TABLE 1.

Laboratory and Diagnostic Workup Leading up to PN Initiation at 3 Months of Age and at 6 Months of Age While on PN

Laboratory or diagnostic test	Result at 3 months of age	Result at 6 months of age
Serum sodium	153 mEq/L	136 mEq/L
Serum chloride	128 mEq/L	103 mEq/L
Serum bicarbonate	13 mEq/L	23 mEq/L
Blood glucose	115 mg/dL	121 mg/dL
Vitamin E level	3.5 mg/L	11.3 mg/L
Vitamin D level	10 ng/mL	15.2 ng/mL
Prothrombin time	16.9 seconds	15.2 seconds
Weight percentile	<0.01 percentile	15.99 percentile
Fecal elastase	38 μg/g	—
Stool α1 anti-trypsin	<0.05 mg/g	—
Fecal fatty acids	>100 droplets/HPF	—
Stool anion gap	128 mOsm/kg	—

Workup revealed a hypernatremic, hyperchloremic metabolic acidosis and low fat-soluble vitamin levels, all of which improved after PN initiation. Weight percentile increased after 3 months on PN. Stool studies at diagnosis were normal except for elevated fecal fatty acids and elevated stool anion gap.

PN = parenteral nutrition.