Liver transplants cure metabolic symptoms of maple syrup urine disease

Children's Hospital of Pittsburgh researchers report that liver transplants cured the metabolic symptoms of 11 individuals with maple syrup urine disease (MSUD), a metabolic disorder. The usual treatment for MSUD is a strict diet almost devoid of protein to try to reduce the toxic accumulation of amino acids in the body. The patients in the study, ranging in age from 1 to 20 years, are all alive and well with normal liver function. Their levels of amino acids stabilized within 6-12 hours of the transplantation and have remained stable since then, despite the participants eating an unrestricted diet. Although liver transplantation carries serious risks, the transplant protects individuals with MSUD from serious complications of the disease. The research was published in the March 2006 issue of American Journal of Transplantation. Children's Hospital has more information about liver transplantation for MSUD.