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September 1993

Acrodermatitis Enteropathica-like Syndrome Secondary to Isoleucine Deficiency During Treatment of Maple Syrup Urine Disease

Am J Dis Child. 1993;147(9):954-956. doi:10.1001/archpedi.1993.02160330044015

• 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. latrogenically 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.

(AJDC. 1993;147:954-956)