PA-LEARNINGS SA ADCON

By: Glaiza Mae 

TUBERCULOSIS
 TB
TB EXPOSURE TB INFECTION TB DISEASE
Exposure Positive Positive Positive

Signs and Negative Negative Positive

Symptoms

Tuberculin skin test Negative Positive Positive

Chest X-ray Negative Negative (may be positive)

Direct sputum Negative Negative (may be positive)

smear microscopy
(DSSM)
Other diagnosis Negative (may be positive) (may be positive)
Algorithm
Treatment
Treatment
IMMUNIZATION
ACUTE GASTROENTERITIS
 Acute Gastroenteritis
• Gastroenteritis, denotes infection of the GI
tract which may be caused by bacteria, virus,
or parasitic pathogens, wherein many of
which are foodborne illnesses.
• Most common manifestations are diarrhea
and vomiting, which may or may not be
associated with abdominal pain and fever.
 Risk Factors
• Environmental contamination
• Increased exposure to enteropathogens
• Young age
• Immunodeficiency
• Malnutrition
 Treatment
A B C

LOOK AT Well, Alert Restless, irritable Lethargic or

CONDITION: unconscious
EYES* Normal Sunken Sunken
THIRST Drinks normally and Thirsty Drinks poorly, or
thirsty drinks eagerly not able to drink
FEEL: SKIN PINCH Goes back quickly Goes back slowly Goes back very
slowly

DECIDE The patient has NO If the patient has If the patient has
SIGNS OF two or more signs two or more signs
DEHYDRATION in B, there is SOME in C, there is
DEHYDRATION SEVERE
DEHYDRATION
TREAT Treatment plan A: Weigh patient, and Weigh patient and
ORS solution use treatment plan use treatment plan
Supplemental Zinc B C
Continue feeding
Acute gastroenteritis
 Acute gastroenteritis
PLAN A PLAN B PLAN C
Give fluids more than Fluids PO:
usual: 75cc/kg over 4 hours
-ORS
-Salted drinks When oral hydration fails
-Soup with salt can give fluids via NGT
<2Y/O: 50-100ml
2-10y/o: 100-200ml Or
>10y/o: as tolerated
LR TIV (75cc/kg over 4
Zinc supplement: OD x 14d hours)
<6mos: 10mg/day
>6mos: 20mg/day

