Newborns born to mothers with a history of recurrent HELLP syndrome or AFLP should be evaluated immediately after birth for which disorder?

• A. Fatty acid oxidation disorder
• B. Maple syrup urine disease
• C. Smith-Lemli-Opitz syndrome
• D. Phenylketonuria

Answer: (A)

Fatty acid oxidation disorders are the key idea here. When a newborn’s mother has a history of recurrent HELLP syndrome or acute fatty liver of pregnancy, it raises concern for a fetal metabolic issue involving fatty acid processing. Fatty acid oxidation disorders impair the infant’s ability to generate energy from fats, which becomes critical after birth during periods of fasting or energy demand. The result is early hypoglycemia, often with low or absent ketones, and liver dysfunction, sometimes with vomiting, poor feeding, or lethargy. Recognizing FAOD right after birth is crucial because timely management—frequent feedings, IV glucose, avoiding prolonged fasting, and targeted dietary therapy—can prevent severe deterioration.

The other listed conditions do not fit this specific perinatal risk pattern. Maple syrup urine disease, phenylketonuria, and Smith-Lemli-Opitz syndrome have different typical presentations and associations and are not the immediate neonatal concern linked to maternal HELLP/AFLP history.