline masses Kimmelstiel-Wilson nodule - most specific lesion of diabetic glomerulosclerosis Q2409:nephrotic syndrome in IV drug user or HIV nephropathy focal segmental glomerulosclerosis Q2410:renal pathology associated with DIC? diffuse cortical necrosis Q2411:Potter's syndrome? bilateral renal agenesis - oligohydraminos; limb & facial deformities;pulmonary hypoplasia Q2412:cause of Potter's syndrome? malformation of ureteric bud Q2413:why do kidneys stay low in abdomen in horseshoe kidney? get trapped under IMA Q2414:RBC casts in urine glomerular inflammation (nephritic syndromes); ischemia; or malignant hypertension Q2415:WBC casts in urine tubulointerstitial disease; acute pyelonephritis; glomerular disorders Q2416:granular casts in urine acute tubular necrosis Q2417:waxy casts in urine advanced renal disease/CRF Q2418:hyaline casts in urine nonspecific Q2419:LM: glomeruli enlarged and hypercellular; neutrophils; lumpy-bumpy; EM: subepithelial humps; IF: granular pattern acute postreptococcal glomerulonephritis Q2420:LM and IF: crescent moon shape rapidly progressive (crescentic) glomerulonephritis Q2421:finding on immunofluorescence in Goodpasture's? linear pattern; anti-GBM IgG Abs Q2422:IF and EM findings in Berger's disease? mesangial IgA deposits (Berger's also known as IgA nephropathy) Q2423:split basement membrane with nerve deafness and lens dislocation or cataracts Alport's syndrome Q2424:mutation in Alport's? characteristic findings? collagen IV; nerve deafness and ocular disorders Q2425:hematuria; hypertension; oliguria; azotemia nephritic syndrome Q2426:findings in nephrotic syndrome massive proteinuria; hypoalbuminemia; peripheral and periorbital edema; hyperlipidemia Q2427:LM: diffuse capillary and BM thickening; IF: granular pattern; EM: spike and dome membranous glomerulonephritis Q2428:findings in minimal change disease/lipoid nephrosis? LM: normal glomeruli; EM: foot process effacement Q2429:LM: segmental sclerosis and hyalinosis focal segmental glomerular sclerosis - most severe disease in HIV patients Q2430:findings in diabetic nephropathy LM: Kimmelstiel-Wilson lesions; basement membrane thickening; glomeruli appear like golf balls Q2431:findings in membranous glomeruloneprhitis in SLE wire-loop lesion with subepithelial deposits Q2432:most common type of kidney stones? calciium oxalate; calcium phosphate; or both Q2433:second most common type of kidney stone; can form staghorn calculi that can be nidus for UTIs ammonium magnesium phosphate (struvite) Q2434:infection with what type of organism leads to struvite kidney stones? urease-positive bugs - proteus vlugaris; staph; klebsiella Q2435:kidney stones often seen as a result of diseases with increased cell turnover; such as leukemia and myeloproliferative disorders uric acid Q2436:types of radiolucent kidney stones? uric acid and cystine Q2437:gene association with renal cell carcinoma? deletion of VHL gene on chromosome 3 Q2438:where does renal cell carcinoma originate? renal tubule cells (polygonal clear cells) Q2439:renal cell carcinoma is associated with what syndromes? paraneoplastic (ectopic EPO; ACTH; PTHrP; and prolactin) Q2440:gene association with Wilm's tumor? deletion of tumor suppressor WT1 on chromosome 11 Q2441:WAGR complex? Wilm's tumor; Aniridia; genitourinary malformation; and mental-motor retardation Q2442:most common tumor of the urinary tract system? transitional cell carcinoma Q2443:transitional cell carcinoma is associated with what? problems in your Pee SAC;phenacetin; smoking; analine dyes; and cyclophosphamide Q2444:white cell casts in urine are pathognomonic for what? acute pyelonephritis Q2445:chronic pyelonephritis clinical manifestations? coarse; asymmetric corticomedullary scarring and blunted calyces; tubules can contain eosinophilic casts (thyroidization of the kidney) Q2446:most common cause of acute renal failure? acute tubular necrosis Q2447:what is acute tubular necrosis associated with? renal ischemia (e.g. shock); crush injury (myoglobinuria); toxins Q2448:when does death most often occur in ATN? during initial oliguric stage Q2449:what is renal papillary necrosis associated with? diabetes; acute pyelonephritis; chronic phenacitin use; sickle cell anemia Q2450:what is uremia? clinical syndrome marked by increased BUN and creatinine and associated symptoms Q2451:metabolic consequences seen in renal failure? 1. Anemia (decreased EPO);2. Renal osteodystrophy (failure of active vitamin D production);3. Hyperkalemia;4. metabolic acidosis due to decreased acid secretion and decreased HCO3- generation;5. Uremic encephalopathy;6. Sodium and H2O excess --> CHF and pulmonary edema;7. Chronic pyelonephritis;8. Hypertension Q2452:low serum chloride concentration is caused by what? is secondary to metabolic alkalosis; hypokalemia; hypovolemia; increased aldosterone Q2453:high serum chloride concentration is secondary to what? non-anion gap acidosis Q2454:most common cause of nephrotic syndrome in adults? membranous glomerulonephritis Q2455:Obese male aged 50-70 years; smoker; with hematuria and palpable mass; fever; weight loss. what is diagnosis? Renal cell carcinoma Q2456:loop diuretic indicated for the treatment of edema associated with CHF; cirrhosis; and renal disease? furosemide (also HTN and hypercalcemia) Q2457:two principal causes of rapidly progressive glomerulonephritis? anti-glomerular basement membrane and primary systemic vasculitis Q2458:drugs implicated in the pathogenesis of acute interstitial nephritis? NSAIDs; beta-lactam antibiotics (penicillins and cephalosporins); sulfonamides; diuretics (furosemide and thiazides); phenytoin; cimetidine; methyldopa Q2459:EM: subendothelial humps; mesangial proliferation (splits BM) membranoproliferative glomerulonephritis Q2460:What does the presence of casts in the urine indicate? hematuria/pyuria is of renal origin Q2461:What do RBCs in urine with no casts indicate? Bladder cancer Q2462:What does WBCs in urine with no casts inddicate? Acute cystitis Q2463:This nephritic syndrome is most frequently seen in children and presents with peripheral and periorbital edema Acute poststreptococcal glomerulonephritis. resolves spontaneously Q2464:Outcome of Membranoproliferative glomerulonephritis? slowly progresses to renal failure Q2465:outcome of Rapidly progressive glomerulonephritis? rapid course to renal failure. number of crescents indicates prognosis Q2466:this is a common cause of recurrent hematuria in young patients IgA nephropathy (Berger's disease) Q2467:Immunofluorescent congo red stain of glomeruli show apple-green birefringence. What is the diagnosis? Amyloidosis Q2468:Amyloidosis is associated with what conditions? multiple myeloma; chronic conditions; TB; rheumatoid arithritis Q2469:Which conditions may lead to hypercalciuria and stones? hypercalcemic conditions: cancer; increased PTH; increased vitamin D; milk-alkali syndrome Q2470:child aged 2-4 presents with huge; palpable flank mass; hemihypertrophy. The mass contains embryonic glomerular structures. What is the tumor? Wilms' tumor Q2471:What causes diffuse cortical necrosis? likely due to a combination of vasospasm and DIC. Associated with obstetric catastrophes and sepsis Q2472:acute interstitial renal inflammation with fever; rash; eosinophilia and hematuria beginning 2 weeks after taking what drugs? Drug-induced interstitial nephritis; penicillins; NSAIDS; diuretics Q2473:Patient presents with acute renal failure. labs show Urine osmolality >500; Urine Na <10; Fe(Na) <1% and BUN/Cr >20. Where is the problem Prerenal Q2474:Patient presents with acute renal failure. labs show Urine osmolality <350; Urine Na >20; Fe(Na) >2% and BUN/Cr <15. Where is the problem Renal Q2475:Patient presents with acute renal failure. labs show Urine osmolality <350; Urine Na >40%; BUN/Cr >15. What is the cause? post-renal; generally outflow obstruction due to stones; BPH or neoplasia Q2476:What is Fanconi's syndrome? What are its complications? Defect in proximal tubule transport. Complications include rickets; osteomalacia; hypokalemia; metabolic acidosis Q2477:patient presents with flank pain; hematuria; hypertension; urinary infection and progressive renal failure. US shows multiple; large; bilateral renal cysts. What is the underlying cause? APKD from AD mutation in APKD1 gene Q2478:low serum Na causes what? disorientation; stupor; coma Q2479:high serum Na causes what? neurologic: irritability; delirium; coma Q2480:low serum K causes what? U waves on ECG; flattened T waves; arrhythmias; paralysis Q2481:high serum K causes what? peaked T waves; wide QRS; arrhythmias Q2482:low serum Ca causes what? tetany; neuromuscular irritability Q2483:high serum Ca causes what? delirium; renal stones; abdominal pain; not necessarily calciuria Q2484:low serum Mg causes what? neuromuscular irritability; arrhythmias Q2485:high serum Mg causes what? delirium; decreased DTRs; cardiopulmonary arrest Q2486:low serum PO4 causes what? low-mineral ion product causes bone loss; osteomalacia Q2487:high serum PO4 causes what? high-mineral ion product causes metastatic calcification; renal stones Q2488:mechanism of mannitol? osmotic diuretic; increased tubular fluid osmolarity; producing increased urine flow. works in PCT Q2489:mannitol contraindications? anuria; CHF Q2490:mechanism of acetazolamide? Carbonic anhydrase inhibitor. Acts in PCT. depletion of HCO3 Q2491:major toxicity of acetazolamide? hyperchloremic metabolic acidosis Q2492:mechanism of furosemide? loop diuretic. inhibits Na/K/Cl cotransport in thick ascending limb. reduces hypertonicity of medulla preventing concentration of urine in the collecting tubule. also promotes loss of Ca due to decreased electrochemical gradient Q2493:major toxicity of furosemide? OH DANG!;Ototoxicity; Hypokalemia; Dehydration; Allergy (sulfa); Nephritis (interstitial); Gout Q2494:mechanism of ethacrynic acid loop diuretic blocks Na/K/Cl cotransporter; reduces ability to concentrate urine Q2495:major toxicity of ethacrynic acid gout Q2496:mechanism of hydrochlorothiazide thiazide diuretic. inhibits NaCl reabsorption in early distal tubule; reducing diluting capacity of nephron. decreases Ca excretion Q2497:toxicities of hydrochlorothiazide hypokalemic metabolic acidosis; hypercalcemia; sulfa allergy Q2498:What are the K sparing diuretics? Spironolactone; Triamterene; Amiloride; eplerenone Q2499:Mechanism of spironolactone? competitive aldosterone receptor antagonist in cortical collecting tubule Q2500:mechanism of Triamterene and amiloride? block Na channels in CCT Q2501:toxicity of K-sparing diuretics? hyperkalemia; antiandrogenic effects Q2502:mechanism of ACE inhibitors? inhibit ACE; reducing angiotensin II and preventing inactivation of bradykinin. Results in increased renin release Q2503:major toxicities of ACE inhibitors? cough; increased renin; hyperkalemia Q2504:What are the ACE inhibitors? Captopril; Enalapril; Lisinopril Q2505:mechanism of Losartan angiotensin II receptor inhibitor (does not produce cough) Q2506:What are podocytes? visceral epithelial cells Q2507:What are the spaces between the podocytes called? split pores Q2508:Which cells synthesis the glomerular BM? visceral epithelial cells (podocytes) Q2509:What keeps albumin out of urine? strong negative charge of Glomerular BM Q2510:What is responsible for charge of GBM? Heparan Sulfate (strong negative charge) Q2511:damage to visceral epithelial cell results in what? damage to BM and leaking of albumin into urine --> nephrotic syndrome Q2512:linear pattern outlining BM on Immunofluorescence goodpasture syndrome Q2513:subendothelial immune complex deposits in glomeruli on EM (granular) lupus Q2514:subepithelial immune complex deposits in glomeruli EM (granular) post-strep glomerulonephritis Q2515:only glomerulonephritis one can diagnose with IF IgA glomerulonephritis Q2516:granular pattern on IF. what does it mean? immunocomplex type III disease Q2517:anti BM antibodies is what type of immune complex disease? Type II Q2518:RBC casts in urine is unique to what class of diseases nephritic syndromes Q2519:serum ANA shows rim pattern. what does that mean? anti-DNA --> lupus Q2520:crescentic glomerulonephritis is most commonly seen in what syndrome? goodpasture's syndrome Q2521:cholesterol casts in urine that when polarized look like maltese crosses. what is the diagnosis? nephrotic syndrome Q2522:why does lipoid nephrosis occur (Minimal change disease)? loss of negative charge of GBM Q2523:Nephrotic syndrome associated with HIV FSGS Q2524:glomerular problem in HBV diffuse membraneous glomerulonephritis Q2525:glomerular problem in HCV Membranoproliferative glomerulonephritis Q2526:vasculitis associated with HBV polyarteritis nodosa Q2527:large golf-ball appearing glomeruli on H&E diabetic nephropathy Q2528:what happens to the GFR and creatinine clearance in early diabetic nephropathy? hyalinization of efferent arterioles; so Cr clearance and GFR increase. Also nonenzymatic glycosylation of BM cause microalbuminuria Q2529:ACE inhibitors do what to glomerular arterioles? less angiotensin II dilates efferent arteriole Q2530:mesangial cells split BM on EM. C3 deposited adjacent to but not within dense deposits. serum C3 is very low. what is the diagnosis? Type II membranoproliferative glomerulonephritis Q2531:properties of BUN blood urea nitrogen - secreted and reabsorbed in PCT Q2532:properties of Creatinine end-product of creatine - only filtered in kidney; neither reabsorbed nor secreted in kidney (can be in other places in very high levels) Q2533:normal BUN and Cr levels BUN - 9-10;Cr - 1 mg/dl Q2534:normal BUN/Cr 10 Q2535:pre-renal azotemia normal kidneys; but reduced Cardiac Output (e.g. CHF); ergo; GFR decreases. BUN/Cr >15 Q2536:renal failure (oliguria; renal tubular casts) affects BUN and Cr equally (increased BUN and Cr in equal proportion) BUN/Cr normal (10/1) Q2537:most common cause of acute renal failure ischemic acute tubular necrosis Q2538:Cardiac output decreases and oliguria; what do you worry about most? ischemic acute tubular necrosis Q2539:most common cause of ischemic acute tubular necrosis not treating pre-renal azotemia Q2540:BUN:Cr ~ 10:1 with oliguria and renal tubular casts acute tubular necrosis Q2541:Why does acute tubular necrosis have such a bad diagnosis? ischemic cause also destroys basement membrane --> loss of structure --> can't regenerate renal tubular cell w/o BM. Even if one recovers; can never recover normal function Q2542:What parts of nephron is most susceptible to ischemia? straight portion of proximal tubule and thick ascending limb (medullary part). Affects Na/K/Cl co-transport Q2543:Nephrotoxic drugs;what are they and where do they affect? prognosis? gentamicin (aminoglycocides); dye from IV pyelograms; damages proximal tubule. prognosis good because they don't damage BM. Q2544:How do you separate pyelonephritis from low UTIs? acute pyelonephritis is infection of kidney proper and has fever with flank pain (CVA tenderness) and WBC casts Q2545:what is the mechanism of all UTIs ascending infection from introitus of urethra. Q2546:scarred kidney with blunted calyces (beneath scarring) chronic pyelonephritis Q2547:fever with rash; oliguria; eosinophiluria after starting drug acute drug-induced interstitial nephritis (methicillin) Q2548:what kinds of hypersensitivity are associated with acute drug-induced interstitial nephritis? combination of type I and type IV hypersensitivities Q2549:empty space on IV pyelogram analgesic nephropathy from acetaminophen and aspirin combo therapy long-term Q2550:what is the mechanism of analgesic nephropathy? acetaminophen --> produces free radicals that damage tubular cells of medulla;aspirin blocks PGE2 so Angiotensin II is unopposed and peritubular capillaries have decreased blood flow causing ischemia in renal papillaries Q2551:causes of renal papillary necrosis aspirin/acetaminophen long-term; diabetes; sickle-cell disease; acute pyelonephritis (from abscess formation) Q2552:BUN/Cr >10 for more than 3 months. what is the diagnosis? Chronic renal failure Q2553:results of chronic renal failure anemia; anion-gap metabolic acidosis; osteoporosis; osteomalacia; secondary hyperparathyroidism Q2554:uncontrolled essential hypertension (over ten years) causes what? nephrosclerosis --> hyaline arteriolosclerosis (cobblestone appearance of kidney) Q2555:person with uncontrolled HTN; wakes up with bad HA; dizzy; blurred vision; BP 240/140; papilledema and flamed hemorrhages; hard and soft exudates; BUN/Cr 80/8. what is diagnosis? malignant hypertension (shows petechia visible on surface of kidney) Q2556:Treatment of malignant hypertension? IV nitroprusside Q2557:pale; depressed-looking lesions on gross examination of kidney. what do you see on LM? pale infarction --> coagulation necrosis Q2558:causes of pale infarcts in kidneys in a patient with irregular irregular pulse? A-fib; causes thromboemboli Q2559:little white dots and microabscesses on gross exam of kidneys probably caused by what? pyelonephritis Q2560:hydronephrosis and increased pressure have what affect on renal cortex and medulla? compression atrophy Q2561:staghorn calculi; alkaline urine and smells of ammonia; what is the cause? urease (+) bacteria: proteus; klebsiella; staph Q2562:most common cause of compression atrophy? stone Q2563:what is the composition of staghorn calculi magnesium ammonium phosphate Q2564:mass in kidney adult what is it? renal adenocarcinoma Q2565:mass in kidney in kid with hypertension? wilm's tumor Q2566:cause of renal adenocarcinoma? derived from proximal tubule; most common cause is smoking Q2567:what are results of renal adenocarcinoma? produce ectopic EPO; PTH-like peptide; and like to invade renal vein Q2568:why hypertension in kid with Wilm's tumor? the tumor makes renin Q2569:findings in Wilm's tumor? embryonic kidney structures; aniridia and hemi-hypertrophy of an extremity --> sign that Wilm's tumor has genetic basis Q2570:genetic abnormality in Wilm's tumor? WT-1 tumor-suppressor gene on chrom 11. AD Q2571:most common organism in cystitis? E. coli Q2572:patient with increased urinary frequency; has positive leukocyte esterase; dysuria; neutrophils in urine; negative bacterial culture; negative nitrites. what is diagnosis? Chlamydia Q2573:causes of sterile pyuria? chlamydia; TB Q2574:most common cause of transitional cell carcinoma of bladder? smoking Q2575:cyclophosphamide is used to treat what? Wegener's Q2576:cyclophosphamide toxicity is prevented by what? Mesna Q2577:hypospadias is caused by what? failure of closure of urethral folds Q2578:most common cancer of penis is what? What is most common cause? squamous cell carcinoma due to lack of hygiene in uncircumcised penis. (shmegma) Q2579:what are two phases of testicular descent? tans-abdominal migration caused by mullerian inhibitory factor; and shortening of gubernaculum due to testosterone and dihydrotestosterone Q2580:undescended testicles before 2 years increased risk for what? seminomas in both testicles (even if appears normal) Q2581:streak ovaries put woman at risk for what? dysgerminomas Q2582:epididymitis at age <35 due to what? neisseria gonorrhea; chlamydia Q2583:epididymitis at ages >35 due to what? pseudomonas; E. coli Q2584:vericocoeles on what side and why? left; due to the spermatic vein on left is connected to left renal vein. Q2585:most common cause of male infertility vericocoele Q2586:what would happen if you block left renal vein? increases pressure on spermatic vein and causes vericocoele. Q2587:torsion of spermatic cord causes what? shortens the cord; so the testicle ascends into inguinal canal; pain; loss of cremasteric reflex Q2588:what is cremasteric reflex? scratching of scrotum causes the cremaster muscle to contract Q2589:hydrocoele is what? persistence of tunica vaginalis Q2590:painless enlargement of testicle has what on it's differential? cancer; cancer; cancer; cancer Q2591:most common cause of testicular cancer seminoma --> highly responsive to radiation Q2592:where do seminomas metastacize? para-aortic LNs Q2593:most common testicular tumor in kid? what's the tumor marker? yolk sac tumor (Alpha fetoprotein) Q2594:25 y/o male presents with unilateral gynecomastia and dyspnea. X-ray of lung shows numerous nodular masses. Where is the primary tumor and what kind? choriocarcinoma of testicle Q2595:why do choriocarcinomas develop gynecomastia? B-HCG is a leutinizing hormone analog --> acts like leutenizing hormone; so stimulates progesterone and causes duct growth in breast tissue Q2596:most common cause of testicular cancer in older men? malignant lymphoma metastaces Q2597:where in prostate gland does hyperplasia occur? periurethral area Q2598:where in prostate gland is cancer located? periphery (that's why you can feel it with your finger on rectal) Q2599:75 y/o man has urinary retention and massive bladder with dribbling urine. what is cause? BPH. Q2600:what hormone is totally responsible for prostate? dihydrotestosterone Q2601:BPH and prostate cancer is under the control of what hormone? dihydrotestosterone Q2602:how do you treat prostate cancer and BPH 5-alpha-reductase inhibitor Q2603:most common cancer in men? prostate cancer Q2604:What is prerenal azotemia? selective rise in BUN during early renal failure Q2605:Define segmental. (kidney pathology) involving part of the glomerular tuft Q2606:hematuria; hypertension; oliguria; azotemia = ? Nephritic Syndrome; "I" = inflammation Q2607:LM findings in post-strep glomerulonephritis? lumpy bumpy and hypercellular w/ neutrophils Q2608:EM findings in post-strep glomerulonephritis? subepithelial humps (from deposition of cationic antigen) Q2609:IF findings in post-strep glomerulonephritis? granular Q2610:Goodpasture's is what type of hypersensistivity? type II (IgG binding directly to BM) Q2611:Goodpasture's IF? linear Q2612:Symptoms of Goodpasture's? hemoptysis; hematuria Q2613:Membranoproliferative glomerulonephritis EM? subendothelial humps; "tram track" Q2614:Course of crescentic glomerulonephritis? rapid (the Red Crescent is the Islamic Red Cross; an organization that provides relief during emergencies; like crescentic glomerulonephritis) Q2615:Course of Membranoproliferative glomerulonephritis? slow Q2616:IgA nephropathy IF and EM? mesangial deposits of IgA (Mes"A"ngium) Q2617:Which can be post infectious; IgA nephropathy or Goodpasture's? IgA nephropathy Q2618:Signs of nephrotic syndrome? massive proteinuria; hypoalbuminemia; generalized edema; hyperlipidemia (think "erotic" = an erection(edema) and ejaculate(massive proteinuria) Q2619:LM of membranous glomerulonephritis? diffuse capillary and basement membrane thickening Q2620:IF of membranous glomerulonephritis? granular Q2621:EM of membranous glomerulonephritis? spike and dome ("if you have a spike in your dome; you must be insane in the "membran"e;" -Chirag) Q2622:Minimal change disease EM? foot process effacement Q2623:Most common cause of childhood nephrotic syndrome? minimal change disease Q2624:Focal segmental glomerular sclerosis LM? segmental sclerosis and hyalinosis Q2625:Diabetic nephropathy LM? Kimmelstiel-Wilson lesions Q2626:SLE (5 patterns of renal involvement) LM? wire-loop appearance w/ extensive granular BM depsits in membranous glomerulonephritis pattern Q2627:What is the most common kidney stone? Calcium (with either oxalate of phosphate) Q2628:What kidney stone is associated with a bacterial infection? Ammonium magnesium phosphate (struivte) stones are associated with urase positive bugs that make ammonium Q2629:What are the two most common stone-forming bacteria? proteus vulgaris and Staph. Q2630:What stones are associated with gout? Uric acid stones Q2631:What else can cause uric acid stones? Any disease with high cell turnover (remember that uric acid is a by-product of DNA formation); so leukemia and myeloproliferative disease Q2632:What stones are associated with cystineuria? cystine stones Q2633:What two stones are radiolucent? Uric acid and cystine (the two purely metabolic causes) Q2634:What are the complications of stones? Hydronephrosis and pyelonephritis Q2635:What causes calcium stones? Anything that increases calcium in the blood; so high PTH; malignancy (bone breakdown; PTH production); vitamin D overdose. Q2636:Renal Cell Carcinoma: Is this a common renal cancer? Yes; the most common Q2637:Renal Cell Carcinoma: Who is prone to this? males; ages 50-70; smokers; gene deletions on chromosome 3 Q2638:Renal Cell Carcinoma: This is a cancer of what cell type? renal tubule cells; histologically they appear clear. Q2639:Renal Cell Carcinoma: What are the clinical signs of this cancer? flank pain; fever; hematuria; palpable mass; secondary polycythemia Q2640:Renal Cell Carcinoma: How does it spread? It invades the renal veins and IVC; to spread hematogenously Q2641:Renal Cell Carcinoma: What hormones can it produce? ACTH; prolactin; Parathyroid-like hormone; gonadotropins and renin. Q2642:Wilm's Tumor: Who gets this cancer? Children 2-4 years Q2643:Wilm's Tumor: Is it common? The most common renal cancer in children Q2644:Wilm's Tumor: What is the chromosomal abnormality that leads to this? deletion of tumor suppressor gene WT1 on chromosome 11 Q2645:Wilm's Tumor: What tetrad is it associated with? WAGR - Wilm's; Anirida (lack of an iris); Genitourinary malformation; and Retardation Q2646:Wilm's Tumor: How does it present? huge palpable mass and hemihypertrophy Q2647:Wilm's Tumor: What is the histological appearance? mixed - with stromal; mesenchymal; tubular; glomerular and fibrous elements Q2648:Transitional Cell Carcinoma: Where does this cancer occur? Urinary spaces (tract; calyces; pelvis; bladder) Q2649:Transitional Cell Carcinoma: Does it recur? yes; often Q2650:Transitional Cell Carcinoma: How does it spread? Local invasion Q2651:Transitional Cell Carcinoma: What can predispose you to it? Pee SAC toxins - Phenacetin; Smoking; Aniline dye (benezenes); and Cyclophsophimide. Q2652:Transitional Cell Carcinoma: How does it present? Hematuria Q2653:Dx;Bilateral renal Agenesis; limb deformities; facial deformities; pulmonary hypoplasia; oligohydraminos Potter's syndrome;[Potters can't Pee in utero] Q2654:where does a horseshoe kidney fuse?;what does it get trapped under? Fuse: Inferior poles;trapped under;Inferior Mesenteric Artery Q2655:Cast in urine;RBC cast;(3) Nephritic syndromes;;Ischemia;;Malignant HTN Q2656:Cast in urine;WBC casts Acute Pyelonephritis Q2657:Cast in urine;Granular casts Acute Tubular Necrosis Q2658:Cast in urine;Waxy cast Chronic Renal Failure Q2659:what is seen in urine w/ acute cystitis?;Bladder CA? Cystitis = WBCs;;Bladder CA = RBCs Q2660:Definition;Hypoalbuminemia; Edema; Hyperlipidemia; massive Proteinuria Nephrotic syndrome Q2661:(6) Nephrotic syndromes Membranous Glomerulonephritis;Minimal change;Focal Segmental Glomerular sclerosis;Diabetic Nephropathy;Renal amyloidosis;SLE nephropathy Q2662:Definition;Oliguria; Azotemia; HTN; hematuria; RBC casts Nephritic syndromes Q2663:Nephritic syndrome;"lumpy-bumpy" LM; subepithelial humps; granular pattern Acute Poststrep Glomerulonephritis Q2664:Nephrotic syndrome;granular pattern w/ "spike and dome" on EM Membranous GN Q2665:Nephritic syndrome commonly seen in children w/ pre-orbital and peripheral edema Acute Poststrep GN Q2666:MC type of kidney stone Calcium Q2667:Radiopaque Kidney stone caused by infection and cause staghorn calculi;(3) bugs causing stones Ammonium Magnesium Phosphate;(Struvite stones);Bugs;Proteus;Staph;Klebsiella Q2668:Kidney stone seen w/ leukemia and myeloproliferative disorders Uric acid stone Q2669:Dx;nephritis; nerve deafness; lens dislocation or cataracts Alport syndrome Q2670:Nephritic syndrome;splitting of the lamina densa Alport syndrome Q2671:(3) causes of renal Papillary Necrosis Diabetes;Acute Pyelonephritis;Chronic Phenacetin/NSAID use Q2672:when is the most critical time for Acute Tubular necrosis?;why? Initial Oliguric phase;can cause cardiac arrest from HyperK Q2673:(3) causes of Acute Tubular Necrosis Renal Ischemia;Crush injury;Toxins Q2674:Dx;PCT reabsorption defect leading to protein and glucose in the urine w/ systemic acidosis Falconi syndrome Q2675:Definition;Impaired tubular reabsorption of Tryptophan leading to Pellagra-like symptoms Hartnup Dz Q2676:Dx;coarse and asymmetric corticomedullary scarring of kidney; interstitial fibrosis; tubular atrophy; thyroidization; eosinophilic casts Chronic Pyelonephritis Q2677:(3) tests to look for to determine patient has Pre-Renal ARF;(and results) Urine Osmolality;(>500);Urine Na;(<10);FeNa;(<1%) Q2678:What test is good to Dx Intrarenal ARF? BUN/creatinine ratio < 15 Q2679:(2) tests to help Dx Post-renal ARF Urine Na;(>40);FeNa;(>4) Q2680:Dx;flank pain; palpable mass; hematuria Renal cell CA Q2681:what cell has cancer in Renal cell CA?;what ectopic hormones can be produced?;(4) Polygonal Clear Cell;hormones;EPO; ACTH; PTH; Prolactin Q2682:(4)* causes of Transitional cell CA of bladder Pee SAC;Phenacetin;;Smoking;;Anline dyes;;Cyclophosphamide Q2683:Dx;child with Aniridia; GU malformation; Retardation; flank mass Wilms Tumor - MC kidney tumor of kids;(WT1 on chrom 11);WAGR complex Q2684:What is the primary disturbance in Metabolic acidosis? a decrease in bicarbonate Q2685:What is the compensation? A drop in CO2 by hyperventilation Q2686:What are common causes? diabetic ketoacidosis (production of ketone acids); diarrhea (loss of GI bicarb); salisylate overdose; acetazoleamide (diuretic) OD; lactic acidosis; renal failure (can't excrete organic acids); ethylene glycol ingestion Q2687:What is the primary disturbance in respiratory acidosis? A build-up in CO2 Q2688:What is the compensation? Increased bicarb reabosrobtion from the kidney Q2689:What are some common causes? COPD; airway obstruction; opiates and sedatives; guillan-barr or ALS; Q2690:What is the primary disturbance in metabolic alkalosis? increased bicarbonate Q2691:What is the compensation? Increased CO2 by decreased respiration Q2692:What are some common causes? Vomiting; hyperaldosteronism (increased H+ secretion); loop or thiazide diuretics (volume contraction) Q2693:What is the primary disturbance in respiratory alkalosis? A drop in CO2 Q2694:What is the compensation? increased excretion of bicarb by the kidney Q2695:What are some common causes? hyperventilation; high altitude; pneumonia and pulmonary embolus (hypoxemia causes hyperventilation_ Q2696:What is the Henderson Haselbach equation? pH = pKa + Log (HCO3-)/(.03*pCO2) Q2697:Is there an awesome graph on page 277 that you should know? Yes Q2698:Low pH; low pCO2 (low HCO3-) metabolic acidosis Q2699:Low pH; high pCO2 (high HCO3-) chronic respiratory acidosis Q2700:High pH; low pC02 (low HC03-) acute respiratory alkalosis Q2701:High pH; high pCO2 (high HCO3-) metabolic alkalosis Q2702:pH < 7.4 acidosis Q2703:pH > 7.4 alkalosis Q2704:Within acidosis: P (CO2) > 40 Respiratory acidosis Q2705:Within acidosis: Hypoventilation or Hyperventilation HYPO Q2706:Within acidosis: Causes chronic lung disease; Acute lung disesae; Drugs (opioids; narcotics; sedatives); Weakening of Resp. muscles Q2707:Within acidosis: P (CO2) < 40 Metabolic acidosis with compensation Q2708:Within acidosis: What should you do next? Check Anion Gap Q2709:Within acidosis: What is the normal anion gap? 8-12 mEq/L Q2710:Within acidosis: Increased Anion Gap - 4 Causes Renal Failure; Lactic acidosis; Ketoacidosis; Aspirin ingestion Q2711:Within acidosis: Normal anion gap (4) Diarrhea; Sniffin' Glue; Renal Tubular acidosis; Hyperchloremia Q2712:pH > 7.4 pH > 7.4 alkalosis Q2713:pH > 7.4 pCO2 < 40 Respiratory alkalosis Q2714:pH > 7.4 Causes (2) HYPERventilation; aspirin ingestion (early) Q2715:pH > 7.4 pCO2 > 40 metabolic alkalosis w/ compensation Q2716:pH > 7.4 Causes (4) vomiting; diuretic use; antacid use; hyperALDOsteronism Q2717:Anion gap acidosis: How do you calculate it Na - Cl - HCO3 Q2718:Anion gap acidosis: What is normal? 8-12 mEq/L Q2719:Anion gap acidosis: What is the mnemonic MUD PILES Q2720:Anion gap acidosis: M Methanol Q2721:Anion gap acidosis: U Uremia Q2722:Anion gap acidosis: D DKA Q2723:Anion gap acidosis: P Paraldehyde or Phenformin Q2724:Anion gap acidosis: I Iron tablets or INH Q2725:Anion gap acidosis: L Lactic Acidosis Q2726:Anion gap acidosis: E Ethanol; Ethylene Glycol Q2727:Anion gap acidosis: S Salicylates Q2728:Acid Base Compensations: Metabolic acidosis pCO2 = 1.5(HCO3) + 8 +/- 2 Q2729:Acid Base Compensations: Metabolic alkalosis pCO2 increases 0.7 mm Hg per 1 mEq/L HCO3 increase Q2730:Acid Base Compensations: Respiratory acidosis (acute) HCO3 increases by 1 mEq/L for every 10 mmHg increase of pCO2 Q2731:Acid Base Compensations: Respiratory acidosis (chronic) HCO3 increases by 3.5 mEq/L for every 10 mmHg increase of pCO2 Q2732:Acid Base Compensations: Respiratory alkalosis (acute) HCO3 decreases by 2 mEq/L for every 10 mmHg decrease of pCO2 Q2733:Acid Base Compensations: Respiratory alkalosis (chronic) HCO3 decreases by 5 mEq/L for every 10 mmHg decrease of pCO2 Q2734:The deep inguinal ring lies lateral or medial to the inferior epigastric artery? Lateral Q2735:What is unusual about Chlamydia's cell wall? Lacks muramic acid Q2736:Symptoms of congenital CMV infection? Hepatosplenomegaly; periventricular brain calcification; petechial hemorrhages; and hydrops;Sensorineural deafness Q2737:Induction of hemolytic anemia in G6PD deficient patients are most often caused by which medications? (make a mnemonic) Sulfonamides; nitrofurantoin; dapsone; primaquine; and quinine Q2738:What is unusual about Chlamydia's cell wall? Lacks muramic acid Q2739:What is unusual about Chlamydia's cell wall? Lacks muramic acid Q2740:Induction of hemolytic anemia in G6PD deficient patients are most often caused by which medications? (make a mnemonic) Sulfonamides; nitrofurantoin; dapsone; primaquine; and quinine Q2741:Intranuclear inclusion bodies surrounded by halos? CMV - "owl eyes" Q2742:Symptoms of congenital CMV infection? Hepatosplenomegaly; periventricular brain calcification; petechial hemorrhages; and hydrops;Sensorineural deafness Q2743:What is located about 1/2 inch above the midpoint of the inguinal ligament? Deep inguinal ring Q2744:Toxoplasma gondii is capable of crossing the placenta in which stage of it's lifecycle? Tachyzoite Q2745:What are important causes of post-gonococcal urethritis? Chlamydia; mycoplasma; and ureaplasma Q2746:Frothy yellow vaginal discharge Trichomonas Q2747:Incomplete fusion of the processus vaginalis in males leads to? Hydrocele of the spermatic cord Q2748:Pleomorphic gram-negative rods in parallel short chains causing painful ulcer on penis? Haemophilus ducreyi Q2749:This space contains the sphincter urethrae muscle and the bulbourethral gland in the male? Deep perineal space Q2750:Granulomatosis infantiseptica? Lysteria monocytogenes in pregnancy Q2751:Which STD organisms induce endocytosis by epithelial cells? Neisseria gonorrhoeae and Chlamydia Q2752:The vagina of prepubertal girls and postmenopausal women is colonized by? Colonic and skin bacteria; including staph epidermidis Q2753:Herpes simplex virus 2 lays dormant in which cells? Neurons of the sacral ganglia Q2754:The filamentous gram-positive rod produces yellow granular deposits (sulfur granules) in the areas of its tissue invasion? Actinomyces israelli Q2755:Testes determining factor does what? Causes the indifferent gonad to develop into a testis with Sertoli cells; which secrete MIF; suppressing the paramesonephric ducts; thus preventing the formation of femal internal reproductive organs Q2756:Males with hyperprolactinemia have what affect on sexual function? interference with testosterone production causing a decrease in sperm count Q2757:Hydatiform mole progresses to choriocarcinoma at a rate of? 2% Q2758:What is adenomyosis? Presence of endometrial glands within the myometrkjm of the uterus in addition to their normal location in the endometrium Q2759:Most spontaneously aborted fetuses in the first trimester are due to? chromosomal abnormalities Q2760:Ovarian metastasis of a mucin-producing adenocarcinoma? Krukenberg tumor Q2761:Hydatiform moles produce high levels of? hCG Q2762:Which type of breast cancer has the best progrnosis? Tubular carcinoma Q2763:XY male born with feminized external genitalia; testes retained within the abdominal cavity and otherwise normal male internal reproductive tracts;what could cause this? 5 alpha reductase deficiency Q2764:How to diagnose chronic abacterial prostatitis? >10 leukocytes per high-power field;no bacteria are isolated from cultures of prostatic secretions Q2765:Flutamide? Mechanism? Chemo;Antiandrogen; inhibits the actions of androgens in target tissues. Used in prostate cancer Q2766:Ovarian tumor - Cystic sac containing thick mucinous fluid within a thin wall;Epithelial lining shows malignant features in the absence of stromal invasion? Borderline mucinous tumor Q2767:Presence of individual adenocarcinoma cells within the squamous epithelium of the skin near the nipple? Paget disease Q2768:Uterine tumor - Well demarcated borders and a whorled pattern on cut surface Leiomyoma Q2769:Classical presentation of a germ cell tumor in a 23 year old man? Non-painful swelling of the testis Q2770:T or F? Condyloma acuminatum contains koilocytes? T Q2771:Most common cause of acute bacterial prostatitis? Escherichia Q2772:This drug acts by inhibiting 5 alpha reductase to treat BPH Finasteride Q2773:T or F? Maturation of germ cells within the seminiferous tubules occurs in a concentric pattern with the less mature spermatogonia near the tubule center and the mature forms near the basal lamina FALSE - the opposite Q2774:Mature teratoma;aka? Dermoid cyst Q2775:Most common causes of maternal death in preeclampsia? Cerebral hemorrhage and ARDS Q2776:Sclerosing adenosis? A type of proliferative fibrocystic disease that is often seen with other variants of fibrocystic disease. Proliferation of small ducts; distorts glands and lobules into a whorled pattern. Increased risk for breast cancer Q2777:Type of endometrium bearing long; narrow; coiled glands lined by a single layer of columnar epithelium showing regular; uniform; small nuclei and clear apical vesicles Secretory endometrium Q2778:Tumor with endodermal sinuses that resemble primitive glomeruli? Yolk sac tumor Q2779:Differentiation of the male external genitals is dependent on? Dihydrotestosterone (via action of 5 alpha reductase on testosterone) Q2780:Key differences between seminomas in men and dysgerminomas in women? 