Offer to feed child every 3-

4hrs (six times a day)
Acute gastroenteritis
CONSTIPATION
HIRSCHSPRUNG VS ILEUS
Hirschsprung
• congenital aganglionic megacolon
• developmental disorder of enteric Nervous system –
absence of ganglionic cell in submucosal an
myenteric plexus – inadequate relaxation of the
bowel wall and hypertonicity
• Rectosigmoid
• In neonates: abdominal distention, failure to pass
meconium, bilious emesis or with feeding
intolerance
• Leads to ENTEROCOLITIS (clostridium difficile, s.
aureus, anaerobes) with associated diarrhea,
abdominal tenderness, sepsis and signs of bowel
obstruction
• Tympanitic, distended abdomen, large fecal mass
palpable in the left lower abdomen
• On DRE: normally place anus, rectum is free of fecal
material, when the finger is removed, there may be
explosive discharge of foul smelling feces and gas.
• Small pellets, ribbon like or fluid consistency feces
• Intermittent attacks may be associated with fever
and pain.
• Urinary retention secondary to urinary compression
• DX: rectal suction biopsy (2 cm above dentate line)
• TX: primary pull through procedure
ILEUS
• Failure of intestinal peristalsis cause by
loss of coordinated gut motility
• Accompanied with metabolic
abnormalities (uremia, hypokalemia,
hypercalcemia, hypermagnesemia,
acidosis)
• Nausea, vomiting, feeding intolerance,
abdominal distention with pain, delayed
passage of stool and bowel gas
• Bowel sounds minimal or absent
• Radiographs: multiple air fluid levels
throughout the abdomen
• TX: correcting underlying abnormalities.
Nasogastric decompression, fluid
correction,
ATOPIC DERMATITS
 ATOPIC DERMATITIS (ECZEMA)
• Atopic Dermatitis (Eczema) is a chronic
condition characterized by pruritus, a
personal/family history of atopy, and an age-
dependent distribution.
• Lesions:
– Typical lesions are pink-red crusted or scaly
plaques or papules.
– Lichenification
 ATOPIC DERMATITIS (ECZEMA)
• Distribution:
• <2y/o: cheeks, face, scalp, trunk, extensor
surface of extremities
• 2-10y/o: Neck, wrist, ankles, flexural surface
of extremities
• After puberty: face, neck, hands and feet
• Complications: Secondary infections
ATOPIC DERMATITIS (ECZEMA)
 ZINC OXIDE + CALAMINE OINTMENT
CLASS/MOA INDICATION CONTRAIN DOSE ADVERSE
DICATION EFFECTS
Drug Class: Soothe and help Hypersensitivity Apply 2-4x daily on Hypersensitivity
Emolient promote healing clean skin (affected
of impared skin area)
MOA: Wound integrity
granulation and
re-
epithelialization
FURUNCULOSIS
 FURUNCULOSIS
• Causative agent: S.aureus
• Risk Factors:
– Obesity
– Hyperhidrosis
– Maceration
– Friction
– Pre-existing dermatitis
– Immunodeficiency
 FURUNCULOSIS
• Causative agent: S.aureus
• Risk Factors:
– Obesity
– Hyperhidrosis
– Maceration
– Friction
– Pre-existing dermatitis
– Immunodeficiency
 FURUNCULOSIS
• Recurrent furunculosis is frequently associated
with carriage of S.aureus in the nares, axillae,
or perineum, or close contact with someone
(e.g., family member) who is a carrier.
• Infection can be spread by crowding
conditions, shared personal hygiene items and
a compromised skin barrier.
 FURUNCULOSIS
• Most commonly located at the lower
abdomen, buttocks and legs.
• Lesions are initially indurated, central necrosis
and suppuration follow leading to rupture and
discharge of a central core of necrotic tissue
and destruction of follicle.
• Healing occurs with scar formation.
 FURUNCULOSIS
• Treatment:
• Regular bathing
• Loose fitting clothes
• Large lesions should be drained
• Systemic antibiotics for numerous furuncles
usually treated with oral antibiotics
 CLOXACILLIN
CLASS/MOA INDICATION CONTRAIN DOSE ADVERSE
DICATION EFFECTS
Drug Class: Staphylococcal Hypersensitivity <40kg -Hypersensitivity
Penicillin infections Mild-Moderate -Cross
infection: 12.5- allergenicity with
MOA: Inhibits 25mkday q6 other penicillins
Binds to PBP, Severe infection: -GI disturbance
inhibits bacterial 50-100mkday q6
cell wall Skin and soft
transpeptidation tissue infection
(MSSA): 25-
50mkday q6
>40kg: 125-
500mg/dose PO q6

