Mismanagement of Phenylketonuria: An Underlying Cause of Kwashiorkor

Objective

Too much restriction of dietary proteins can cause severe protein malnutrition,which can occur in adjusting the diet for some kinds of aminoacidopathies, urea cycle disorder and organic academia. This report presents the case of a 1.5-year-old boy with history of phenylketonuria with a three weeks history of erythematous scaly plaques and edema of his extremities; he had a history of similar skin manifestations three months earlier that resolved spontaneously. The patient had been on very restricted phenylalanine diet. Diagnosed with Kwashiorkor, a phenylalanine level of 0.4 mg/dl, the child was hospitalized and put on a special diet and given the appropriate antibiotic; after a few days of treatment his condition improved. We underscore the importance of education for those considering prescription of diet restriction and emphasize the regulation of balanced diet in such patients.