1. Seminomas are most common in the fourth decade; dysgerminomas in the third decade;2. Seminomas are relatively common; while dysgerminomas are rare Q2781:These agents induce the formation of surfactant lipids in the prevention of RDS in preterm babies; Corticosteroids Q2782:Pseudomyxoma peritonei is a potential complication of this tumor? Mucinous cystadenoma Q2783:T or F? Elevation of LDH is common in seminomas. True Q2784:Koilocytotic atypia is commonly observed with infection with? HPV Q2785:Most cases of male pseudohermaphrodites are due to? Testicular feminization syndrome Q2786:Most common variant of breast adenocarcinomoa? Invasive ductal carcinoma Q2787:Which phase accounts for most of variability in the length of the menstrual cycle? Follicular phase Q2788:T or F? Basal body temperature falls precipitously a few days prior to menstruation True Q2789:Intraductal papillomas - describe the favorable pathology? Presence of fibrovascular core; and both epithelial and myoepithelial cells in thepaipllary fronds Q2790:Alpha fetoprotein is a good marker for which cancers? Non-seminomatous germ cell tumors of the testes; endodermal sinus (yolk sac) ovarian tumors; hepatocellular carcinomas Q2791:Malignant mixed mullerian tumor? Tumor with 2 components (stromal and epithelial (endometrial glands) Q2792:Maternal blood levels of ; are dependent on a viable fetus? Estrioll Q2793:This tumor; in combination with ascites and hydrothorax; is referred to as Meigs' syndrome Thecoma-fibroma Q2794:Serous paipllary cystadenocarcinomas of the ovaries express which marker? CA-125 Q2795:Three stages of spermatogenesis? Spermatocytogenesis; meiosis; and spermiogenesis Q2796:What are two types of ovarian tumors that can produce large amounts of steroid hormones? Sertoli-Leydic cell tumors (testosterone); and granulosa cell tumors (estrogen) Q2797:36 y/o man with painless enlargement of right testis. Biopsy - Round nests of cells with conspicuous nucleoli and clear cytoplasm; nests are separated by delicate fibrous septa containing numerous lymphocytes; Seminoma Q2798:Causative organism of lymphogranuloma venereum? Chlamydia Q2799:Bright reddish-pink; acellular ring around the ovum? Zona pellucida Q2800:Forms of penile carcinoma in situ? Bowen's disease; Bowenoid papulosis; and erythroplasia of Queyrat Q2801:Describe the lesions of Bowenoid paulosis? Multiple reddish-brown pauplar lesions Q2802:Most cases of dysfunctional uterine bleeding are due to? Anovulatory cycles Q2803:Histology of inflammatory breast cancer? Dermal lymphatic invasion by cancer cells; can cause orange peel appearance Q2804:Koilocytic? The characteristic nuclear and cytoplasmic changes shown by squamous epithelial cells infected by HPV Q2805:Name the mutations of the following syndromes: LiFraumeni; Cowden; and ataxia-telangiectasia LiFraumeni - p53;Cowden - gene of chromosome 10;Ataxia- telangiectasia - ATM gene Q2806:Gynecomastia; golden-brown testicular tumor; large uniform cells with indistinct cell borders; and rod-shaped crystals Leydig cell tumor (crystals of Reinke) Q2807:Struma ovarii? Monodermal teratomas can develop an appearance of mature thyroid tissue Q2808:T or F? Ductal carcinoma in situ can produce Paget disease? True Q2809:The first set of lymph nodes to which lymph from the testes drain? Para-aortic nodes; near where the testicular artery arises Q2810:First event in preeclampsia is thought to be? Placental ischemia Q2811:Condyloma acuminatum Verrucous wartlike lesions on vulva; perineum; vagina and cervix associated with HPV serotypes 6 and 11 Q2812:Pelvic inflammatory disease Vaginal discharge (cervicitis); vaginal bleeding (endometritis); bilateral lower abdomainal and pelvic pain (salpingitis). Caused by n. gonorrhea and/or chlamydia. Complications: tubo-ovarian abscess; tubal scarring (granulomatous inflamation) with infertility and ectopic pregnancies Q2813:Cervical cancer risk factors Early age of first intercourse; multiple sexual partners; multiple pregnancies; oral contraceptives; smokking Q2814:Cervical cancer clinical features 45 years old. Asymptomatic or postcoital bleeding; dyspareunia (painful intercourse); malodorous discharge. Caused by HPV types 16; 18; 31 and 33. Precursor lesion is cervical intraepithelial neoplasia Q2815:Endometriosis Presence of endometrial glands and stroma outside the uterus in ovaries; ligaments and pouch of Douglas. Presents with chronic pelvic pain; dysmenorrhea; dyspareunia (painful intercourse); rectal pain; constipation; infertility Q2816:Endometrial carcinoma risk factors Early menarche; late menapause; nulliparity; hypertension; diabetes; anovulation; estrogen-producing tumors; estrogen- replacement therapy; endometrial hyperplasia Q2817:Endometrial carcinoma clinical features 55 year old with postmenopausal vaginal bleeding Q2818:Polycystic ovarian disease Females of reproductive age; oligomenorrhea; hirsutism; infertility. Lab: elevated LH; low FSH; high testosterone. Predisposes to endometrial cancer Q2819:Ovarian cystadenocarcinoma 65 year old with malignant bilateral ovarian enlargement. Risk factors: BRCA-1. Marker: CA125 Q2820:Metastatic tumors to the ovary primary sites Breast; colon; endometrial; gastric "signet-cell" Krukenberg tumor Q2821:Hydatidiform mole Tumor of placental trophoblastic tissue/ Excessive uterine enlargement; vaginal bleeding; high B-HCG. Complete mole: fertilization of an ovum without chromosomes. Partial mole: fertilization by two sperms (one 23X; one 23Y) Q2822:Choriocarcinoma Malignant germ cell tumor derived from the trophoblast Q2823:Fibrocystic change presentation Bilateral painful mass in young woman with menstrual variation Q2824:Fibroadenoma Movable mass that changes with mentrual cycles Q2825:Breast carcinoma risk factors BRCA-1; BRCA-2; p53; prior breast cancer; old age; nulliparity; obesity Q2826:Breast carcinoma clinical features Solitary painless mass in old woman with nipple retraction or skin dimpling or fixation to chest wall. Calcification on mamogram. MC variation is invasive ductal carcinoma Q2827:Benign prostatic hyperplasia Decreased caliber and force of stream; urgency; frequency; nocturia; dysuria. PSA is elevated. Rx.: 5-alpha reductase inhibitor Q2828:Prostate cancer Asymptomatic or lower back pain secondary to metastasis. High PSA. Metastasis to pelvic lymph nodes and lumbar spine with high alkaline phosphatase Q2829:The Adrenal cortex derives from what embryonic tissue? Mesoderm Q2830:The Adrenal medulla derives from what embryonic tissue? Neural creast Q2831:The Adrenal Zona Glomerulosa produces what? Aldosterone Q2832:The Adrenal Zona Fasciculata produces what? Cortisol; and some sex hormones Q2833:The Adrenal Zona Reticularis produces what? Sex hormones (androgens) Q2834:The Adrenal Medulla produces what? Catecholamines (Epinephrine and Norepinephrine) Q2835:What do beta islet cells produce? Insulin Q2836:What do alpha islet cells produce? Glucagon Q2837:What do delta islet cells produce? Somatostatin Q2838:What is the neurohypophysis? Posterior pituitary Q2839:What is the adenohypophysis? Anterior pituitary Q2840:What is the posterior pituitary derived from? Neuroectoderm Q2841:What is the anterior pituitary derived from? Oral ectoderm Q2842:What anterior pituitary cells are acidophils? The ones that produce GH or prolactin Q2843:What anterior pituitary cells are basophils? "B-FLAT";The ones that produce FSH; LH; ACTH; TSH Q2844:What hormones share a common alpha-subunit? TSH; LH; FSH and hCG Q2845:What regulates TSH release? Increased by TRH;Decreased by Somatostatin Q2846:What regulates Prolactin release? Increased by TRH;Decreased by Dopamine Q2847:What regulates ACTH release? Increased by CRH Q2848:What regulates GH release? Increased by GHRH;Decreased by Somatostatin Q2849:What regulates FSH release? Increased by GnRH Q2850:What regulates LH release? Increased by GnRH Q2851:What is the most common form of congenital adrenal hyperplasia? 21-beta-hydroxylase deficiency Q2852:How many carbons do mineralocorticoids have? 21 carbons Q2853:How many carbons do glucocorticoids have? 21 carbons Q2854:How many carbons do androgens have? 19 carbons Q2855:How many carbons do estrogens have? 18 carbons Q2856:Where is PTH secreted from? Chief cells of parathyroid Q2857:What is the active form of Vitamin D 1;25-(OH)2 vitamin D Q2858:What is the inactivated form of Vitamin D 24;25-(OH)2 vitamin D Q2859:How is Vitamin D activated? The liver converts Vitamin D to 25-OH Vitamin D(storage form) and the kidney then converts it to 1;25(OH) Vitamin D Q2860:What is the storage form of Vitamin D? 25-OH Vitamin D Q2861:Where is calcitonin secreted from? Parafollicular cells(C cells) of thyroid Q2862:What is the role of calcitonin? Oppose the actions of PTH;Secreted when serum Ca increases and it decreases bone resorption of calcium Q2863:What hormones are steroid hormones? "PET CAT";Progesterone; Estrogen; Testosterone; Cortisol; Aldosterone; Thyroxine and T3 Q2864:Secretion and cell type of;Somatotrope cells Secretes GH;Acidophil Q2865:Secretion and cell type of;Mammotrope cells Secretes Prolactin;Acidophil Q2866:Secretion and cell type of;Corticotrope cells Secretes ACTH;Basophil Q2867:Secretion and cell type of;Gondadotrope cells Secretes LH & FSH;Basophil Q2868:Secretion and cell type of;Thyrotrope cells Secretes TSH;Basophil Q2869:What is the main form of thyroid hormone secreted by the thyroid? T4-Tetraiodothyronine Q2870:What is the most active form of thyroid hormone? T3-Triiodithyronine Q2871:What condition leads to increased Thyroxine-binding globulin(TBG)? Pregnancy;Increased estrogen leads to increased TBG Q2872:What condition leads to decreased Thyroxine-binding globulin(TBG)? Hepatic failure Q2873:What is Conn's syndrome? Primary hyperaldosteronism- an aldosterone secreting tumor Q2874:What is the most common tumor of the adrenal medulla in adults? Pheochromocytoma Q2875:What is the most common tumor of the adrenal medulla in children? Neuroblastoma Q2876:What is a/w pheochromocytomas? Neurofibromatosis; MEN types II & III Q2877:What is Wermer's syndrome? MEN type I Q2878:What is Sipple's Syndrome? MEN type II Q2879:Which type of MEN?;Pancreatic; parathyroid & pituitary tumors MEN type I Q2880:Which type of MEN?;Medullary carcinoma of the thyroid; pheochromocytoma & parathyroid tumors MEN type II Q2881:Which type of MEN?;Medullary carcinoma of the thyroid; pheochromocytoma; & oral & intestinal ganglioneuromatosis MEN type III Q2882:Which thyroid cancer?;Most common Papillary carcinoma of the thyroid Q2883:Which thyroid cancer?;"Ground-glass" nuclei Papillary carcinoma of the thyroid Q2884:Which thyroid cancer?;From parafollicular "C cells" Medullary carcinoma of the thyroid Q2885:Which thyroid cancer?;Produces calcitonin Medullary carcinoma of the thyroid Q2886:Which thyroid cancer?;a/w MEN types II & III Medullary carcinoma of the thyroid Q2887:Which thyroid cancer?;usually only in older patients Undifferentiated/anaplastic cancer of the thyroid Q2888:Which thyroid cancer?;a/w Hashimoto's thyroiditis Lymphoma of the thyroid Q2889:ret gene mutations are a/w what? MEN types II & III Q2890:Type I or Type II DM?;Viral or Immune destruction of beta-cells Type I DM Q2891:Type I or Type II DM?;a/w HLA-DR3 & DR4 Type I DM Q2892:Type I or Type II DM?;Ketoacidosis more common Type I DM Q2893:Type I or Type II DM?;"Classic" symptoms of Diabetes more common Type I DM;Classic symptoms-polyuria; polydipsia; thirst; weight loss Q2894:Type I or Type II DM?;a/w obestity Type II DM Q2895:Type I or Type II DM?;Strong genetic predisposition Type II DM Q2896:What drug class?;Lispro Insulin- short acting Q2897:What drug class?;NPH Insulin-intermediate acting Q2898:What drug class?;Lente Insulin-long acting Q2899:What drug class?;Tolbutamide First generation sulfonylurea Q2900:What drug class?;Chlorpropamide First generation sulfonylurea Q2901:What drug class?;Glyburide Second generation sulfonylurea Q2902:What drug class?;Glimepiride Second generation sulfonylurea Q2903:What drug class?;Glipizide Second generation sulfonylurea Q2904:What drug class?;Metformin Biguanides Q2905:What drug class?;Pioglitazone Glitazones Q2906:What drug class?;Rosiglitazone Glitazones Q2907:What drug class?;Acarbose alpha-glucosidase inhibitors Q2908:What drug class?;Miglitol alpha-glucosidase inhibitors Q2909:What diabetes drug type is;Lispro Short-Acting Insulin Q2910:What diabetes drug type is;Insulin Short-Acting Insulin Q2911:What diabetes drug type is;NPH Intermediate-Acting Insulin Q2912:What diabetes drug type is;Lente Long-Acting Insulin Q2913:What diabetes drug type is;Ultralente Long-Acting Insulin Q2914:What diabetes drug type is;Tolbutamide First generation sulfonylurea Q2915:What diabetes drug type is;Chlorpropamide First generation sulfonylurea Q2916:What diabetes drug type is;Glyburide Second generation sulfonylurea Q2917:What diabetes drug type is;Glimepiride Second generation sulfonylurea Q2918:What diabetes drug type is;Glipizide Second generation sulfonylurea Q2919:What diabetes drug type is;Metformin Biguanide Q2920:What diabetes drug type is;Pioglitazone Glitazones Q2921:What diabetes drug type is;Rosiglitazone Glitazones Q2922:What diabetes drug type is;Acarbose alpha-glucosidase inhibitors Q2923:What diabetes drug type is;Miglitol alpha-glucosidase inhibitors Q2924:What structure contains the ovarian vessels? Suspensory ligament of ovaries Q2925:What structure contains the uterine vessels? Transverse cervical(cardinal);ligament Q2926:What structure contains the round ligament of the uterus & the uterine tubules & vessels Broad ligament Q2927:What does the acrosome of sperm derive from? Golgi apparatus Q2928:What is the food supply of sperm? Fructose Q2929:What kind of DNA do Spermatogonium have? 2N diploid Q2930:What kind of DNA do Primary spermatocyte have? 4N diploid Q2931:What kind of DNA do Secondary spermatocyte have? 2N haploid Q2932:What kind of DNA do Spermatid have? 1N haploid Q2933:What course do sperm take from production to ejaculation? SEVEN UP;Seminiferous tubules; Epididymis; Vas deferens; Ejaculatory ducts; (Nothing); Urethra; Penis Q2934:FSH stimulates which cells in the male? Sertoli cells;Aid in development of sperm Q2935:FSH stimulates which cells in the female? Granulosa cells;Aromatization of androgens Q2936:LH stimulates which cells in the male? Leydig cells;Testosterone release Q2937:LH stimulates which cells in the female? Thecal cells;Produces androgens Q2938:Which cell; leydig or sertoli; is found in interstitium between seminiferous tubules? Leydig cells Q2939:Which cell; leydig or sertoli; is found in the seminiferous tubules at the edge of the basement membrane? Sertoli cells Q2940:What converts testosterone to DHT? 5-alpha-reductase Q2941:What inhibits 5-alpha-reductase? Finasteride;Blocks conversion of testosterone to DHT Q2942:What is the most potent androgen? Dihydrotestosterone(DHT) Q2943:Which androgen cannot be aromatized to an estrogen? DHT;Both Testosterone and androstenedione can be converted to estrogens Q2944:Which is the most potent estrogen? Estradiol Q2945:Which estrogen increases the most during pregnancy? Estriol Q2946:In what stage of the cell cycle do eggs arrest in for most of their life? Prophase of meiosis I Q2947:In what stage of the cell cycle do eggs arrest in after ovulation and until fertilization? Metaphase of meiosis II Q2948:How many days after fertilization does hCG become detectable in the urine? 8 days Q2949:What is a/w Epispadias? Exstrophy of the bladder Q2950:What is the genotype of a complete mole? 46; XX w/ all genes coming from the father Q2951:What is the genotype of an partial mole? Usually triploid Q2952:What strains of HPV are a/w cervical dysplasia? 16 & 18 Q2953:Which ovarian tumor;Equivalent to male seminoma Dysgerminoma Q2954:Which ovarian tumor;Sheets of uniform cells Dysgerminoma Q2955:Which ovarian tumor;Aggresive malignancy in ovaries & sacrococcygeal area of young children Yolk sac (endodermal sinus) tumor Q2956:Which ovarian tumor;Large; hyperchromatic syncytiotrophoblastic cells Choriocarcinoma Q2957:Which ovarian tumor;90% of ovarian germ cell tumors Teratoma Q2958:Which ovarian tumor;Contains cells from 2 or 3 germ layers Teratoma Q2959:Which ovarian tumor;May contain struma ovarii Teratoma;Struma ovarii- contains functional thyroid tissue Q2960:Which ovarian tumor;20% of ovarian tumors Serous cystadenoma Q2961:Which ovarian tumor;50% of ovarian tumors Serious cystadenocarcinoma Q2962:Which ovarian tumor;Frequently bilateral & lined w/ fallopian tube-like epithelium Serous cystadenoma Q2963:Which ovarian tumor;Multilocular cyst lined by mucus-secreting epithelium Mucinous cystadenoma Q2964:Which ovarian tumor;a/w Pseudomyxoma peritonei Mucinous cystadenocarcinoma Q2965:Which ovarian tumor;Resembles bladder epithelium Brenner tumor Q2966:Which ovarian tumor;Bundles of spindle-shaped fibroblasts Ovarian fibroma Q2967:Which ovarian tumor;Part of Meig's syndrome Ovarian fibroma;Meig's syndrome- triad of ovarian fibroma; ascites & hydrothorax Q2968:Which ovarian tumor;Can lead to precocious puberty Granulosa cell tumor Q2969:Which ovarian tumor;Secretes estrogen Granulosa cell tumor Q2970:Which ovarian tumor;Can cause endometrial hyperplasia/carcinoma in adults Granulosa cell tumor Q2971:Which ovarian tumor;Call-exner bodies Granulosa cell tumor;Call-exner bodies- small follicles filled w/ eosinophilic secretions Q2972:What is Pseudomyxoma peritonei intraperitoneal accumulation of mucinous material from ovarian or appendiceal tumor;a/w Mucinous cystadenocarcinoma Q2973:What breast condition?;Most common tumor in women under 25 Fibroadenoma Q2974:What breast condition?;Lump w/ increased size and tenderness w/ pregnancy Fibroadenoma Q2975:What breast condition?;Benign tumor of lactiferous ducts Intraductal papilloma Q2976:What breast condition?;Presents w/ serous or bloody nipple discharge Intraductal papilloma Q2977:What breast condition?;Large; bulky mass of connective tissue & cysts Cystosarcoma phyllodes Q2978:What breast condition?;"Leaflike" projections Cystosarcoma phyllodes Q2979:What breast condition?;Early malignancy w/o basement membrane penetration Ductal carcinoma in situ Q2980:What breast condition?;Worst and most invasive breast cancer Invasive ductal carcinoma Q2981:What breast condition?;Cheesy consistency due to central necrosis Comedocarcinoma Q2982:What breast condition?;Fleshy; cellular; lymphocytic infiltrate Medullary carcinoma Q2983:What breast condition?;Eczematous patches on nipple Paget's disease of the breast Q2984:What breast condition?;Large cells w/ clear halo Paget's disease of the breast;Cells are called paget cells Q2985:What breast condition?;MCC of breast lumps age 25- menopause Fibrocystic disease Q2986:What breast condition?;Diffuse breast pain & multiple lesions; often bilateral Fibrocystic disease Q2987:What breast condition?;Increased number of epithelial cell layers in terminal duct lobule Epithelial hyperplasia- Fibrocystic disease Q2988:What breast condition?;Increased acini & intralobular fibrosis Sclerosing type of Fibrocystic disease Q2989:What breast condition?;Hyperplasia of breast stroma Fibrosis type of Fibrocystic disease Q2990:What breast condition?;Breast abscess during breast feeding Acute mastitis Q2991:What breast condition?;Benign painless lump that forms due to injury to breast tissue Fat necrosis Q2992:Risk factors for Breast tumors Gender; age; early 1st menarche(<12 y.o.); delayed 1st pregnancy(>30 y.o.); late menopause(>50 y.o.); family history of 1st degree relative w/ breast cancer at young age Q2993:Most common pathogen in acute mastitis Staphylococcus aureus Q2994:What testicular tumor?;Painless testicular enlargement Seminoma Q2995:What testicular tumor?;Most common testicular tumor Seminoma Q2996:What testicular tumor?;Worst prognosis Embryonal carcinoma of the testicle Q2997:What testicular tumor?;Reinke crystals Leydig cell tumor Q2998:What testicular tumor?;Androgen producing tumor Leydig cell tumor Q2999:What testicular tumor?;Causes gynecomastia in men & precocious puberty in boys Leydig cell tumor Q3000:What testicular tumor?;Androblastoma from sex cord stroma Sertoli cell tumor Q3001:What testicular tumor?;Most common testicular cancer in older men Testicular lymphoma Q3002:What is the typical age of presentation of a seminoma? 15-35 years old Q3003:What penile tumor?;Solitary crusty plaque on shaft or on scrotum Bowen disease- type of penile carcinoma in situ Q3004:What penile tumor?;Peak incidence in 5th decade of life Bowen disease- type of penile carcinoma in situ Q3005:What penile tumor?;Becomes invasive SCC in <10% of cases Bowen disease- type of penile carcinoma in situ Q3006:What penile tumor?;Red velvety plaques; usually involving the glans Erythroplasia of Queyrat- type of penile carcinoma in situ Q3007:What penile tumor?;Similar to Bowen disease Erythroplasia of Queyrat- type of penile carcinoma in situ Q3008:What penile tumor?;Multiple papular lesions Bowenoid papulosis- type of penile carcinoma in situ Q3009:What penile tumor?;Affects a younger age group than other penile tumors Bowenoid papulosis- type of penile carcinoma in situ Q3010:What penile tumor?;a/w HPV Penile squamous cell carcinoma Q3011:What penile tumor?;Rare in circumcised men Penile squamous cell carcinoma Q3012:What are the three layers of the skin Epidermis; dermis; subcutaneous tissue Q3013:What types of cells compose the epidermal layer of the skin Stratified squamous epithelium Q3014:What are the four layers of the epidermis Stratum Corneum;Stratum Granulosum;Stratum Spinosum;Stratum Basalis;Cocky Guys Save Babes Q3015:Hyperkeratosis affects which layer of the epidermis Stratus corneum Q3016:What types of cells compose the stratum basalis Columnar basal cells Q3017:What is a plaque Raised area of skin >1cm in diameter Q3018:What is a papule Raised area of skin <1cm in diameter Q3019:What is a macule Flat; discolored area of skin <1cm in diameter Q3020:What is a vesicle A raised; fluid-filled blister measuring <0.5cm in diameter Q3021:What are the small; encapsulated sensory receptors found in the dermis of the palms; soles; and digits of the skin (Hint: they are also involved in light discriminatory touch of hairless skin) Meissner’s corpuscles Q3022:What is the name of tactile disks that mediate light crude touch Merkel corpuscles Q3023:What are the large encapsulated sensory receptors found in deeper layers of skin that are involved in pressure; coarse touch; vibration; and tension Pacinian corpuscles Q3024:Where are pacinian corpuscles found Deep layers of skin;Joint capsules;Serous membranes;Mesenteries Q3025:What structures prevent diffusion across intracellular spaces Zona occludens (tight junctions) Q3026:What structures allow adjacent cells to communicate rapidly Gap junctions Q3027:What structure connects cells to underlying extracellular matrix Hemidesmosomes Q3028:What are structures that join adjacent cells together and provide anchoring points for intermediate filaments Desmosomes (macula adherens) Q3029:What is the function of Langerhans cells Antigen-presenting cells; Main inducers of antibody response Q3030:From where does the epidermis regrow after trauma or removal Hair follicles and sweat glands in the dermis Q3031:What is the term that describes the replacement of one adult cell type by another; often secondary to irritation and/or environmental exposure Metaplasia (reversible) Q3032:Which term describes an increased number of cells Hyperplasia (reversible) Q3033:What do you call the change of a cell to a less differentiated form Anaplasia Q3034:What is the term for abnormal growth with loss of orientation; shape; and size compared to normal cells Dysplasia (reversible) Q3035:Which enzymes do malignant cells use to metastasize Collagenases and hydrolases Q3036:Which rash is often described as a target lesion that has a red center; pale zone; and a dark outer ring Erythema multiforme Q3037:What are common causes of erythema multiforme Infections;Antibiotics;Radiation;Chemicals;Malignancy Q3038:What is the term for dilated; superficial blood vessels Telangiectasia Q3039:Which test is designed to ascertain whether a skin lesion will blanch as a result of pressure Diascopy. Used to determine whether a red lesion is blood- filled or hemorrhagic Q3040:What are the most common causes of nonscarring alopecia Telogen effluvium;Androgenic alopecia;Alopecia areata;Tinea capitis;Traumatic alopecia Q3041:What are the most common causes of scarring alopecia Cutaneous lupus;Lichen planus;Folliculitis planus;Linear scleroderma Q3042:What is the easiest and quickest way to determine if a skin condition is fungal KOH preparation Q3043:An 8-month old presents with large; easily ruptured flaccid bullae; with large areas of desquamation of skin and a positive Nikolsky’s sign. What is the most likely diagnosis Staphylococcal scalded skin syndrome Q3044:What is the infectious agent that causes scalded skin syndrome Staphylococcus aureus Q3045:What condition is described as having thin-walled vesicles or pustules that rupture to form golden-yellow crusts (honey crusts) Impetigo Q3046:What is the most common bacterial infection of the skin in children Impetigo Q3047:What bacteria cause impetigo Staph aureus or Strep pyogenes Q3048:What test is helpful to determine the organism involved in impetigo Culture and catalase tests Q3049:A sexually active 23 year old patient presents with painful vesicles on his penis and a slight fever. He refers to having the same type of vesicles multiple times a year. Multinucleated giant cells and ballooning of nuclei are seen microscopically. What is the likely diagnosis Herpes simplex infection Q3050:What is the treatment for herpes simplex type I Oral and topical acyclovir;Valacyclovir;Famciclovir Q3051:What infection causes unilateral; painful vesicles along a dermatome of the face or trunk Shingles (Herpes Zoster) Q3052:Which test can be used to assist in the diagnosis of herpes virus infection Tzank smear Q3053:Which disease of childhood presents with acute vesicular eruptions that occur in successive crops; so that the rash typically consists of vesicles at different stages of resolution Varicella or chicken pox Q3054:A 16 year old presents with multiple dome-shaped; umbilicated; waxy papules on the face and chest. What is the most likely diagnosis Molluscum contagiosum Q3055:How does molluscum contagiosum appear microscopically Epidermal hyperplasia producing a basin with moluscum bodies (Henderson-Patterson bodies) Q3056:What type of virus causes molluscum contagiosum Pox virus Q3057:A 35 year old patient with AIDS presents with multiple brownish/purplish macules on the trunk and lower extremities. What is the most likely diagnosis Kaposi’s sarcoma (HHV8) Q3058:A 7 year old present with multiple hard; rough- surfaced papules on his fingers and elbows. Diagnosis? Verruca vulagris (common wart) Q3059:What is the causative agent of verruca vulgaris HPV Q3060:What is the typical primary syphilis skin manifestation Painless indurated genital or lip ulcer (chancre) Q3061:What are the typical secondary syphilis skin manifestations Cutaneous lesions that are maculopapular or erythematosquamous; lesions on palms and soles; warts (condylomata lata) on anogenital region; and alopecia Q3062:What HPV serotypes cause the common wart HPV-1;2;4;7 Q3063:A 24 year old man from the Northeast; visits the physician because of a centrifugally spreading; erythematous lesion on his right leg. The patient noticed the rash after he went hiking. Diagnosis? Erythema chronicum migrans (Lyme disease) Q3064:A mother brings her 5 year old son to the physician because she noted her son scratching a pinkish lesion on his neck. Upon examination; the physician notes a ring-shaped scaling plaque with central clearing and elevated borders. Diagnosis? Tinea corporis (ringworms) Q3065:How is ringworm acquired Tinea corporis is not due to a worm but due to a fungal infection Q3066:Skin infections are typically caused by what bacterium S. aureus Q3067:A 22 year old man presents with a rash that first appeared on his palms and soles; and then spread to his face and trunk. Diagnosis? Rocky Mountain spotted fever Q3068:What type of rash is seen in spotted fever An inward or centripetal spreading rash Q3069:What is used to test for typhus and Rocky mountain spotted fever Weil-Felix reaction Q3070:What is the treatment for Rocky mountain spotted fever Tetracyclines or chloramphenicol Q3071:A 26 year old female from Texas complains of small hypopigmented spots on her upper back that usually disappear in the winter months. Diagnosis? Tinea versicolor Q3072:What is the confirmatory test for tinea versicolor KOH preparation Q3073:Which diseases cause hand and foot lesions Syphilis;Hand-foot-and-mouth disease;Rocky Mountain spotted fever Q3074:A 25 year old sexually active male presents with a painful; nonindurated genital ulcer; and tender regional lymphadenopathy. Diagnosis? Chancroid Q3075:A 35 year old homeless woman visits a shelter physician because of multiple; extremely pruritic papules in her axilla; groin; and finger webs. The patient indicates her husband also has the same lesions. Diagnosis? Scabies Q3076:A 15 year old male on the school swim team visits the dermatologist because of itchiness on both his feet. He states he is not on any medication and has not had it before. Upon inspection; the physician notes erythematous; dry scaling lesions on both feet. Diagnosis? Tinea pedis Q3077:What is the treatment for tinea pedis Topical or oral antifungals Q3078:What is the rapid developing infection of the skin and fascia that may lead to death if not treated quickly Necrotizing fasciitis Q3079:What are the organisms responsible for necrotizing fasciitis Group A streptococci or Clostridium perfringens Q3080:A 67 year old Caucasian woman visits her dermatologist because of small reddish papules/pustules predominantly on her cheeks; nose; chin; and forehead. She states that her face becomes worse if she uses hot water or is in warm weather. Diagnosis? Rosacea Q3081:What is the treatment for Rosacea Avoid precipitating factors;Topical metronidazole;Sulfur lotions;Oral tetracyclines;Isotretinoin Q3082:A 12 year old male visits his physician because of a slap-like red mark on his cheek and a rash on his arms that appeared 1 day after the cheek rash. Upon physical examination; the physician notes malar erythema and a maculopapular rash on his extremities. Diagnosis? Fifth diseas (erythema infectiosum) Q3083:What causes fifth disease Parvovirus B19 Q3084:What are the other complications of fifth disease Nonimmune fetal hydrops (virus infects and destroys fetal red blood cells);More severe anemia in patients with other type of chronic anemia (like aplastic crisis in sickle cell patient) Q3085:Which bacteria cause erythrasma Corynebacterium Q3086:What bacterium causes scarlet fever S. pyogenes (group A β-hemolytic) Q3087:What type of rash is seen in typhus An outward or centrifugal-spreading rash Q3088:How do the rickettsiae cause severe tissue damage Organisms infect endothelial cells and cause vascular leakage; which results in hypovolemic shock; pulmonary edema; renal failure; and CNS damage Q3089:What does disseminated disease of coccidioidomycosis manifest as on the skin Verrucous plaques (usually on face);Subcutaneous abscesses;Pustular lesions Q3090:A 23 year old farmhand presents to the dermatologist with multiple red-violaceous nodules on the hand; fever; and history of diarrhea. During the examination; the patient states he is in charge of the cows on the farm. Diagnosis? Milker’s nodules Q3091:What causes milker’s nodules Paravaccinia virus Q3092:What group is paravaccinia in Double-stranded parapoxvirus Q3093:What disease may follow paravaccinia infection Bullous pemphigoid Q3094:A 16 year old female complains of fatigue; fever; sore throat; and lymphadenopathy. She leaves with a prescription for medication. Two days later she returns with a cutaneous rash on her face. What did the physician prescribe the patient Ampicillin Q3095:What is the most likely diagnosis for a patient who develops a rash after treatment with ampicillin Mononucleosis Q3096:What is the etiologic agent for mononucleosis EBV (HHV 4) Q3097:What are the classic criteria for diagnosing mononucleosis Lymphocytosis; presence of at least 10% atypical lymphocytes on peripheral smear; and a positive serologic test for EBV Q3098:What organism is responsible for tinea versicolor Malassezia furfur Q3099:What is used to treat tinea versicolor Topical miconazole;Selenium sulfide Q3100:A 45 year old rancher visits a dermatologist because of a black 2-cm lesion on his hand. He states that the lesion was itchy and had a reddish color a day or two ago. What is the most likely diagnosis Anthrax Q3101:What is the causative agent of anthrax Bacillus anthracis Q3102:A 36 year old migrant worker from Mexico visits the physician because of small disfiguring nodules forming on his ears and hands. The patient also states that he is losing sensation in the affected areas. Diagnosis? Leprosy Q3103:What is the causative agent of leprosy Mycobacterium leprae Q3104:What agent causes hand-foot-and-mouth disease Coxsackie virus type A-16 Q3105:What are the signs and symptoms of hand-foot-and- mouth disease Fever and malaise with small oval vesicles along creases of palms; soles; and lips Q3106:What is the most common type of bacterial infection in burn victims Pseudomonas aeruginosa infections Q3107:Dermatophytes include members of which genera Trichophyton; Microsporum; Epidermophyton Q3108:An inner-city child is brought to the physician because of patches of hair loss. His mother states that he has had this problem for at least a month. The lesions are painless and have some scaling. Diagnosis? Tinea capitis Q3109:What is the most likely etiologic organism of tinea capitis Trichophyton tonsurans Q3110:What is the treatment for tinea capitis Griseofulvin;Terbinafine Q3111:Which form of M. furfur generally causes disease Hyphal form Q3112:What is the most common type of skin cancer Basal cell carcinoma Q3113:What is the neoplasm that is often described as