Max dose: 2g/24hr

 MUPIROCIN
CLASS/MOA INDICATION CONTRAIN DOSE ADVERSE
DICATION EFFECTS
Drug Class: Gram positive Hypersensitivity Topical (>mons- -Epistaxis
Topical cocci including adult): APPLY TID -Stinging or
Antibiotics methicillin- TO AFFECTED AREA burning
susceptible and sensation
MOA: Inhibits MRSA for minor Intranasal for -Mild skin rash
staphylococcal skin infections elimination of -Headache
isoleucyl tRNA nasal colonization:
synthetase; Infant and child:
Bactericidal small amount
intranasally BID 5-
10days
Child >12y/o:
500mg intransally
BID x 5-10days
CHICKENPOX/VIRAL EXANTHEM
 VARICELLA ZOSTER
• Double stranded DNA
• Most children were infected by 10
• Transmission: direct contact, airborne
(household and school classroom)
• Contagious: 24-48 hours before the rash is
evident until vesicles are crusted, usually 3-7
days after onset of rash
• Inoculation of virus onto the mucosa of upper
respiratory tract and tonsillar lymphoid tissue
• Virus replicates in the local lymphoid tissue
• Latency of VZV occurs only in ganglionic
neurons
• Herpes zoster – Reactivation of VZV, usually
manifested by a vesicular rash that is
unilateral and dermatomal in distribution
 MANIFESTATIONS
• IN UNVACCINATED
– Begins 14 to 16 days after exposure
– Fever, malaise, anorexia, headache may occur 24-48 hr
before the rash appears
– RASH: appear first on the scalp, face, trunk
– Pruritic erythematous macules that evolve through the
papular stage to form clear, fluid filled vesicles
– The simultaneous presence of lesions in various stages of
evolution is characteristics of varicella
– Distribution: central or centripetal, greatest concentration
on the trunk and proximally on the extremities
– Ulcerative lesions involving mucosa of the oropharynx and
vagina are common
 MANIFESTATIONS
• VACCINATED
– Wild type or vaccine strain – occurs 0-42hrs after vaccination
– BREAKTHROUGH VARICELLA – occurs in more than 42 days
• NEONATAL VARICELLA
– Pregnant mother is exposed during the first half of
pregnancy (8th-20th week)
– Virus spreads to all fetal organs
– Scars and skin loss
– Microcephaly, seizure, encephalitis, cortical and spinal
atrophy, mental retardation, cerebral calcification
– Cataracts, optic atrophy, nystagmus and horner syndrome
– Limb hypoplasia, prematurity and IUGR
 MANIFESTATIONS
• CONGENITAL VARICELLA SYNDROME
– Pregnant mother infection during the last 3 weeks
of pregnancy (rash appears 5 days before delivery
& 2 days post partum)
– Centripetal rash that spares the extremities
– Macules that progress to vesicles and encrustation
– May develop varicella pneumonia
 ACYCLOVIR
CLASS/MOA INDICATION CONTRAIN DOSE ADVERSE
DICATION EFFECTS
Drug Class: Herpes simplex 1 Hypersensitivity Preparations: Nausea
Antiviral and 2 (cold sores 200mg/5ml Vomiting
400mg/5ml
Synthetic and genital 200mg/tab
Diarrhea
nucleoside herpes) 400mg/tab Headache
analogues 800mg/tab
Varicella zoster ORAL: 20mkdose,
MOA: inhibits maximum 800mg/dose,
DNA polymerase, Herpes zoster given 4x a day for 5 days
incorporate into
and terminates
the growing viral
DNA chain, and
inactivates viral
DNA polymerase
 POSTEXPOSURE PROPHYLAXIS
• Vaccination 3-5 days after exposure
• HIGH TITER ANTI VZV immune globulin – for
immunocompromised, pregnant women,
newborns
• Varicella IG – for newborns whose mothers
have varicella 5 days before or 2 days after
delivery
 MEASLES (RUBEOLA)
Paramyxovirus (IP 8-12 days)
• High-grade fever with cough,
conjunctivitis and colds (3-5 days)
and photophobia
• Enanthem: Koplik spots (grayish
white dots with red border
opposite lower molars) appear
before the rash
• Rashes usually appear at the
height of fever
• Craniocaudal dissemination:
maculopapular rash begins in face
• Rash fades downward in the same
sequence in which it appeared
(branny desquamation),
disappears in 7-10 days
• Airborne
• Communicability: 4 days before to
4 days after onset of rash
• Diagnosis: Warthin-Finkeldey
giant cells (fusion of infected
cells)
• Supportive
• Most common complication: otitis •
media
• Most common cause of mortality: •
GERMAN MEASLES
(RUBELLA)
Togavirus (IP 14-21 days)
• Most characteristic sign:
retroauricular, posterior cervical &
postoccipital symmetrical
lymphadenopathy (begins 24
hours before the rash & remains
for 1 week)
• Enanthem: Forscheimer spots
(discrete rose spots onthe soft
palate) appear at the same time as
rash
• No photophobia
• Fever with decrease in
temperature on 3rd-4th day as
rashes appear
Maculopapular rash begins at face
•
and neck and spreads
centrifugally as discrete macules
• Droplet or transplacental
• Communicability: 7 days before to
6-7 days after onset of rash
• Fourfold increase in IgM and IgG
antibody is diagnostic
• Supportive
Thrombocytopenia 2 weeks after
rash
Arthritis of the small joints of the
ROSEOLA INFANTUM
Human herpes virus (HHV) 6 & 7
(IP 9-10 days)
• Most often among <3 years
old, peak at 6-15 months old
• Fever for 3-5 days with
fussiness
• Rash appears within 12-24
hours of fever resolution:
discrete, small pink lesions on
the trunk, spreads in a
centripetal pattern, fades in 1-
3 days
• Ulcers in uvulopalatoglossal
junction (Nagayama spots)
• Bulging of anterior fontanelle
• Convulsions
• Probably acquired from the saliva
of healthy persons, enters host
through mucosa
• Communicability: Unknown
• HHV-6 serology, PCR, virus culture
• LOW WBC count, neutrophils,
lymphocytes
• Supportive
• Excellent with no obvious
sequelae HHV-6 can suppress all
cellular lineages within the bone
SCABIES
SCABIES
 Itchy skin condition caused by a tiny burrowing mite called Sarcoptes scabiei
 SITES: scalp, face, neck, palms of the hands, soles of the feet
 MANIFESTATIONS: intense pruritus at night. 1-2mm red papules. Threadlike burrows are
the classic lesion of scabies but may not be seen in infants. In infants, bullae and pustules
are relatively common.

