Gp. Giacoia et Gt. Berry, ACRODERMATITIS-ENTEROPATHICA LIKE SYNDROME SECONDARY TO ISOLEUCINE DEFICIENCY DURING TREATMENT OF MAPLE-SYRUP-URINE-DISEASE, American journal of diseases of children [1960], 147(9), 1993, pp. 954-956
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.