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 . A 1-week-old newborn has had poor feeding,
vomiting, and
progressive lethargy over the past 4 days. She was
born at term; pregnancy,
labor, and delivery were uncomplicated, and she had no
anomalies. She is being breast-fed. She has a
healthy 2-year-old brother; a
sister died at 10 days of age after a full-term birth.
shows decreased muscle tone and poor responsiveness;
reflexes are normal.
Serum bicarbonate level is 8 mEq/L, pH is 7.15, and
plasma ammonia
level is 10 times the upper limit of normal. Which of
the following is the
most likely cause?

) Mitochondrial disorder

) Mucopolysaccharidoses disorder

) Organic acid metabolism disorder

) Renal tubular acidosis
e, x linked leukodistrophy




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