a pearly; red macule; papule; or nodule that is found on sun- exposed areas of the head or neck Basal cell carcinoma Q3114:What skin cancer is microscopically characterized by nests of palisading cells Basal cell carcinoma Q3115:Which neoplasm is often described as a red papule; nodule; or plaque that may be hyperkeratotic or ulcerated on sun-exposed skin Squamous cell carcinoma Q3116:Arsenic causes which type of skin cancer Squamous cell carcinoma Q3117:Which neoplasm is microscopically characterized by nest of atypical squamous epithelial cells and keratin Squamous cell carcinoma Q3118:Which type of skin cancer results as a complication of severe burns 20-40 years later and has a higher risk of metastasis compared to its regular skin version Squamous cell carcinoma Q3119:Actinic keratosis lesions may transform into what type of skin cancer if left untreated Squamous cell carcinoma Q3120:What are some risk factors for squamous cell carcinoma Sun exposure;Ionizing radiation;Actinic keratosis;Immunosuppression;Arsenic;Industrial carcinogens Q3121:A 54 year old male visits the dermatologist because of a dark brown-black 5mm freckle in between his third and fourth toe. The patient stated that he noticed the freckle about a year and a half ago. The dermatologist biopsies the lesion and under the microscope; sees atypical confluent melanocytes with asymmetrical proliferation. Diagnosis? Melanoma Q3122:Which stains are used to test for melanoma S-100;HMB-45;MART-1 Q3123:What is the most sensitive stain that could be used to test for melanoma and is almost always positive S-100 Q3124:Large congenital nevi and dysplastic nevi may be precursor lesions for what type of cancer Melanoma Q3125:Which clinical criteria are used to help diagnose melanomas Aysmmetry;Border irregularity;Color variation;Diameter;ABCDs of melanoma Q3126:What is the term for a lesion that is a precursor or marker to melanoma Dysplastic nevus Q3127:How is dysplastic nevus syndrome inherited Autosomal dominant inheritance Q3128:What chromosome is dysplastic nevus syndrome located on Chromosome 1 Q3129:What are the peak ages for melanoma 40-70 years of age Q3130:What are risk factors for melanoma Sunburns;Chronic sun exposure;Fair skin;Dysplastic nevi Q3131:What is the most common subtype of melanoma Superficial spreading melanoma Q3132:Which type of melanoma has the best prognosis Lentigo maligna melanoma Q3133:Which type of melanoma has the worst prognosis Nodular melanoma Q3134:What is the most common type of melanoma in dark- skinned individuals Acral-lentiginous melanoma Q3135:What is the most important prognostic parameter for melanoma Depth (Breslow’s thickness) Q3136:In what condition would you find cytoplasmic Birbeck granules through electron microscopy Histiocytosis X (Langerhans cell histiocytosis) Q3137:In histiocytosis X; proliferations of which cells is usually found in the epidermis Langerhans cells (macrophages) Q3138:What term describes full-thickness epithelia atypia with an intact basement membrane in a cancer biopsy Carcinoma in situ Q3139:A 40 year old male has rash of scaly red patches on his trunk; face; and extremities. A biopsy is taken and superficial dermal infiltrates of T-lymphocytes and a collection of atypical lymphocytes are seen within the epidermis. Diagnosis? Mycosis fungoides (cutaneous T-cell lymphomas) Q3140:What is mycosis fungoides called where there is blood involvement Sezary syndrome Q3141:Skin carcinogenesis is thought to be caused by the accumulation of mutations in which tumor suppressor gene p53 Q3142:What type of carcinoma microscopically resembles metastatic small cell carcinoma from the lung or certain lymphomas Merkel cell carcinoma Q3143:Which HPV serotypes cause condyloma acuminatum HPV 6 and 11 Q3144:What are tan/brown plaques or papules that have a stuck on appearance and may be found anywhere on the body of adults; except the palms and soles Seborrheic keratosis Q3145:What is the most common type of collagen in a keloid Type III collagen Q3146:What are the risk factors for keloid formation African-American race; <30 years of age; and increased skin tension in a wound Q3147:Single or multiple bright red papules measuring a few millimeters in diameter that occur predominantly on the trunks and limbs of patients over 40 years are what type of lesions Senile angiomas (cherry angiomas) Q3148:A 59 year old male visits his family physician because of loss of appetite; weight loss; and fatigue. During the physical examination; the physician notes dark; rough-looking skin in the axilla region. What should the physician suspect Internal cancer or an endocrine disorder Q3149:What skin condition is described as dark; rough- looking or velvety skin in the axilla or on the back of the neck Acanthosis nigricans Q3150:Which autosomal recessive disease is characterized by defective DNA repair and photosensitivity Xeroderma pigmentosa Q3151:Patients with xeroderma pigmentosa usually develop which skin lesions Basal cell carcinoma;Squamous cell carcinoma;Actinic keratosis;Melanoma in childhood Q3152:Exposure to UV light causes what type of dimmers in the skin Thymine-thymine dimmers Q3153:What are the multiple; light-brown; freckle-like lesions found in neurofibromatosis Café au lait spots Q3154:Café au lait spots usually grow along what structures Peripheral nerves Q3155:Hypopigmented macules or ash-leaf spots on the trunk or lower extremities are associated with what disease Tuberous sclerosis Q3156:What is the tuberous sclerosis triad Mental retardation;Epilepsy;Multiple angiofibromas Q3157:A unilateral port-wine stain of the forehead and upper eyelid is associated with what condition Sturge-Weber syndrome (encephalotrigeminal angiomatosis) Q3158:Which nerve is associated with Sturge-Weber syndrome Ophthalmic branch of the trigeminal nerve Q3159:What condition has whitish-red nodules especially on digits and over joints; and is associated with uric acid accumulation Gout Q3160:A 39 year old man comes to the physician because he has noted a bluish-black color on his ears; nose; and sclera. The patient also states that his urine is sometimes very dark. Diagnosis? Alkaptonuria Q3161:How is alkaptonuria inherited Autosomal recessive inheritance Q3162:What accounts for the discoloration of the skin and urine in alkaptonuria Deposition of homogentisic acid Q3163:Hemochromatosis has what manifestation in the skin Hyperpigmented bronze skin Q3164:Which connective tissue disorder is associated with hyperextensible fragile skin; loose joints; and a tendency toward easy bruising and bleeding Ehlers-Danlos syndrome Q3165:What is the cause of death for a patient with Ehlers- Danlos disease Arterial or intestinal rupture Q3166:What is the name of the group of autosomal recessive diseases of premature aging Progeria Q3167:Marfan syndrome is due to a defect in which gene Fibrillin-1 (FBN-1) Q3168:On which chromosome is the defect for Marfan syndrome 15q21 Q3169:What is the most common cause of death in a Marfan patient Ascending aortic dissection Q3170:How is albinism generally inherited Autosomal recessive inheritance Q3171:What is lacking in the epidermis of albino patients Melanin Q3172:A 35 year old woman visits her physician because she recently noticed multiple small nodules on the back of her ankle. The patient’s vital signs are normal and she has no prior clinical illnesses. A blood test is taken that reveals Na 142; glucose 100; creatinine 1; BUN 17; cholesterol 310; triglycerides 412. What do the small nodules likely represent Xanthomas Q3173:What is the most likely cause of xanthomas Hyperlipidemia Q3174:A mother brings her 7 year old son to the physician because of two small masses on his right chest. The mother states that the child is adopted and does not know the child’s family history. A 4 and 3 cm mass is palpated on his right pectoral; small tan lesions are seen on his back and right arm; and a small growth is seen on his iris. Diagnosis? Neurofibromatosis 1 Q3175:On what chromosome is NF type 1 found Chromosome 17 Q3176:What are other manifestations associated with NF type 1 Optic gliomas;Bone abnormalities;Freckling of the axillary or genital area Q3177:What is the most common neurocutaneous disorder Neurofibromatosis Q3178:What condition is associated with multiple neuromas on the eyelid; lips; distal tongue; and/or oral mucosa MEN; Type 2b (III) Q3179:Epidermolysis bullosa acquisita (EBA) is associated with which disease Inflammatory bowel disease; especially Crohn’s disease Q3180:What haplotype is frequently found in patients with EBA HLA-DR2 Q3181:Which drugs cause erythema multiforme or “target” lesions Aspirin;Penicillin;Sulfonamides;Phenytoin;Corticosteroids;Ci metidine;Allopurinol;Oral contraceptives Q3182:Which drugs cause Stevens-Johnson syndrome Sulfa drugs;Carbamazepine;Phenytoin;Valproic acid;Phenobarbital;Quinolones;Cephalosporins;Allopurinol;C orticosteroids;Aminopenicillins Q3183:Which drugs induce acne Lithium;Steroids;Androgens;Oral contraceptive pills Q3184:Which marker is associated with a genetic susceptibility to fixed-drug reactions HLA-B22 Q3185:A 16 year old male with a clinical history of acne visits his family physician because he has a red rash on his face and various exposed parts. During the exam; he states the rash occurred after he spent a couple of hours outside playing football. What medication is he most likely taking Tetracyclines Q3186:A 75 year old female heart patient visits her dermatologist because her skin has begun to turn a light blue color. She is embarrassed to go in public because children say she looks like a “smurf.” What medication did her cardiologist most likely give her Amiodarone Q3187:What type of drug is Amiodarone Class III antiarrhythmic Q3188:What do you call a symmetrical; hyperpigmented lesion of the forehead and cheeks that occurs in women who are on oral contraceptives or pregnant Melasma Q3189:What are some common drugs that are associated with hyperpigmentation Bleomycin; Minocycline; Miodarone; Chloroquine; Gold; Chlorpromazine; 5-Fluorouracil; Daunorubicin; Busulfan Q3190:Which groups of patients have an increased risk of adverse drug reaction Women;Patients with Sjogren’s syndrome;AIDS patients Q3191:Which drug causes red man syndrome usually during rapid IV infusion Vancomycin Q3192:A 24 year old female presents to the dermatologist because of target-like lesions on her right arm. The patient states she is taking some type of antibiotic for a urinary tract infection. What drug class most likely caused the lesions Sulfonamides Q3193:What is the most common cause of burns in children Scalds from hot liquids Q3194:What is the most common cause of burns in adults Accidents with flammable liquids Q3195:Which kind of burn affects only the epidermis First-degree burn Q3196:Which kind of burn usually blisters and affects the dermis and adnexal structures Second-degree burn Q3197:Which type of burn involves the entire thickness of the skin; including variable amount of underlying fat and causes loss of sensation in affected area Third-degree burn Q3198:The scar that follows a deep second and third degree burn is composed of what Hyalinized collagen Q3199:Sharply demarcated; silvery-white plaques on a patient’s elbows and knees are most likely what disorder Psoriasis Q3200:What conditions can trigger psoriasis Trauma; Infection; Drugs Q3201:Which major histocompatibility markers are associated with psoriasis HLA-CW6;B13;B17;B27 Q3202:What disorder has scaly; thickened plaques that develop in response to persistent rubbing of pruritic sites Lichen simplex chronicus Q3203:Which disease results from the deposition of collagen in skin that causes a “hardened” and “thickened” appearance and is associated with Raynaud’s phenomenon Scleroderma Q3204:Which antibodies are associated with scleroderma Scl-70 (diffuse);Anticentromere antibodies (localized) Q3205:What other conditions are associated with scleroderma Hypertension;Gastrointestinal disease;Pulmonary fibrosis;Kidney disease Q3206:Which type of collagen is defective in osteogenesis imperfecta Type I collagen Q3207:Which disease is associated with a rash on the face; particularly the malar areas Systemic lupus erythematosus Q3208:Atopic dermatitis is associated with what conditions Asthma and allergic rhinitis Q3209:Which test is often helpful in the evaluation of patients with chronic contact dermatitis Patch test Q3210:What is the most common presentation of contact dermatitis Hand eczema; most likely due to occupational exposure Q3211:Contact dermatitis is what type of hypersensitivity reaction Type IV-delayed hypersensitivity Q3212:Eruptive forms of what condition may be associated with Reiter syndrome Psoriasis Q3213:Touch normal-appearing skin with a sliding motion and having the epidermis layer separated from the basal layer is what skin test Nikolsky’s sign Q3214:Which HLA types are increased in frequency in patients with dermatitis herpetiformis HLA-B8; DR3; DQW2 Q3215:Which rheumatologic disease is associated with a diffuse red rash of trunk; periungual telangiectasis; proximal weakness; myositis on muscle biopsy; and elevated CPK and aldolase Dermatomyositis Q3216:Vitiligo is most commonly associated with what conditions Thyroid disease;Pernicious anemia;Addison’s disease;Diabetes mellitus type 1 Q3217:What are some clinical manifestations of type I hypersensitivity reactions Anaphylaxis;Urticaria;Exanthema;Angioedema Q3218:Urticaria is what type of hypersensitivity reaction IgE-mediated; type I hypersensitivity reaction Q3219:Autoantibodies to the intercellular junction of epidermal cells are found in which disease Pemphigus vulgaris Q3220:Pemphigus vulgaris is associated with which antibody IgG Q3221:What do the autoantibodies in pemphigus vulgaris target Desmocollins and desmogleins (transmembrane desmosomal glycoproteins) Q3222:Patients with pemphigus vulgaris have an increased incidence of which haplotypes HLA-DR4; DRw6 Q3223:What do the autoantibodies in bullous pemphigoid target BP1 and BP2 in basement membrane Q3224:Bullous pemphigoid is an autoimmune disorder that rarely affects which part of the body in contrast to pemphigus vulgaris; which affects it frequently Oral mucosa Q3225:Dermatitis herpetiformis is associated with what condition Gluten-sensitive enteropathy Q3226:TB skin test; transplant rejection; and contact dermatitis are what type of hypersensitivity reaction Delayed type hypersensitivity reaction; type IV Q3227:What is the most likely vitamin deficiency that manifests as petechiae; echymoses; abnormal hair growth; bleeding gums; and poor wound healing Vitamin C (scurvy) Q3228:A 45 year old patient presents with intense hyperpigmentation; areas of epithelial desquamation; diarrhea; and confusion. What is the most likely deficient vitamin Niacin (nicotinic acid) Q3229:Which vitamin deficiencies have cutaneous manifestations Vitamin C;Vitamin A;Nicotinic acid;Riboflavin;Pyridoxine Q3230:What are some skin manifestations of kwashiorkor Dry skin;Patches of hypopigmentation;Skin peeling;Peripheral edema;Thin hair shafts Q3231:What condition would cause an increase in skin glycogen and acid α-glucosidase in cultured fibroblasts Pompe’s disease (type II) Q3232:A 3 year old male presents to the physician with a photosensitive rash; cerebellar ataxia; mental disturbances; and aminoaciduria. Niacin levels are within normal range. What is the most likely diagnosis Hartnup disease Q3233:How is Hartnup disease inherited Autosomal recessive inheritance Q3234:What are the cutaneous manifestations in Whipple’s disease Hyperpigmentation of scars and sun-exposed skin Q3235:A 32 year old woman visits her family physician because of rapid weight gain; profuse sweating; sudden abnormal hair growth; and easy bruising. Upon physical examination; the physician notes purplish striae on the abdomen; telangiectasia; thin skin; and an increase of fatty tissue on her back at the level of C6. What is the most likely diagnosis Cushing’s syndrome Q3236:Which test is used to diagnose Cushing’s disease Dexamethasone suppression test and 24 hour urinary measurement for cortisol Q3237:What is the most common cause of Cushing’s syndrome Iatrogenic Q3238:What hormone is increased in Cushing’s syndrome Cortisol Q3239:Patients with carcinoid syndrome may have which skin manifestation Episodes of flushing of the head; neck; and sometimes trunk Q3240:What are the metabolic causes of hyperpigmentation Porphyria cutanea tarda;Hemochromatosis;Vitamin B12 deficiency;Folic acid deficiency;Pellagra;Mallabsorption;Whipple’s disease Q3241:A 56-year-old man experienced chest pain and was admitted to the hospital suffering from an acute myocardial infarction. Five days later; he developed ventricular tachycardia; which progressed to ventricular fibrillation; and could not be resuscitated. The microscopic section shows an area of myocardium between an established infarct and normal myocardium. The myocytes in the figure show ;A. cell injury with undetermined ultimate fate ;B. early coagulation necrosis ;C. early liquefaction necrosis ;D. irreversible injury and will die within hours Option A (cell injury with undetermined ultimate fate) is correct. The cells show vacuolation due to loss of the ability to synthesize ATP via aerobic respiration. This; in turn; shuts down the Na+-K+ pump of the cell membrane. Sodium is no longer excluded from the cytosol; and water follows it into the cell; causing swelling and vacuolation. This is a reversible process. Q3242:. A 31-year-old woman has a mass on the right side of the neck lateral to the larynx. The mass is determined to be a well-differentiated tumor containing psammoma bodies. The tumor is most likely classified as a;A. follicular adenoma ;B. follicular carcinoma ;C. papillary adenoma ;D. papillary carcinoma Option D (papillary carcinoma) is correct. The presence of psammoma bodies is virtually diagnostic of papillary carcinoma; because these bodies are rarely seen in other thyroid neoplasms. Papillary carcinoma occurs most frequently in women between the ages of 20 and 50 years. Papillary carcinoma is the most common thyroid cancer and spreads via the lymphatic system. This type of thyroid cancer is more common in patients with a history of radiation exposure. Q3243:A total thyroidectomy is performed on a 42-year-old woman with a thyroid mass that appears to be encapsulated. There is evidence of both recent and past hemorrhage and numerous small; follicle-like structures composed of very uniform-appearing cells. Which of the following distinguishing features would suggest that this tumor is malignant?;A. Absence of papillary growth ;B. Absence of psammoma bodies ;C. Extension through the capsule ;D. Microfollicular pattern ;E. Presence of hemorrhage Option C (extension through the capsule) is correct. Invasion through the capsule of the tumor is one of the core characteristics used to determine if a follicular neoplasm of the thyroid is benign or malignant. Another characteristic is invasion of the tumor into the vasculature. Q3244:A 61-year-old man with chronic renal failure is being considered for a renal transplant. His serum calcium level is 7.6 mg/dL. Which of the following findings is most likely to be typical in this patient?;A. Bilateral adrenal hyperplasia ;B. Enlargement of all four parathyroid glands ;C. Functional parathyroid adenoma ;D. Hyperplasia of the adenohypophysis ;E. Hyperplasia of the thyroid follicular cells Option B (enlargement of all four parathyroid glands) is correct. The patient has secondary hyperparathyroidism associated with chronic renal failure. In such cases; the hypocalcemia caused by renal retention of phosphate leads to compensatory hyperplasia of the parathyroid glands. Typically; all four parathyroid glands are enlarged. Q3245:Two months ago; a 19-year-old man was brought to the emergency department following an automobile accident in which he sustained severe lacerations and a ruptured spleen. He immediately received four units of packed red blood cells. He now has developed mild jaundice. Except for vague symptoms of fatigue; he is generally asymptomatic. Both his ALT (alanine aminotransferase) and AST (aspartate aminotransferase) are 40 U/L. His alkaline phosphatase is within normal limits. Which of the following types of hepatitis is most likely to have caused this infection?;A. Hepatitis A (HAV) ;B. Hepatitis B (HBV) ;C. Hepatitis C (HCV) ;D. Hepatitis D (HDV) ;E. Hepatitis E (HEV) Option C (hepatitis C) is correct. HCV has become the major cause of transfusion-related hepatitis since adequate screening methods for HBV have been developed. HCV was previously referred to as non-A; non-B hepatitis or chronic active hepatitis. Q3246:For the past several weeks; a 39-year-old woman has experienced fatigue; weakness; poor appetite; and weight loss. Physical examination shows hyperpigmentation of the skin and dark patches on the mucous membranes. Laboratory studies show her serum sodium level is 125 mEq/dL; and her serum potassium level is 6.0 mEq/dL. Which of the following is the most likely diagnosis?;A. Atrophic adrenals with dense lymphocytic infiltrate ;B. Carcinoid tumor of the vermiform appendix ;C. Functional follicular adenoma of the thyroid ;D. Granulosa cell tumor of the right ovary ;E. Hyperplasia of all four parathyroid glands Option A (atrophic adrenals with dense lymphocytic infiltrate) is correct. This patient has Addison's disease; as suggested by the fatigue; weakness; poor appetite; and weight loss. Other characteristics include hyperpigmentation of the skin and dark patches on the mucous membranes. In developed countries; the most common cause of Addison's disease is autoimmune destruction of the adrenals; suggested by lymphoid infiltrates in the adrenal glands plus circulating antiadrenal antibodies. In developing countries; tuberculosis would also be a major cause. Q3247:For the past 6 months; an 18-year-old woman has had diarrhea; a fever; and cramping and left lower quadrant pain. Flexible sigmoidoscopy shows mucosal ulceration in the sigmoid colon. A biopsy shows transmural inflammation with occasional granulomas. Which of the following would differentiate Crohn's disease from ulcerative colitis?;A. History of fever ;B. History of prolonged diarrhea ;C. Involvement limited to the colon ;D. Presence of gross mucosal ulcerations ;E. Presence of transmural inflammation with granular formation Option E (presence of transmural inflammation with granular formation) is correct. Ulcerative colitis is essentially a disease of the mucosa; whereas Crohn's disease involves all layers of the bowel wall. A biopsy shows that this patient has transmural inflammation with occasional granulomas. Q3248:A 56-year-old man with a history of gastric ulceration and Helicobacter pylori infection has a lesion involving the stomach wall. A biopsy shows that the lesion is a mucosa- associated lymphoid tissue (MALT) lymphoma. Which of the following is the most likely source of this tumor?;A. B cells ;B. Histiocytes ;C. Natural killer (NK) cells ;D. T cells Option A (B cells) is correct. Mucosa-associated lymphoid tissue (MALT) lymphomas are low-grade B-cell lymphomas. Gastric MALT lymphoma has a strong association with H. pylori infection. Q3249:. A 18-year-old woman with mildly icteric sclerae has a total bilirubin of 5.5 mg/dL and a direct bilirubin of 0.4 mg/dL. She has no associated symptoms; and no history of exposure to hepatotoxins; blood products; or persons with known hepatitis. A brother is unaffected; but an uncle has a similar condition. This patient most likely has which of the following congenital hyperbilirubinemias? ;A. Crigler-Najjar syndrome; type I ;B. Crigler-Najjar syndrome; type II ;C. Dubin-Johnson syndrome ;D. Gilbert syndrome ;E. Rotor's syndrome Option D (Gilbert syndrome) is correct. Patients with this mild; inherited form of conjugated hyperbilirubinemia are healthy and have no functional or structural evidence of liver disease; except for an increase in unconjugated bilirubin. Q3250:A 51-year-old man with an 8-year history of cirrhosis has a mass lesion in the left lobe of the liver. A needle biopsy determines the lesion is hepatocellular carcinoma (HCC). In the United States; development of HCC is most commonly associated with which of the following?;A. Alcoholism ;B. Exposure to aflatoxin ;C. Hepatitis C (HCV) infection ;D. Microvesicular steatosis ;E. Neonatal hepatitis Option C (hepatitis C infection) is correct. HCV infection is most commonly associated with hepatocellular carcinoma. There is a marked reduction of cases of hepatitis B (HBV) in the United States due to the screening of blood used for transfusions. Q3251:A 12-year-old girl has metabolic acidosis and severe ketosis. Her blood glucose level is 460 mg/dL. Serologic studies for antibodies against b cells show a high titer of anti b cell antibodies. Which of the following is most significant concerning the finding of a high titer of these antibodies?;A. Are a serum marker for the destruction of b cells ;B. Are an indication of the development of type 2 diabetes mellitus ;C. Have caused the destruction of the b cells in the pancreatic islets;D. Form a complex with insulin that causes hyperglycemia Option A (are a serum marker for the destruction of b cells) is correct. It is currently thought that anti b cell antibodies arise from antigens released into the blood by the destruction of b cells; probably by cytotoxic T cells. Thus; they are a marker; not a cause; of b cell injury. Q3252:A 62-year-old woman with bilateral palpable masses in her abdomen has a creatinine level of 3.7 mg/dL and her blood urea nitrogen (BUN) is 32 mg/dL. During the past year; she has had hematuria with occasional small blood clots in her urine. Which of the following is the most likely diagnosis?;A. Medullary sponge kidney ;B. Nephronophthisis ;C. Polycystic kidney disease; autosomal dominant ;D. Polycystic kidney disease; autosomal recessive ;E. Renal dysplasia Option C (polycystic kidney disease; autosomal dominant) is correct. This clinical scenario of bilateral palpable abdominal masses and marked increase in creatine and BUN represents a fairly classic presentation of autosomal dominant (adult) polycystic disease. Q3253:A 24-year-old woman has painful urination with frequency and urgency. A physical examination shows an increase in heart rate and a temperature of 39.2\\'b0C (102.5\\'b0F). She appears ill and somewhat diaphoretic. A urine specimen is grossly red; and red blood cells (RBCs) are seen on microscopic examination. A urinalysis shows bacteria; polymorphonuclear leukocytes; and leukocyte casts. A nitrite test on a urine dipstick is positive. Which of the following is the most likely diagnosis?;A. Acute pyelonephritis ;B. Acute tubular necrosis ;C. Cystitis ;D. Nephrotic syndrome Option A (acute pyelonephritis) is correct. Leukocyte casts are formed only in the kidneys; and the presence of these casts is ample evidence of an infection of the renal parenchyma; characteristic of acute pyelonephritis. Q3254:A urinalysis of an asymptomatic 59-year-old man shows microscopic hematuria and mild prostatic hypertrophy. A CT scan of the abdomen shows a large mass in the left kidney. A needle biopsy confirms a diagnosis of renal cell carcinoma; clear-cell type. Radical nephrectomy indicates that the tumor appears to have invaded the man's left renal vein. If the tumor has metastasized; the most likely site of metastasis is;A. adrenal glands ;B. bones ;C. liver ;D. lungs ;E. regional lymph nodes Option D (lungs) is correct. Renal cell carcinoma is angioinvasive and tends to disseminate via the vascular system early in its history. If metastases occur; the lungs are involved in more than 50% of cases. Q3255:A 16-year-old girl whose mother was given diethylstilbestrol (DES) during her pregnancy because of threatened abortion wants to be evaluated in anticipation of problems associated with her mother's medical history. Which of the following conditions would represent a risk as a result of maternal use of DES?;A. Adenocarcinoma of the Bartholin's glands ;B. Adenocarcinoma of the endometrium ;C. Adenocarcinoma of the vagina ;D. Squamous cell carcinoma of the cervix ;E. Squamous cell carcinoma of the vulva Option C (adenocarcinoma of the vagina) is correct. A small percentage of female children born to women treated with DES (< 0.14%) eventually develop adenocarcinoma of the vagina. The tumor is usually detected between 15 and 20 years of age. The tumor cells contain abundant glycogen producing a so-called clear-cell carcinoma. Q3256:A 30-year-old woman fears that she will develop ovarian cancer because of her family history and is tested for the BRCA1 gene. This gene has a strong association with which of the following ovarian neoplasms?;A. Dysgerminoma ;B. Mixed M\\'fcllerian tumor ;C. Serous cystadenocarcinoma ;D. Yolk sac tumor Option C (serous cystadenocarcinoma) is correct. In women who are positive for the BRCA1 gene; the vast majority of ovarian tumors are serous cystadenocarcinomas. A woman who has a family history of ovarian cancer would be at risk for carrying the BRCA1 gene. Q3257:A 50-year-old woman with an infiltrating ductal carcinoma of the breast has a lumpectomy; followed by adjuvant radiation and chemotherapy. Tissue is sent to the laboratory to determine if any prognostic factors might be present in this woman. Which of the following findings would be associated with an extremely poor prognosis for this patient?;A. Estrogen receptors ;B. HER-2 neu amplification ;C. Mutated p53 oncoprotein ;D. Progesterone receptors Option B (HER-2 neu amplification) is correct. Tumors with oncogene amplification (HER-2 neu) tend to have a less favorable prognosis than those without this factor. Q3258:A 55-year-old slightly obese woman has had several bouts of intense right upper quadrant pain and now has scleral icterus. A cholecystectomy shows numerous spongy; laminated brown stones in the gallbladder. Brown gallstones are almost always associated with which of the following conditions?;A. Alcoholic cirrhosis ;B. Escherichia coli infection of the gallbladder ;C. History of mild b-thalassemia ;D. Hypercholesterolemia Option B (Escherichia coli infection of the gallbladder) is correct. Brown gallstones are almost always associated with E. coli cholecystitis. Bacteria may secrete b-glucuronidase; which hydrolyzes conjugated bilirubin to unconjugated bilirubin. The increase in concentration of unconjugated bilirubin is believed to produce the stones. Q3259:An endometrial biopsy of a 47-year-old woman with a history of irregular vaginal bleeding shows a well- differentiated adenocarcinoma of the endometrium. A hysterectomy confirms tumor involvement of the corpus of the uterus and the cervix. Which of the following is a known risk factor for the development of endometrial carcinoma?;A. History of anovulatory menstrual cycles ;B. History of cervical human papillomavirus (HPV) infection ;C. Multiple pregnancies ;D. Thin body habitus Option A (history of anovulatory menstrual cycles) is correct. A history of irregular vaginal bleeding corroborates anovulatory menstrual cycles and is strongly associated with the development of endometrial cancer; probably because of prolonged estrogen stimulation. Q3260:A 40-year-old woman has enlarged lymph nodes in the axillae; groin; and cervical triangles. Biopsy of an axillary node shows complete effacement of the architecture of the nodes by nodular aggregates of lymphoma cells. Which of the following is the cell line of origin of this type of lymphoma?;A. B cell ;B. CD4+ T cell ;C. CD8+ T cell ;D. Histiocyte ;E. Natural killer (NK) cell Option A (B cell) is correct. The woman has follicular (nodular) lymphoma; a non-Hodgkin's (NHL) lymphoma that expresses B cell cluster of differentiation (CD) markers. All follicular (nodular) lymphomas are of B cell lineage. Q3261:A 64-year-old woman with a saccular aneurysm of the ascending aorta has ataxia and loss of joint position sense. She confabulates when the physician attempts to obtain a history. Which of the following organisms is capable of producing this constellation of findings?;A. Chlamydia trachomatis ;B. Haemophilus ducreyi ;C. Neisseria gonorrhoeae ;D. Treponema pallidum ;E. Trichomonas vaginalis Option D (Treponema pallidum) is correct. An aortic aneurysm with ataxia and impaired proprioception are characteristic of the tertiary stage of syphilis. The primary stage involves a painless genital ulcer and regional lymphadenopathy; and the secondary stage involves skin and mucosal lesions as well as possible meningeal; hepatic; renal; bone; and joint invasion. Q3262:When asked to speak to a women's group about risk factors for the development of breast cancer; the physician should tell the group that the histopathologic finding that carries the strongest risk factor for developing breast cancer is;A. apocrine metaplasia ;B. atypical lobular hyperplasia ;C. epithelial hyperplasia ;D. sclerosing adenosis Option B (atypical lobular hyperplasia) is correct. Atypical lobular hyperplasia is a moderate risk factor for the development of breast cancer. Q3263:A 51-year-old man has adenocarcinoma of the lesser curvature. Which of the following is the most important prognostic factor when evaluating a patient with this type of tumor?;A. Degree of differentiation ;B. Depth of invasion ;C. Gross growth pattern ;D. Histologic subtype of the carcinoma ;E. Presence of Helicobacter pylori Option B (depth of invasion) is correct. The morphologic feature with the greatest effect on clinical outcome in adenocarcinoma of the lesser curvature is depth of tissue invasion. Patients with carcinoma in the early stages (limited to the mucosa and submucosa) have a far better prognosis (90% survival with surgery) than patients with advanced carcinoma (<10% survival with surgery). Q3264:Individuals taking phenobarbital may be more susceptible to free radical injury by toxins such as carbon tetrachloride (CCl4). Phenobarbital induces which of the following subcellular changes? ;A. Additional receptor sites for CCl4 on the cell membrane of the hepatocytes;B. Atrophy of the Golgi zone within the hepatocytes ;C. Atrophy of the smooth endoplasmic reticulum (SER) within the;hepatocytes ;D. Increased formation of G proteins within the hepatocytes ;E. Marked hypertrophy of the SER within the hepatocytes Option E (marked hypertrophy of the SER within the hepatocytes) is correct. Phenobarbital induces hypertrophy of the SER and hence a marked increase in cytochrome p450 activity. The conversion of CCl4 into the toxic free radical CCl3 by the cytochrome p450 system causes the injury. Q3265:A 67-year-old retired airline pilot slipped on the ice; striking his head on the sidewalk. His history is unremarkable. A CT scan of the head shows no sign of hematoma; but does show atrophy of the cerebral hemispheres. The atrophy is most likely due to;A. decreased daily workload ;B. diminished blood supply ;C. inadequate nutrition ;D. loss of endocrine stimulation ;E. loss of innervation Option B (diminished blood supply) is correct. Atherosclerosis leads to reduced blood flow to the brain and is thought to be the major culprit in the etiology of cerebral atrophy in the elderly. Q3266:A 50-year-old man who has smoked two packs of cigarettes a day for 30 years has squamous metaplasia of the respiratory epithelium. His physician tells him that smoking is a major cause of metaplasia; but there are other causes for this change. Which of the following may also cause squamous metaplasia of bronchial mucosa?;A. Deficiency of folate ;B. Deficiency of vitamin A ;C. Excess vitaminB2 ;D. Excess vitamin C ;E. Excess vitamin D Option B (deficiency of vitamin A) is correct. Vitamin A and retinoids are necessary in maintaining the differentiation of epithelial cells. Deficiency of vitamin A causes squamous metaplasia of the respiratory tract epithelium as well as impaired vision; night blindness; and xerophthalmia. Q3267:When an acute inflammatory reaction develops in response to an injurious stimulus; endothelial cells and macrophages produce nitric oxide (NO). During acute inflammation; NO functions to ;A. cause pain ;B. induce smooth muscle contraction ;C. opsonize bacteria ;D. produce fever ;E. promote vasodilation Option E (promote