TRANSMISSION SYMPTOMS DIAGNOSIS CONTROL AND

PREVENTION
 From infested host  Raised rash and  Microscopically  Controlled by
to a new host by intense itching from skin early diagnosis
skin to skin caused by allergic scrapings, needle  Early treatment
contact reaction to the removal of the with scabicide and
 From infested mites, burrow, mite or by the environment and
clothing, beddings eggs and fecal adhesive tape test textile cleaning,
or environment pellets under the contact
skin prophylaxis,
education
 Starve the mite by
placing items can’t
be washed in a
sealed plastic bag
and leave for
couple weeks
DRUG ACTION INDICATION CONTRA- ADVERSE
INDICATION EFFECTS

GENERIC Acts on the active against Hypersensiti Itching

NAME: nerve cell a broad range
membrane to of pests, vity Redness
Permethrin disrupt the including lice,
cream sodium ticks, fleas, Numbness or
channel mites, and tingling of the
DRUG CLASS: current that other skin
scabicides and regulates the arthropods
polarization of Rash
pediculicides the
membrane.
 HAND-FOOT-MOUT DISEASE
ETIOLOGY MANIFESTATION TRANSMISSION
 <10 years of age  Fecal-oral and  Diagnosis: Viral
COXSACKIE A 16  1-2 days low respiratory routes culture (gold
grade fever fever  Communicability: standard)
ENTEROVIRUS 71 then appearance viral shedding
of enanthem from the  Treatment:
(vesicles and respiratory tract Supportive
IP: 4-6 DAYS ulcerations in the 1-3 weeks and
oral cavity) fecal shedding up  Complications:
followed by to 7-11 weeks neurologic
exanthem post-infection disease,
 Tender, vesicular encephalomyelitis
skin lesions with , pulmonary
surrounding edema,
erythema on the hemorrhage
hands (more than
feet), buttocks,
groin
APPENDICITIS
DRUG ACTION INDICATION CONTRA- ADVERSE
INDICATION EFFECTS

GENERIC NAME: INHIBITS INFECTIONS OF HYPERSENSITIVITY CNS: SEIZURES IN

BACTERIAL CELL THE FF: TO CEFOXITIN, HIGH DOSES
CEFOXITIN WALL SYNTHESIS -LOWER ANY COMPONENT
BY BINDING TO RESPIRATORY OF THE GI:
DRUG CLASS: ONE OR MORE TRACT FORMULATION, PSEUDOMEMBRAN
OUS COLITIS,
SECOND OF THE INFECTION OR OTHER DIARRHEA, NAUSEA,
GENERATION PENICILLIN- -SKIN CEPHALOSPORINS VOMITING
BINDING INFECTIONS SKIN: RASHES AND
CEPHALOSPORIN PROTEINS; -BONE AND URTICARIA
INHIBITS FINAL JOINT HEMATOLOGIC:
TRANSPEPTIDATI INFECTIONS BLEEDING,
ON STEP OF -UTIs EOSINOPHILIA,
PEPTIDOGLYCAN -GYNECOLOGIC HEMOLYTIC
SYNTHESIS -INTRA- ANEMIA,
RESULTING IN ABDOMINAL LEUKOPENIA,
THROMBOCYTOPENI
CELL WALL -SEPTICEMIA A
DEATH. -PERIOPERATIVE MISC:
PROPHYLAXIS ANAPHYLAXIS/ALLE
RGIC REACTIONS
DENGUE FEVER
 DENGUE FEVER
• Most significant vector borne infection
• Causative agent: Dengue Virus
• Vector: Aedes aegypti
• Incubation period: 3-14days
DENGUE FEVER
DENGUE FEVER
PCAP
PCAP
MENINGITIS
 TB MENINGITIS BACTERIAL MENINGITIS
Rapid progression Headache, nasuea, vomiting, anorexia,
More common in infants and young children irritability, fever, neck pain and rigidity,
obtundation, coma, focal neurologic deficits
1st stage Nuchal rigidity secondary to inflammation
-Last 1-2 weeks of spinal nerves and roots produce
-non specific SSX (Fever, headache, meningeal signs of irritation (Brundzinski
irritability, drowsiness, malaise) and kernig sign)

