Transplant surgeons at Children's Hospital of Pittsburgh and UPMC have saved two patients with life-threatening liver conditions utilizing a technique known as a domino transplant. It is only the nation's second domino transplant involving a patient with maple syrup urine disease (MSUD).
Domino transplants are so named for the sequential nature of the transplants an organ from a deceased donor is transplanted into the first recipient. The first recipient's organ then is transplanted into a second recipient. Domino transplants are a rare but effective way of overcoming the shortage of organs available for transplant.
In this case, a liver from a cadaveric donor was transplanted into Nickolai Rudd, an adult patient at Children's with MSUD, a rare and potentially life-threatening genetic disease. Mr. Rudd's liver was transplanted into James Paulshock, an adult suffering from liver failure caused by primary sclerosing cholangitis. The MSUD that afflicted Mr. Rudd was not passed onto Mr. Paulshock through his donated liver, while Mr. Rudd's new liver metabolically cured his MSUD. Because MSUD does not originate in the liver, and is caused by a lack of enzymes, the second patient will not inherit the disease. Both patients' new livers are now functioning normally.
Mr. Rudd's transplant was led by George V. Mazariegos, MD, director of Pediatric Transplantation at Children's Hillman Center for Pediatric Transplantation and an associate professor of surgery at the University of Pittsburgh School of Medicine. Mr. Paulshock's transplant surgery was led by Amadeo Marcos, MD, clinical director of transplantation at the Thomas E. Starzl Transplantation Institute and a professor of surgery at the University of Pittsburgh School of Medicine. The transplants were performed May 30, 2006, and both patients have since been discharged.
"Domino transplants are rare because there are a very limited number of diseases in which the organ of a sick pa
Contact: Marc Lukasiak
Children's Hospital of Pittsburgh