vasodilation) is correct. NO is produced by nitric oxide synthase and is somewhat cytotoxic. NO causes the relaxation of vascular smooth muscle; leading to vasodilation. Q3268:A 61-year-old woman with a lengthy history of hypertension dies of an acute myocardial infarction (MI). At autopsy; the heart shows gross hypertrophy of the left ventricle. The adaptive mechanism responsible for the increase in the mass of the ventricle is most likely;A. fusion of individual muscle fibers into larger; stronger units ;B. increased cycling of cells out of G 0 ;1 phase ;C. increased individual cell size; with no mitotic activity ;D. increased mitotic rate and the production of more cells ;E. primarily an increase in individual cell size; with normal mitotic activity Option C (increased individual cell size; with no mitotic activity) is correct. The increase in cardiac and skeletal mass is due to an increase in the size of individual muscle fibers. These cells are not capable of significant mitotic activity. Q3269:Several weeks after sustaining a gunshot wound; the bullet is surgically removed from the shoulder of a 20-year-old man. Histologically; the lesion resembles a chronic inflammatory infiltrate; with numerous macrophages surrounding the bullet. In the chronic inflammatory response; macrophages are especially important because they are;A. capable of local proliferation ;B. easily recruited from the circulation ;C. involved in the production of numerous biologically active substances ;D. permanently localized to the site of inflammation Option C (involved in the production of numerous biologically active substances) is correct. Macrophages produce an abundance of biologically active substances (e.g; toxic free radicals; proteases; cytokines; growth factors; and angiogenesis factor); and all are involved in chronic inflammation. Q3270:A 30-year-old black woman who resides in North Carolina develops increasing shortness of breath. A physical examination shows lymphadenopathy in the axillae and groin. X-ray film of the chest shows a marked degree of hilar lymph node enlargement. A biopsy of an enlarged axillary lymph node shows numerous noncaseating granulomas. No organisms are identifiable; and acid-fast stains are negative. These findings are most likely caused by which of the following?;A. Coccidioidomycosis ;B. Cryptococcosis ;C. Histoplasmosis ;D. Sarcoidosis ;E. Tuberculosis Option D (sarcoidosis) is correct. Sarcoidosis typically produces noncaseating ("hard") granulomas; rather than lesions characterized by central caseous necrosis. In addition; multisystem involvement; including skin; lungs; lymph nodes; liver; spleen; eyes; and the small bones of the hand and feet; is typical. Sarcoidosis occurs mainly in individuals between the ages of 20 and 40 years; risk is higher in the black population. Q3271:A 63-year-old man has a history of congestive heart failure due to severe arteriosclerosis of the coronary arteries. He now has pitting edema of the ankles that extends about halfway up the lower part of his legs. The edema in this patient is most likely caused by which of the following mechanisms?;A. Decreased oncotic pressure in the vascular compartment ;B. Impaired lymphatic drainage of the legs ;C. Increased hydrostatic pressure in the vascular compartment ;D. Increased oncotic pressure in the extracellular compartment ;E. Increased vascular permeability Option C (increased hydrostatic pressure in the vascular compartment) is correct. In this patient; the increased pressure is the main cause of dependent edema. The failing right side of the heart (right atrium and ventricle) has led to an increase in intravascular hydrostatic pressure; which eventually causes a net movement of fluid from the vascular space to the extravascular space. Q3272:An 18-year-old man scheduled to undergo surgery to repair a hernia has a history of easy bruising and a tendency to bleed for an abnormally long time after an injury. Laboratory studies show prolonged bleeding time; normal prothrombin time; and slightly prolonged partial thromboplastin time. Which of the following is the most specific laboratory test that would confirm the suspected diagnosis of von Willebrand's disease?;A. Clot retraction test ;B. Factor VIII assay ;C. Ristocetin aggregation test ;D. Thrombin time Option C (ristocetin aggregation test) is correct. Ristocetin binds to platelets; activating von Willebrand's factor (vWF) receptors on the surface. If vWF is present; it forms a bridge between receptors on different platelets; causing platelet aggregation. Q3273:A 40-year-old man who was involved in an automobile accident is being treated for a fracture of the pelvis. The man is at increased risk of developing pulmonary emboli because of relative stasis of venous flow in his legs. Which of the following most accurately describes these emboli?;A. Most are clinically silent ;B. They are rare ;C. They commonly cause chronic pulmonary hypertension ;D. They typically cause pulmonary infarction ;E. They usually cause immediate death Option A (most are clinically silent) is correct. The majority of pulmonary emboli (60\\'9680%) are small; thus; they are clinically silent. Autopsy series are the source of most of the data; and the frequency of finding emboli tends to be related to the diligence of the pathologist. Q3274:A 54-year-old woman with severe right upper quadrant pain; fever; and jaundice is in shock when brought to the emergency department. A blood culture grows Escherichia coli. The presumptive diagnosis is acute cholecystitis. The most likely cause of shock is ;A. anaphylactic reaction ;B. cardiogenic shock ;C. endotoxemia ;D. hemorrhage ;E. neurogenic shock Option C (endotoxemia) is correct. Endotoxin (lipopolysaccharide); a component of the cell walls of gram- negative bacteria; binds to CD14 receptors. It causes a cascade of events that result in shock; metabolic failure; disseminated intravascular coagulopathy; and eventually multiple organ failure. Q3275:A 46-year-old woman delivers a newborn with the physical features associated with Down's syndrome (trisomy 21). A FISH (fluorescent in situ hybridization) assay shows the presence of three number 21 chromosomes. The most common mechanism causing trisomy 21 is;A. failure of lyonization ;B. formation of a ring chromosome ;C. meiotic nondisjunction of chromosome 21 in the ovum ;D. mitotic nondisjunction of chromosome 21 early in embryogenesis ;E. robertsonian translocation of the long arm of chromosome 21 to an acrocentric chromosome Option C (meiotic nondisjunction of chromosome 21 in the ovum) is correct. Down's syndrome typically affects children born of women over 35 years of age; and is of maternal origin but of undetermined etiology. The most common mechanism of trisomy 21 (>90% of cases) involves the meiotic nondisjunction of chromosome 21. Q3276:A 27-year-old man is a diagnosed with a diffuse large cell lymphoma. Genetic analysis of DNA from the lymphoma cells shows rearrangement of the immunoglobulin genes. Which of the following is the most likely origin of this lymphoma? ;A. B-cell lymphoma ;B. Histiocytic lymphoma ;C. Natural killer (NK) cell lymphoma ;D. T-cell lymphoma Option A (B-cell lymphoma) is correct. Somatic rearrangement of the immunoglobulin genes marks this lesion as a lymphoma of B cell origin. Lymphoma is a monoclonal lesion; so all of the tumor cells exhibit identical patterns of immunoglobulin gene rearrangement. Q3277:A 32-year-old woman has a family history of a high incidence of neoplasia involving multiple organ systems. She undergoes genetic screening for several tumor suppressor genes and oncogenes and is found to have Li-Fraumeni syndrome. The genetic defect associated with this syndrome is loss of one allele of which of the following genes?;A. bcl-2 ;B. NF-1 ;C. P53 ;D. RB ;E. WT1 Option C (P53) is correct. Loss of one of the alleles of the P53 gene is the basis of the Li-Fraumeni syndrome. Patients with this syndrome have an increase in the development of malignant neoplasms arising in various anatomic locations. P53; a tumor suppressor gene that also has oncogene-like properties when mutated; produces an abnormal gene product that is not degraded with normal speed. Patients with a mutated or missing P53 gene have an increased risk of developing malignancies in various tissues. Q3278:The mechanism thought to be responsible for the increasingly aggressive behavior of malignant tumors is that they;A. are genetically labile ;B. cause the production of tumor necrosis factor-a (TNF-a) ;C. induce angiogenesis ;D. produce paraneoplastic syndromes Option A (are genetically labile) is correct. Malignant tumors are constantly mutating and forming new clones. Many of the clones die; but some will have a selective advantage with regard to growth; invasion; or metastasis. Q3279:A 32-year-old man has had vague abdominal discomfort; bloating; and diarrhea since returning from a camping trip 2 weeks ago where he routinely drank mountain stream water. Stool smears show triangular-shaped organisms containing two nuclei. Which of the following is the most likely cause of the diarrhea?;A. Balantidium coli ;B. Entamoeba histolytica ;C. Giardia lamblia ;D. Vibrio cholerae Option C (Giardia lamblia) is correct. G. lamblia is the most prevalent pathogenic intestinal protozoan. It attaches to the mucosa rather than invading it; thus; it tends to cause irregular (intermittent) watery diarrhea rather than dysentery. In some patients; it causes constipation; in others; it causes steatorrhea. Symptoms of giardiasis generally occur 1\\'963 weeks after infection. Q3280:A 50-year-old woman who has been taking estrogen daily; without interruption; for a long period of time is at significant risk for the development of ;A. endometrial adenocarcinoma ;B. endometrial atrophy ;C. enlarged corpora lutea ;D. ovarian teratoma Option A (endometrial carcinoma) is correct. The risk for development of endometrial adenocarcinoma is about 1.7\\'962.0 times the normal rate in patients who take unopposed estrogen on a continuous basis; without interruption. Q3281:A 17-year-old girl with Hodgkin's disease was treated successfully with aggressive chemotherapy; and the disease has been in remission for 2 years. She is now at increased risk for the development of a second malignant tumor. Which of the following changes is most likely caused by antineoplastic agents?;A. Depression of immune surveillance ;B. Depression of the mononuclear phagocyte system ;C. Induction of nonlethal mutations ;D. Induction of telomerase ;E. Promotion of tumor angiogenesis Option C (induction of nonlethal mutations) is correct. Most antineoplastic agents act on dividing cells at some specific point in the cell cycle. In damaging the reproductive function of the cell; accumulating nonlethal mutations may occur; which may result in the formation of a second neoplasm. Q3282:An 18-year-old college student who was seen drinking heavily at a party is found in his room completely unresponsive. When he is admitted to the emergency department; his blood alcohol level is 325 mg/dL. In a nonhabituated drinker; this level of blood alcohol is associated with;A. coma and respiratory collapse ;B. congestive cardiomyopathy ;C. development of acute yellow atrophy of the liver ;D. Korsakoff's syndrome ;E. Wernicke's encephalopathy Option A (coma and respiratory collapse) is correct. Alcohol acts as a central nervous system (CNS) depressant via mechanisms that are still unclear; but possibly by action on GABA receptors. At alcohol levels of 300\\'96400 mg/dL; coma and respiratory collapse are likely. Individuals who chronically abuse the substance will tolerate higher levels. Q3283:A 47-year-old man is brought to the emergency department after being exposed to an unknown dose of radiation due to malfunction of the industrial radiographic unit he operates. His coworkers think he may have received whole-body radiation exposure. Which of the following would be most sensitive to radiation exposure?;A. Bladder epithelium ;B. Cartilage ;C. Hair follicles ;D. Kidney ;E. Lymphoid tissue Option E (lymphoid tissue) is correct. Lymphoid tissue; hematopoietic tissue; spermatogonia; and ovarian follicles are all highly sensitive to radiation. Q3284:A 24-year-old man has a history of fever; weight loss; hypertension; and abdominal pain. Stool and urinalysis are positive for the presence of blood. Several nodular skin lesions are found. A biopsy shows necrotizing arteritis involving medium-sized muscular arteries. This constellation of findings is most suggestive of;A. Kawasaki disease ;B. polyarteritis nodosa ;C. syphilis ;D. systemic lupus erythematosus (SLE) ;E. Takayasu's arteritis Option B (polyarteritis nodosa) is correct. Necrotizing inflammation of affected arteries is characteristic of polyarteritis nodosa; a disease that typically involves medium-sized muscular arteries. This disease may involve fever and weight loss; and hypertension is common. Gastrointestinal lesions are the source of the abdominal pain. The disease may affect individuals of any age. Q3285:A 52-year-old man complains of general malaise. Physical examination shows an enlarged liver; and an MRI shows a mass in the left lobe of the liver. A liver biopsy confirms the mass as an angiosarcoma. The patient most likely has been exposed to which of the following toxins?;A. Carbon tetrachloride (CCl4) ;B. b-naphthylamine ;C. Nickel carbonyl ;D. Organophosphates ;E. Polyvinyl chloride Option E (polyvinyl chloride) is correct. Polyvinyl chloride; a known carcinogen; is associated with the development of hepatic angiosarcoma. Q3286:A 48-year-old man comes to the emergency department because of severe chest pain. He is 5 ft 8 in tall and weighs about 90 kg (200 lb). He describes the pain as sudden in onset; producing a sensation of "tearing." Nitroglycerin spray provides no relief. An ECG shows no changes suggestive of an acute myocardial infarction (MI). Angiography shows a dissecting aneurysm of the aorta. The most likely antecedent condition associated with development of a dissecting aneurysm is;A. hypertension ;B. Marfan's syndrome ;C. severe atherosclerosis ;D. syphilis ;E. systemic lupus erythematosus (SLE) Option A (hypertension) is correct. Over 90% of patients who develop a dissecting aneurysm; and who have neither Marfan's syndrome nor a localized connective tissue disorder; are hypertensive. Q3287:A 29-year-old woman has a soft midsystolic click on auscultation of the heart. Ultrasound examination shows a mitral valve prolapse. The most likely eventual outcome of this condition is ;A. embolism by platelet aggregates ;B. mitral valve incompetence ;C. mitral valve stenosis ;D. normal life Option D (normal life) is correct. A mitral value prolapse generally is benign. About 97% of female patients with the condition experience no significant untoward effects. Q3288:As a result of the decreased incidence of patients with chronic rheumatic fever; consequent damage to the cardiac valves has diminished. The most common antecedent condition leading to infectious myocarditis presently is;A. congenital heart disease;B. endocardial trauma;C. hypercoagulable state;D. leukemia;E. mucin-producing adenocarcinoma Option A(congenital heart disease) is correct. Rheumatic heart disease was once considered the most common condition that could lead to infectious endocarditis. Presently; congenital lesions; particularly those that produce a "jet" effect such as a small ventricular septal defect; are more common and are a risk factor for infective endocarditis. Q3289:A 63-year-old woman with a history of type 2 diabetes mellitus and an increasing exercise limitation is admitted to the hospital with severe influenza pneumonitis. She subsequently develops adult respiratory distress syndrome (ARDS) and dies 4 days after admission. Autopsy shows the heart encased in a 1-cm dense collagenous scar. Which of the following is the most likely cause of constrictive pericarditis?;A. Cryptococcosis ;B. Histoplasmosis ;C. Idiopathic ;D. Oat cell carcinoma ;E. Tuberculosis Option C (idiopathic) is correct. A well-defined history of a previous suppurative; hemorrhagic; or caseous pericarditis is often absent in the patient who dies of constrictive pericarditis. Q3290:A 72-year-old woman complains of feeling weak. Laboratory studies show a hematocrit of 18%; macrocytosis; hypersegmentation of neutrophils; and occasional giant platelets. Her lactate dehydrogenase (LDH) level is 2950 U/L; and her serum vitamin B12 is 27 pg/mL. Which of the following conditions most likely caused the increase in LDH? ;A. Cardiomyopathy ;B. Hypersplenism ;C. Ineffective erythropoiesis ;D. Intravascular hemolysis ;E. Liver failure Option C (ineffective erythropoiesis) is correct. Patients with vitamin B12 deficiency have hyperplasia of the red blood cell (RBC) elements in the bone marrow; but the developing RBCs are destroyed in the marrow before they can be released into the circulation. When RBCs are destroyed; they release their large quantity of LDH into the plasma. Q3291:A 22-year-old medical student volunteers for a hematology research project and is found to be lacking one of four a-globin genes. Previously; he had several hematologic studies that were within normal limits. The significance of this unexpected finding suggests that the student;A. has a moderate degree of erythroid hyperplasia in bone marrow ;B. has an abnormal hemoglobin concentration ;C. has numerous target cells in the peripheral blood ;D. is an asymptomatic carrier of a-thalassemia ;E. may develop severe hemolytic anemia if exposed to oxidant drugs Option D (is an asymptomatic carrier of a-thalassemia) is correct. Deletion of one of the four a-globin genes produces a silent carrier state; and no hematologic abnormalities are evident. One of the chromosome 16 genes carries this deficiency. If such an individual has offspring with another carrier; the children could have the a-thalassemia trait. There are four functional a-globin genes; thus; there are four possible degrees of a-thalassemia (based on the loss or one to four genes). This individual has the mildest form; the most severe form is associated with fetal death in utero. Q3292:A 36-year-old man has generalized lymphadenopathy. A histologic examination of a lymph node shows a marked degree of follicular hyperplasia. Which of the following findings would best determine that this condition is a benign reactive process rather than neoplastic?;A. Polyclonality on DNA hybridization studies ;B. Presence of broad increase in IgG on high-resolution electrophoresis ;C. Presence of surface IgG on the cell membrane of the proliferating cells ;D. Presence of translocation t (14;18)(q32;q21) Option A (polyclonality on DNA hybridization studies) is correct. Polyclonality is a sign of a benign process. Lymphoma is the result of monoclonal expansion of a single cell that has become malignant. Q3293:A patient has vague symptoms of weakness and fatigue and decreased exercise tolerance. Laboratory studies show a leukocyte count of 11;000/mm with 7% blasts on the peripheral smear. Bone marrow aspirate shows 40% myeloblasts. Acute myeloblastic leukemia is suspected. This patient is most likely between the ages of;A. birth to 2 years;B. 2-10 year;C. 10-15 years;D. 15-40 years;E. >40 years Option D is correct. Acute myeloblastic leukemia (AML) is primarily a disease of young adults. Q3294:A 67-year-old woman with a 15-year history of severe coronary artery disease and multiple infarcts of the left ventricle and interventricular septum is hospitalized with acute pulmonary edema. Which of the following conditions is the primary physiologic event leading to pulmonary edema?;A. Decreased plasma oncotic pressure ;B. Left-sided congestive heart failure ;C. Lymphatic obstruction ;D. Right- sided congestive heart failure ;E. Volume overload Option B (left-sided congestive heart failure) is correct. Failure of the left ventricle causes increased pressure in the pulmonary circulation; which leads to a net movement of fluid from the vascular to the extravascular compartment (Starling's hypothesis). Q3295:A 29-year-old man is brought to the emergency department after sustaining third-degree burns over 60% of his body. Twenty-four hours later; he develops adult respiratory distress syndrome (ARDS). Which of the following physiologic features would suggest a diagnosis of restrictive lung disease in this patient?;A. Decreased PaO2 ;B. Decreased pulmonary elastic recoil ;C. Decreased total lung volume ;D. Increased PaCO2;E. Increased pulmonary blood flow Option C (decreased total lung volume) is correct. All of the restrictive lung diseases; including ARDS; have a unifying physiologic abnormality\\: reduction in total lung capacity. This is often due to parenchymal lung disease; but it can also be the result of neuromuscular disease; skeletal diseases; pleural disease; and acute trauma such as bilaterally fractured ribs that produce a flail chest. Q3296:A 12-year-old child develops wheezing and dyspnea after petting a cat. The child is suffering from which of the following forms of asthma?;A. Allergic bronchopulmonary aspergillosis ;B. Atopic asthma ;C. Intrinsic nonreaginic asthma ;D. Intrinsic pharmacologic asthma ;E. Occupational asthma Option B (atopic asthma) is correct. Atopic; or allergic; asthma involves a type I hypersensitivity reaction. The binding of an inhaled specific allergen; such as cat dander; to IgE on the surface of a mast cell induces an attack of typical atopic asthma. Q3297:A 17-year-old boy works for a contractor sandblasting painted concrete surfaces during his summer vacation. In a dusty environment such as this; which of the following sizes of potentially inhaled dust particles has the greatest capacity for causing pulmonary injury?;A. <1 mm ;B. 1mm ;C. 5mm ;D. 10mm ;E. >20mm Option B (1 mm) is correct. Dust particles within the size range of 1mm have great potential for causing pulmonary injury. Bacteria fit nicely into this size range. Shape is also an important factor in considering capacity for pulmonary injury. Although objects such as asbestos fibers are quite long; they can also gain access to the terminal airways and alveoli because they are thin. Q3298:A 67-year-old woman is admitted to the hospital with symptoms of pleuritic chest pain; cough productive of rusty looking sputum containing innumerable neutrophils; and a temperature of 39.2\\'b0C (102.5\\'b0F). She is an alcoholic. X-ray film of the chest shows that most of the right middle lobe is opacified. Which of the following is the most likely cause of the infection?;A. Haemophilus influenzae ;B. Klebsiella pneumoniae ;C. Mycoplasma pneumoniae ;D. Staphylococcus aureus ;E. Streptococcus pneumoniae Option E 9Streptococcus pneumoniae) is correct. The x-ray film of the chest shows the presence of an intra-alveolar exudate with lobar or segmental consolidation;S. pneumoniae (pneumococcus) accounts for 90-95% of cases of lobar pneumonia. Alcoholism is a major risk factor for pneumonia and can lead to increased morbidity and mortality. Q3299:A 69-year-old man sees his physician because of increasing shortness of breath. X-ray film of the chest shows that most of the right lung is encased by a thick layer of moderately dense tissue. Pleural fluid is blood-tinged with numerous atypical cells. The patient is most likely to have a history of occupational exposure to which of the following substances?;A. Asbestos ;B. Formalin ;C. Hematite ;D. Nickel carbonyl ;E. Silica Option A (asbestos) is correct. About 90% of malignant mesotheliomas are related to occupational exposure to asbestos. Individuals who have been exposed to asbestos have an 8% risk of developing malignant mesothelioma. There is a long latent period; with the tumor appearing from about 20-40 years after exposure to the substance. Q3300:A 65-year-old woman is undergoing a physical examination when the physician notices her mucous membranes are somewhat pale and her tongue is beefy red to magenta colored. There is slight cracking of the skin around the angles of her mouth; and her fingernails are slightly spoon- shaped. She has difficulty swallowing. Laboratory studies show a red blood cell count of 12 mL/kg and a hematocrit of 30%. The most likely cause of this patient's condition is a deficiency of;A. iron ;B. niacin ;C. pyridoxine ;D. riboflavin ;E. vitamin B12 Option A (iron) is correct. The history and physical findings suggest that the woman has Plummer-Vinson syndrome; which is characterized by iron-deficiency anemia; glossitis; cheilosis; and dysphagia due to formation of upper esophageal webs. Q3301:A 43-year-old woman sees her physician because of a swollen gland on the left side of her neck. Examination shows a firm mass in the left parotid gland. On palpation; the mass does not move with the overlying skin. The most likely diagnosis is;A. acinic cell carcinoma ;B. adenoid cystic carcinoma ;C. mucoepidermoid carcinoma ;D. pleomorphic adenoma ;E. Warthin's tumor Option D (pleomorphic adenoma) is correct. This benign tumor accounts for about 50% of salivary gland tumors. The location of the tumor (in the left parotid gland) and the sex of the patient (female) increase the likelihood of the tumor being a pleomorphic adenoma. Q3302:A 54-year-old man with a history of gastroesophageal reflex disease (GERD) is prescribed a proton pump inhibitor. He says he cannot afford the medication and is unable to adhere to the recommended treatment regimen; but instead relies on over-the-counter medications such as antacids and herbal remedies. Brush cytology of the esophagus shows marked reactive changes of the squamous cells as well as the presence of reactive glandular cells consistent with origin in the body of the stomach. This patient is most seriously at risk of developing ;A. adenocarcinoma ;B. esophageal candidiasis ;C. esophageal stenosis ;D. esophageal varices ;E. ulceration of the esophageal mucosa Option A (adenocarcinoma) is correct. The presence of glandular cells in the esophageal brushing indicates that the patient has glandular metaplasia of the esophageal mucosa; or Barrett's esophagus (metaplastic columnar epithelium); a complication of chronic GERD. Adenocarcinoma of the esophagus is 30-40 times more likely to occur in patients with Barrett's esophagus. Q3303:A 42-year-old man has a boring midepigastric pain and occasional tarry stools. Over-the-counter antacids relieve the pain; and aspirin or other nonsteroidal anti-inflammatory drugs (NSAIDs) generally aggravate it. X-ray film of the upper gastrointestinal tract shows the presence of a 1- to 2- cm erosion in the duodenal mucosa about 4-cm distal to the pylorus. Which of the following is the most likely cause of this patient's condition?;A. Gastrinoma ;B. Helicobacter pylori infection ;C. Insulinoma ;D. NSAID ingestion ;E. Vitamin B12 deficiency Option B (Helicobacter pylori infection) is correct. Infection with ;H. pylori causes the development of most duodenal and many gastric ulcers. The mechanism by which the organism causes peptic ulcer disease is not well understood. Q3304:An autopsy of a 7-year-old boy who died of irreversible heart failure shows a patchy but focally dense infiltrate of chronic inflammatory cells; predominantly lymphocytes; in the myocardium. Cardiac myocytes in the vicinity of the lymphoid aggregates show focal necrosis. No abnormalities of the cardiac vasculature are evident. Light microscopy shows no organisms. Which of the following is most commonly associated with these findings?;A. Chronic toxoplasmosis ;B. Corynebacterium diphtheriae ;C. Lyme disease ;D. Recent acute viral illness ;E. Rubella Option D (recent acute viral illness) is correct. Infectious myocarditis is most often associated with an acute viral illness of recent occurrence (possibly with the past 5-30 days) involving an organ other than the heart. Offending organisms are typically coxsackievirus; echovirus; poliovirus; and influenza. Diagnosis of the etiologic agent often requires demonstration of a rising titer of antibody against the suspected virus. Q3305:Meckel's diverticulum is an uncommon congenital malformation that is usually a clinically silent condition. In some cases; Meckel's diverticulum can be confused with appendicitis in the presence of;A. heterotopic gastric tissue ;B. heterotopic pancreatic tissue ;C. obstruction of the small intestine ;D. roundworm infestation ;E. ulcerative colitis Option A (heterotopic gastric tissue) is correct. Meckel's diverticulum is a congenital malformation of the small intestine; and occurs in about 2% of the population. It is uncommon; but not rare. If the diverticulum contains heterotopic gastric mucosa; it may ulcerate and cause significant bleeding. It can produce symptoms similar to appendicitis (e.g; right lower quadrant pain). Q3306:A 4-year-old boy develops hemarthrosis of the right knee with no known preceding traumatic episode. He has not shown any previous abnormal bleeding tendencies associated with the usual childhood trauma. The patient's history suggests an X-linked recessive trait on his mother's side of the family. Which of the following studies would most likely be abnormal?;A. Bleeding time ;B. Partial thromboplastin time (PTT) ;C. Prothrombin time (PT) ;D. Thrombin time Option B (partial thromboplastin time) is correct. PTT is a measure of the intrinsic clotting pathway and is abnormal if factor VIII or factor IX activity is reduced. Although PTT is generally adequate to screen for hemophilia; factor VIII or factor IX assay are required to confirm the diagnosis. Hemophilia A and hemophilia B are not clinically distinguishable. Q3307:A febrile 23-year-old woman complains of fatigue; right upper quadrant pain; and difficulty swallowing. Physical examination shows exudative tonsillitis; palatal petechia; tender cervical lymphadenopathy; splenomegaly; and tender hepatomegaly. A complete blood cell count shows mild microcytic anemia; lymphocytic leukocytosis with ~20% of the lymphocytes having atypical features; and a mild thrombocytopenia. Which of the following laboratory findings is expected in this patient?;A. Low total iron-binding capacity ;B. Normal serum ferritin ;C. Normal serum transaminases ;D. Positive hepatitis B surface antigen ;E. Positive heterophile antibody test Option E (positive heterophile antibody test) is correct. The patient most likely has infectious mononucleosis (IM); which is caused by the Epstein-Barr virus (EBV). The virus infects B cells initially by attaching to CD 21 receptors on the cell surface. Circulating T lymphocytes interact with the infected B cells and become antigenically stimulated; resulting in atypical lymphocytosis. The key screening test for IM is the Monospot test; which detects heterophile antibodies in the patient's serum. Heterophile antibodies unique to IM are IgM antibodies directed against horse red blood cells (RBCs). An agglutination reaction against horse RBCs is the basis for a positive Monospot test. Q3308:A routine physical examination of an asymptomatic; normotensive 21-year-old African American woman is normal; however; a urinalysis shows red blood cells (RBCs) with no casts. The patient says that she occasionally has had blood in her urine in the past. A urine culture is negative. Laboratory studies show;Serum blood urea nitrogen (BUN) 10 mg/dL ;Serum creatinine 1.0 mg/dL ;Hemoglobin 11.0 g/dL ;Mean corpuscular volume 78 ;Reticulocyte count; corrected 2% ;The peripheral smear shows occasional hypochromatic RBCs. Renal ultrasonography is normal. Which of the following is the next best step in the management of this patient?;A. Bone marrow examination ;B. Cystoscopy ;C. Renal biopsy ;D. Sickle cell preparation ;E. No further investigation is necessary Option D (sickle cell preparation) is correct. The patient most likely has sickle cell trait; which causes recurrent microscopic hematuria. Although the percentage of sickle hemoglobin in sickle cell trait is only ~40%; with the remainder representing hemoglobin A; the oxygen tension in the renal medulla is low enough to induce sickling of the RBCs in the peritubular capillaries. This results in microinfarctions in the renal medulla and the potential for renal papillary necrosis and loss of both concentration and dilution of urine. Q3309:A 72-year-old man complains of sudden onset of left flank pain and dizziness when he stands up quickly. When he is lying down; his blood pressure is 100/80 mm Hg; and his pulse is 110/min. When he is moved to a sitting position; his blood pressure is 80/60 mm Hg; and his pulse is 160/min. A pulsatile mass is palpated in the abdomen. The pathogenesis of the patient's flank pain and hypotension is most closely attributed to structural weakness of the aorta due to ;A. genetic defect in collagen ;B. genetic defect in fibrillin ;C. immunocomplex-mediated disease ;D. normal changes associated with aging ;E. severe atherosclerosis Option E (severe atherosclerosis) is correct. The patient has the classic triad of a ruptured abdominal aortic aneurysm: sudden onset of left flank pain; hypotension; and a pulsatile abdominal mass. Atherosclerotic damage of the abdominal aorta weakens the vessel wall; leading to outpouching of the aorta and the potential for rupture as the expansion increases wall stress. Q3310:An afebrile 35-year-old sheepherder who is living in a Basque community in southern Arizona complains of recurrent right upper quadrant pain. The sheepherder says that he and his dog spend their days together tending the sheep. A complete blood cell count shows a marked increase in the percentage of eosinophils. An ultrasound of his liver shows a cystic mass with calcifications in the lining of the cyst. Which of the following most accurately describes the epidemiology of this patient's liver disease;;A. The dog ate infected sheep ;B. The dog is an intermediate host ;C. The sheep is a definitive host ;D. The sheepherder ate infected sheep ;E. The sheepherder is a definitive host Option A (the dog ate infected sheep) is correct. The patient has echinococcosis due to the tapeworm Echinococcus granulosis or E. multilocularis. In the normal developmental cycle of the Echinococcus species; adult worms mate and lay eggs that develop into larvae. The larvae mature into adult worms; and the cycle repeats. Hosts containing the larval form are called intermediate hosts; while hosts containing the adult worms are called definitive hosts. Q3311:A 24-year-old professional weight lifter develops sudden onset of abdominal pain while bench-pressing 550 pounds. Within 10 minutes of the onset of pain; he becomes hypotensive and collapses. During emergency surgery; the surgeon finds clotted and unclotted blood filling the peritoneal cavity. Which of the following best explains the likely origin of the patient's intra-abdominal bleeding?;A. Ruptured abdominal aortic aneurysm ;B. Ruptured cavernous hemangioma of the liver ;C. Ruptured liver cell (hepatic) adenoma ;D. Ruptured splenic artery aneurysm Option C (ruptured liver cell adenoma) is correct. Professional weight lifters commonly use anabolic steroids to increase muscle mass and strength. One of the complications of anabolic steroids is the development of liver cell (hepatic) adenomas; which are benign tumors arising from hepatocytes. These tumors tend to rupture and produce intraperitoneal hemorrhage. Women taking estrogen-containing medications are subject to the same complication. Q3312:An 84-year-old man complains of lower back pain and inability to void urine over the past 24 hours. Physical examination shows point tenderness over the lower lumbar vertebrae and an enlarged bladder extending to the level of the umbilicus. The physician suspects metastatic prostate cancer. Which of the following is indicated as the first step in the management of this patient?;A. Digital rectal examination ;B. Prostate-specific antigen (PSA) ;C. Radionuclide bone scan ;D. Serum alkaline phosphatase ;E. Transrectal ultrasound with biopsy Option A (digital rectal examination) is correct. Prostate cancer is the most common cancer in men. Point tenderness over the vertebral bodies in an elderly patient is highly suggestive of metastatic prostate cancer; especially coupled with clinical evidence of urinary retention. Because prostate