Only transplant center that developed comprehensive protocol for liver transplants in kids with MSUD

One year ago, Children's Hospital of Pittsburgh became the first and only transplant center in the world to develop a comprehensive, multidisciplinary medical protocol for performing liver transplants in patients with Maple Syrup Urine Disease (MSUD). Today, seven children who suffered from the debilitating and life-threatening disease have been transplanted and are now free from symptoms.

No other center in the country has experienced as many successful transplants as Children's Hospital of Pittsburgh for MSUD. MSUD is a metabolic disorder that causes amino acids from proteins to accumulate in the body, which can have a toxic effect and lead to brain swelling, neurological damage and death. MSUD derives its name from the sweet smell of the urine.

For the first time in these young patients' lives, they are able to go on vacation without fear of metabolic crisis and to eat foods, like ice cream and chicken nuggets, that normal children enjoy. All of the seven patients with MSUD who have been transplanted at Children's are showing excellent results.

Jakob Jasin, now 5, son of Chris and Susan Jasin of Fairfax, Va., became the first patient with MSUD to be transplanted at Children's on May 30, 2004. Jakob, who was 4 when transplanted and named his new liver "Tommy," is now free from the symptoms of the disease. Jakob was discharged from Children's 11 days after his transplant -- approximately six months after Children's established a comprehensive protocol that provides guidelines and safety nets for MSUD transplantation.

"Without this transplant, Jakob would have followed a strict diet for the rest of his life, and even then, he was still at risk for going into metabolic crisis and suffering irreparable brain damage," said George V. Mazariegos, MD, director of Pediatric Transplantation at Children's and associate professor at the University of Pittsburgh School of Medicine in the departments of Surgery, Anesthesiology and Critical Care

Contact: Melanie Finnigan
Children's Hospital of Pittsburgh

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