PEDIATRIC COMAT REVIEW 2024-2025 |COMPLETE QUESTIONS AND
CORRECT DETAILED ANSWERS (VERIFIED ANSWERS ) AGRADE.
clinical presentations of fetal hydantoin syndrome - ANSWER-due to
phenytoin, carbamazepine, or valproate use
- cleft lip and palate
- wide anterior fontanelle
- distal phalange hypoplasia
- cardiac anomalies (pulmonary stenosis, Aortic stenosis)
- microcephaly
risk factors for pediatric ischemic stroke - ANSWER-congenital heart
defects (paradoxical embolism), bacterial endocarditis, arterial
dissection (head/neck trauma), vasculitis (takayasu arteritis, SLE),
infection (bacterial meningitis), sickle cell, hypercoagulable states
(protein C or S deficiency)
to diagnose this = MRA - more sensitive than CT head for ischemic
stroke
Clinical manifestations of Wilsons Disease - ANSWER-due to
defective hepatic copper transport
1. CNS- parkinson-like sx (bradykinesia, tremor, rigidity, pooled
saliva) dementia. personality and behavioral changes including
psychosis
2. liver disease- hepatitis, hepatosplenomegaly, cirrhosis,
hemolytic anemia
,3. corneal copper- Kayser-fleischer rings (brown or green
pigmentation in cornea
most cases of pulmonic regurg is due to ? - ANSWER-iatrogenic -
following repair of congenital defects including TOF
Postural kyphosis vs. Scheuermann Disease Kyphosis? – ANSWER-
kyphosis fixes with forward bending with postural kyphosis but
doesn't with Scheuermann
Scheuermann is due to abnormal anterior vertebral wedging --> rigid
curve in the spine
diagnosis requires lateral spine xrays showing kyphosis >40 degrees
and >5 degrees of anterior wedging in 3 or more adjacent vertebrae
common cause of iron deficiency anemia in children – ANSWER-
excessive cows milk intake (>700mL daily)
consequences of myelomeningocele - ANSWER-CSF is lost through
the open neural tube defect --> collapse of cranial structures -->
inferior displacement of the medulla and cerebellum through the
foramen magnum = Chiari II malformation
the early stages of ALL can show what labs - ANSWER-pancytopenia -
the leukocyte count initially decreases due to concurrent production
,with the blastic cells, but as time goes on those blastic cells reach the
periphery leading to increased leukocytes
clinical presentation of septic arthritis in infants - ANSWER-fever, pain
with hip movement, and leukocytosis
the affected hip will often be flexed, abducted, and externally rotated
normal liver size in children - ANSWER-1-2 cm below the costal
margin
lactation failure jaundice vs breast milk jaundice - ANSWER-lactation
failure jaundice = age <1 week, insufficient intake of breast milk -->
dec bilirubin elimination and inc enterohepatic circulation - presents
with signs of dehydration and suboptimal breastfeeding
tx: feed more frequently (every 2-3 hours)
breast milk jaundice = age > 1 week (peaks at 2 weeks), increased
beta-glucuronidase in breast milk --> inc deconjugation of intestinal
bilirubin and inc enterohepatic circulation - can result in significantly
elevated unconjugated bilirubin levels
- presents with well hydrated baby with adequate breastfeeding
tx: if baby is feeding adequately and gaining weight, and bilirubin
levels are trending down - continued breastfeeding is recommended
and the jaundice will resolve by week 12
, Clinical features of neuroblastoma - ANSWER--Median age < 2 -
abdominal mass, fever, weight loss, signs of secondary mets (ie
bone pain), tumor compression symptoms. -Periorbital
ecchymoses (due to orbital mets)
-Spinal cord compression from epidural invasion (dumbbell tumor)
- opsoclonus-myoclonus syndrome
-increased VMA and HVA in urine
-Small round blue cells on histo
-N-myc gene amplification
Mullerian agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome) -
ANSWER-abnormal development of uterus, cervix, and upper third of
vagina due to failure of paramesonephric duct development
- primary amenorrhea
- normal female external genitalia
- blind vaginal pouch
- absent or rudimentary uterus
- bilateral functioning ovaries (normal FSH)
WAGR syndrome manifestations - ANSWER-Wilms Tumor, Aniridia,
Genitourinary abnormalities, mental retardation
CORRECT DETAILED ANSWERS (VERIFIED ANSWERS ) AGRADE.