cancers develop in the peripheral zone of the prostate; they are palpated easily by digital rectal examination; especially if they have already spread beyond the gland. Q3313:A 35-year-old pharmacist complains of recurrent episodes of forgetfulness and tiredness. Physical examination is essentially unremarkable. Laboratory studies show a serum glucose level of 20 mg/dL. Additional studies on the same serum sample show a high serum insulin level and a low C- peptide level. Based on these findings; which of the following is the most likely diagnosis?;A. Benign tumor involving b-islet cells in the pancreas;B. Ectopic secretion of an insulinlike factor ;C. Malignant tumor involving a-islet cells in the pancreas ;D. Surreptitiously injected human insulin Option D (surreptitiously injected human insulin) is correct. Normally; b-islet cells first synthesize preproinsulin in the rough endoplasmic reticulin. Preproinsulin then is delivered to the Golgi apparatus; where proteolytic reactions generate insulin and a cleavage peptide called C peptide. Hence; C peptide is a marker for endogenous synthesis of insulin. In this case; the patient has been injecting himself with insulin. This increases the serum insulin level; however; serum C peptide is decreased due to suppression of the b-islet cells by the exogenously administered insulin. Q3314:An autopsy performed on a 40-year-old man with dementia shows atrophy of the frontal and temporoparietal lobes of the brain. Histologic examination shows senile plaques. The pathogenesis of the dementia in this patient is most closely related to which of the following?;A. b-amyloid protein ;B. Decreased dopamine levels ;C. Slow virus disease ;D. Triplet repeat mutation Option A (b-amyloid protein) is correct. Senile plaques are characteristic of Alzheimer's disease (AD); which is the most common cause of dementia in patients over 65 years of age. Senile plaques contain a core of A-b-amyloid surrounded by neurites. The explanation for the age disparity in this case is that the patient has Down syndrome with three functioning chromosome number 21 (e.g; trisomy 21). Chromosome 21 codes for an Alzheimer precursor protein (APP); part of which is amyloid-b (A- b) protein. This protein is toxic to neurons; hence the extra chromosome 21 codes for more of the protein; which results in AD at an early age. Q3315:A 30-year-old woman states that she often burns her hands without feeling any pain. Physical examination shows decreased pain and temperature sensation in the upper extremities; atrophy of the intrinsic muscles of the hands; and abnormal deep tendon reflexes in the upper extremities. The pathogenesis of this patient's neurologic problems is most closely associated with which of the following?;A. Autoimmune destruction of myelin ;B. Fluid-filled cavity in the cervical spinal cord ;C. Superoxide free radical destruction of upper and lower motor neurons ;D. Tumor in the cervical spinal cord ;E. Vitamin B12 deficiency Option B (fluid-filled cavity in the cervical spinal cord) is correct. The patient has syringomyelia; the development of an expanding fluid-filled cavity in the cervical spinal cord. This results in destruction of the crossed lateral spinothalamic tracts (loss of pain and temperature sensation); anterior horn cells (loss of the intrinsic muscles of the hand); and other tracts as the cavity expands. It is often confused with amyotrophic lateral sclerosis (ALS); however; in ALS there are no sensory abnormalities. Q3316:Common congenital malformations Heart defects;Hypospadias;Cleft lip;Congenital hip dislocation;Spina bifida;Anencephaly;Pyloric stenosis Q3317:Congenital malformation associated with projectile vomitting Pyloric stenosis Q3318:Right to left shunts (early cyanosis) blue babies - name 3 causes 3 T's;Tetralogy of Fallot;Transposition of great vessels;Truncus arteriosus Q3319:Child may squat to increase venous return - what type of shunt? Right to left Q3320:Left to right shunts (late cyanosis) - blue kids - name 3 causes VSD;ASD;PDA Q3321:Close with indomethacin PDA Q3322:Most common congenital cardiac anomaly VSD Q3323:Loud S1; wide; split; fixed S2 ASD Q3324:Uncorrected VSD; ASD or PDA leads to progressive pulmonary hypertension. As pulmonary resistance increases shunt changes from Lto R to R to L which causes late cyanosis (clubbing and polycythemia);Diagnosis Eisenmengers syndrome Q3325:Name 4 parts of tetralogy of Fallot PROVe;Pulmonary stenosis;Right ventricular hypertrophy;Overriding aorta;VSD Q3326:What do you see on xray in patient with tetralogy of Fallot Boot shaped heart due to RVH Q3327:Cause of tetralogy of Fallot Anterosuperior displacement of infundibular septum Q3328:Patient suffers from "cyanotic spells"; x ray shows boot shaped heart - diagnosis? Tetraology of Fallot Q3329:Aorta leaves RV (anterior) and pulmonary trunk leaves LV (posterior) --> separation of systemic and pulmonary circulation. Not compatible with life unless a shunt is present to allow adequate mixing of blood (VSD; PDA or patent foramen ovale);Diagnosis? Transposition of great vessels Q3330:Cause of transposition of great vessels Failure of aorticopulmonary septum to spiral Q3331:Prognosis for transposition of great vessels Without surgical correction infants die within 1st month of life Q3332:In infantile type of coarctation of aorta - aortic stenosis is proximal or distal to insertion of ductus arteriosus? Proximal (preductal);INfantile = IN close to heart Q3333:In adult type of coarctation of aorta stenosis is distal or proximal to ductus arteriosus Distal (postductal);ADult= Distal to Ductus Q3334:This condition is associated with notching of ribs; hypertension in upper extremities and weak pulses in lower extremities Coarctation of aorta Q3335:What should you check in physical exam if suspect coarctation of aorta Femoral pulses Q3336:Male to female ratio for coarctation of aort 3:01 Q3337:In fetal period shunt is right to left (normal). In neonatal period; lung resistance decreases and shunt becomes left to right with subsequent RVH and failure (abnormal). Associated with a continuous "machine like" murmur - Name condition Patent ductus arteriosus Q3338:Patency of ductus arteriosus is maintained by? PGE synthesis and low O2 tension Q3339:What drug is used to close PDA Indomethacin Q3340:Name drug used to keep PDA open which may be necessary to sustain life in conditions such as transposition of great vessels PGE Q3341:Name defect associated with 22q11 syndromes Truncus arteriosus; tetralogy of Fallot Q3342:Name congenital cardiac defect associated with Down syndrome ASD;VSD Q3343:Name congenital cardiac defect associated with congenital rubella Septal defects;PDA Q3344:Name congenital cardiac defect associated with Turners syndrome Coarctation of aorta Q3345:Name congenital cardiac defect associated with offspring of a diabetic mother Transposition of great vessels Q3346:Most common chromosomal disorder and cause of congenital mental retardation Down syndrome Q3347:GI defect associated with Downs Duodenal atresia Q3348:Which cancer is in increased risk for patients with Downs ALL Q3349:Most common cardiac malformation in patients with Downs - cause? Septum primum type ASD due to endocardial cushion defects Q3350:Decreased levels of alpha fetoprotein;Increased beta HCG;Increased nuchal translucency - diagnosis Downs Q3351:Mental retardation; flat facial profile; prominent epicantal folds; simian crease - diagnosis? Downs trisomy 21 Q3352:95% of Downs are caused by _ ;4% ?;1% Nondisjunction;4% - Robertsonian translocation;1% - mosaicism Q3353:Edwards syndrome is also called? Trisomy 18 (Election age) Q3354:Baby is born with severe mental retardation; rocker bottom feet; low set ears; micrognathia (small jaw); congenital heart disease; clenched hands and prominent occiput- diagnosis + prognosis Edwards syndrome - trisomy 18;Death within 1 year Q3355:Pataus syndrome is also called Trisomy 13 Q3356:Baby is born with severe mental retardation; microphthalmia; cleft lip/palate; abnormal forebrain structures; polydactyly; congenital heart disease - diagnosis + prognosis Pataus trisomy 13;Death within 1 year Q3357:Klinefelters genotype XXY Q3358:Testicular atrophy; eunuchoid body shape; tall; long extremities; gynecomastia; female hair distribution; presence of inactivated X chromosome (Barr body) Klinefelter syndrome Q3359:Common cause of hypogonadism see in infertility workup Klinefelter syndrome Q3360:Genotype for Turners syndrome XO Q3361:Short stature; ovarian dysgenesis (streak ovary); webbing of neck; coarctation of aorta; no Barr body Turners Q3362:Most common cause of primary amenorrhea Turners Q3363:Phenotypically normal; very tall; severe acne; antisocial behavior; observed with increased freuency among inmates of penal institutions Double Y males XYY Q3364:What type of mutation is Duschennes Frameshift mutation --> deletion of dystrophin gene --> accelerated muscle breakdown Q3365:Inheritance for Duschennes X linked Q3366:Where does weakness begins in patients with Duschennes Pelvic girdle and moves superiorly Q3367:5 year old male presents with pseudohypertrophy of calf muscles and cardiac myopathy - name condition and cause Duschennes - replacement of muscle by fibrofatty tissue Q3368:7 year old male requires assistance of the upper extremities to stand up (proximal lower limb weakness) - name phenomenon and condition Gowers maneuver - Duschennes muscular dystrophy Q3369:How do you diagnose muscular dystrophies By increased CPK and muscle biopsy Q3370:Disagreement between phenotypic (external genitalia) and gonadal (testes vs ovaries) sex Pseudohermaphroditism Q3371:Ovaries are present but external genitalia are virilized or ambiguous - name condition + cause Female pseudohermaphroditism;Due to excessive and inappropriate exposure to androgenic steroids during early gestation (congenital adrenal hyperplasia or exogenous administration of androgens during pregnancy) Q3372:Testes present but external genitalia are female or ambiguous - name condition + cause Male pseudohermaphroditism - androgen insensitivity syndrome (testicular feminization) Q3373:Both ovary and testicular tissue present; ambiguous genitalia - very rare;Possible genotypes? True hermaphrodite;46 XX or 47 XXY Q3374:Defect in androgen receptor resulting in normal appearing female; female external genitalia with rudimentary vagina; uterus and uterine tubes are absent. develops testicular tissue(often found in labia majora; surgically removed to prevent malignancy);Name condition + genotype + what happens to levels of estrogen; testosterone and LH Androgen insensitivity syndrome;46 XY;Levels of testosterone; estrogen and LH are all high Q3375:Unale to convert testosterone to DHT; ambiguous genitalia until puberty; when increased testosterone causes masculinization of genitalia;Name condition + what happens to levels of estrogen; testosterone and LH 5 alpha-reductase defficiency;Testosterone and estrogen levels are normal; LH normal or increased Q3376:Congenital deletion of short arm of chromosome 5 leads to _ Cri-du-chat syndrome Q3377:Microcephaly; severe mental retardation; high pitched crying/meowing; epicanthal folds; cardiac abnormalities Cri-du-chat syndrome Q3378:2nd most common cause of genetic mental retardation Fragile X Q3379:X linked defect affecting methylation and expression of FMR1 gene; associated with macro-orchidism (enlarged testes); long face with large jaw; large everted ears and autism Fragile X Q3380:What type of mutation is in Fragile X Triple repeat disorder (CGG) that may show genetic anticipation (germlike expansion in females) Q3381:Cleft palate;Abnormal facies;Thymic aplasia;Cardiac defects;Hypocalcemia;2 types of disorders 22q11 syndromes - DiGeorge syndrome (thymic; parathyroid and cardiac defects) or velocardiofacial syndrome (palate; facial and cardiac defects) Q3382:Adult polycystic kidney disease - unilateral or bilateral ALWAYS bilateral Q3383:Patient presents with pain; hematuria; hypertension; progressive renal failure; 90% of mutations are due to mutation in APKD1 (chromosome 16) Adult polycystic kidney disease - massive enlargement of kidneys due to multiple large cysts Q3384:Name conditions associated with adult polycystic kidney disease Polycystic liver disease;BERRY ANEURYSMS;Mitral valve prolapse Q3385:What is the inheritance for adult form of polycystic kidney disease? ;juvenile? Adult - autosomal dominant;Juvenile - autosomal recessive Q3386:Patient has high cholesterol (heterozygotes 300; homozygotes - rare- 700); severe atherosclerotic disease early in life and tendon xanthomas (classically in Achilles tendon); MI may develop before age 20;Name disease and cause Familial hypercholesterolemia (hyperlipidemia type IIa);Elevated LDL owing to defective or absent LDL receptor Q3387:Cause of Marfans disease Fibrillin gene mutation -> connective tissue disorders Q3388:Skeletal abnormalities in Marfans syndrome Tall with long extremities; hyperextensive joints; and long; tapering fingers and toes Q3389:Cardiovascular abnormalities in Marfans syndrome Cystic medial necrosis of aorta --> aortic incometence and dissecting aortic aneurysms;Floppy mitral valve Q3390:Occular abnormality in Marfans syndrome Subluxation of the lens Q3391:Findings in neurofibromatosis type I (von Riecklinghausen disease) Cafe-au-lait spots;Neural tumors;Lisch nodules (pigmented iris hamartomas);Also marked by skeletal disorders (scoliosis) and increased tumor susceptibility Q3392:Gene coding for NFT type I (von Riecklinghausen disease) located where? Long arm of chromosome 17 (17 letters in Recklinghausen) Q3393:Gene coding for NFT type II is located where? On chromosome 22 Q3394:Patient presents with bilateral acoustic neuromas; optic pathway gliomas; juvenile cataracts - diagnosis? Type II NFT Q3395:Findings: facial lesions (adenoma sebaceum); hypopigmented "ash leaf spots" on skin; cortical and retinal hamartomas; seizures; mental retardation; renal cysts; cardiac rhabdomyomas. Incomplete penetrance; variable presentation Tuberous sclerosis Q3396:Findings: hemangioblastomas of retina/cerebellum/medulla; about half of affected individuals develop multiple bilateral renal cell carcinomas and other tumors - name disease; which gene affected and inheritance Von Hippel Lindau disease;Deletion of VHL gene (tumor suppressor) on chromosome 3 (3p);Autosomal dominant Q3397:Findings - depression; progressive dementia; choreiform movements; caudate atrophy and decreased levels of GABA and Ach in brain; symptoms manifest between 20 and 50 - name disorder; what type of disorder; inheritance + which chromosome affected Huntingtons;Triplet repeat disorder;Autosomal dominant;Gene on chromosome 4 Q3398:Colon becomes covered wiith adenomatous polyps after puberty. Progresses to cancer unless resected - name disease and chromosome on which deletion occurs+ inheritance pattern Familial adenomatous polyposis - deletion on chromosome 5; autosomal dominant Q3399:Spheroid erythrocytes; hemolytic anemia; increased MCHC - name disease + what is curative + inheritance pattern Hereditary spherocytosis;Splenectomy is curative;Autosomal dominant Q3400:Autosomal dominant cell-signalng defect of fibroblast growth factor (FGF) receptor 3. Results in dwarfism; short limbs but head and trunk are normal size - name disease Achondroplasia Q3401:Inheritance pattern for cystic fibrosis Autosomal recessive Q3402:Inheritance pattern for albinism Autosomal recessive Q3403:Inheritance pattern for alpha1-antitrypsin defficiency AR Q3404:Inheritance pattern for PKU AR Q3405:Inheritance pattern for thalassemias and sickle cell anemias AR Q3406:Inheritance pattern for glycogen storage disorders AR Q3407:Inheritance pattern for mucopolysaccharidoses - ?;EXCEPT - > AR;EXCEPT Hunters Q3408:Inheritance pattern for sphingolipidoses- ?;EXCEPT- ? AR;EXCEPT Fabrys Q3409:Inheritance pattern for infant polycystic kidney disease AR Q3410:Inheritance pattern for hemochromatosis AR Q3411:Infertility in males with cystic fibrosis is due to? Absence of vas deferens Q3412:Which vitamins are defficient in patients with cystic fibrosis Fat soluble ADEK Q3413:Cystic fibrosis can present as _ in infancy Failure to thrive Q3414:Most common lethal genetic disease of Caucasians Cystic fibrosis Q3415:Treatment of cystic fibrosis N-acetylcysteine to loosen mucous plugs Q3416:Cause of cystic fibrosis + how it presents Autosomal recessive defect in CFTR gene on chromosome 7. Defective Cl channel -->secretion of abnormally thick mucus that plugs lungs; pancreas and liver --> recurrent pulmonary infections (Pseudomonas and S aureus); chronic bronchitis; bronchiectasis; pancreatic insufficiency (malabsorption and steatorrhea); meconium ileus in newborns Q3417:Diagnostic test for cystic fibrosis Increased concentration of Cl ions in sweat Q3418:Associated with low folic acid intake during pregnancy; elevated alpha fetoprotein in amniotic fluid and maternal serum Neural tube defects Q3419:Failure of bony spinal canal to close but no structural herniation; usually seen at lower vertebral levels Spina bifida occulta Q3420:Meninges herniate through spinal canal defect Meningocele Q3421:Meninges and spinal cord herniate through spinal canal defect Meningomyelocele Q3422:Mechanism of fetal alcohol syndrome Inhibition of cell migration Q3423:Newborn presents with postnatal developmental retardation; microcephaly; facial abnormalities; limb dislocation and heart and lung fistulas - probable cause? Fetal alcohol syndrome Q3424:# 1 cause of congenital malformation in USA Fetal alcohol syndrome Q3425:Increase in number of cells (reversible) Hyperplasia Q3426:One adult cell type is replaced by another (reversible) - often secondary to irritation and/or environmental exposure Metaplasia Q3427:Abnormal growth with loss cellular orientation; shape; and size in comparison to normal tissue maturation; commonly preneoplastic (reversible) Dysplasia Q3428:Abnormal cells lacking differentiation; like primitive cells of same tissue; often equated with undifferentiated malignant neoplasms; tumor giant cells may be formed Anaplasia Q3429:Clonal proliferation of cells that is uncontrolled and excessive Neoplasia Q3430:S-100 Marker for melanoma; neural tumors; and astrocytomas Q3431:Gastric Adenocarcinoma markers CEA and bombesin Q3432:HCC markers AFP and alpha-1-AT Q3433:Plummer-Vinson syndrome triad Dysphagia (due to webs in the upper esophagus); atrophic glossitis; iron-deficiency anemia Q3434:Pulseless disease Takayasu arteritis - stenosis of aortic arch and its large branches with ischemia to upper part of the body Q3435:Trousseau Sign Migratory thromophlebitis associated with tumors of pancreas; lung; and colon Q3436:Colon Cancer tumor suppression genes APC; DCC; p53 Q3437:Pancreatic Cancer tumor suppression gene DPC Q3438:Stomach Cancer tumor suppressor gene DCC Q3439:Peripheral nerve tumor suppressor gene NF-1 (neurofibromatosis 1) Q3440:Inadequate oxygenation is called _ HYPOXIA Q3441:Most common cause of hypoxia is _ Coronary artery atherosclerosis Q3442:CO and CN both inhibit _ Cytochrome oxidase Q3443:How do you treat CO poisoning ? 100% O2 Q3444:Most adversely affected cell in tissue hypoxia _ NEURONS Q3445:What do you see on ECG in subendocardial ischemia ST segment depression Q3446:Cytochrome C activates _ Apoptosis Q3447:Most common cause of drug induced fulminant hepatitis ACETAMINOPHEN Q3448:Reperfusion injury can be caused by ? O2 and increase in cytosolic Ca Q3449:What is the difference between dystrophic and metastatic calcification Dystrophic calcification - calcification of necrotic tissue;Metastatic calcification - calcification of normal tissue Q3450:Decrease in size of tissue or organ is called _ ATROPHY Q3451:Increase in cell size is called _ HYPERTROPHY Q3452:Increase in number of cells _ HYPERPLASIA Q3453:One cell type replaces another - this is called _ METAPLASIA Q3454:Disordered cell growth is called _ DYSPLASIA Q3455:In cerebral infarction - what type of necrosis? LIUEFACTIVE (NOT coagulative) Q3456:Most common cause of caseous necrosis Tuberculosis Q3457:What type of necrosis in acute pancreatitis Enzymatic fat necrosis Q3458:Programmed cell death is called _ Apoptosis Q3459:Name apoptosis gene BAX Q3460:Name anti apoptosis gene BCL2 Q3461:These cells release preformed histamine Mast cells Q3462:Primary leukocytes in acute inflammation Neutrophils Q3463:Responsible for "rolling" of neutrophils Selectins Q3464:Neutrophil adhesion molecules Beta 2 integrins Q3465:Directed migration of neutrophils is called _ CHEMOTAXIS Q3466:Name 2 opsonins IgG and C3b Q3467:In Butons agammogloblinemia there is a defect in _ OPSONIZATION Q3468:In Chediak Higashi syndrome patient cannot form _ PHAGOLYSOSOMES Q3469:Most potent microbicidal system O2 dependent MPO system Q3470:End product of O2 dependent MPO system BLEACH Q3471:In chronic granulomatous disease there is absent _ NADPH OXIDASE AND RESPIRATORY BURST Q3472:Most important chemical mediator of acute inflammation HISTAMINE Q3473:Most common cause of skin abscess Staph aureus Q3474:Most common cause of chronic inflammation INFECTION Q3475:Primary leukocytes in chronic inflammation Monocytes and macrophages Q3476:Granulation tissue is converted to _ Scar tissue Q3477:Key adhesion glycoprotein in ECM Fibronectin Q3478:cell types in granuloma macrophages and CD4 helper T cells Q3479:Macrophages activated by gamma interferon from CD4 Th cells are called _ Epithelioid cells Q3480:Most variable stage in cell cycle G1 Q3481:Most critical phase in cell cycle G1 to S Q3482:Genes controlling G1 to S phases of cell cycle RB and TP53 Q3483:Key adhesion glycoprotein in basement membrane LAMININ Q3484:IN Ehlers Danlos syndrome which collagen is defective Type I and type III Q3485:Cofactor in collagenase Zn Q3486:Most common cause of impaired wound healing Infections Q3487:In which vitamin defficiency is there decreased cross linking of collagen Vitamin C defficiency Q3488:Which drugs prevent scar formation Glucocorticoids Q3489:In keloids which type collagen is in excess type III Q3490:In lung injury which cell is repair cell TYPE II PNEUMOCYTE Q3491:What happens in brain with injury Proliferation of astrocytes and microglial cells Q3492:With peripheral nerve transection which cell is key cell in reinnervation Schwann cell Q3493:Predominant immunoglobulin in acute inflammation IgM Q3494:Predominant immunoglobulin in chronic inflammation IgG Q3495:Corticosteroid effect in blood Increased neutrophils;Decreased lymphocytes and eosinophils Q3496:Marker of necrosis and disease activity CRP Q3497:Marker of acute inflammation IgM Q3498:Sign of chronic inflammation Polyclonal gammopathy; increased IgG Q3499:Large granular lymphocytes in peripheral blood Natural killer cells Q3500:When does IgM and IgG synthesis begin After birth Q3501:Class I MHC is present on which cells All nucleated cells Q3502:Name antigen presenting cells B cells;Macrophages;Dendritic cells Q3503:Which HLA protein is associated with ankylosing spondylitis HLA B27 Q3504:IgE activation of mast cells happens in which type of hypersensitivity Type I Q3505:Name potentially fatal type I hypersensitivity reaction Anaphylactic shock Q3506:Antibody dependent cytotoxic reactions are what type of hypersensitivity Type II Q3507:Activation of compliment by circulating antigen- antibody complexes is what type of hypersensitivity Type III Q3508:Name antibody activated hypersensitivity reactions Type I; II; III Q3509:Which type of hypersensitivity has to do with cellular immunity Type IV Q3510:What is the most important requirement for successful transplantation ABO blood group compatibility Q3511:Type of transplant with best survival rate Autograft (self to self) Q3512:Hyperacute rejection of transplant - is it reversible? what type of hypersensitivity? Irreversible; type II hypersensitivity Q3513:Most common type of transplant rejection - what typ of hypersensitivity? Acute rejection - type IV and type II hypersensitivity Q3514:Is chronic rejection of transplant reversible Irreversible Q3515:What are symptoms and signs of graft versus host reactions Jaundice;Diarrhea;Dermatitis Q3516:Most common cardiac finding in SLE Fibrinous pericarditis with effusion Q3517:Most common drug associated with drug induced lupus Procainamide Q3518:What type of antibodies are present in drug induced lupus Antihistone antibodies Q3519:What two tests can confirm SLE Anti double stranded DNA;Anti Smith antibodies Q3520:In systemic sclerosis there is excess _ Collagen deposition Q3521:Most common initial sign of systemic sclerosis Raynaud phenomenon Q3522:What type of antibodies are present with systemic sclerosis Anti-topoisomerase antibodis Q3523:What does CREST syndrome stand for Calcinosis;Raynauds phenomenon;Esophageal dysfunction;Sclerodactyly;Telangiectasia Q3524:In dermatomyositis and polymyositis there is increased _ Serum creatine kinase Q3525:Im MCTD (mixed connective tissue disease) what kind of antibodies are present Anti RNP antibodies Q3526:Most common congenital immunodefficiency IgA defficiency Q3527:Most common acquired immunodefficiency disease worldwide AIDS Q3528:Pediatric AIDS is mostly due to _ transmission Vertical (mother to child) Q3529:HIV is cytotoxic to what type of cells? which immunity is lost? CD4 T cells;Loss of cell mediated immunity Q3530:Which protein is detected in ELISA test screen for HIV anti gp 120 Q3531:Which test confirms HIV Western blot Q3532:Which cells are reservoir cells for HIV Follicular dendritic cells in lymph nodes Q3533:Most common CNS fungal infection in AIDS Cryptococcosis Q3534:Most common malignancy in AIDS Kaposis sarcoma Q3535:Most common cause of blindness in AIDS CMV Q3536:In hereditary angioedema there is defficiency of what? C1 esterase inhibitor Q3537:Which protein exhibits apple green birefringence in polarized light Amyloid Q3538:Which protein is associated with Alzheimers disease in Down syndrome Beta amyloid Q3539:Major cation of extracellular fluid Na Q3540:Major cation of intracellular fluid K Q3541:Water movement between extracellular and intracellular fluid compartments is called? What is it controlled by? OSMOSIS - controlled by serum Na Q3542:Formula for calculating serum Na Total body Na/ Total body water Q3543:What is the sign of volume depletion Decreased total body Na Q3544:In which conditions would you see increase in total body Na Pitting edema;Body cavity effusions Q3545:What happens to Na in isotonic loss or gain Serum Na normal Q3546:What two signs describe hypotonic disorders Hyponatremia;ICF expansion Q3547:In which conditions would you see pitting edema Right sided heart failure;Cirrhosis;Nephrotic syndrome Q3548:Which signs describe hypertonic disorders Hypernatremia or hyperglycemia;ICF conraction Q3549:What happens to Posm and Na in hyperglycemia Increased Posm;Decreased serum Na Q3550:What happens to K in alkalosis Shifts into cell Q3551:What happens to K in acidosis Shifts out of cell Q3552:Most common cause of hypokalemia Loop and thiazide diuretics Q3553:What does ECG show in hypokalemia U wave Q3554:Most common cause of hyperkalemia Renal failure Q3555:What does ECG show in hyperkalemia Peaked T waves Q3556:What does PaCO2 have to be to diagnose respiratory acidosis >45 mm Hg Q3557:What does PaCO2 have to ne to diagnose respiratory alkalosis < 33 mmHg Q3558:What happens in increased anion gap metabolic acidosis Anions of acid replace HCO3 Q3559:What happens in normal anion gap metabolic acidosis Cl anions replace HCO3 Q3560:What is the level of HCO3 have to be to diagnose metabolic acidosis < 22 mE/L Q3561:Most common cause of metabolic alkalosis Loop and thiazide diuretics Q3562:What does HCO3 have to be to diagnose metabolic alkalosis > 28 mEq/L Q3563:With _ intoxication there is often mixture of primary metabolic acidosis and primary respiratory alkalosis SALICYLATE Q3564:Excess fluid in interstitial space is called _ EDEMA Q3565:Protein poor and cell poor fluid is called _ TRANSUDATE Q3566:Protein rich and cell rich fluid is called _ EXUDATE Q3567:Is there transudate or exudate in pitting edema? What happens to hydrostatic and oncotic pressures? Transudate;Increased hydrostatic pressure ;Decreased oncotic pressure Q3568:Most common site for venous thrombosis Deep vein of lower extremity under knee Q3569:Name anticoagulants that are used for prevention of venous thrombosis Warfarin and Heparin Q3570:Prevents formation of arterial thrombi Aspirin Q3571:How do you prevent mixed thrombus Aspirin + anticoagulant therapy Q3572:Majority of pulmonary thromboembolism originate in _ Femoral veins Q3573:Majority of systemic embolisms originate in _ Left side of the heart Q3574:Most common cause for fat embolism Fracture of long bones Q3575:Signs and symptoms of amniotic fluid embolism Abrupt onset dyspnea;Hypotension;Bleeding (DIC) Q3576:What happens in decompression sickness Nitrogen gas bubbles occlude vessel lumens Q3577:Two serious potential complications of scuba diving Pneumothorax and pulmonary embolism Q3578:Most common cause of hypovolemic shock Blood loss Q3579:Most common cause of cardiogenic shock acute MI Q3580:What happens to cardiac output in hypovolemic shock? Cardiogenic shock? septic shock? Hypovolemic shock - decreased CO;Cardiogenic shock - decreased CO;Septic shock - increased CO Q3581:What happens to left ventricular end-diastolic pressure in hypovolemic shock? cardiogenic shock? septic shock? Decreased in hypovolemic and septic shock; increased in cardiogenic shock Q3582:What happens to peripheral vascular resistance in hypovolemic shock? cardiogenic shock? septic shock? Increased in cardiogenic and hypovolemic shock; decreased in septic shock Q3583:Most common cause of death in shock Multiorgan failure Q3584:What type of mutation occurs with sickle cell disease/trait Missense mutation Q3585:With beta thalassemia major what type of mutation Nonsense mutation with stop codon Q3586:In Tay Sachs disease what type of mutation Frameshift mutation Q3587:Additional trinucleotide repeats increases disease severity in future generations - this is called _ ANTICIPATION Q3588:Most common type of mendelian disorder Autosomal recessive Q3589:Both parents must have mutant gene - what type of inheritance Autosomal recessive Q3590:Enzyme defficiencies disorders usually involve what type of inheritance AR Q3591:In PKU what is increased and what is decreased Phenylalanine increased;Tyrosine decreased Q3592:What is defficient in Von Gierkes disease Glucose 6 phosphatase Q3593:Most common AR disorder Hemochromatosis Q3594:Heterozygotes with dominant mutant gene express disease - type of inheritance AD Q3595:Individual with mutant gene doesnt express disease - this is called Reduced penetrance Q3596:Most common AD disorder Von Willebrand disease Q3597:Asymptomatic female carrier transmits mutant gene to 50% of sons - what type of inheritance XR Q3598:Most common x linked disorder Fragile X syndrome Q3599:In which x linked inheritance femaler carriers are symptomatic XD Q3600:Number of Barr bodies? Number of chromosomes - 1 Q3601:Unequal separation of chromosomes in meiosis is called _ Nondisjunction Q3602:Nondisjunction of chromosomes in mitosis is called _ Mosaicism Q3603:Which chromosomal defect is present in cri du chat syndrome Deletion short arm chromosome 5 Q3604:Most cases of Down syndrome occur due to _ Nondisjunction Q3605:Most common genetic cause of mental retardation Down syndrome Q3606:Most important risk factor for bearing offspring with trisomy syndromes Advanced maternal age Q3607:45; x caryotype is present in which condition Turner syndrome Q3608:Most common genetic cause of primary amenorrhea Turners syndrome Q3609:"Menopause before menarche" Turners syndrome Q3610:Which hormones are decreased and which are increased in Klinefelters syndrome Testosterone and inhibin decreased;LH and FSG increased Q3611:Which chromosome determines genetic sex of individual Y chromosome Q3612:Most common cause of pseudohermaphroditism? What is defficient in this disease? Testicular feminization;Androgen receptors Q3613:Most common pathogen causing congenital infection CMV Q3614:Name TORCH infections Toxoplasmosis;Other agents;Rubella;CMV;Herpes simplex Q3615:Most common cause of stillbirth Abruptio placentae Q3616:Common cause of spontaneous abortions Trisomy 16 Q3617:Most common cause for large for gestational age babies Maternal diabetes Q3618:Most common cause of neonatal morbidity/mortality Prematurity Q3619:Triple marker for Down syndrome Decreased AFP;Increased hCG;Decrease in unconjugated urine Q3620:Most important preventable cause of disease and death in US Smoking Q3621:Do women or men have less gastric alcohol dehydrogenase and therefore reach hight alcohol levels Women Q3622:Most common cause of thiamine defficiency Alcohol abuse Q3623:Increased anion gap metabolic acidosis is caused by _ Lactic acid and beta-hydroxybutyric acid Q3624:Most common systemic complication of intravenous drug use Hepatitis B Q3625:Oral contraceptives decrease risk for which cancers Endometrial and ovarian Q3626:Most common cause of hypertension in young woman Oral contraceptives Q3627:Most common cause of death in burn victims sepsis caused by Pseudomonas Q3628:Least sensitive tissue to radiation Bone Q3629:First hematological sign of total body radiation Lymphopenia Q3630:Most frequent type of cancer caused by radiation Acute leukemia Q3631:Which UV light is most damaging and how UVB - increase in pyrimidine dimers distorts DNA helix Q3632:Most common UVB related skin cancer Basal cell carcinoma Q3633:Where does carbohydrate digestion begin Mouth Q3634:Where does protein digestion begin Stomach Q3635:Where does fat digestion begin Small intestine Q3636:What is characteristic feature of kwashiorkor Pitting edema Q3637:What is characteristic feature of marasmus Extreme muscle wasting Q3638:Most common cause of death in anorexia nervosa Ventricular arrythmia Q3639:What is the complication caused by vomitting in bulimia nervosa Hypokalemic metabolic alkalosis Q3640:Which gene is usually defective in obesity Leptin gene Q3641:First sign of vitamin A defficiency Night blindness Q3642:Most common cause of vitamin D defficiency Renal failure Q3643:Vitamin E toxicity causes _ Decreased synthesis of vitamin K dependent coagulation factors Q3644:Most common cause of vitamin K defficiency in hospital Broad spectrum antibiotics Q3645:Rat poison contains _ derivatives Coumarin Q3646:People on corn based diets are defficient in _ Tryptophan and niacin Q3647:Three D's of pellagra Dermatitis ;Diarrhea;Dementia Q3648:Most common cause of vitamin B12 defficiency Perncious anemia Q3649:Most common cause of folate defficiency Alcohol abuse Q3650:Defficiency of ascorbic acid is called _ Scurvy Q3651:Poor wound healing; poor taste and perioral rash are signs of what defficiency Zn Q3652:Iodide defficiency results in _ Multinodular goiter Q3653:Benign tumors are of what origin Epithelial or connective tissue Q3654:Tumor that has derivatives of ectoderm; endoderm and mesoderm is called_ Teratoma Q3655:Carcinomas derive from? Squamous;Glandular (adenocarcinoma);Transitional epithelium Q3656:Sarcoma derives from? Connective tissue Q3657:Non neoplastic overgrowth of tissue is called _ Hamartoma Q3658:Which cancer invades tissue but doesnt metastasize Basal cell carcinoma of skin Q3659:Which metastasis have greater prognostic significance - nodal or extranodal Extranodal (liver; etc) Q3660:Name 3 routes of metastasis Lymphatic;Hematogenic;Seeding of body cavities Q3661:2 types of bone metastasis Osteoblastic (radiodense);Osteolytic (radioluscent) Q3662:Most common cause of cancer deaths in adults Lung cancer Q3663:Precursor of squamous cell carcinoma Actinic (solar) keratosis Q3664:How can you reduce risk for developing gastric lymphoma and adenocarcinoma Treat H pylori Q3665:Most common type of mutation in cancer Point mutation Q3666:Which genes protect from unregulated cell growth? Which are involved in normal growth and repair? Suppressor genes;Proto- oncogenes Q3667:What is the most effective host defense against cancer Cytotoxic CD8 T cells Q3668:What is the most common cause of death in cancer Gram negative sepsis Q3669:What is the most common paraneoplastic syndrome Hypercalcemia Q3670:Diet derived triglyceride Chylomicron Q3671:Liver derived