Pediatrics COMAT
Bernard-Soulier syndrome: scientific mechanism of disease - platelet aggregation defect due to
deficiency in gp Ib complex --> can't bind VWF
- autosomal recessive
Bernard-Soulier syndrome: h&p - abnormally large platelets
- thrombocytopenia / abnormal platelet function testing
- easy / prolonged mucosal bleeding
- ecchymoses
- petechiae
difference between hemophilia a and b A- factor VIII deficiency, aPTT prolonged
B- factor IX defiency, clinicaly indistinguishable from A (prolonged aPTT but other values normal)
intussusception: h&p sudden onset intermittent severe, cramps, progressive abdominal pain with
inconsolable crying and drawing up the legs towards the abdomen, often with pallor; vomiting; sausage-
mass; currant jelly stool
pathognomonic finding for necrotizing enterocolitis X-ray shows pneumatosis intestinalis - extravasation
of bowel gas into damaged bowel wall
hereditary spherocytosis: diagnostic technologies (pertinent lab findings) (4) - increased mean
corpuscular hemoglobin concentration
- negative Coombs
- increased osmotic fragility on acidified glycerol lysis test
- abnormal eosin-5-maleimide binding test
,hereditary spherocytosis: management - complications - pigment gallstones
- aplastic crises from parvovirus B19
difference between wilm's tumor / nephroblastoma and neuroblastoma neuroblastoma can cross the
midline of abdomen, nephroblastoma canNOT
what condition can develop after viral gastroenteritis and why secondary lactase deficiency due to
intestinal epithelial cell damage
secondary lactase deficiency: h&p - after viral gastroenteritis ep
- abdominal pain
- bloating
- diarrhea with stools that are malodorous and bulky
greatest risk factor of primary nocturnal enuresis positive family history
age to diagnose primary nocturnal enuresis as abnormal development greater than 5 years old
euvolemic hypernatremia: management hypotonic solution = 5% dextrose
hypovolemic hypernatremia: management isotonic solution = 0.9% saline bolus if symptomatic until
euvolemia is achieved
spermatocele: h&p cystic, mobile, smooth, nodule superior and posterior to the testis (upper pole) in
sexually mature males
,hydrocele anatomical location collection of fluid between parietal and visceral layers of tunica vaginalis
balanitis inflammation of the glans penis
what infection should be considered when thick white discharge is present? Candida albicans
a child with impaired adaptation to darkness, photophobia, dry scaly skin, Bitot spots vitamin A
deficiency
fever, HA, seizures, altered mental status, temporal lobe (uni or bilateral) hyper intensity on imaging
suggests HSV encephalitis
viral meningitis CSF findings:
- WBC predominance
- glucose
- protein - lymphocytosis
- normal glucose
- mildly increased protein
HSV encephalitis: management (tx) IV acyclovir
complication of measles and when it normally shows up subacute sclerosing panencephalitis; 5-7 years
after initial infection
migratory joint pain, carditis, fever onset after untreated URI acute rheumatic fever
acute rheumatic fever is what type of hypersensitivity reaction type II
, molluscum contagiosum: management reassurance
molluscum contagiosum is caused by what and spread by what a poxvirus
contaminated fomites (like towels), direct contact, autoinoculation
molluscum contagiosum: diagnostic techniques none - diagnosed clinically
molluscum contagiosum: h&p round, raised, flesh-colored lesions with central umbilication
impetigo: management - topical antibiotics (mupirocin) for few lesions
- oral antibiotics for many lesions or ecthyma (deep form with ulcers)
tinea corporis: h&p erythematous scaling rings with central clearing; ringworm
fixed arthritis for more than 6 weeks, quotidian (daily) fever for more than 2 weeks, rash that worsens
with fever, hepatosplenomegaly, anemia, inflammation markers, lymphadenopathy systemic juvenile
idiopathic arthritis
joint pain, daily fevers, splenomegaly, normocytic anemia, leukocytosis; contender LAD, fatigue, weight
loss ALL
Ehlers-Danlos syndrome - common cardiovascular complications aortic aneurysms
Friedreich ataxia - common cardiovascular complication hypertrophic cardiomyopathy
