• 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.
Giacoia GP, Berry GT. 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