triglyceride VLDL #NAME? LDL Q3673:Removes cholesterol from plaques for disposal in the liver HDL Q3674:Pathognomonic lesion of atherosclerosis Fibrous cap Q3675:Excellent marker of disrupted fibrous plaques CRP Q3676:Most common site for atherosclerosis Abdominal aorta Q3677:2 conditions associated with hyaline arteriosclerosis DM;Hypertension Q3678:Most common aneurysm in men>55 years of age Abdominal aortic aneurysm Q3679:Rupture of aneurysm triad Left flank pain;Hypotension;Pulsatile mass Q3680:2 conditions of aortic arch aneurysm Tertiary syphillis;Vasculitis of vasa vasorum Q3681:Most common cause of death in Marfan syndrome and EDS Aortic dissection Q3682:Most common cause of death with aortic dissection Cardiac tamponade Q3683:Symptoms and signs of small vessel vasculitis Palpable purpura Q3684:Symptoms and signs of medium sized vessel vasculitis Thrombosis;Aneurysm formation Q3685:Most common type of hypertension Essential hypertension Q3686:Most common cause of secondary hypertension Renovascular hypertension Q3687:Ventricular hypertrophy occurs due to_ Increased afterload or increased preload Q3688:Pulmonary edema is seen in which heart failure Left Q3689:First cardiac sign of LHF S3 Q3690:Increases intensity for left sided heart murmurs and abnormal heart sounds Expiration Q3691:Increases intensity of right sided heart murmurs and abnormal heart sounds Inspiration Q3692:Inrease in venous hydrostatic pressure occurs in which heart failure Right Q3693:Most common manifestation of CAD Angina pectoris Q3694:Subendocardial ischemia with ST segment depression is what type of angina Stable Q3695:Vasospasm with transmural ischemia and ST segment elevation is what type of angina Prinzmetals angina Q3696:Most common cause of death in acute MI Ventricular fibrillation Q3697:Most common cause of death in CHF Ventricular aneurysm Q3698:Gold standard for diagnosis of acute MI Cardiac troponin I and T Q3699:ECG findings in acute MI Inverted T waves; elevated ST segments; Q waves Q3700:Stimuli for EPO Hypoxemia;Left-shifted OBC;High altitude Q3701:Where is EPO synthesized? In endothelial cells of peritubular capillaries Q3702:Measure of effective erythropoiesis; corrected for the degree of anemia Reticulocyte count Q3703:Extramedullary hematopoiesis most often occurs where? In liver and spleen Q3704:What happens to Hb; Hct and RBC count in thalassemia Hb; Hct decreased;RBC count increased Q3705:What happens to RDW in iron defficiency Increased Q3706:End product of heme degradation in macrophage Unconjugated bilirubin Q3707:What is the main source of energy in mature RBC Anaerobic glycolysis Q3708:Serum ferritin is _ in iron defficiency anemia Decreased Q3709:Serum ferritin is _ in ACD; iron overload disease Increased Q3710:Serum iron is _ in iron defficiency; ACD Decreased Q3711:Serum iron is _ in iron overload disease Increased Q3712:If serum total iron binding capacity is decreased it means that transferin is _ Decreased Q3713:In microcytic anemia there is defect in _ Synthesis of Hb (heme + globin chain) Q3714:Iron defficiency most often caused by _ Bleeding Q3715:Most common anemia in hospitalized patients ACD - anemia of chronic disease Q3716:With sideroblastic anemia there is defect in _ Heme synthesis in mitochondria; ringed sideroblasts Q3717:Most common cause of sideroblastic anemia Alcohol Q3718:Most common cause of pyridoxine defficiency Isoniazid Q3719:In lead poisoning where does lead deposit Epiphyses Q3720:Most common cause of vitamin B12 defficiency Pernicious anemia Q3721:Which drug inhibits intestinal conjugase Phenytoin Q3722:Monoglutamate reabsorption is inhibited by _ Alcohol and oral contraceptives Q3723:Most common cause of folate defficiency Alcohol Q3724:McArdle glycogen storage disease;muscle Q3725:von Gierke glycogen storage disease;liver and kidneys Q3726:Wilson Copper metabolism #;liver and brain Q3727:Galactosemia galactose metabolism #;liver; lens; brain Q3728:Turcot CNS: gliomas;GI tract: adenomas Q3729:Caroli congenital malformation bile ducts Q3730:May-White familial progressive myoclonus epilepsy with lipomas; deafness; ataxia Q3731:Crigler-Najjar Unconjugated bili- severe Q3732:Gilbert Unconjugated bili- mild Q3733:Dubin-Johnson conjugated bili- mild Q3734:Rotor conjugated bili- mild Q3735:Sturge-weber leptomeningial angiomatosis; port-wine stain; mental retardation; seizures; hemiplegia Q3736:Von hippel lindau capillary hemangioblastoma;retinal angiomas;renal ca ;cysts or ang# any organ Q3737:Gardner fam. colon ca; and osteomas; epidermal cysts; fibromatosis; abnormal teeth; thyroid ca; duodenal ca; pigmented ocular fundus; desmoid tumors Q3738:Menkes copper deficiency -> #collagen crosslinking -> depigmented lusterless hair; facial/ocular/vascular/cerebral # Q3739:Milroy congenital lymphatic malformation / lymphedema Q3740:Wermer MEN 1;parathyroid (hypercalcemia; kidney stones);pituitary;pancreas (ulcers) Q3741:Sipple MEN 2a;pheochromo;parathyroid;thyroid Q3742:shy-drager ANS failure -> orthostatic hypotension and parkinsonism Q3743:Budd-Chiari IVC or hepatic vein occlusion (caused by malignancy or congenital IVC webs) Q3744:Bloom's auto rec = small body size; immunodeficiency; light-sensitive facial erythema; predisp cancer Q3745:Rett only females; severe impairment; characteristic hand wringing movements Q3746:Churg-Strauss allergic granulomatosis and angiitis -> asthma and eosinophilia and vasculitis Q3747:Reye postviral/aspirin -> enlarged distorted mitochrondria -> esp. liver (disruption biochemical pathways -> microvesicular steatosis); brain (edema). 75% mild; 25% coma; liver failure or cns#; incr ammonia Q3748:Wiskott-Aldrich thrombocytopenia; eczema; recurrent sinopulmonary infections;low IgM; high IgE; IgG; IgA; defective response to polysaccharide antigens (defect T cells prevents binding to B cells);12% chance of non-hodgkin lymphoma! Q3749:Chediak-Higashi no lysosomal enzymes in phagosomes -> giant inclusions -> infections (neutropenia); albinism; nerve deficits; bleeding diathesis Q3750:Tuberous sclerosis MR; seizures; hypopigment (!!); angiofibromata (adenoma sebaceum); large cortical hamartomas; renal angiomyolipomas; cardial rhabdomyomas Q3751:de Quervain postviral destruction thyroid causing hyper than hypo Q3752:Nelson extreme hyperpigmentation after adrenalectomy Q3753:Eisenmenger shift from L-R shunt to R-L dus to pulm HT Q3754:Dressler's complication MI -> autoimmune fibrinous pericarditis with fever and peripleural chest pain (several weeks after MI) Q3755:Peutz-jeghers polyps (complex branching pattern; benign); melanin spots and incr risk breast/pancreas/overy/uterus/lung ca Q3756:Potter bilateral renal agenesis -> oligohydramnios Q3757:Lesch-Nyhan purine metabolism# (no HGPRT);aggressive; self-mutilation; gout; gouty nephropathy; yellow-orange crystals of uric acid in diaper Q3758:maple syrup disease branched chain amino acid #;motor abnormalities and seizures Q3759:phenylketonuria aromatic amino acid metabolism #;mental retardation Q3760:orotic aciduria pyrimidine metabolism #;retarder growtha nd development and megaloblastic anemia Q3761:homocystinuria sulfur containing amino acid #;mental retardation; dislocation of lenses; osteoporosis; thrombosis Q3762:Binswanger subcortical leukencephalopathy ;Diffuse loss of deep white matter - ass hypertension Q3763:Albinism copper-dependent tyrosinase deficiency -> block production melanin from aromatic amino acid tyrosine Q3764:Pickwickian syndrome = obesity hypoventilation syndrome;obesity and sleep apnea Q3765:What are the risk factors for HTN? increased age;obesity;diabetes;smoking;genetics;blacks > whites > Asians Q3766:What does HTN predispose? atherosclerosis;stroke;CHF;renal failure;retinopathy;aortic dissection Q3767:Monckeberg atherosclerosis calcification of the arteries; esp. radial or ulnar;usually benign Q3768:atreriolosclerosis hyaline thickening of small arteries in essential HTN;hyperplastic "onion-skinning" in malignant HTN Q3769:atherosclerosis disease of elastic arteries and large and medium-sized muscular arteries Q3770:What are the risk factors in developing atherosclerosis? smoking;HTN;diabetes mellitus;hyperlipidemia;family history Q3771:What are the complications of atherosclerosis? aneurysms;ischemia;infarcts;peripheral vascular disease;thrombus;emboli Q3772:Where do atherosclerotic plaques tend to form? abdominal aorta > coronary artery > popliteal artery > carotid artery Q3773:What is the progression of atherosclerosis? fatty streaks -> proliferative plaque -> complex atheromas Q3774:stable angina most common form of angina;pain precipitated by exertion and is relieved by rest or vasodilators (e.g; nitroglycerine) Q3775:Prinzmetal's angina intermittent chest pain at rest;caused by vasospasm Q3776:unstable/cresendo angina prolonged or recurrent pain at rest;often indicative of imminent MI Q3777:Which type of angina has pain that is relieved by rest? stable angina Q3778:Which type of angina has pain that gets worse during rest Prinzmetal's angina;unstable/cresendo angina Q3779:Which type of angina has intermittent chest pain at rest? Prinzmetal's angina Q3780:Which type of angina is caused by vasospasms? Prinzmetal's angina Q3781:Which type of angina is relieved by vasodilators? stable angina Q3782:Which type of angina is indicative of imminent MI? unstable/cresendo angina Q3783:Which type of angina is a result of severe narrowing of atheroscclerotic coronary vessels? stable angina Q3784:What is the most common form of angina? stable angina Q3785:Where do red (hemorrhagic) infarcts occur? loose tissue w/ collaterals (e.g; lungs; intestine) or following reperfusion Q3786:Where do pale infarcts occur? in solid tissues w/ single blood supply (e.g; brain; heart; kidney; spleen) Q3787:Which coronary arteries are occluded the most? the least? LAD > RCA > circumflex Q3788:When is an MI maximally yellow and soft? 10 days post-MI Q3789:When are contraction bands visible on an MI? 4 hours post-MI Q3790:When is ECG the best method of diagnosis of an MI? w/in the first 6 hours post-MI Q3791:When does cardiac troponin I begin to rise? How long does it stay elevated? rises after 4 hours;elevated for 7-10 days Q3792:What is the test of choice for MI in the first 24 hours post-MI? CK-MB Q3793:How long does LDH stay elevated post-MI? 2-7 days Q3794:Is AST specific for liver? no;can also be found in cardiac; liver and skeletal muscle cells Q3795:ST elevation transmural infarct Q3796:ST depression subendocardial infarct Q3797:Q waves transmural infarct Q3798:What are the complications of an MI? cardiac arrhythmia;LV failure and pulmonary edema;cardiogenic shock;rupture of ventricular free wall; interventricular septum; papillary muscle; cardiac tamponade;thromboembolism - mural thrombus;fibrinous pericarditis (friction fub);Dressler's syndrome (autoimmune pheenomenon resulting in fibrinous pericarditis) Q3799:What is the most common cardiomyopathy? dilated (congestive) cardiomyopathy - 90% Q3800:What type of cardiomyopathy causes systolic dysfunction? dilated (congestive) cardiomyopathy Q3801:What type of cardiomyopathy causes diastolic dysfunction? hypertrophic cardiomyopathy Q3802:hypertrophic cardiomyopathy walls of LV are thickened (esp. the ventricular septum) and chamber becomes banana shaped;cause of sudden death in young athletes;50% inheriited as autosomal dominant trait;characterized microscopically by disoriented and tangled myocardial fibers Q3803:dilated cardiomyopathy most comon form of cardiomyopathy;characterized by dilation of both ventricles and by both right- and left-sided heart failure Q3804:restrictive cardiomyopathy caused by infiltrative processes w/in myocardium that results in stiffening of the heart muscle -> interferes w/ pumping action;exemplified by cardiac amloidosis -> right- and left- sided heart failure Q3805:mitral regurgitation holosystolic high-pitched "blowing murmur";loudest at apex Q3806:aortic stenosis crescendo-decrescendo systolic ejection murmur following ejection click;LV >> aortic pressure during systole;radiates to carotids/apex Q3807:VSD holosystolic murmur Q3808:mitral prolapse late systolic murmuc w/ midsystolic click;most frequent valvular lesion Q3809:aortic regurgitation immediate high-pitched "blowing" diastolic murmur;wide pulse pressure Q3810:mitral stenosis follows opening snap;delayed rumbling late diastolic murmur;LA >> LV pressure during diastole Q3811:patent ductus arteriosus continusos machine-like murmur;loudest at time of S2 Q3812:What are the causes of serous pericarditis? SLE;rheumatoid arthritis;infection;uremia Q3813:What are the causes of fibrinous pericarditis? uremia;MI;rheumatic fever Q3814:What are the causes of hemorrhagic pericarditis? TB;malignancy Q3815:What are the findings in hemorrhagic pericarditis? pericardial pain;friction rub;ECG chances (diffuse ST elevations in all leads);pulsus paradoxus;distant heart sounds Q3816:syphilitic heart disease tertiary syphilis disrupts the vasa vasorum of the aorta -> dilation of the aorta and valve ring;"tree bark" appearance of the aorta Q3817:Buerger's disease;(aka thromboangiitis obliterans) acute inflammation involving small to medium size arteries of the extremities; extending to adjacent veins and nerves;clearly assoc. w/ smoking Q3818:What is the treatment for Buerger's disease? quit smoking Q3819:Takayasu's arteritis inflammation and stenosis of medium and large sized arteries w/ freq. involvement of the aortic arch and its branches -> arotic arch syndrome;assoc. w/ elevated ESR;primarily affects young Asian females Q3820:What are the clinical symptoms of Tayakasu's arteritis? (FAN ON MY SKIN On Wednesday);Fever;Arthritis;Night sweats;MYalgia;SKIN nodules;Ocular disturbances;Weak pulses in upper extremities Q3821:Takayasu's arteritis primarily affects what population? young Asian females Q3822:Buerger's diease affects what vessels? small and intermediate vessels of the extremities Q3823:Takayasu's arteritis involves what vessels? medium and large arteries w/ frequent involvement of the aortic arch and its branches Q3824:temporal arteritis;(giant cell arteritis) most common vasculitis that affects medium and small arteries; usually branches of carotid artery Q3825:What are the clinical symptoms of temporal arteritis? unilateral headache;jaw claudication;imparied vision (occlusion of opthalmic artery -> blindness) Q3826:What population does temporal arteritis affect most? elderly females Q3827:Temporal arteritis is associated w/ what lab finding? elevated ESR Q3828:polyarteritis nodosa necrotizing immue complex inflammation of medium-sized muscular arteries; typically involving renal and visceral vessels Q3829:What is the treatment for polyarteritis nodosa? corticosteroids;cyclophosphamide Q3830:Wegner's granulomatosis characterized by focal necrotizing vasculitis and necrotizing granulomas in the lung and upper airway and by necrotizing glomerulonephritis Q3831:What is a strong marker for Wegner's granulomatosis? C-ANCA Q3832:What is the treatment for Wegner's granulomatosis? cyclophosphamide and corticosteroids Q3833:microscopic polyangiitis similar to Wegner's granulomatosis but lacks granulomas;P- or C-ANCA Q3834:Churg-Strauss syndrome granulomatous vasculitis w/ eosinophilia Q3835:What organs are involved in Churg-Strauss syndrome? lung; heart; skin; kidneys; nerves Q3836:Kawasaki's disease acute; self-limiting disease of infants/kids;acute necrotizing vasculitis of small/medium-sized vessels Q3837:People w/ Kawasaki's disease may develop what? coronary aneurysms Q3838:what does uremia do to the bone marrow? makes it less responsive to EPO Q3839:what is hydroxyurea used for? increases production of fetal hemoglobin HbF Q3840:what are Howell Jolly bodies? inclusions of nuclear chromatin remnants seen in RBCs of pts with hypoactive splenic function Q3841:why should sickle cell patients consider prophylactice cholecystectomy? 70% of sickle cell patients develop symptomatic cholelithiasis Q3842:why does hepatosplenomegaly develop in thalassemia pts? increased hemolysis of abnormal RBCs by macrophages in the spleen and liver; extramedullary hematopoiesis Q3843:where is dietary iron absorbed and in what form? absorbed in proximal duodenum; we eat Fe+++ and absorb as Fe++ Q3844:why do pts with chronic disease develop anemia? 1. chronic inflammation leads to more cytokines in blood and resulting increase in phagocytic activity destroying RBCs;2. cytokines inhibit renal secretion of EPO;3. lactoferrin released by inflammatory cells binds serum iron and makes it unavailable for erythropoiesis Q3845:what's the big picture problem in anemia of chronic disease and iron? high iron stores; but unavailable to the body Q3846:what does B12 do? 1. catalyzes conversion of homocysteine to methionine;2. catalyzes conversion of methyltetrahydrofolate to tetrahydrofolate;3. converts methylmalonic acid to succinyl CoA Q3847:what is tetrahydrofolate? form of folic acid used in DNA synthesis Q3848:what is G6PD? the rate-limiting step in HMP pathway for NADPH production Q3849:what is glutathione? after reduction by NADPH; it combats oxidatative damage in cells Q3850:what is primary hemostasis? production of the temporary platelet plug Q3851:how does tPA work as a clot buster? it's an enzyme that activates plasminogen; converting it to plasmin which cleaves fibrin strands Q3852:how does heparin work? stimulates antithrombin III; which inhibits thrombin (factor II) Q3853:what is microangiopathic hemolytic anemia? hemolytic anemia due to intravascular fracture of RBCs Q3854:E. coli 0157 and Shigella species are known for causing what heme dysfunction? TTP or HUS Q3855:what's the main problem in TTP or HUS? endothelial damage leads to platelet consumption Q3856:what does desmopressin acetate (DDAVP) do? stimulates the liver to produce plasma clotting factors Q3857:what are 2 common causes of acquired platelet dysfunction? uremia and NSAIDs Q3858:how do clopidogrel and ticlopidine work? these 2 antiplatelet drugs block platelet-ADP receptor activation Q3859:how do abciximab; eptifibatide and tirofiban work? inhibit platelet GpIIb-IIIa receptor which mediates aggregation via a "fibrinogen bridge" Q3860:what anticoagulant can cause thrombocytopenia? heparin in 1-3% of pts;;can cause 2 syndromes;HITS: heparin induced thrombocytopenia syndrome;HITT: heparin induced thrombocytopenia thrombosis Q3861:how can ITP be treated? prednisone to slow the immune-mediated destruction of platelets Q3862:What is the term for an abnormal amount of collagen type III that produces a large bulging scar; seen primarily in blacks? Keloid Q3863:True or false? Klinefelter syndrome cannot be diagnosed until puberty. True Q3864:What form of sunlight is the most carcinogenic? Ultraviolet B (UVB) sunlight Q3865:What renal pathology involves uniform thickening of the glomerular capillary wall; granular appearance under the microscope; and effacement of foot processes? Membranous glomerulonephritis Q3866:What enveloped RNA retrovirus infects CD4 T cells and uses the enzyme reverse transcriptase? HIV Q3867:What enzyme is deficient in chronic granulomatous disease of childhood? NADPH oxidase Q3868:What rare disorder presents as a large; hard; irregular thyroid gland due to fibrous proliferation of connective tissue in the thyroid gland and extends to adjacent structures? Riedel thyroiditis Q3869:Rheumatic fever most commonly follows pharyngeal infections with what bacteria? Group A beta -hemolytic streptococci Q3870:What benign cardiac tumor is associated with tuberous sclerosis? Rhabdomyoma Q3871:What are the rules of 10 regarding pheochromocytoma? 10% are bilateral; 10% malignant; and 10% familial; 10% in children; 10% outside the adrenal gland; and 10x more likely to be seen on the USMLE than in real life Q3872:What vascular pathology is associated with HTN in the upper extremities; hypotension in the lower extremities; and a radial-femoral delay? Postductal coarctation of the aorta (adult) Q3873:What seronegative spondyloarthropathy is seen in HLA-B27–positive young females and presents with the triad of conjunctivitis; urethritis; and arthritis affecting the knees and ankles? Reiter syndrome Q3874:What AD disease involves hyperkeratosis of the palms and soles in association with esophageal carcinoma? Tylosis Q3875:A 20-year-old woman who was recently diagnosed with a sexually transmitted disease goes to the ER with a tender; painful; swollen; and erythematous knee (monoarticular). What organism is the likely culprit? Neisseria gonorrhea (history of STD in patient with monoarticular infectious arthritis: think gonococcus) Q3876:What vasculitis is characterized by systemic vasculitis in small to medium- size vessels (except the lung); affecting young males; 30% HBsAg-positive; P-ANCA and autoantibodies against myeloperoxidase? Polyarteritis nodosa Q3877:What malignant bone tumor is associated with familial retinoblastoma? Osteosarcoma Q3878:What bilateral AR disorder seen in infancy as progressive renal failure has multiple small cysts at right angles to the cortical surface? Polycystic kidney disease of childhood Q3879:In what syndrome does the patient have angiomatosis; renal cell carcinomas; pheochromocytomas; retinal; cerebellar; medulla; or spinal cord hemangioblastomas; and epidermal cysts? von Hippel-Lindau syndrome Q3880:What is the term for hyperextension of the PIP and flexion of the DIP joints in rheumatoid arthritis? Swan-neck deformities Q3881:What is the term for white retinal spots surrounded by hemorrhage? In what condition are they seen? Roth spots; and they are seen in bacterial endocarditis. Q3882:A 70-year-old man complains of urinary urgency; nocturia; hesitancy; postvoid dribbling; urinary retention; and a PSA result of 6.5 ng/mL. What is your diagnosis? BPH. Although an argument can be made for prostatic cancer (you should expect a much higher PSA result); these are buzzwords for BPH. Prostatic cancer is usually silent until late in the disease; when obstructive symptoms begin to occur. Q3883:What triad consists of endothelial injury; changes in laminar flow; and hypercoagulation? Virchow triad; associated with the formation of a thrombus. Q3884:What bone cell has receptors for PTH? Osteoblasts (Remember; they modulate the function of osteoclasts.) Q3885:What type of peptic ulcer disease is characterized by the onset of burning epigastric pain immediately after eating? Gastric ulcer Q3886:What is the term for normal cellular genes associated with growth and differentiation? Proto-oncogenes Q3887:Blue sclera is seen in what hereditary bone disorder? Osteogenesis imperfecta Q3888:What form of anemia is diagnosed with sucrose lysis test and Ham test? Paroxysmal nocturnal hemoglobinuria Q3889:In what rare AR disorder do you see neutropenia; defective degranulation; and delayed microbial killing due to a problem in chemotaxis and migration? Chédiak-Higashi syndrome Q3890:What myeloid disorder is characterized by increased hematocrit; blood viscosity; basophils; and eosinophils; intense pruritus; and gastric ulcers due to histamine release from basophils; increased left atrial pressure; and plethora? Polycythemia vera (Remember; polycythemia vera is a risk factor for acute leukemias.) Q3891:If you order a V/Q scan for suspected pulmonary emboli; is the filling defect seen on the ventilation or perfusion side? Ventilation of an unperfused lung segment is highly suspicious for pulmonary embolism. Q3892:What transports iron in the blood? Transferrin Q3893:What hematological malignancy is particularly likely to affect patients with Down syndrome? ALL (nearly 15–20 times the normal risk) Q3894:What childhood pathology involves anterior bowing of the tibia; epiphyseal enlargements; and costochondral widening; with the endochondral bones being affected? Rickets Q3895:A Japanese man has weight loss; anorexia; early satiety; epigastric abdominal pain; and a palpable left supraclavicular lymph node. On endoscopy you find a large; irregular ulcer with elevated margins on the lesser curvature of the stomach. What is your diagnosis? Gastric carcinoma Q3896:What drug causes a sixfold increase in schizophrenia; can impair motor activity; and can cause lung problems? Marijuana Q3897:What is the term for neurologic signs consistent with a cerebrovascular accident but lasting 24 hours with full recovery? Transient ischemic attack Q3898:What is the name of the tumor when gastric carcinoma spreads to the ovaries? Krukenberg tumor Q3899:What condition results from a deficiency in the enzyme hexosaminidase A? Tay-Sachs disease Q3900:Which cerebral herniation results in compression of the anterior cerebral artery? Cingulate gyrus herniation (subfalcine) Q3901:What pathology involves excessive fibrosis throughout the body via increased fibroblast activity; occurs in women more than men; and is most commonly seen in the third to the fifth decade? Scleroderma Q3902:What is the term for the syndrome consisting of hepatomegaly; ascites; and abdominal pain due to hepatic vein thrombosis? Budd-Chiari syndrome Q3903:What form of angina is characterized by;? Coronary artery vasospasm; symptom occurrence at rest; ST segment elevation (during episode); and no signs on ECG? Prinzmetal variant angina Q3904:What form of angina is characterized by;? Coronary artery luminal narrowing; symptom occurrence during exertion; ST segment depression on ECG? Stable angina Q3905:What form of angina is characterized by;? Coronary artery nonocclusive thrombus; symptom occurrence with increasing frequency; duration; intensity; and decreasing activity; frequently at rest? Unstable (crescendo) angina Q3906:What skin condition is a localized proliferation of melanocytes presenting as small; oval; light brown macules? Benign lentigo Q3907:What is the term for nonneoplastic abnormal proliferation of cell size; shape; and cellular organization? Dysplasia Q3908:What diagnosis ensues from finding well-demarcated erythematous plaques with silvery scales on the knees; elbows; and scalp along with nail bed pitting and discoloration? Psoriasis Q3909:What renal disease in diabetic patients is seen as a halo of capillaries around the mesangial nodules? Kimmelstiel-Wilson disease Q3910:What autoimmune liver disease is characterized by affecting a middle-aged woman with jaundice; pruritus; fatigue; xanthomas; increased direct bilirubin levels; and antimitochondrial Abs? Primary biliary cirrhosis Q3911:What cell in chronic inflammation is derived from blood monocytes? Macrophages Q3912:True or false? Pancreatic insufficiency results in vitamin B12 malabsorption. True. Pancreatic enzymes begin the breakdown of vitamin B12-R complex in the duodenum. Q3913:What neuroendocrine tumor produces excess serotonin; is associated with diarrhea; flushing; bronchospasms; wheezing; and is diagnosed by findings of elevated urinary 5-HIAA levels? Carcinoid tumor Q3914:What tumor constitutes 40% of testicular tumors in children? Teratoma Q3915:What urinary metabolite is elevated in pheochromocytoma? Vanillylmandelic acid (VMA) Q3916:A 25-year-old black woman presents with nonproductive cough; shortness of breath; fatigue; and malaise; she has bilateral hilar lymphadenopathy on chest radiography and elevated ACE levels. What do you diagnose? Sarcoidosis Q3917:What are the four reasons for hypochromic microcytic anemia with a low MCV? 1. Sideroblastic anemias (i.e; porphyrin and heme synthesis disorders) ;2. Thalassemia ;3. Iron deficiency ;4. Lead poisoning Q3918:What is characterized by an intense inflammatory reaction; an increase in the amounts of granulation tissue and wound contraction by myofibroblasts? Healing by secondary intention Q3919:What thyroiditis presents as a tender; enlarged; firm thyroid gland; usually preceded by an upper respiratory viral illness? de Quervain thyroiditis Q3920:What disorder leads to IgG autoantibodies to the TSH receptor? Graves disease Q3921:What intrauterine deficiency leads to failure to thrive; mental retardation; motor incoordination; and stunted growth? Iodine; resulting in congenital hypothyroidism Q3922:What pancreatic islet cell tumor is associated with MEN I syndrome? Gastrinoma Q3923:What type of PUD is classically described by the onset of burning epigastric pain 1 to 3 hours after eating that is relieved by food? Duodenal ulcer Q3924:What disease arises from the adrenal medulla; displaces and crosses the midline; metastasizes early; is the most common solid tumor; and is seen in the 2-to 4-year-old age group? Neuroblastoma Q3925:What AD disease associated with chromosome 19 involves a defect in the LDL receptors that leads to skin and tendon xanthomas? Familial hypercholesterolemia Q3926:A 20-year-old woman goes to the ER with ptosis; diplopia; weakness in her jaw muscles when chewing; and muscle weakness with repeated use. What is your diagnosis? Myasthenia gravis Q3927:What is the term for RBCs with smooth undulations on the surface of their membrane; commonly seen in uremia? Burr cells (echinocytes) Q3928:Name the cancer associated with the following tumor markers. (Some may have more than one answer.);? Beta-hCG Choriocarcinomas and trophoblastic tumors Q3929:Name the cancer associated with the following tumor markers. (Some may have more than one answer.);? CA-125 Ovarian cancer Q3930:Name the cancer associated with the following tumor markers. (Some may have more than one answer.);? CA-19.9 and CEA Pancreatic cancer Q3931:Name the cancer associated with the following tumor markers. (Some may have more than one answer.);? alpha- Fetoprotein Hepatoma and nonseminomatous testicular germ cell tumors Q3932:Name the cancer associated with the following tumor markers. (Some may have more than one answer.);? Calcitonin Medullary carcinoma of the thyroid Q3933:Name the cancer associated with the following tumor markers. (Some may have more than one answer.);? PSA and prostatic acid phosphatase Prostate cancer Q3934:Name the cancer associated with the following tumor markers. (Some may have more than one answer.);? Placental alkaline phosphatase Seminoma Q3935:Name the cancer associated with the following tumor markers. (Some may have more than one answer.);? CEA Cancer of the lung; stomach; colon; and breast Q3936:What seronegative spondyloarthropathy is seen in HLA-B27–positive young men; involves the sacroiliac joints; has no subcutaneous nodules; and has a bamboo spine appearance on radiograph? Ankylosing spondylitis Q3937:What disorder is associated with decreased platelet count; prolonged PT and PTT; decreased fibrinogen; and increased fibrin split products (D-dimers)? Disseminated intravascular coagulation (DIC) Q3938:What spirochete is responsible for Lyme disease? Borrelia burgdorferi Q3939:What very aggressive lung cancer metastasizes early and is associated with smoking and paraneoplastic syndromes? Small cell carcinoma (oat cell) Q3940:What bone disorder is characterized by brown tumors; bone pain; deformities; and fractures due to excessive PTH? Osteitis fibrosa cystica (von Recklinghausen disease) Q3941:What glycogen storage disease is due to the following enzyme deficiencies?;? Lysosomal glucosidase (acid maltase) Pompe's disease Q3942:What glycogen storage disease is due to the following enzyme deficiencies?;? Muscle phosphorylase McArdle's syndrome Q3943:What glycogen storage disease is due to the following enzyme deficiencies?;? Glucose-6-phosphatase von Gierke's disease Q3944:What ovarian disease involves psammoma bodies? Serocystadenocarcinoma Q3945:What cystic swelling of the chorionic villi is the most common precursor of choriocarcinoma? Hydatidiform mole Q3946:In what condition do you see dimpling on the kidney's surface? Pyelonephritis Q3947:What are the most common causes of osteomyelitis;? Overall? Staphylococcus aureus Q3948:What are the most common causes of osteomyelitis;? In neonates? Streptococcus agalactiae Q3949:What are the most common causes of osteomyelitis;? In patients with sickle cell disease? Staphylococcus aureus (but they are more prone to salmonella infections) Q3950:What are the most common causes of osteomyelitis;? In drug addicts? Pseudomonas Q3951:What malignant neoplasm of the bone is associated with Homer-Wright pseudorosettes; onion skinning of the periosteum on radiographs of the femur; pelvis; and tibia; and chromosome 11;22 translocation? Ewing's sarcoma Q3952:What lymphoma is characterized by CD19; CD20; CD5; CD23-negative; and chromosome 11;14 translocations? Mantle cell lymphoma Q3953:What components of the complement cascade form the MAC? C5b–C9 Q3954:True or false? HPV infection increases the risk of developing squamous cell carcinoma of the penis. True. HPV serotypes 16 and 18 are risk factors for squamous cell carcinoma. Q3955:What form of coarctation of the aorta is associated with Turner syndrome? Preductal (infantile) Q3956:True or false? An elevated serum osteocalcin level is a marker for increased bone formation. True. Increased alkaline phosphatase levels also are associated with increased bone formation. Q3957:What is the most common opportunistic infection of the CNS in HIV? Toxoplasmosis Q3958:What hereditary bone disorder is due to decreased osteoclast function; resulting in thick; sclerotic bones that fracture easily? Osteopetrosis (Albers-Sch?nberg disease) Q3959:True or false? Pancreatic delta-cell tumors inhibit CCK secretion; leading to gallstones and steatorrhea. True. Delta-Cell tumors produce excess somatostatin; which inhibits CCK; gastrin (hypochlorhydria); and insulin secretion (diabetes). Q3960:What is the term for the speckled appearance of the iris in patients with Down syndrome? Brushfield spots Q3961:What is the term for the collapse of the vertebral body due to TB? Pott disease Q3962:What organ must metastasize for carcinoid heart disease to occur? Liver Q3963:Which subset of MEN syndrome is associated with the following?;? Medullary carcinoma of the thyroid; pheochromocytoma; and mucocutaneous neuromas MEN III (or IIb) Q3964:Which subset of MEN syndrome is associated with the following?;? Medullary carcinoma of the thyroid; pheochromocytoma; and parathyroid adenomas (or hyperplasia) MEN IIa (or Sipple syndrome) Q3965:Which subset of MEN syndrome is associated with the following?;? Parathyroid; pancreatic; and pituitary gland tumors and Zollinger-Ellison syndrome MEN I (or Wermer syndrome) Q3966:What X-linked recessive disease involves mental retardation; self-mutilation; choreoathetosis; spasticity; a decrease in HGPRT; and an increase in uricemia? Lesch-Nyhan syndrome Q3967:What disorder is associated with spider angiomas; palmar erythema; gynecomastia; testicular atrophy; encephalopathy; abnormalities in clotting factors; and portal HTN? Cirrhosis Q3968:What is your diagnosis of a young; thin asymptomatic female with a midsystolic click on cardiac auscultation? Mitral valve prolapse Q3969:What infection is associated with ring-enhancing lesions seen on computed tomography (CT) of the brain in an HIV-positive individual? Toxoplasmosis (although you should rule out cerebral abscess due to other organisms) Q3970:What is the term for a reversible change in one cell type to another? Metaplasia (usually to a more protective cell type) Q3971:What liver tumor is associated with oral contraceptive pill use? Hepatic adenomas Q3972:What CNS developmental abnormality is associated with downward displacement of the cerebellar vermis and medulla compressing the fourth ventricle and leading to obstructive hydrocephalus? Arnold-Chiari malformation type 2 Q3973:What disease involves a lack of both T cell-mediated and humoral immune responses that can be either X-linked or AR? Severe combined immunodeficiency disease Q3974:What condition results in the following CSF results?;? Opening pressure 70 to 180 mm H2O; 0–10 WBCs (monocytes); glucose 45 to 85; protein 15 to 45 Normal values Q3975:What condition results in the following CSF results?;? Opening pressure 450 mm H2O; 5 WBCs (90% lymphocytes); normal glucose and protein levels Brain abscess Q3976:What condition results in the following CSF results?;? Opening pressure 100 mm H2O; 120 WBCs (90% lymphocytes); normal glucose levels; protein 17 Viral meningitis Q3977:What condition results in the following CSF results?;? Opening pressure 250 mm H2O; WBCs 250 (90% lymphocytes); glucose 35; protein 100 TB meningitis Q3978:What condition results in the following CSF results?;? Opening pressure 400 mm H2O; WBCs 8500 (90% PMNs); glucose 15; protein 120 Bacterial meningitis Q3979:True or false? Removal of the ileum results in vitamin B12 deficiencies. True. The ileum is the site where vitamin B12 is absorbed. Q3980:What is the term for edema that has LDH below 200; protein level 2.5; and a specific gravity below 1.020? Transudative; exudative has the opposite values and has an elevated cellular content. Q3981:What is the term for thickened; hyperpigmented skin in the axillae; groin; and skin folds associated with malignancies; obesity; and DM? Acanthosis nigricans Q3982:How many grams of protein must be excreted in 24 hours to produce the diagnosis of nephrotic syndrome? >3.5 g/day of protein; along with generalized edema; hypoalbuminemia; and hyperlipidemia Q3983:What illicit drug can cause amyloidosis and focal segmental glomerulosclerosis in the kidney? Heroin Q3984:What catecholamine-hypersecreting tumor; a secondary cause of HTN; results in headache; diaphoresis; anxiety; tachycardia; and palpitations? Pheochromocytoma Q3985:What is the term for flattened nose; low-set ears; and recessed chin seen in patients with bilateral renal agenesis? Potter facies Q3986:What type of healing occurs in a clean surgical incision? Primary intention Q3987:What are the two most common viral infections in HIV? CMV retinitis and HSV-2 Q3988:What disorder is defined by inability of the lower esophageal sphincter to relax with swallowing and a bird beak barium swallow result? Achalasia. (Think Chagas disease if it presents in a person from Central or South America.) Q3989:Does Cushing syndrome or Cushing disease have elevated ACTH levels and cortisol suppression with dexamethasone? Cushing's disease (pituitary) has elevated ACTH and cortisol suppression with dexamethasone; whereas Cushing's syndrome (adrenal adenoma) has decreased ACTH and no cortisol suppression with dexamethasone. Q3990:What CD4 T-cell receptor does the HIV virus bind to? gp120 Q3991:What is the term for RBC fragments? Schistocytes Q3992:What disorder is due to a deficiency in tyrosinase? Albinism Q3993:What two Abs are used to diagnose Hashimoto's thyroiditis? Antithyroglobulin and antimicrosomal Abs Q3994:What urease-producing gram-negative curved rod is associated with PUD and chronic gastritis? Helicobacter pylori; which is also associated with an increased risk of gastric carcinoma Q3995:Name the cancer associated with the following chemical agents. (Some may have more than one answer.);? Alkylating agents Leukemias and lymphomas Q3996:Name the cancer associated with the following chemical agents. (Some may have more than one answer.);? Aromatic amines and azo dyes Hepatocellular carcinoma Q3997:Name the cancer associated with the following chemical agents. (Some may have more than one answer.);? Arsenic Squamous cell carcinoma (skin; lung) and angiosarcoma of the liver Q3998:Name the cancer associated with the following chemical agents. (Some may have more than one answer.);? Asbestos Mesothelioma and bronchogenic carcinoma Q3999:Name the cancer associated with the following chemical agents. (Some may have more than one answer.);? Naphthylamine Bladder cancer Q4000:Name the cancer associated with the following chemical agents. (Some may have more than one answer.);? Benzenes Leukemias Q4001:Name the cancer associated with the following chemical agents. (Some may have more than one answer.);? Vinyl chloride Angiosarcoma of the liver Q4002:Name the cancer associated with the following chemical agents. (Some may have more than one answer.);? Chromium and nickel Bronchogenic carcinoma Q4003:Name the cancer associated with the following chemical agents. (Some may have more than one answer.);? Polycyclic aromatic hydrocarbons Bronchogenic carcinoma Q4004:Name the cancer associated with the following chemical agents. (Some may have more than one answer.);? Nitrosamines Gastric cancer Q4005:What are the five conditions associated with normochromic normocytic anemia with a normal MCV and an elevated reticulocyte count? 