Bernard-Soulier syndrome: scientific mechanism of disease - platelet aggregation defect due to
deficiency in gp Ib complex --> can't bind VWF
- autosomal recessive
Bernard-Soulier syndrome: h&p - abnormally large platelets
- thrombocytopenia / abnormal platelet function testing
- easy / prolonged mucosal bleeding
- ecchymoses
- petechiae
difference between hemophilia a and b A- factor VIII deficiency, aPTT prolonged
B- factor IX defiency, clinicaly indistinguishable from A (prolonged aPTT but other values normal)
intussusception: h&p sudden onset intermittent severe, cramps, progressive abdominal pain with
inconsolable crying and drawing up the legs towards the abdomen, often with pallor; vomiting; sausage-
mass; currant jelly stool
pathognomonic finding for necrotizing enterocolitis X-ray shows pneumatosis intestinalis - extravasation
of bowel gas into damaged bowel wall
hereditary spherocytosis: diagnostic technologies (pertinent lab findings) (4) - increased mean
corpuscular hemoglobin concentration
- negative Coombs
- increased osmotic fragility on acidified glycerol lysis test
- abnormal eosin-5-maleimide binding test
,hereditary spherocytosis: management - complications - pigment gallstones
- aplastic crises from parvovirus B19
difference between wilm's tumor / nephroblastoma and neuroblastoma neuroblastoma can cross the
midline of abdomen, nephroblastoma canNOT
what condition can develop after viral gastroenteritis and why secondary lactase deficiency due to
intestinal epithelial cell damage
secondary lactase deficiency: h&p - after viral gastroenteritis ep
- abdominal pain
- bloating
- diarrhea with stools that are malodorous and bulky
greatest risk factor of primary nocturnal enuresis positive family history
age to diagnose primary nocturnal enuresis as abnormal development greater than 5 years old
euvolemic hypernatremia: management hypotonic solution = 5% dextrose
hypovolemic hypernatremia: management isotonic solution = 0.9% saline bolus if symptomatic until
euvolemia is achieved
spermatocele: h&p cystic, mobile, smooth, nodule superior and posterior to the testis (upper pole) in
sexually mature males
,hydrocele anatomical location collection of fluid between parietal and visceral layers of tunica vaginalis
balanitis inflammation of the glans penis
what infection should be considered when thick white discharge is present? Candida albicans
a child with impaired adaptation to darkness, photophobia, dry scaly skin, Bitot spots vitamin A
deficiency
fever, HA, seizures, altered mental status, temporal lobe (uni or bilateral) hyper intensity on imaging
suggests HSV encephalitis
viral meningitis CSF findings:
- WBC predominance
- glucose
- protein - lymphocytosis
- normal glucose
- mildly increased protein
HSV encephalitis: management (tx) IV acyclovir
complication of measles and when it normally shows up subacute sclerosing panencephalitis; 5-7 years
after initial infection
migratory joint pain, carditis, fever onset after untreated URI acute rheumatic fever
acute rheumatic fever is what type of hypersensitivity reaction type II
, molluscum contagiosum: management reassurance
molluscum contagiosum is caused by what and spread by what a poxvirus
contaminated fomites (like towels), direct contact, autoinoculation
molluscum contagiosum: diagnostic techniques none - diagnosed clinically
molluscum contagiosum: h&p round, raised, flesh-colored lesions with central umbilication
impetigo: management - topical antibiotics (mupirocin) for few lesions
- oral antibiotics for many lesions or ecthyma (deep form with ulcers)
tinea corporis: h&p erythematous scaling rings with central clearing; ringworm
fixed arthritis for more than 6 weeks, quotidian (daily) fever for more than 2 weeks, rash that worsens
with fever, hepatosplenomegaly, anemia, inflammation markers, lymphadenopathy systemic juvenile
idiopathic arthritis
joint pain, daily fevers, splenomegaly, normocytic anemia, leukocytosis; contender LAD, fatigue, weight
loss ALL
Ehlers-Danlos syndrome - common cardiovascular complications aortic aneurysms
Friedreich ataxia - common cardiovascular complication hypertrophic cardiomyopathy
