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1. 1 wk newborn girl, poor feeding, vomiting, progressive lethargy over past 4 days. Born at term, no complication/cnongenital anomaly. Breast-fed. Healthy 2 yr brother, sister died at 10 days after full-term birth. PE: decreased muscle tone & poor responsiveness. Reflex normal. Serum bicarbonate 8, pH 7.15, plasma ammonia 10x the upper limit. Cause?
a. Mitochondrial disorder
b. Muscopolysaccharidoses disorder
c. Organic acid metabolism disorder
d. Renal tubular acidosis
e. X-linked leukodystrophy

2. 13 yr boy, frequent headaches over past 5 wks. Dull pain across forehead, four or five times weekly. Acetaminophen not relieving pain. Occasional nausea w/ no vomiting. Schizophrenial well controlled with resperidone. MRI of brain show which?
a. Bilateral increased caudate nuclei
b. Decreased cerebellar volume
c. Hippocampal symmetry
d. Increased lateral ventricle size
e. Multiple white matter hyperintensitiies
mnhakeem


1-??
2- D
abc

1)organic acid metabolism disorder
2)increased lateral ventricle size
carlos canova - teresacanova

22(d)
Me

1-C
2-D