2nd stage
-focal neurologic signs (Nuchal rigidity,
seizures, positive kernig and brudzinski,
cranial nerve palsies)

3rd stage
-coma
-hypertension
-deteriorating vital signs
DIAGNOSTICS: DIAGNOSTICS
-CSF analysis -LUMBAR PUNCTURE
-CT or MRI -blood culture
-high CRP, ESR(differentiates bacterial from
viral)
 SUBARACHNOID HEMORRHAGE
• Subarachnoid hemorrhage (SAH) is a
type of stroke
• Head trauma is the most common cause
• In patients without head trauma, SAH is
most commonly caused by a brain
aneurysm.
• There is bleeding in the space that
surrounds the brain, between the pia
and arachnoid membrane
• Most often, it occurs when a weak area
in a blood vessel (aneurysm) on the
surface of the brain bursts and leaks.
• The blood then builds up around the
brain and inside the skull increasing
pressure on the brain.
• sudden severe headache
• a stiff neck
• feeling and being sick
• sensitivity to light (photophobia)
• blurred or double vision
INTRACRANIAL MASS
• Tumors arising from tissue other than
brain parenchyma, such as the meninges,
dura, calvarium, ventricle, choroid
plexus, pineal gland, or pituitary gland
• Meningioma being the most common
type of neoplasm
• New onset or change in pattern of
headaches
• Headaches that gradually become more
frequent and more severe.
• Unexplained nausea or vomiting.
• Vision problems, such as blurred vision,
double vision or loss of peripheral vision.
SEXUALLY TRANSMITTED DISEASE
OTITIS
JUVENILE NASOPHARYNGIOMA
 JUVENILE NASOANGIOFIBROMA
• Benign, vascular neoplasm located at nasopharynx of
adolescent males
• Most commonly in the 2nd decade 7-21 years, mean age of 14
• Cause: history of Familial adenomatous polyposis are slightly
more likely to have JNA
– Desmoplastic response of the nasopharyngeal periosteum or
– Embryonic fibrocartilage between basiocciput and the
basisphenoid
– Vestiges of atrophied stapedial artery
• Location: Superior lip of the sphenopalatine foramen at the
junction of the pterygoid process of the sphenoid bone and
the sphenoid process of the palatine bone
 JUVENILE NASOANGIOFIBROMA
• Usually lobulated, rubbery and red-pink to tan-gray
in appearance
• Usually is encapsulated and composed of vascular
tissue and fibrous stroma with coarse or fine collagen
fibers.
• Slow growing and initially expand intranasally into
the nasopharynx and nasal cavity and then into the
pterygomaxillary space.
• Over time, will eventually erode bone and invade the
infratemporal fossa, orbit, and middle cranial fossa.
 JUVENILE NASOANGIOFIBROMA
• The blood supply to these benign tumor is
most commonly from the internal maxillary
artery
• May also be supplied by the:
– External carotid artery
– Internal carotid artery
– Common carotid artery
– Ascending pharyngeal artery
 JUVENILE NASOANGIOFIBROMA
PRESENTATIONS
• Unilateral nasal obstruction
• Epistaxis
• Nasopharyngeal mass in adolescent males with an average
age of onset of 15 years of age
• Conductive hearing loss
• Dacrocystits – inflammation of nasolacrimal gland
• Rhinolalia – nasal tone speech due to excessive closure od
posterior nares
• Hard and soft palate deformity
• Hyposmia or anosmia
 JUVENILE NASOANGIOFIBROMA

