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. 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.