Pathomorph Lab 30- Diseases of infancy and childhood

1. Erythroblastosis fetalis (651).

a. Is hemolytic disease of the fetus or newborn
b. Caused by transplacental passage of maternal antibodies against feta
erythrocyte antigens
c. Fetus enherits red cell antigens from the father
d. Most cases involve Rh-D antigen
i. Mother Rh neg and newborn Rh positive
e. Fetab RBCs gain access to maternal circulation
f. Since IgM antibodies are involved in primary response
g. Mother forms IgG antibodies which cross the planeta and destroy fetal
RBCs
h. Clinical:
i. Fetal develops sever anemia with congestive hear failure and
compensoatory increased hematopoiesis
ii. Hydrops fetalis edema secondary to CHF
iii. Blulirubin encephalopathy (kernicterus)
i. Micro
i. Nests of myeloid precursors
ii. Cells with small dark nuclei are erythroblasts, NOT
LYMPHOCTES!
2. Cystic fibrosis (mucoviscidosis) (the pancreas) (659).
a. Autosomal recessive disease in whites
b. Widespread disortder in epithelial chloride transport affecting fluid
retention in:
i. Exocrine glands
ii. Epithelial lining of the repiratory, GI and reproductive tracts
c. Abnormally viscid mucus secretions
d. Most patients on live up to slightly more than 30
e. Pancreas
i. Atrophy of exocrine pancreas with progressive fibrosis loss of
pancreatic exocrine secretion trouble digesting food, chronic
pancreatitis, avitaminosis A
f. Liver
i. Plugging of bile canaliculi with portal inlfam
g. Repro
h. Symptoms
i. Coughing or wheezing
ii. Weigh loss
iii. Salty tasting skin
iv. Recurrent pulmonary infections, lung abcess, bronchiectasis
v. Greast stool (steatorrhea); distended small instestine
i. Chloride channel defect and effects
i. CC defect in sweat glands
ii. In the airway, CF patients have decreasded chloride secretion and
increased sodium and waater reabsorption leading to dehydration

Pathomorph Lab 30- Diseases of infancy and childhood

of the mucuus layer coating epithelial cells, defective mucociliary
action, and mucus plugging of airways trouble breathing,
frequent lung infections
j.
3. Neonatal respiratory distress syndrome (RDS) (653).
a. Life threatening disorder of the lungs caused by a deficiency of surfactant
b. A failure of type II pneumocytes maturation
c. Infants develop hypoxemia and resp acidosis- baby becomes cyanotic
d. Respiratory difficulty (tachypnea) begins within a few hours of birth
e. Risk factors
i. Prematurity (greatest risk factor)
ii. Maternal diabetes mellitus
iii. Cesarean delivery
f. Micro
i. Thick alveoli membranes
ii. Hyaline membrane lines the alveoli ducts
iii. Eosinophilic rich
4. Neonatal hepatitis (657).
a. Inflammation of the liver that occurs only in early infancry. 1-2 months
after birth
b. Usualy due to prolonged conjugated hyperbilirubinemia; jaundice
c. May be idiopathic
d. Associated with congenital infections- CMV, rubella, hepatitis (A, B, or C)
e. Many neonates recover in a couple of months
5. Osteochondroma (exostosis) (804).
a. Developmental defect of the growth plate
b. Occurs <30 years of age
c. Cartilage grows laterally toward the metaphysis into the soft tissue,
vessels originating from bone marrow cavity extend
d. Benign tumor near the end of a long bone (knee and humerous)
e. Micro
i. Cartilage
ii. Outer layer composed of benign hyaline cartilage, varying in
thickness
iii. The stalk is formed of bone and the medullary cavity of the
osteochondroma is in continuity with the parent bone
iv.
6. Retinoblastoma (the eyeball) (403).
a. Most common primary intraocular malignant neoplasm of childhood
b. Is a rapidly growing cancer that develops in the cells of retina
c. Arises from immature retinal neurons
d. Mostly only one eye is affected (unilateral)

Pathomorph Lab 30- Diseases of infancy and childhood

e. Fatal if left untreated
f. Clinical signs
i. White pupil
ii. Sqint
iii. Poor vision
iv. Light entering the eye commonly reflects a yellowish color similar
to that from the tapetum of a cat (Cats eye reflex)
g. Micro
i. Neoplastic cells- invade blood vessels
ii. Flexner-Wintersteiner rosettes
7. Wilms tumor (the kidney) (368).
a. Is a malignant neoplasm of embryonal nephrogenic elemts
b. The tumor may arise as a result of a failure of blastemal tissue
c. An abdominal solid tumor in children under the age of 5. Diagnosed
between 2-5.
d. Clinical features
i. Abnormally large abdomen
ii. Abdominal pain
iii. Nausea and vomiting
iv. Hematuria
v. High BP
vi. Fever caused by tumor necrosis
e. Micro
i. Blastemic component: sheets of small undifferentiated round blue
cells with scanty cytoplasm
ii. Epithelial elements: abortivetubules and glomeruli
iii. Stromal elemnts: spindle shaped cells with skeletal muscle, display
smooth muscle or fibroblast differentiation
iv. All three parts are made of malignant tumor cells