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 acrode rmatitis enteropathica-like syndrome developed while he was receiving a branched-chain amino acid-free formula. latrogenically induced isole ucine deficiency developed and resulted in a decreased protein accreti on 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 wi thout adequate supplementation of deficient amino acids.