Acrodermatitis enteropathica-like syndrome secondary to isoleucine deficiency during treatment of maple syrup urine disease.
We describe a patient with maple syrup urine disease in whom an acrodermatitis enteropathica-like syndrome developed while he was receiving a branched-chain amino acid-free formula. Iatrogenically induced isoleucine deficiency developed and resulted in a decreased protein accretion and persistent increase in the plasma concentrations of leucine. A rapid clinical response to isoleucine supplementation was noted. This observation underscores the risks of using amino acid-free formulas without adequate supplementation of deficient amino acids.
Giacoia
G P
GP
Department of Pediatrics, University of Oklahoma College of Medicine, Tulsa 74136.
Berry
G T
GT
D000169:Acrodermatitis / Q000209:etiology*; D006801:Humans; D007049:Iatrogenic Disease; D007225:Infant Food; D007231:Infant, Newborn; D007532:Isoleucine / Q000172:deficiency*; D008297:Male; D008375:Maple Syrup Urine Disease / Q000150:complications* / Q000178:diet therapy; D013577:Syndrome
D007532:Isoleucine