To the Editor:
Kwashiorkor, or protein-calorie malnutrition, is a common issue in developing countries subject to starvation. In economically advanced nations, however, kwashiorkor is extremely rare and may appear in children placed on restrictive diets instituted by well-meaning guardians. Kwashiorkor also may occur because of gastrointestinal malabsorption. We present a unique case of kwashiorkor that revealed an underlying diagnosis of pancreatic insufficiency.
A 12-year-old girl presented to the hospital with 4 days of watery nonbloody diarrhea occurring with every feeding as well as new onset of presumed diaper dermatitis that had not responded to nystatin cream. Facial swelling also was noted the day prior to admission. Her medical history was notable for cerebral palsy secondary to nonaccidental trauma, leaving the patient nonverbal and quadriplegic. She had numerous prior admissions for sepsis with marked hypotension and more recently was diagnosed with insulin-dependent type 2 diabetes mellitus. She had never lived outside the United States and resided at home with her adoptive parents.
Physical examination revealed a nonverbal underweight girl (weight, 25 kg). Large areas of denudation with surrounding desquamated skin resembling flaking enamel paint covered the buttocks and posterior legs bilaterally (Figure). She had linear hyperpigmented patches on the dorsal hands with one superficial erosion on the left wrist. Marked periorbital edema as well as nonpitting edema of the face, arms, and legs were present.
A, The right buttocks and posterior thigh showed patchy reddish brown discoloration with marked exfoliation and areas of hypopigmentation, giving an appearance of flaking enamel paint. There also was notable edema of the patient’s fingers. B, The lateral right lower extremity showed areas of hyperpigmented desquamation in a linear configuration.
Upon additional questioning, the patient’s adoptive parent reported a diet of formula containing 1.0 cal/mL with 200-mL feedings 3 times daily through a Geiger-Müller tube, providing a daily protein intake of approximately 17.7 g per day (0.7 g/kg per day). On the day of admission, abnormal laboratory findings included low protein and albumin levels at 4.6 g/dL (reference range, 5.7–8.2 g/dL) and 2.1 g/dL (reference range, 3.2–4.8 g/dL), respectively; an elevated aspartate aminotransferase level of 73 U/L (reference range, 10–34 U/L); and an elevated alanine aminotransferase level of 80 U/L (reference range, 10–40 U/L). Based on the patient’s characteristic clinical findings and abnormal laboratory values, a diagnosis of acute kwashiorkor was made. Although the zinc level was low at 0.29 µg/mL (reference range, 0.66–1.10 µg/mL), the patient did not have any periorificial involvement to support a diagnosis of acrodermatitis enteropathica.
Upon further workup, stool elastase was measured at less than 50 µg per gram of stool (reference range, >200 µg pancreatic elastase per gram of stool), confirming a diagnosis of severe pancreatic insufficiency. Pancreatic enzyme supplementation was initiated along with an increase in protein intake to 1.5 g/kg per day. The patient’s hospital course was complicated by respiratory distress and sepsis, leading to a prolonged hospital stay. A component of refeeding syndrome may have contributed to the patient’s respiratory distress.