1. Autoimmune hypersplenism ;2. Trauma ;3. Anemia ;4. Spherocytosis ;5. Sickle cell anemia Q4006:What pancreatic islet cell tumor is associated with hypoglycemia; sweating; hunger; confusion; and increased C- peptide levels? Insulinoma Q4007:What substance is used to test platelets' response in patients with von Willebrand disease? Ristocetin Q4008:What X-linked recessive disorder that is due to an abnormality in the dystrophin gene; has onset by age 5 with progressive proximal muscle weakness; calf pseudohypertrophy; and elevated CPK levels? Duchenne muscular dystrophy (Remember; Becker's is slower in progress; less severe; later in onset; and lacks cardiac involvement.) Q4009:What subset of adenocarcinoma arises from the terminal bronchioles and/or alveolar walls? Bronchioloalveolar carcinoma Q4010:What estrogen-producing tumor of the female genital tract is characterized by Call-Exner bodies? Granulosa cell tumor of the ovary Q4011:What AD syndrome involves 1000 or more edematous polyps; most commonly affects the colorectal area; and is associated with chromosome 5q21? Familial polyposis coli Q4012:What is the term for osteophyte formation at the proximal interphalangeal (PIP) joints in osteoarthritis? In the distal interphalangeal (DIP) joints? Bouchard nodes in the PIP joints; Heberden nodes in the DIP joints. Q4013:What prostaglandin is associated with maintaining patency of the ductus arteriosus? PGE (along with low oxygen tension) Q4014:What is the term for dilated veins within the spermatic cord? Varicocele Q4015:What type of hemostasis in an intravascular space consists of fibrin; platelets; RBCs; and WBCs? Thrombus Q4016:What is the most common primary malignant tumor in bone? Osteosarcoma Q4017:What is the most common infectious agent in HIV? Pneumocystis carinii Q4018:What myeloid disorder is characterized by dry bone marrow aspirations; splenomegaly; leukoerythroblastosis; teardrop RBCs; and hyperuricemia due to increased cell turnover? Myelofibrosis with myeloid metaplasia Q4019:What disease that involves mental retardation; flat face; muscle hypotonia; and a double-bubble sign on radiograph poses an increased risk of Alzheimer's disease and ALL? Down syndrome (trisomy 21) Q4020:What form of anemia is associated with IgG Abs against Rh antigens; positive direct Coombs test; and splenomegaly? Autoimmune hemolytic anemia Q4021:What urinary metabolite is increased in patients with carcinoid syndrome? 5-hydroxyindoleacetic acid (5-HIAA) Q4022:What chronic liver disease has a beaded appearance of the bile ducts on cholangiogram? Primary sclerosing cholangitis Q4023:What three LTs are associated with bronchospasms and an increase in vessel permeability and vasoconstriction? LC4; LD4; and LE 4 Q4024:What are the three Bs of adult polycystic kidneys? 1. Big ;2. Bilateral ;3. Berry aneurysm Q4025:What AR disease involves a defect in AA 508 on chromosome 7; causing a defect in Cl- transportation that leads to recurrent pulmonary infections and an increase in viscid mucoid secretions along with pancreatic insufficiencies? Cystic fibrosis. (Parents are usually the first to find out because the baby tastes salty.) Q4026:What law states that an enlarged; palpable gallbladder is more likely due to cancer than stone obstruction? Courvoisier's law Q4027:What is the term for air in the pleural space? Pneumothorax Q4028:What disorder of bone remodeling results in thick; weak bones and is associated with high-output cardiac failure? Paget disease (osteitis deformans) Q4029:What is the term to describe the increase in organ size due to the increase in cell size and function? Hypertrophy Q4030:What slow-growing primary CNS tumor that affects mostly females is associated with psammoma bodies? Meningioma Q4031:True or false? Ethyl alcohol induces the cytochrome P-450 enzymes. True Q4032:What are the five components of portal HTN? 1. Caput medusae ;2. Esophageal varices ;3. Ascites ;4. Splenomegaly ;5. Hemorrhoids Q4033:What syndrome results when there is a deletion to paternal chromosome 15? Maternal? Prader-Willi syndrome and Angelman syndrome; respectively Q4034:What CNS tumor arises from Rathke's pouch? Craniopharyngioma Q4035:What is the triad of Reiter syndrome? 1. Conjunctivitis;2. Nongonococcal urethritis ;3. Peripheral arthritis ;Can't see; can't wee; can't kick with your knee Q4036:Which of the following is not a risk factor for cholesterol gallstones: pregnancy; OCP use; female gender; hemolytic anemia; cirrhosis; and obesity? (May be more than one answer.) Cirrhosis and hemolytic anemia; which are risk factors for pigmented gallstones. Q4037:Name the nephritic disease based on the immunofluorescent staining;? Mesangial deposits of IgA and C3 IgA nephropathy (Berger disease) Q4038:Name the nephritic disease based on the immunofluorescent staining;? Smooth and linear pattern of IgG and C3 in the GBM Goodpasture disease Q4039:Name the nephritic disease based on the immunofluorescent staining;? Granular deposits of IgG; IgM; and C3 throughout the glomerulus Postinfectious GN Q4040:What glycoprotein allows platelets to adhere to each other through the use of fibrinogen? GP IIb/IIIa; which is why GP IIb/IIIa inhibitors are used in the treatment of acute coronary syndromes Q4041:What virus is associated with body cavity large B-cell lymphomas? HHV-8 Q4042:What germ cell tumor is seen in the 15-to 35-year-old age group; peaks when the person is 35 years of age; and is a bulky mass that spreads via the lymphatic system? Seminoma Q4043:What transmural inflammatory bowel disease can be found from the mouth to anus; has noncaseating granulomas; is discontinuous (skip lesions); and has a cobblestone appearance; thickening of the bowel wall; linear fissures; and aphthous ulcers with normal mucosa between? Crohn disease Q4044:Does ELISA or Western blot confirm whether a patient is HIV-positive? ELISA screens and Western blot confirms the diagnosis. Q4045:What GN is highly associated with hepatitis B and C infections? Membranoproliferative glomerulonephritis (MPGN) Q4046:What is the term for squamous to columnar metaplasia of the distal esophagus secondary to chronic inflammation? Barrett esophagus has an increased risk of developing adenocarcinoma of the esophagus. Q4047:What is the term for excessive amounts of granulation tissue that can block re-epithelialization and wound healing? Proud flesh Q4048:What is released from the mitochondria to trigger apoptosis? Cytochrome c Q4049:How much of a vessel must be stenosed to cause sudden cardiac death? More than 75% of the vessel Q4050:Oxidation of Hgb forms what bodies in patients with G-6-PD deficiency? Heinz bodies Q4051:What syndrome that is due to an adrenal gland adenoma produces excess aldosterone resulting in HTN; hypokalemia; and low rennin levels? Conn syndrome (primary hyperaldosteronism) Q4052:What virus is associated with both nasopharyngeal carcinoma and Burkitt lymphoma? EBV Q4053:What normochromic; normocytic AD anemia has splenomegaly and increased osmotic fragility? Hereditary spherocytosis Q4054:What does prepubertal hypersecretion of growth hormone lead to? Gigantism Q4055:What enzyme is deficient in alkaptonuria? Homogentisic oxidase Q4056:What sex cell tumor causes precocious puberty; masculinization; gynecomastia in adults; and crystalloids of Reinke? Leydig cell tumor Q4057:Name four major risk factors for atherosclerosis. DM; hypercholesterolemia; smoking; and HTN are major risk factors. Being male; obesity; sedentary lifestyle; homocysteine elevation; oral contraceptive pills; and genetics are minor risk factors for atherosclerosis. Q4058:Name the AD disease associated with chromosome 15 in which the patient has long extremities; lax joints; pigeon chest; and posterior mitral leaflet prolapse and is prone to developing dissecting aortic aneurysm. Marfan syndrome Q4059:What is the term for a large VSD that leads to pulmonary HTN; RVH; and cyanosis due to right-to-left reversal of the shunt? Eisenmenger syndrome; which can also occur with any left-to- right shunt Q4060:Eating fava beans can produce the Mediterranean type of what deficiency? G-6-PD deficiency Q4061:What form of hemophilia is X-linked recessive and due to a deficiency in factor VIII? Hemophilia A Q4062:What leukemia affects a 4-year-old child with 3 months of fever; fatigue; generalized lymphadenopathy; CNS involvement; hepatosplenomegaly; bleeding; and platelet count below 100; 000? ALL Q4063:What protein deficiency results in respiratory distress syndrome of newborns? Deficiency in surfactant Q4064:What are the three components of amyloid? 1. Fibrillary protein ;2. Amyloid protein ;3. Glycosaminoglycans Q4065:What autoimmune disorder is due to Abs directed to ACh receptors at the NMJ? Myasthenia gravis Q4066:What are the three left-to-right shunts? 1. VSD ;2. ASD ;3. PDA Q4067:What protein causes fibrinolysis? Plasmin Q4068:What pancreatic islet cell tumor is associated with watery diarrhea; hypokalemia; and achlorhydria? VIPoma Q4069:True or false? Obesity; DM; HTN; multiparity; early menarche; and late menopause are all risk factors for endometrial carcinoma. False. They are all risk factors for endometrial carcinoma except multiparity. Nulliparity; estrogen-producing tumors; and estrogen replacement therapy are also risk factors for endometrial carcinoma. Q4070:What is the term for pigmented iris hamartomas seen in patients with neurofibromatosis type 1? Lisch nodules Q4071:What GI pathology is associated with a positive string sign; an increase in the number of bloody stools; RLQ pain; skip lesions; terminal ileum most commonly affected; occurrence in women more than men; and an increased thickness of the bowel? Crohn disease Q4072:What is the most common fungal infection in HIV? Candida Q4073:True or false? GERD is a cause of asthma. True. Don't forget this in your differential diagnosis of an asthmatic. Q4074:Name the product or products of arachidonic acid;? Vasodilation and inhibition of platelet aggregation produced by vascular endothelium PGI2 Q4075:Name the product or products of arachidonic acid;? Vasodilation PGD2; PGE2; and PGF 2 Q4076:Name the product or products of arachidonic acid;? Pain and fever PGE2 Q4077:Name the product or products of arachidonic acid;? Vasoconstriction and platelet aggregation produced by platelets TXA2 Q4078:Name the product or products of arachidonic acid;? Chemotactic for neutrophils LTB4 Q4079:Name the product or products of arachidonic acid;? Vasodilation; bronchospasm; and increased vascular permeability LTC4; LTD4; and LTE4 Q4080:Which hepatitis B Ab indicates low transmissibility? HBeAb Q4081:What pneumoconiosis is associated with exposure to the following occupations or materials?;? Miners; metal grinders; and sandblasters Silicosis;Note: Coal worker's pneumoconiosis is synonymous with black lung disease; an upper lobe occupational disorder Q4082:What pneumoconiosis is associated with exposure to the following occupations or materials?;? Aerospace industry; nuclear reactors Berylliosis Q4083:What pneumoconiosis is associated with exposure to the following occupations or materials?;? Shipyards; brake linings; insulation; and old building construction Asbestosis Q4084:What is the term for calcification of the gallbladder seen on radiograph due to chronic cholecystitis or adenocarcinoma of the gallbladder? Porcelain gallbladder Q4085:What is the term to describe a decrease in the cell size and function usually associated with disuse? Atrophy. Disuse can also be due to immobilization; ischemia; aging; and a host of other causes. Q4086:Which B-cell neoplasm has the following cell surface markers: CD19; CD20; CD5 (T-cell marker); CD23; and are CD10-negative? CLL (B-cell origin) Q4087:What disease caused by decompression sickness leads to multiple foci of ischemic necrosis that affect the head of the femur; humerus; and tibia? Caisson disease Q4088:What are the four DNA oncogenic viruses? 1. HPV ;2. EBV ;3. Hepatitis B ;4. Kaposi sarcoma Q4089:Is the AD or AR form of osteopetrosis malignant? The AR form is malignant and AD is benign. Q4090:What carcinoma produces hematuria; flank pain; and a palpable mass? This is the triad of renal cell carcinoma Q4091:Name at least three causes of metastatic calcification. Remember the mnemonic PAM SMIDT;P; (hyper) Parathyroid/ Paget disease;A; Addison's disease;M; Milk- alkali syndrome/metastatic cancer;S; Sarcoidosis;M; Multiple myeloma;I; Immobilization/idiopathic;D; Vitamin D intoxication;T; Tumors Q4092:What is the only subtype of Hodgkin's lymphoma that is most commonly seen in females? Nodular sclerosis Q4093:What is the leading cause of preventable premature death and illness in the United States? Smoking Q4094:What prion-associated CNS pathology produces rapidly progressive dementia with myoclonus; involuntary movements; and death within 6 to 12 months? Creutzfeldt-Jakob disease Q4095:What breast malignancy has tumor cells with a halo surrounding the nucleus and is an ulceration of the nipple and areola with crusting; fissuring; and oozing? Paget disease of the breast Q4096:What breast pathology involves malignant cells with halos invading the epidermis of the skin? Paget disease of the breast Q4097:Macro-ovalocytes in the peripheral blood smear are formed from what cell in the bone marrow? Megaloblasts Q4098:Name the type of exudate; given the following examples;? Sunburn Serous exudates Q4099:Name the type of exudate; given the following examples;? Uremic pericarditis Fibrinous exudates Q4100:Name the type of exudate; given the following examples;? Parasitic infection Eosinophilic exudates Q4101:Name the type of exudate; given the following examples;? Diphtheria infection Pseudomembranous exudates Q4102:Name the type of exudate; given the following examples;? Meningococcal infection Purulent exudates Q4103:Name the type of exudate; given the following examples;? Rickettsial infection Hemorrhagic exudates Q4104:What parasite is associated with squamous cell carcinoma of the urinary bladder? Schistosoma haematobium Q4105:What malabsorption syndrome produces abdominal distention; bloating; flatulence; diarrhea; steatorrhea; and weight loss shortly after eating bread products? Celiac sprue (gluten-sensitive enteropathy) Q4106:What herpes virus is associated with Kaposi sarcoma? HHV 8 Q4107:What is the term for the copper corneal deposits found in Wilson's disease? Kayser-Fleischer rings Q4108:Name the six vitamin K–dependent coagulation factors. Factors II; VII; IX; and X and proteins C and S. Q4109:What ovarian carcinoma is characterized by psammoma bodies? Cystadenocarcinoma Q4110:A marfanoid patient presents with tearing retrosternal chest pain radiating to her back. What is your first diagnosis? Dissecting aortic aneurysm. MI is also high on the list; but these are buzzwords to look for dissection. Q4111:What malignant neoplasm of the skin is associated with keratin pearls? Squamous cell carcinoma Q4112:Name four chemotactic factors for neutrophils. N-formyl-methionine;LTB4;C5a;IL-8 Q4113:What is the term for granuloma at the lung apex in TB? Simon focus Q4114:What are the three platelet aggregating factors? 1. ADP ;2. PG ;3. TXA2 Q4115:What syndrome is due to a Neisseria sp. infection in a child resulting in bilateral hemorrhagic infarcts of the adrenal glands? Waterhouse-Friderichsen syndrome Q4116:What foci of fibrinoid necrosis are surrounded by lymphocytes and macrophages throughout all the layers of the heart? Aschoff bodies of rheumatic fever Q4117:What is the leading cause of primary hyperparathyroidism? Chief cell adenoma (80%) Q4118:In what X-linked recessive disease is there a decrease in the HMP shunt; along with Heinz body formation? G-6-PD deficiency Q4119:What is the term for a RBC that has a peripheral rim of Hgb with a dark central Hgb-containing area? Target cell Q4120:True or false? Raynaud's phenomenon has no underlying pathology. False. The disease has no associated pathology; the phenomenon is arterial insufficiency due to an underlying disease. Q4121:What benign solitary papillary growth within the lactiferous ducts of the breast commonly produces bloody nipple discharge? Intraductal papilloma Q4122:What form of hemophilia is X-linked recessive and is due to a deficiency in factor IX? Hemophilia B Q4123:What are the two reasons for megaloblastic anemia with elevated MCV? Vitamin B12 deficiency and folate deficiency Q4124:Is cigarette smoking associated with transitional cell carcinoma of the bladder? Yes. It is also a cause of cancers of the lung; esophagus; ureter; and kidney; just to name a few. Q4125:What disease has multiple schwannomas; café-au-lait spots on the skin; and Lisch nodules and is associated with chromosome 17q? Neurofibromatosis I (chromosome 22q is with neurofibromatosis II and no Lisch nodules) Q4126:What syndrome is due to Abs directed to presynaptic calcium channels and causes axial and girdle muscle weakness that improves with repeated use? Eaton-Lambert syndrome Q4127:In what rare lung malignancy have 90% of patients had an occupational exposure to asbestos? Malignant mesothelioma Q4128:What is the term for cytoplasmic remnants of RNA in RBCs; seen in lead poisoning? Basophilic stippling Q4129:What is the triad of fat embolism? 1. Petechiae ;2. Hyperactive mental status ;3. Occurrence within 24 to 48 hours of the initial insult (e.g; long bone fracture) Q4130:Upon seeing negatively birefringent needle-shaped crystals from a joint aspiration of the great toe; what form of arthritis do you diagnose? Gout Q4131:What condition is manifested by bilateral sarcoidosis of the parotid glands; submaxillary gland; and submandibular gland with posterior uveal tract involvement? Mikulicz syndrome Q4132:What female genital tract disorder is characterized by obesity; hirsutism; infertility; amenorrhea; elevated LH and testosterone levels; and low FSH levels? Polycystic ovary disease (Stein-Leventhal syndrome) Q4133:What bronchogenic carcinoma is associated with an elevated level of Ca2+; involves keratin pearls; occurs in men more than women; is associated with smoking; occurs in the major bronchi; and is seen in the central areas of the lung? Squamous cell carcinoma Q4134:What disorder is due to a deficiency in the enzyme phenylalanine hydroxylase? PKU Q4135:True or false? Being a white male increases your risk factor for testicular cancer. Oddly enough; it is true. Cryptorchidism; Klinefelter syndrome; testicular feminization; and family history of testicular cancer are all risk factors. Q4136:Can an acute MI be diagnosed only by looking at an ECG? No. Remember; tests do not diagnose; they confirm or refute your diagnosis. Also; diagnosis of MI requires two of three criteria: chest pain consistent and characteristic of MI; elevated cardiac enzymes consistent with MI; and ST segment elevation of 2 mm or more in at least two contiguous leads. Q4137:What autoimmune syndrome is characterized by keratoconjunctivitis; corneal ulcers; xerostomia; and an increased risk of high-grade B-cell lymphomas? What two Ab tests are used in making the diagnosis? Sj?gren's syndrome; SS-A (Ro) and SS-B (La) Q4138:True or false? Sickle cell anemia; Caisson disease; chronic steroid use; and Gaucher disease are causes of avascular necrosis of bone. True. Fractures and trauma; however; are the most common causes. Q4139:What gene stimulates apoptosis when DNA repair is unable to be done? p-53 Q4140:Is ulcerative colitis or Crohn disease more commonly associated with primary sclerosing cholangitis? Ulcerative colitis Q4141:What test uses p24 protein when diagnosing HIV? ELISA test Q4142:How many café-au-lait spots are necessary for the diagnosis of neurofibromatosis type 1? At least six Q4143:What is the term for severe and protracted vomiting resulting in linear lacerations at the gastroesophageal junction? Mallory-Weiss syndrome Q4144:What is the term for hypercalcemia resulting in precipitation of calcium phosphate in normal tissue? Metastatic calcification Q4145:What is the term for a twisting of the bowel around its vascular axis resulting in intestinal obstruction? Volvulus Q4146:What form of poisoning is associated with bitter almond–scented breath? Cyanide Q4147:Name the type of hypersensitivity reaction based on the following properties;? Circulating Ab-Ag immune complexes deposited in the tissue result in neutrophil attraction and the release of lysosomal enzymes. Type III hypersensitivity (immune complex) Q4148:Name the type of hypersensitivity reaction based on the following properties;? IgE-mediated release of chemical mediators from basophils and mast cells; need prior exposure to Ag in the past; eosinophils amplify and continue reaction; can be system or localized. Type I hypersensitivity (anaphylactic) Q4149:Name the type of hypersensitivity reaction based on the following properties;? IgG or IgM Abs against a specific target cell or tissue; complement-dependent or ADCC. Type II hypersensitivity (cytotoxic) Q4150:Name the type of hypersensitivity reaction based on the following properties;? Reaction-mediated by sensitized T- cells Type IV hypersensitivity (cell-mediated) Q4151:What highly undifferentiated aggressive CNS tumor of primordial neuroglial origin develops in children and is associated with pseudorosettes? Primitive neuroectodermal tumors (i.e; medulloblastomas and retinoblastomas) Q4152:What syndrome is due to anti-GBM Abs directed against the lung and kidneys? Goodpasture syndrome Q4153:What pathway of the coagulation cascade is activated when it is in contact with foreign surfaces? Intrinsic. The extrinsic pathway is activated by the release of tissue factors. Q4154:What tumor is seen in the 2-to 4-year-old age group; does not cross the midline; has immature glomeruli; tubules; and stroma; and metastasizes late to the lungs? Wilms tumor Q4155:What CNS tumor commonly produces tinnitus and hearing loss? Schwannoma Q4156:True or false? Anticentromere Abs are used in diagnosing CREST syndrome. True. Scl-70 Abs are used in diagnosing diffuse scleroderma. Q4157:What AR CNS disorder presents early in childhood with gait ataxia; loss of deep tendon reflexes; impaired vibratory sensation; hand clumsiness; and loss of position sense? Friedreich ataxia Q4158:What potent platelet aggregator and vasoconstrictor is synthesized by platelets? TXA2 Q4159:Which type of cerebral herniation is associated with CN III palsy? Transtentorial (uncal) Q4160:What form of vasculitis involves the ascending arch and causes obliterative endarteritis of the vasa vasorum? Syphilitic Q4161:What is the main type of cell involved in cellular immunity? T lymphocyte Q4162:What skin condition has irregular blotchy patches of hyperpigmentation on the face commonly associated with OCP use and pregnancy? Melasma Q4163:What is the classic triad of TB? Fever; night sweats; and hemoptysis Q4164:True or false? Blood clots lack platelets. True. A thrombus has platelets; but clots do not. Q4165:What malignant tumor of the skin is associated with Birbeck granules? Histiocytosis X Q4166:What type of anemia is the result of a deficiency in intrinsic factor? Pernicious anemia (secondary to a lack of vitamin B12 absorption) Q4167:What cancer is particularly likely to affect English chimney sweeps? Scrotal cancer; due to the high exposure to polycyclic aromatic hydrocarbons Q4168:What is the term for a raised fluid-filled cavity greater than 0.5 cm that lies between the layers of the skin? Bulla Q4169:What virus is associated with the endemic form of Burkitt lymphoma? EBV Q4170:With which pituitary adenoma is an elevated somatomedin C level associated? GH-producing adenoma Q4171:What three criteria allow you to differentiate an ulcer from an erosion or carcinoma? 1. Less than 3 cm ;2. Clean base ;3. Level with the surrounding mucosa Q4172:Name the four right-to-left congenital cardiac shunts. Truncus (1) arteriosus;Transposition of the (2) great vessels;Tri(3)cuspid atresia;Tetra(4)logy of Fallot;They all begin with T Q4173:What do low levels of Ca2+ and PO4- along with neuromuscular irritability indicate? Hypoparathyroidism Q4174:Does PT or PTT test the extrinsic coagulation pathway? PT for extrinsic and PTT for intrinsic (remember: wPeT and hPiTT; which means warfarin; extrinsic; PT; heparin; intrinsic; PTT) Q4175:What leukemia is associated with four-leaf-clover lymphocytes on peripheral blood smear? Adult T-cell leukemia Q4176:What ring is a weblike narrowing of the gastroesophageal junction? Schatzki ring Q4177:With what disease do you see IgA deposits in small vessels of the skin and the kidneys? Henoch-Sch?nlein purpura Q4178:What rapidly progressive and aggressive T-cell lymphoma affects young males with a mediastinal mass (thymic)? Lymphoblastic lymphoma Q4179:What is the term for the appearance of the kidney in malignant hypertension (it has petechiae on its surface)? Flea-bitten kidney (can also be seen in pyelonephritis) Q4180:True or false? Psammoma bodies are seen in medullary carcinoma of the thyroid. False. Elevated calcitonin levels are seen in medullary carcinoma of the thyroid. Psammoma bodies are seen in papillary carcinoma of the thyroid and ovaries; as well as meningiomas. Q4181:What is the lecithin:sphingomyelin ratio in respiratory distress syndrome of newborns? <2 Q4182:What syndrome has loss of deep tendon reflexes; muscle weakness; and ascending paralysis preceded by a viral illness? Guillain-Barré syndrome Q4183:What form of endocarditis do patients with SLE commonly encounter? Libman-Sacks endocarditis Q4184:What is the term for black pigmentation of the colon associated with laxative abuse? Melanosis coli Q4185:Are the following major or minor Jones criteria of rheumatic fever?;? Fever Minor Q4186:Are the following major or minor Jones criteria of rheumatic fever?;? Migratory polyarthritis Major Q4187:Are the following major or minor Jones criteria of rheumatic fever?;? Subcutaneous nodules Major Q4188:Are the following major or minor Jones criteria of rheumatic fever?;? Elevated acute phase reactants (e.g; ESR) Minor Q4189:Are the following major or minor Jones criteria of rheumatic fever?;? Arthralgias Minor Q4190:Are the following major or minor Jones criteria of rheumatic fever?;? Pericarditis Major Q4191:Are the following major or minor Jones criteria of rheumatic fever?;? Erythema marginatum Major Q4192:Are the following major or minor Jones criteria of rheumatic fever?;? Sydenham chorea Major Q4193:What gene inhibits apoptosis by preventing the release of cytochrome c from mitochondria? Bcl-2 Q4194:Which hepatitis strain is a defective virus that can replicate only inside HBV-infected cells? Hepatitis D Q4195:What are the three main components of amyloid? Fibrillary protein; amyloid protein; and glycosaminoglycans (heparin sulfate mainly) Q4196:What leukemia is characterized by Philadelphia chromosomal translocation (9;22); massive splenomegaly; peripheral leukocytosis (commonly > 100; 00); decreased LAP levels; and nonspecific symptoms of fatigue; malaise; weight loss; and anorexia? CML Q4197:What is the difference between a Ghon focus and a Ghon complex? A Ghon focus is a TB tubercle; whereas a Ghon complex is a focus with hilar lymph node involvement. Q4198:In what disease do you see horseshoe kidneys; rockerbottom feet; low-set ears; micrognathia; and mental retardation? Edward syndrome (trisomy 18) Q4199:What parasitic infection is associated with cholangiocarcinoma? Clonorchis sinensis Q4200:What disorder is associated with loss of polarity; anaplasia; pleomorphism; discohesiveness; increase in the nuclear:cytoplasmic ratio; hyperchromasia; and increase in the rate of mitosis? Malignancy Q4201:What is the term for telescoping of the proximal bowel into the distal segment presenting as abdominal pain; currant jelly stools; and intestinal obstruction? Intussusception Q4202:What mushroom poisoning is associated with fulminant hepatitis with extensive liver necrosis? Amanita phalloides Q4203:What type of erythema do you see in;? Ulcerative colitis? Erythema nodosum Q4204:What type of erythema do you see in;? Rheumatic fever? Erythema marginatum Q4205:What type of erythema do you see in;? Stevens- Johnson syndrome? Erythema multiforme Q4206:What benign bone tumor is associated with Gardner syndrome? Osteoma Q4207:What renal calculus is associated with urea-splitting bacteria? Magnesium ammonium phosphate (struvite) Q4208:What lymphoma is associated with bleeding and cryoglobulin precipitation at low temperatures; headache and confusion due to hyperviscosity; IgM M-protein spike on serum electrophoresis; and Russell bodies? Waldenstr?m's macroglobulinemia Q4209:What type of acute metal poisoning involves stomach and colon erosion and acute tubular necrosis? Mercury Q4210:What slow-growing CNS tumor in 30-to 50-year-old patients with a long history of seizures has fried egg cellular appearance in a network of chicken wire? Oligodendroglioma Q4211:Goodpasture Ag is a component of what type of collagen? Type IV collagen Q4212:If a peripheral blood smear shows schistocytes; reticulocytes; and thrombocytopenia; is it more commonly seen in patients with ITP or TTP? TTP; thrombocytopenia with megathrombocytes is more characteristic of ITP. Q4213:After traveling in a plane across the Atlantic Ocean; an obese male goes to the ER with swollen right leg and sudden onset of shortness of breath. What do you immediately diagnose? Pulmonary embolism due to a DVT; this is not absolute but a classic description. Q4214:What cell type involves humoral immunity? B lymphocyte Q4215:What is the first sign of megaloblastic anemia on a peripheral blood smear? Hypersegmented neutrophils Q4216:What is the term for gastric ulcers associated with severely burned or traumatic patients? Curling ulcers (think curling iron = burn) Q4217:What syndrome arises from mutation in the fibrillin gene (FBN1) on chromosome 15q21? Marfan syndrome Q4218:What AD disorder is characterized by degeneration of GABA neurons in the caudate nucleus resulting in atrophy; chorea; dementia; and personality changes? Huntington disease Q4219:What atypical pneumonia can be diagnosed with elevated cold agglutinin titers? Mycoplasma pneumoniae Q4220:What is the triad of Felty syndrome? Neutropenia; splenomegaly; and rheumatoid arthritis Q4221:What is the term for the unidirectional attraction of cells toward a chemical mediator released during inflammation? Chemotaxis Q4222:What is the term for a benign melanocytic tumor associated with sun exposure that presents as tan-to-brown colored and has sharply defined well-circumscribed borders? Benign nevus (mole) Q4223:What small- to medium- sized vasculitis is seen in a 35-year-old man who is a heavy smoker presenting with claudication symptoms in the upper and lower extremities? Buerger disease (thromboangiitis obliterans) Q4224:What is the term for pelvic inflammatory disease of the fallopian tubes? Salpingitis Q4225:What disease with familial mental retardation produces large; everted ears and macro-orchidism? Fragile X syndrome Q4226:What type of skin carcinoma occurring on sun- exposed sites has a low level of metastasis? Squamous cell carcinoma Q4227:What is the tetrad of tetralogy of Fallot? VSD; RVH; overriding aorta; and pulmonary stenosis Q4228:What is the term for chronic necrotizing pulmonary infections resulting in permanent airway dilation and associated with Kartagener syndrome? Bronchiectasis Q4229:What is an elevated; fluid-filled cavity between skin layers up to 0.5 cm? Vesicle (e.g; poison ivy) Q4230:What is the term for panhypopituitarism secondary to ischemic necrosis and hypotension postpartum? Sheehan syndrome Q4231:What disease is diagnosed by findings of ANAs and anti-SCL-70 antibodies? Scleroderma Q4232:What is the name of the ovarian cyst containing ectodermal; endodermal; and mesodermal elements (i.e; skin; hair; teeth and neural tissue)? Teratoma (dermoid cyst) Q4233:What syndrome is seen in iron-deficient middle-aged women with esophageal webs? Plummer-Vinson syndrome Q4234:What are the three causes of transudate? CHF; cirrhosis; and nephrosis Q4235:A chronic alcohol abuser goes to the ER with weakness; a sore; beefy red tongue; loss of vibration and position sense; arm and leg dystaxia; elevated levels