• CT SCAN with CONTRAST – excellent for

evaluation
• Holman-Miller sign
– The characteristic anterior bowing of the posterior
maxillary wall due to the presence of a mass in the
pterygomaxillary space
• Should NOT undergo BIOPSY due to risk of
bleeding
 JUVENILE NASOANGIOFIBROMA
Staging: Classification according to Fisch
• Stage I - Tumors limited to nasal cavity, nasopharynx
with no bony destruction
• Stage II - Tumors invading pterygomaxillary fossa,
paranasal sinuses with bony destruction
• Stage III - Tumors invading infratemporal fossa, orbit
and/or parasellar region remaining lateral to
cavernous sinus
• Stage IV - Tumors invading cavernous sinus, optic
chiasmal region, and/or pituitary fossa
 JUVENILE NASOANGIOFIBROMA
TREATMENT
• Surgery – Gold standarad
• Radiation therapy
• Chemotherapy
• Hormone therapy

Preoperative selective arterial embolization of feeding vessels

from the external carotid artery has significantly decreased
intraoperative blood loss and facilitated resection of larger tumors.
• Embolization is typically performed 24-72 hours prior to
resection.
NEPHRITIC VS NEPHROTIC
 NEPHROTIC vs NEPHRITIC
NEPHROTIC NEPHRITIC
Persistent heavy proteinuria Proteinuria
(>3.5g/24hr)
Hypoalbuminemia Gross hematuria
(<2.5g/dL)
Hyperlipidemia Oliguria
(cholesterol >200mg/dl
Edema Edema
-/+ Hypertension + Hypertension
UTI
 URINARY TRACT INFECTION
• Most common in children under 1 year old
• Afebrile symptomatic UTI over 1 year of age is 8%
• Febrile UTI is 7%
• 1st year of life M:F ratio 2.8:5.4
• Beyond 1-2 year old, FEMALE predominance 1:10
• In MALES, UTI occur during the 1st year of life, more
common in uncircumcised (20%)
• In FEMALES, occurs usually by the age of 5, peaks during
infancy, toilet training and onset of sexual activity
• MCC: Escherichia coli (56-67%), followed by Klebsiella
spp, Proteus spp, Enterococcus, and Pseudomonas
 URINARY TRACT INFECTION
• 2 basic forms: PYELONEPHRITIS and CYSTITIS
• Pyelonephritis
– Abdominal pain, back or flank pain, fever, malaise,
nausea, vomiting and occasionally diarrhea
– Fever maybe the only manifestation (>39C) without
another source lasting for more than 24 hours
• Cystitis
– Bladder involvement only
– Includes dysuria, urgency, frequency, suprapubi pain,
incontinence and possible malodorous urine
• Pathogenesis: ascending infection
 URINARY TRACT INFECTION
• URINE CULTURE – gold standard
• Sample collection
– In toilet trained – mid stream urine (>100,000 colonies)
– 2-24 months not – catheterized or suprapubic aspirate
urine (>50,000 colonies or 10,000 if symptomatic)
• Nitrites and Leukocyte often positive
• Pyuria – leukocytes on urine
• WBC above 3-6 is indicative of infection
• Sterile pyuria – positive leukocytes, negative
culture
DUODENAL ATRESIA
 DUODENAL ATRESIA
• Pathogenesis
– Failure to recanalize the lumen during 4th-5th week of gestation
– Obstruction usually distal to the ampulla of vater
– More common in preterm infants
• Presentation
– BILIOUS vomiting (hallmark) without abdominal distention
usually noted on 1st day of life
– Prenatal Exam: polyhydramnios in 50% due to failure of
absorption of amniotic fluid in the distal intestine
– Associated with Down syndrome, esophageal atresia and
imperforate anus
 DUODENAL ATRESIA
• Diagnosis
– Abdominal radiograph: “double bubble sign” –
pathognomonic of duodenal obstruction where two
lucencies are seen in the stomach and the other in
the first portion of duodenum
• Management
– Gastric decompression (NGT, OGT) to control
vomiting
– Surgical repair: duodenoduodenostomy with
gastrostomy tube
 HIRSCHSPRUNG DISEASE
• Congenital aganglionic megacolon causing loss of ability of the
muscles in the bowels to move stool through the intestine
• Most common cause of lower intestinal obstruction in neonates
• PATHOGENESIS
– More common in MALES
– Due to arrest of neuroblast migration from the proximal to distal bowel,
leading to absence of ganglion cells in the bowel wall beginning in the
internal anal sphincter
– Absence of Meisnsner and Aurbach plexus and hypertrophied bundles
with high concentrations of acetylcholinesterase between the muscular
and submucosa layers that leads to:
• Decreased motility in the affected bowel segment
• Lack of progression of peristaltic waves into the aganglion colon
• Abnormal or absent relaxation of this segment and of the internal anal sphincter
 HIRSCHSPRUNG DISEASE
• SYMPTOMS
– Distended abdomen, failure to pass meconium, bilious emesis of
aspirates, feeding intolerance
– Suspected in any full term infants with delayed passage of stool
(>48hours of life)
– Some may present with chronic constipation
• SIGNS
– Tympanitic and distended abdomen
– Large fecal mass palpable in the left lower quadrant
– Pellet-like or ribbon like stool or with fluid consistency
– RECTAL EXAM: empty feces on exam with normal anal tone and
may have explosive discahrge (gush of air) once finger is removed
 HIRSCHSPRUNG DISEASE
• DIAGNOSIS
– RECTAL BIOPSY – gold standard
– Abdominal xray with contrast enema:
• transition zone between normal dilated proximal colon and the smaller-
calliber obstructed distal colon due to nonrelaxation of the aganglionic
bowel
• A rectal diameter that is the same as or smaller than the sigmoid colon
– Anorectal manometry 0 difficult to perform in infants
• Evaluated the internal anal sphincter while a balloon is distended in the
rectum
• Normal response: relaxation of the internal anal sphicter
• MANAGEMENT
– Primary pull-through procedure unless there is associated
enterocolitis or other complications
SLE
SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