clinical presentations of fetal hydantoin syndrome - ANSWER-due to
phenytoin, carbamazepine, or valproate use
- cleft lip and palate
- wide anterior fontanelle
- distal phalange hypoplasia
- cardiac anomalies (pulmonary stenosis, Aortic stenosis)
- microcephaly
risk factors for pediatric ischemic stroke - ANSWER-congenital heart
defects (paradoxical embolism), bacterial endocarditis, arterial
dissection (head/neck trauma), vasculitis (takayasu arteritis, SLE),
infection (bacterial meningitis), sickle cell, hypercoagulable states
(protein C or S deficiency)
to diagnose this = MRA - more sensitive than CT head for ischemic
stroke
Clinical manifestations of Wilsons Disease - ANSWER-due to
defective hepatic copper transport
1. CNS- parkinson-like sx (bradykinesia, tremor, rigidity, pooled
saliva) dementia. personality and behavioral changes including
psychosis
2. liver disease- hepatitis, hepatosplenomegaly, cirrhosis,
hemolytic anemia
,3. corneal copper- Kayser-fleischer rings (brown or green
pigmentation in cornea
most cases of pulmonic regurg is due to ? - ANSWER-iatrogenic -
following repair of congenital defects including TOF
Postural kyphosis vs. Scheuermann Disease Kyphosis? – ANSWER-
kyphosis fixes with forward bending with postural kyphosis but
doesn't with Scheuermann
Scheuermann is due to abnormal anterior vertebral wedging --> rigid
curve in the spine
diagnosis requires lateral spine xrays showing kyphosis >40 degrees
and >5 degrees of anterior wedging in 3 or more adjacent vertebrae
common cause of iron deficiency anemia in children – ANSWER-
excessive cows milk intake (>700mL daily)
consequences of myelomeningocele - ANSWER-CSF is lost through
the open neural tube defect --> collapse of cranial structures -->
inferior displacement of the medulla and cerebellum through the
foramen magnum = Chiari II malformation
the early stages of ALL can show what labs - ANSWER-pancytopenia -
the leukocyte count initially decreases due to concurrent production
,with the blastic cells, but as time goes on those blastic cells reach the
periphery leading to increased leukocytes
clinical presentation of septic arthritis in infants - ANSWER-fever, pain
with hip movement, and leukocytosis
the affected hip will often be flexed, abducted, and externally rotated
normal liver size in children - ANSWER-1-2 cm below the costal
margin
lactation failure jaundice vs breast milk jaundice - ANSWER-lactation
failure jaundice = age <1 week, insufficient intake of breast milk -->
dec bilirubin elimination and inc enterohepatic circulation - presents
with signs of dehydration and suboptimal breastfeeding
tx: feed more frequently (every 2-3 hours)
breast milk jaundice = age > 1 week (peaks at 2 weeks), increased
beta-glucuronidase in breast milk --> inc deconjugation of intestinal
bilirubin and inc enterohepatic circulation - can result in significantly
elevated unconjugated bilirubin levels
- presents with well hydrated baby with adequate breastfeeding
tx: if baby is feeding adequately and gaining weight, and bilirubin
levels are trending down - continued breastfeeding is recommended
and the jaundice will resolve by week 12
, Clinical features of neuroblastoma - ANSWER--Median age < 2 -
abdominal mass, fever, weight loss, signs of secondary mets (ie
bone pain), tumor compression symptoms. -Periorbital
ecchymoses (due to orbital mets)
-Spinal cord compression from epidural invasion (dumbbell tumor)
- opsoclonus-myoclonus syndrome
-increased VMA and HVA in urine
-Small round blue cells on histo
-N-myc gene amplification
Mullerian agenesis (Mayer-Rokitansky-Kuster-Hauser syndrome) -
ANSWER-abnormal development of uterus, cervix, and upper third of
vagina due to failure of paramesonephric duct development
- primary amenorrhea
- normal female external genitalia
- blind vaginal pouch
- absent or rudimentary uterus
- bilateral functioning ovaries (normal FSH)
WAGR syndrome manifestations - ANSWER-Wilms Tumor, Aniridia,
Genitourinary abnormalities, mental retardation