of methylmalonic acid in the urine; and anemia with an MCV above 105 fL. What is your diagnosis; and how will you monitor his response to treatment? Subacute combined degeneration of the spinal cord is treated with IM vitamin B12 injections. If treatment is working; you will see an increased reticulocyte count on the peripheral smear in about 5 days. Q4236:What form of GN is characteristically associated with crescent formation? Rapidly progressive glomerulonephritis (RPGN) Q4237:What vitamin deficiency may result in sideroblastic anemia? Vitamin B6 Q4238:What is the term for TB with the cervical lymph node involved? Scrofula Q4239:Influx of what ion is associated with irreversible cell injury? Massive influx of calcium Q4240:What pathology is associated with elevated levels of Ca2+; cardiac arrhythmias; bone resorption; kidney stones; and metastatic calcifications? Primary hyperparathyroidism Q4241:What type of metal poisoning causes mental retardation; somnolence; convulsions; and encephalopathy? Lead Q4242:What syndrome is rheumatoid arthritis with pneumoconiosis? Caplan syndrome Q4243:True or false? All of the following are risk factors for cervical cancer: multiple pregnancies; early age of intercourse; multiple sexual partners; OCP use; smoking; HIV; and STDs. True. Don't forget this list; you will be asked. Q4244:What is the term for precipitation of calcium phosphate in dying or necrotic tissue? Dystrophic calcification Q4245:What congenital small bowel outpouching is a remnant of the vitelline duct? Meckel diverticulum Q4246:What type of crystals are associated with gout? Monosodium urate crystals Q4247:What is the term for transverse bands on the fingernails seen in patients with chronic arsenic poisoning? Mees lines Q4248:What is the tumor at the bifurcation of the right and left hepatic ducts? Klatskin tumor Q4249:IgE-mediated mast cell release; C3a and C5a; and IL-1 all trigger the release of what vasoactive amine? Histamine Q4250:What disease is seen in the 20-to 40-year-old age group; is more prevalent in women than men; involves diarrhea with or without bloody stools; starts in the rectum and ascends without skipping areas; includes pseudopolyps; and has a thickness of the bowel that does not change? Ulcerative colitis Q4251:What disorder causes joint stiffness that worsens with repetitive motion; crepitus; effusions; and swelling and commonly affects the knees; hips; and spine? Osteoarthritis Q4252:What condition is characterized by a 46XY karyotype; testes present; and ambiguous or female external genitalia? Male pseudohermaphrodite (dude looks like a lady!) Q4253:What is the term for RBC remnants of nuclear chromatin in asplenic patients? Howell-Jolly bodies Q4254:What is the term for gastric ulcers associated with increased intracranial pressure? Cushing's ulcers Q4255:What platelet disorder is characteristically seen in children following a bout of gastroenteritis with bloody diarrhea? Hemolytic uremic syndrome Q4256:Are elevated alkaline phosphatase and decreased phosphorus and calcium levels more consistent with osteoporosis or osteomalacia? Osteomalacia. Osteoporosis has normal levels of calcium; phosphorus; and alkaline phosphatase. Q4257:What vascular tumor associated with von Hippel- Lindau syndrome involves the cerebellum; brainstem; spinal cord; and retina? Hemangioblastoma Q4258:How many segments in a neutrophilic nucleus are necessary for it to be called hypersegmented? At least 5 lobes Q4259:Name the type of regeneration (i.e; labile; stable; or permanent) based on the following examples;? Epidermis Labile Q4260:Name the type of regeneration (i.e; labile; stable; or permanent) based on the following examples;? Skeletal muscle Permanent Q4261:Name the type of regeneration (i.e; labile; stable; or permanent) based on the following examples;? Pancreas Stable Q4262:Name the type of regeneration (i.e; labile; stable; or permanent) based on the following examples;? CNS neurons Permanent Q4263:Name the type of regeneration (i.e; labile; stable; or permanent) based on the following examples;? Fibroblasts Stable Q4264:Name the type of regeneration (i.e; labile; stable; or permanent) based on the following examples;? Hematopoietic cells Labile Q4265:Name the type of regeneration (i.e; labile; stable; or permanent) based on the following examples;? Liver Stable Q4266:Name the type of regeneration (i.e; labile; stable; or permanent) based on the following examples;? Smooth muscle Stable Q4267:Name the type of regeneration (i.e; labile; stable; or permanent) based on the following examples;? Cardiac muscle Permanent Q4268:Name the type of regeneration (i.e; labile; stable; or permanent) based on the following examples;? Mucosal epithelium Labile Q4269:Name the type of regeneration (i.e; labile; stable; or permanent) based on the following examples;? Kidney Stable Q4270:Name the type of regeneration (i.e; labile; stable; or permanent) based on the following examples;? Osteoblasts Stable;(Labile cells proliferate throughout life; stable cells have a low level of proliferation; and permanent cells as the name states; do not proliferate.) Q4271:What CNS tumor cells stain positive for glial fibrillary acidic protein (GFAP)? Astrocytoma Q4272:True or false? Elevated ASO titers and serum complement levels are associated with poststreptococcal GN. False. ASO titers are elevated; but serum complement levels are decreased. Q4273:What glycoprotein allows platelets to adhere to von Willebrand factor? GP Ib Q4274:What encephalitis is associated with the JC virus? Progressive multifocal leukoencephalopathy Q4275:Hereditary angioneurotic edema (AD) produces local edema in organs (e.g; GI; skin; respiratory tract). What enzyme deficiency causes increased capillary permeability due to a release of vasoactive peptides? C1 esterase inhibitor (C1INH) Q4276:Is an anti-HAV IgG Ab associated with immunization or recent infection? Anti-HAV IgG Abs are associated with immunization or a prior infection. Anti-HAV IgM is associated with acute or recent infection. Q4277:Which integrin mediates adhesion by binding to lymphocyte function– associated Ag 1 (LFA-1) and MAC-1 leukocyte receptors? Intercellular adhesion molecule (ICAM) 1 Q4278:Name the cerebral vessel associated with the following vascular pathologies;? Subarachnoid hemorrhage Berry aneurysm in the circle of Willis Q4279:Name the cerebral vessel associated with the following vascular pathologies;? Subdural hemorrhage Bridging veins draining into the sagittal sinus Q4280:Name the cerebral vessel associated with the following vascular pathologies;? Epidural hemorrhage Middle meningeal artery Q4281:True or false? Live vaccines are contraindicated in patients with SCID. True Q4282:What is the term for the round intracytoplasmic eosinophilic inclusions containingɑ-synuclein found in the dopaminergic neurons of the substantia nigra? Lewy bodies Q4283:In which form of emphysema; panacinar or centriacinar; is the effect worse in the apical segments of the upper lobes? Centriacinar worse in upper lobes; panacinar worse in base of lower lobes Q4284:What syndrome results if the enzyme ɑ-1-iduronidase is deficient? L-iduronate sulfatase deficiency? Hurler syndrome and Hunter syndrome; respectively Q4285:What percentage of the bone marrow must be composed of blast for leukemia to be considered? At least 30% blast in the bone marrow Q4286:What is the term for the heart's inability to maintain perfusion and meet the metabolic demands of tissues and organs? CHF Q4287:What syndrome occurs when pelvic inflammatory disease ascends to surround the liver capsule in violin string adhesions? Fitz-Hugh-Curtis syndrome Q4288:True or false? Patients with Turner syndrome have no Barr bodies. True. Remember; the second X chromosome is inactivated; and so is the Barr body. Turner syndrome has only one X chromosome. Q4289:What is the term for the sign revealed when a psoriatic scale is removed and pinpoint bleeding occurs? Auspitz sign Q4290:What type of Hgb is increased in patients with sickle cell anemia who take hydroxyurea? Hgb F Q4291:What vasculitis affects a 30-year-old Asian female having visual field deficits; dizziness; decreased blood pressure; and weakened pulses in the upper extremities? Takayasu arteritis (medium-size to large vessels) Q4292:A 20-year-old college student has fever; grey-white membranes over the tonsils; posterior auricular lymphadenitis; and hepatosplenomegaly. What is your diagnosis? What test do you order to confirm your diagnosis? EBV infections resulting in infectious mononucleosis can be diagnosed by the Monospot test. (Remember; it may be negative in the first week of the illness; so retest if you have a high index of suspicion.) Q4293:What cell type is commonly elevated in asthma? Eosinophil Q4294:What pathology is associated with deposition of calcium pyrophosphate in patients older than 50 years? Pseudogout Q4295:What thyroid carcinoma secretes calcitonin and arises from the parafollicular C cells? Medullary carcinoma of the thyroid Q4296:What illegal drug can cause rhabdomyolysis; MI; cerebral infarct; and lethal cardiac arrhythmias? Cocaine Q4297:What AA is substituted for glutamic acid at position 6 on the Beta-chain in patients with sickle cell anemia? Valine Q4298:What endogenous pigment found in the substantia nigra and melanocytes is formed by the oxidation of tyrosine to dihydroxyphenylalanine? Melanin Q4299:What tumor marker is associated with seminomas? Placental alkaline phosphatase Q4300:What type of GN; associated with celiac disease and dermatitis herpetiformis; has mesangial deposits of IgA; C3; properdin; IgG; and IgM? Berger disease (IgA nephropathy) Q4301:What AD disease is associated with chromosome 4p; does not present until the person is in his or her 30s; and involves atrophy of the caudate nucleus; dilatation of the lateral and third ventricles; and signs of extrapyramidal lesions? Huntington disease Q4302:What pattern of inheritance is G-6-PD deficiency? X-linked recessive Q4303:What adenocarcinoma presents with elevated levels of acid phosphatase; dihydrotestosterone; PSA; and bone pain? Prostatic carcinoma Q4304:Is Dubin-Johnson or Rotor syndrome associated with black pigmentation of the liver? Both are AR with conjugated hyperbilirubinemia; but Dubin- Johnson syndrome is differentiated from Rotor by the black pigmentation of the liver. Q4305:What oxygen-dependent killing enzyme requires hydrogen peroxide and halide (Cl-) to produce hypochlorous acid? Myeloperoxidase Q4306:What condition results in a strawberry gallbladder? Cholesterolosis Q4307:What three chemical agents are associated with angiosarcomas of the liver? Arsenic; thorotrast; and vinyl chloride Q4308:What is the term for programmed cell death? Apoptosis (Remember; there is a lack of inflammatory response.) Q4309:What potentially fatal disease occurs in children who are given aspirin during a viral illness? Reye syndrome Q4310:What metal poisoning produces microcytic anemia with basophilic stippling? Lead poisoning Q4311:What inflammatory bowel disorder is continuous; with extensive ulcerations and pseudopolyps; and is associated with HLA-B27? Ulcerative colitis Q4312:What is the pentad of TTP? Neurologic symptoms;Renal failure;Thrombocytopenia;Fever;Microangiopathic hemolytic anemia;(Don't forget it. When I was an intern; my senior resident asked me this question more times than I would like to remember.) Q4313:Which hepatitis B Ag correlates with infectivity and viral proliferation? HBeAg Q4314:What disease involves cold skin abscesses due to a defect in neutrophil chemotaxis and a serum IgE level higher than 2000? Job syndrome Q4315:What female pathology is associated with endometrial glands and stroma outside the uterus commonly affecting the ovaries as chocolate cysts? Endometriosis Q4316:What is the karyotype in Turner syndrome? 45XO Q4317:What is the term for a congenital absence of the ganglionic cells of the Auerbach and Meissner plexus in the rectum and sigmoid colon? Hirschsprung disease (aganglionic megacolon) Q4318:What syndrome is associated with gastrin-producing islet cell tumor resulting in multiple intractable peptic ulcers? Zollinger-Ellison syndrome Q4319:What type of collagen is associated with keloid formation? Type III Q4320:The "tea-and-toast" diet is classically associated with what cause of megaloblastic anemia? Folate deficiency (very common in the elderly) Q4321:What is the term for ascending bacterial infection of the renal pelvis; tubules; and interstitium causing costovertebral angle tenderness; fever; chills; dysuria; frequency; and urgency? Pyelonephritis Q4322:How can a deficiency in adenosine deaminase be a bone marrow suppressor? It causes a buildup of dATP; which inhibits ribonucleotide reductase and leads to a decrease in deoxynucleoside triphosphate; a precursor of DNA; resulting in overall bone marrow suppression. Q4323:Which phenotype of osteogenesis imperfecta is incompatible with life? Type II Q4324:With what is cherry red intoxication associated? Acute CO poisoning Q4325:What are the four most common causes of femoral head necrosis? 1. Steroids ;2. Alcohol ;3. Scuba diving ;4. Sickle cell anemia Q4326:What are the four signs of acute inflammation? Rubor (red); dolor (pain); calor (heat); tumor (swelling); also sometimes there is loss of function Q4327:Name the hypochromic microcytic anemia based on the following laboratory values;? Increased iron; decreased TIBC; increased percent saturation; increased ferritin Sideroblastic anemia Q4328:Name the hypochromic microcytic anemia based on the following laboratory values;? Decreased iron; TIBC; and percent saturation; increased ferritin Anemia of chronic disease Q4329:Name the hypochromic microcytic anemia based on the following laboratory values;? Decreased iron; percent saturation; and ferritin; increased TIBC Iron deficiency anemia Q4330:Name the hypochromic microcytic anemia based on the following laboratory values;? Normal iron; TIBC; percent saturation; and ferritin Thalassemia minor Q4331:Which form of emphysema is associated with an alpha1-antitrypsin deficiency? Panacinar Q4332:An 80-year-old woman presents to you with right- sided temporal headache; facial pain and blurred vision on the affected side; and an elevated ESR. Your diagnosis? Temporal arteritis (giant cell arteritis) Q4333:What type of neurofibromatosis is associated with bilateral acoustic schwannomas? Type 2 Q4334:What disorder is due to a deficiency in the enzyme glucocerebrosidase? Gaucher disease Q4335:What is the term for flexion of the PIP and extension of the DIP joints seen in rheumatoid arthritis? Boutonnière deformities Q4336:True or false? Atelectasis is an irreversible collapse of a lung. False. Atelectasis is a reversible collapse of a lung. Q4337:What viral infection in patients with sickle cell anemia results in aplastic crisis? Parvovirus B 19 Q4338:What syndrome has elevated FSH and LH levels with decreased testosterone levels and 47XXY karyotype? Klinefelter syndrome Q4339:What CNS developmental abnormality is associated with 90% of syringomyelia? Arnold-Chiari malformation type 2 Q4340:What is the term for fibrinoid necrosis of the arterioles in the kidney secondary to malignant hypertension? Onion skinning Q4341:A 30-year-old woman goes to your office with bilateral multiple breast nodules that vary with menstruation and have cyclical pain and engorgement. What is your diagnosis? Fibrocystic change of the breast. This highlights the distinguishing features from breast cancer; which is commonly unilateral; single nodule; no variation with pregnancy. Q4342:What disease is X-linked recessive; is associated with eczema thrombocytopenia and an increased chance of developing recurrent infections; involves a decrease in serum IgM and in the T cell-dependent paracortical areas of the lymph nodes; and means that the patient is likely to develop malignant lymphoma? Wiskott-Aldrich syndrome Q4343:Which form of melanoma carries the worst prognosis? Nodular melanoma Q4344:Patients with sickle cell anemia are at increased risk for infection from what type of organisms? Encapsulated bacteria Q4345:How many major and/or minor Jones criteria are required for the diagnosis of rheumatic fever? Two major or one major and two minor Q4346:What skin carcinoma is a superficial dermal infiltrate of T lymphocytes seen in males more than 40 years old and presents as scaly red patches or plaques? Mycosis fungoides (cutaneous T-cell lymphoma) Q4347:What Hgb-derived endogenous pigment is found in areas of hemorrhage or bruises? Hemosiderin Q4348:What is a palpable; elevated solid mass up to 0.5 cm? Papule Q4349:True or false? Monocytosis is seen in TB. True Q4350:What pathology is associated with podagra; tophi in the ear; and PMNs with monosodium urate crystals? Gout Q4351:What GI pathology can be caused by a patient taking clindamycin or lincomycin or by Clostridium difficile; ischemia; Staphylococcus; Shigella; or Candida infection? Pseudomembranous colitis Q4352:What do the risk factors late menopause; early menarche; obesity; nulliparity; excessive estrogen; genetic factor p53; and brc-abl characterize? Breast cancer Q4353:What thyroid carcinoma is associated with radiation exposure; psammoma bodies; and Orphan Annie eye nuclei? Papillary carcinoma of the thyroid Q4354:Name three opsonins. Fc portion of IgG; C3b; and mannose-binding proteins Q4355:What chemical can be dangerous if you work in the aerospace industry or in nuclear plants? Beryllium Q4356:Which hepatitis B serology markers—HBcAb IgG; HBcAb IgM; HBeAg; HBsAb IgG; HBsAg; HBV-DNA—are associated with the following periods?;? Window period HBcAb IgM Q4357:Which hepatitis B serology markers—HBcAb IgG; HBcAb IgM; HBeAg; HBsAb IgG; HBsAg; HBV-DNA—are associated with the following periods?;? Immunization HBsAb IgG Q4358:Which hepatitis B serology markers—HBcAb IgG; HBcAb IgM; HBeAg; HBsAb IgG; HBsAg; HBV-DNA—are associated with the following periods?;? Prior infection HBcAb IgG and HBsAb IgG Q4359:Which hepatitis B serology markers—HBcAb IgG; HBcAb IgM; HBeAg; HBsAb IgG; HBsAg; HBV-DNA—are associated with the following periods?;? Acute infection HBcAb IgM; HBV-DNA; HBeAg; HBsAg Q4360:Which hepatitis B serology markers—HBcAb IgG; HBcAb IgM; HBeAg; HBsAb IgG; HBsAg; HBV-DNA—are associated with the following periods?;? Chronic infection HBcAb IgG; HBV-DNA; HBeAg; HBsAg Q4361:Name the type of necrosis;? The most common form of necrosis; denatured and coagulated proteins in the cytoplasm Coagulative necrosis Q4362:Name the type of necrosis;? Seen as dead tissue with coagulative necrosis Gangrenous necrosis Q4363:Name the type of necrosis;? Seen as dead tissue with liquefactive necrosis? Liquefaction necrosis Q4364:Name the type of necrosis;? Due to lipase activity and has a chalky white appearance Fat necrosis Q4365:Name the type of necrosis;? Soft; friable; cottage- cheese appearing; characteristically seen in TB Caseous necrosis Q4366:Name the type of necrosis;? Histologically resembles fibrin Fibrinoid necrosis Q4367:Name the three enzymes that protect the cell from oxygen-derived free radicals. Superoxide dismutase; glutathione peroxidase; catalase Q4368:What are the rules of 2 for Meckel diverticulum? 2% of population; 2 cm long; 2 feet from ileocecal valve; 2 years old; and 2% of carcinoid tumors Q4369:What aneurysm of the circle of Willis is associated with polycystic kidney disease? Berry aneurysm Q4370:A 20-year-old black woman goes to you with nonspecific joint pain; fever; and a malar rash over the bridge of her nose and on her cheeks. This is a classic example of what autoimmune disease? What are three autoantibody tests you could order to make the diagnosis? SLE; ANA; anti-dsDNA and anti-Sm (anti-Smith) Q4371:True or false? Excess lead deposits in the oral cavity. True. It deposits at the gingivodental line; known as the lead line. Q4372:What is the term for increased iron deposition resulting in micronodular cirrhosis; CHF; diabetes; and bronzing of the skin? Hemochromatosis Q4373:What AR disorder is due to a deficiency in glycoprotein IIb-IIIa; resulting in a defect in platelet aggregation? Glanzmann syndrome Q4374:What protein-losing enteropathy has grossly enlarged rugal fold in the body and fundus of the stomach in middle- aged males; resulting in decreased acid production and an increased risk of gastric cancer? Ménétrier's disease Q4375:What myopathy due to autoantibodies to ACh receptors can present with thymic abnormalities; red cell aplasia; and muscle weakness? Myasthenia gravis Q4376:Which subtype of AML is most commonly associated with Auer rods? M3 (promyelocytic leukemia) Q4377:What condition results from a 46XX karyotype and female internal organs with virilized external genitalia? Female pseudohermaphrodite Q4378:Two weeks after her son has a throat infection; a mother takes the boy to the ER because he has fever; malaise; HTN; dark urine; and periorbital edema. What is your diagnosis? Poststreptococcal GN Q4379:What X-linked recessive immune disorder is characterized by recurrent infections; severe thrombocytopenia; and eczema? Wiskott-Aldrich syndrome Q4380:What form of arthritis is associated with calcium pyrophosphate crystals? Pseudogout Q4381:What is the term for excessive production of collagen that flattens out and does not extend beyond the site of the injury? Hypertrophic scar Q4382:What is the term for inflamed; thickened skin on the breast with dimpling associated with cancer? Peau d'orange Q4383:What rare vasculitis has the following characteristics: males aged 40 to 60; affecting small arteries and veins; involving nose; sinuses; lungs; and kidneys; C-ANCA and autoantibodies against proteinase 3? Wegener granulomatosis Q4384:What retrovirus is associated with adult T-cell leukemia? HTLV-1 Q4385:What disease is seen in children younger than 5 years of age and is characterized by X-linked recessive cardiac myopathies; calf pseudohypertrophy; lordosis; protuberant belly; an increase then a decrease in CPK; and death commonly in the second decade of life? Duchenne muscular dystrophy Q4386:What malignant neoplasm of the bone has a soap bubble appearance on radiograph? Giant cell bone tumor (osteoclastoma) Q4387:What nephrotic syndrome has effacement of the epithelial foot processes without immune complex deposition? Minimal change disease Q4388:What is the term for tissue-based basophils? Mast cells Q4389:What malignant bone tumor is characterized by Codman triangle (periosteal elevation) on radiograph? Osteosarcoma Q4390:Is splenomegaly more commonly associated with intravascular or extravascular hemolysis? Extravascular hemolysis if it occurs in the spleen; if in the liver; it results in hepatomegaly. Q4391:What cancer of the male genitourinary system is associated with osteoblastic bony metastasis? Prostatic carcinoma Q4392:What stromal tumor in males is characterized histologically with crystalloids of Reinke? Leydig cell tumor Q4393:What pulmonary disease; most commonly associated with smoking; results in enlarged; overinflated lungs owing to the destruction of the alveolar walls with diminished elastic recoil? Emphysema Q4394:What factor gets activated in the intrinsic pathway of the coagulation cascade? Extrinsic pathway? Factor XII for the intrinsic; factor VII for the extrinsic pathway Q4395:What chronic systemic inflammatory disease commonly seen in women aged 20 to 50 is a progressive; symmetric arthritis affecting the hands; wrists; knees; and ankles that improves with increased activity? Rheumatoid arthritis Q4396:Name the following descriptions associated with bacterial endocarditis;? Retinal emboli Roth spots Q4397:Name the following descriptions associated with bacterial endocarditis;? Painful subcutaneous nodules on fingers and toes Osler nodes Q4398:Name the following descriptions associated with bacterial endocarditis;? Painless hyperemic lesions on the palms and soles Janeway lesions Q4399:What two CD cell surface markers do Reed- Sternberg cells stain positive for? CD15 and CD30 Q4400:What two lysosomal storage diseases have cherry-red spots on the retina? Niemann-Pick and Tay-Sachs diseases Q4401:True or false? Increased leukocyte alkaline phosphatase (LAP) is associated with CML. False. Increased LAP is seen in stress reactions and helps differentiate benign conditions from CML; which has low LAP levels. Q4402:What syndrome has multiple adenomatous colonic polyps and CNS gliomas? Turcot syndrome Q4403:What is the term for a venous embolus in the arterial system? Paradoxic emboli most commonly enter the arteries through a patent septal defect in the heart. Q4404:Are hemorrhagic cerebral infarcts more commonly associated with embolic or thrombotic occlusions? Embolic Q4405:What is the name for the following RBC indices?;? The coefficient of variation of the RBC volume Red blood cell distribution width index (RDW) Q4406:What is the name for the following RBC indices?;? Average mass of the Hgb molecule/RBC Mean corpuscular hemoglobin (MCH) Q4407:What is the name for the following RBC indices?;? Average volume of a RBC Mean corpuscular volume (MCV) Q4408:What is the name for the following RBC indices?;? Average Hgb concentration/given volume of packed RBCs Mean corpuscular hemoglobin concentration (MCHC) Q4409:What cardiomyopathy is due to a ventricular outflow obstruction as a result of septal hypertrophy and leads to sudden cardiac death in young athletes? Hypertrophic cardiomyopathy (hypertrophic subaortic stenosis; or IHSS) Q4410:Which HPV serotypes are associated with condyloma acuminatum? HPV serotypes 6 and 11 Q4411:Which form of melanoma carries the best prognosis? Lentigo maligna melanoma Q4412:What is the term for an increase in the number of cells in a tissue? Hyperplasia Q4413:A 60-year-old man has back pain (compression spinal fracture); hypercalcemia; increased serum protein; Bence- Jones proteinuria; and monoclonal M-spike on serum electrophoresis. What is your diagnosis? Multiple myeloma Q4414:What is the term for pus in the pleural space? Empyema Q4415:What is flat; circumscribed nonpalpable pigmented change up to 1 cm? Macule (e.g; a freckle) Q4416:Name the macrophage based on its location;? Liver macrophages Kupffer cells Q4417:Name the macrophage based on its location;? Bone macrophages Osteoclasts Q4418:Name the macrophage based on its location;? Brain macrophages Microglia Q4419:Name the macrophage based on its location;? Lung macrophages Pulmonary alveolar macrophages Q4420:Name the macrophage based on its location;? Connective tissue macrophages Histiocytes Q4421:Name the macrophage based on its location;? Epidermal macrophages Langerhans cells Q4422:What is the term for a large; immature RBC that is spherical; blue; and without a nucleus? Reticulocyte Q4423:What testicular tumor of infancy is characterized by elevated-fetoprotein levels and Schiller-Duval bodies? Yolk sac tumor Q4424:Starry sky appearance of macrophages is pathognomonic of what lymphoma? Burkitt lymphoma Q4425:In which region of the lung are 75% of the pulmonary infarcts seen? Lower lobe Q4426:For what disease are SS-A(Ro); SS-B(La); and R-ANA diagnostic markers? Sj?gren disease Q4427:What HPV serotypes are associated with increased risk of cervical cancer? HPV serotypes 16; 18; 31; and 33 Q4428:Do the following structures pick up stain from hematoxylin or eosin?;? Nuclei Hematoxylin Q4429:Do the following structures pick up stain from hematoxylin or eosin?;? Nucleoli Hematoxylin Q4430:Do the following structures pick up stain from hematoxylin or eosin?;? Cytoplasm Eosin Q4431:Do the following structures pick up stain from hematoxylin or eosin?;? Collagen Eosin Q4432:Do the following structures pick up stain from hematoxylin or eosin?;? RBCs Eosin Q4433:Do the following structures pick up stain from hematoxylin or eosin?;? Calcium Hematoxylin Q4434:Do the following structures pick up stain from hematoxylin or eosin?;? Bacteria Hematoxylin Q4435:Do the following structures pick up stain from hematoxylin or eosin?;? Fibrin Eosin Q4436:Do the following structures pick up stain from hematoxylin or eosin?;? Thyroid colloid Eosin Q4437:What commonly encountered overdose produces headache; tinnitus; respiratory alkalosis; metabolic acidosis; confusion; vomiting; and tachypnea? ASA (salicylate) Q4438:What AR syndrome is due to a deficiency of glycoprotein Ib; resulting in a defect in platelet adhesion? Bernard-Soulier syndrome Q4439:What AD renal disorder is associated with mutations of the PKD 1 gene on chromosome 16 and berry aneurysms in the circle of Willis and presents in the fifth decade with abdominal masses; flank pain; hematuria; HTN; and renal insufficiency? Adult polycystic kidney disease Q4440:Based on the following information; is the renal transplantation rejection acute; chronic; or hyperacute?;? Months to years after transplantation; gradual onset of HTN; oliguria; and azotemia; seen as intimal fibrosis of the blood vessels and interstitial lymphocytes Chronic rejection Q4441:Based on the following information; is the renal transplantation rejection acute; chronic; or hyperacute?;? Immediately after transplantation; seen as a neutrophilic vasculitis with thrombosis Hyperacute rejection Q4442:Based on the following information; is the renal transplantation rejection acute; chronic; or hyperacute?;? Weeks to months after transplantation; abrupt onset of oliguria and azotemia; seen as neutrophilic vasculitis and interstitial lymphocytes Acute rejection Q4443:What type of collagen is abnormal in patients with osteogenesis imperfecta? Type I (makes sense; since they have a predisposition for fractures and type I collagen is associated with bones and tendons) Q4444:What coronary artery vasculitis is seen in Japanese infants and children less than 4 years old with acute febrile illness; conjunctivitis; maculopapular rash; and lymphadenopathy? Kawasaki disease Q4445:What disease has IgG autoantibodies; occurs in women more than men; and includes exophthalmos; pretibial myxedema; nervousness; heart palpitations; and fatigue? Graves disease Q4446:What condition is defined by both testicular and ovarian tissues in one individual? True hermaphrodism Q4447:A mother takes her 2-week-old infant to the ER because the baby regurgitates and vomits after eating and has peristaltic waves visible on the abdomen and a palpable mass in the right upper quadrant. What is your diagnosis? Pyloric stenosis Q4448:What variant of polyarteritis nodosa is associated with bronchial asthma; granulomas; and eosinophilia? Churg-Strauss syndrome Q4449:What component of the basement membrane binds to collagen type IV and heparin sulfate and is a cell surface receptor? Laminin Q4450:What B-cell neoplasm is seen in males with massive splenomegaly; produces dry tap on bone marrow aspirations; and stains positive for tartrate-resistant acid phosphatase (TRAP)? Hairy cell leukemia Q4451:What form of nephritic syndrome is associated with celiac sprue and Henoch- Sch?nlein purpura? IgA nephropathy Q4452:What syndrome is characterized by embryologic failure of the third and fourth pharyngeal pouches resulting in hypocalcemia; tetany; and T-cell deficiency? DiGeorge syndrome Q4453:What is the treatment for physiologic jaundice of newborns? Phototherapy Q4454:How many months in how many years must a person cough with copious sputum production for the diagnosis of chronic bronchitis to be made? 3 months of symptoms in 2 consecutive years Q4455:What chronic inflammatory WBC is associated with IgE-mediated allergic reactions and parasitic infections? Eosinophils Q4456:What AD syndrome produces hamartomatous polyps in the small intestine and pigmentation of the lips and oral mucosa? Peutz-Jeghers syndrome Q4457:What is the term for formation of a stable fibrin– platelet plug to stop bleeding? Hemostasis Q4458:True or false? All of the following are risk factors for breast cancer: early menses; late menopause; history of breast cancer; obesity; and multiparity. False. All except multiparity are risk factors for breast cancer. Nulliparity; increasing age; and family history in first-degree relative are also risk factors. Memorize this list! Q4459:Is jugular venous distention a presentation of isolated left or right heart failure? Right-sided Q4460:Name the hepatitis virus based on the following information;? Small circular RNA virus with defective envelope Hepatitis D Q4461:Name the hepatitis virus based on the following information;? Enveloped RNA flavivirus Hepatitis C Q4462:Name the hepatitis virus based on the following information;? Naked capsid RNA calicivirus Hepatitis E Q4463:Name the hepatitis virus based on the following information;? Enveloped DNA hepadnavirus Hepatitis B Q4464:Name the hepatitis virus based on the following information;? Naked capsid RNA picornavirus Hepatitis A Q4465:What AR disease involves a decreased amount of sphingomyelinase; massive organomegaly; zebra bodies; and foamy histiocytes and is associated with chromosome 11p? Niemann-Pick disease Q4466:What is the term for hypoperfusion of an area involving only the inner layers? Mural infarct Q4467:What are the three causes of normochromic normocytic anemia with a normal MCV and a low reticulocyte count? Marrow failure; cancer; and leukemia Q4468:Notching of the ribs; seen on chest radiograph in patients with postductal coarctation of the aorta; is due to collateralization of what arteries? Dilation of the internal mammary arteries results in erosions on the inner surface of the ribs and is seen as notching. Q4469:What is the physiologic storage form of iron? Ferritin Q4470:What is the term for occlusion of a blood vessel due to an intravascular mass that has been carried downstream? Embolism Q4471:What AR disorder of copper metabolism can be characterized by Kayser- Fleischer rings; decreased ceruloplasmin levels; and increased urinary copper excretion and tissue copper levels? Wilson disease. (Remember; patients commonly present with psychiatric manifestations and movement disorders but may be asymptomatic.) Q4472:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? c-myc Burkitt lymphoma Q4473:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? L-myc Small cell cancer of the lung Q4474:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? hst-1 and int-2 Melanoma; cancer of the stomach and bladder Q4475:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? et MEN II and III syndromes Q4476:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? Ki-ras Pancreas and colon Q4477:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? cyclin D Mantle cell lymphoma Q4478:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? N-myc Neuroblastoma Q4479:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? CDK4 Melanoma Q4480:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? abl CML and ALL Q4481:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? hst-1; int-2; erb-2; and erb-3 Breast cancer Q4482:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? sis Astrocytoma Q4483:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? Ki-ras and erb-2 Lung cancer Q4484:Name the cancer associated with the following oncogenes. (Some may have more than one answer);? erb-1 Squamous cell carcinoma of the lung Q4485:What AD GI neoplasia produces multiple adenomatous polyps; osteomas; fibromas; and epidural inclusion cysts? Gardner syndrome Q4486:What disease involves microcephaly; mental retardation; cleft lip or palate; and dextrocardia? Patau syndrome (trisomy 13) Q4487:What type of GN occurs most commonly in children after a pharyngeal or skin infection; is immune complex– mediated; and is seen as lumpy-bumpy subepithelial deposits? Postinfectious GN Q4488:What are the three most common sites for left-sided heart embolisms to metastasize? Brain; spleen; and kidney Q4489:With what two pathologies is a honeycomb lung associated? Asbestosis and silicosis Q4490:What AD disorder due to a mutation in fibroblast growth factor receptor 3 results in normal-size vertebral column and skull and short; thick extremities? Achondroplasia
Fast Facts: Deficit di piruvato chinasi per pazienti e sostenitori: Una rara malattia genetica che colpisce I globuli rossi Informazioni + assunzione del controllo = migliore risultato
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