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 patient can be transplanted into another individual without passing on the disease. MSUD is one such disease,” Dr. Mazariegos said. “Because Children’s and UPMC are the leading transplant centers in the world for patients with MSUD, we foresee the opportunity to continue performing domino transplants in appropriate situations. We have performed more than 20 liver transplants in patients with MSUD, and in each and every patient, liver function has returned to normal, metabolically curing these patients. Domino transplants are an extremely resourceful approach to overcome the scarcity of organs available for transplant.”
Only 109 domino transplants have been performed in the United States, all involving heart or liver transplants, according to the United Network for Organ Sharing.
“Our novel immunosuppression therapies have enabled our live donor liver transplant recipients to recover more quickly without dependency on heavy anti-rejection medications,” commented Amadeo Marcos, M.D., professor of surgery, chief, division of transplantation and clinical director of the Starzl Transplantation Institute. “Furthermore, through our exceptional partnership with surgeons at Children’s Hospital, we have been able to expand the donor pool to adult recipients who can benefit by receiving an MSUD liver, since the disease is not transferred to the recipient.
Children’s has been the nation’s leading pediatric transplant center since becoming the first in 1981 under the leadership of transplant pioneer Thomas E. Starzl, MD. Children’s has performed more pediatric transplants than any other center and is achieving survival rates that are among the highest in the world. For instance, Children’s one-year survival rate for liver transplant recipients is 98 percent, compared with a national average of 90 percent.
In 2004, Children’s became the first transplant center to develop a specific, comprehensive protocol to develop liver transplantation as a potential cure for MSUD. Since then, it has performed more than 20 MSUD-related liver transplants, more than any other center.
The transplant programs at the University of Pittsburgh Medical Center’s Thomas E. Starzl Transplantation Institute comprise the world’s largest and busiest where surgeons perform more types of organ transplants than at any other institution. The mission of the Starzl Transplantation Institute is to foster a multidisciplinary approach to the continual improvement of the clinical, scientific and social aspects of transplantation to improve the lives of patients with end-stage organ failure. The Starzl Institute places a strong emphasis on the advancement of basic science and clinically applied research, as well as the teaching and training of multinational specialists.
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