• Autoantibody production against self-antigens

resulting in inflammatory damage to organs
• Female predominance
• Median age of diagnosis: 11-12 yo
• Fibrinoids deposit found in blood vessel walls
of affected organs, skin, joints, kidneys, blood-
forming cells, blood vessels, and CNS
 MANAGEMENT
• MILD:
– Who do not have renal or other life threatening involvement
– NSAIDS, Hydroxychloroquine, low dose glucocorticoids

• MODERATE TO SEVERE:
– With organ involvement
– -high dose glucocorticoid or pulse therapy with methyprednisone
– Hydroxychloroquine
– Cyclophosphomide
– Rituximab
– If persistent: methothrexate, leflunomide, azathioprine to limit
cumulative steroid exposure
DESMOID TUMOR
•
DESMOID
Known as Fibromatosis
TUMOR
• From connective tissue, from fibroblast cells
• Caused by gene alteration - Adenomatous polypsosis coli or
APC. This causes too much of protein called beta-catenin, this
cause cells to grow when they shouldn’t
• Occur anywhere in the body
• Often found in the ABDOMEN, as well as the shoulders, upper
arms and thighs
• Benign
• Invades nearby tissue and often are very painful
• SYMPTOMS: pain, swelling in the area of the tumor, difficulty
moving
• DIAGNOSTICS: CT, MRI, UTZ; Biopsy
 DESMOID TUMOR
• TREATMENT
– Observation: grows slowly, or can shrink and go
away on their own, or remain the same size to
grow quickly
– Surgery: tumor often returns to the same location
after surgery
– Radiation therapy
– Chemotherapy: can shrink the tumor
• Complication: obstruction
ASTROCYTOMA
 Right Frontal Lobe biopsy result done on 10/1/21:

• HIGH-GRADE GLIOMA, FAVOR HIGH GRADE ASTROCYTOMA

• IMMUNOHISTOCHEMISTRY STUDIES:
• ATRX: RETAINED NUCLEAR STAINING IN CELL5 OF INTEREST.
• P53: NEGATIVE, NO STAINING IN CELLS OF INTEREST.
• GFAP: POSITIVE. STRONG, DIFFUSE CYTOPLASMIC
STAINING IN CELLS OF INTEREST.
• SYNAPTOPHYSIN: DIFFUSE CYTOPLASMIC STAINING IN
SURROUNDING NON-DYSPLASTIC NEURONS,
• K167: 10 TO 15% PROLIFERATION INDEX