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Sunday, February 10, 2008

Domino Transplant Allows A Child With Rare Liver Disease To Receive New Organ

Liver Disease

Transplant surgeons have performed their first-ever domino liver transplant involving a pediatric recipient.

Domino transplants allow surgeons to perform two life-saving transplants from one deceased organ donor.

In Children's first-ever pediatric case, performed late last month, a liver from a deceased donor was transplanted into a 9-year-old with maple syrup urine disease (MSUD). His liver was then transplanted into a 24-year-old patient from Saudi Arabia without passing on the disease. The transplants were led by surgeon George V. Mazariegos, MD, director of Pediatric Transplantation at Children's Hillman Center for Pediatric Transplantation.

Domino transplants are so named for the sequential nature of the transplants รข€” an organ from a deceased donor is transplanted into the first recipient. The first recipient's organ is then transplanted into a second recipient. Last year, Children's surgeons were involved in a domino transplant involving two adult recipients.

This is only the third domino transplant ever involving a patient with MSUD and only 67 domino liver transplants have been performed in the United States, according to the United Network for Organ Sharing. "Domino transplants are rare because there are very few conditions for which you can cure one patient with a transplant and then transplant his or her organ into someone else without passing on the disease. MSUD is one such disease," Dr. Mazariegos said. "Children's Hospital is uniquely poised to begin offering domino transplants as an option to more and more families."

In 2004, Children's developed the nation's first comprehensive protocol for performing transplants in MSUD patients, and has performed more than 26 since then, more than any other center in the world. All of Children's MSUD patients who have received liver transplants since its first in 2004 have been metabolically cured, with liver function returning to normal, according to Dr. Mazariegos.

On Saturday, June 23, and Sunday, June 24, 2007, Children's team transplanted a liver from a deceased donor into Johnathan Devantier, 9, of St. Louis. After nearly dying as a newborn, Johnathan was diagnosed with MSUD at 7 days old. Since then, he has suffered numerous metabolic crises, and something as simple as inadvertently eating protein or catching a common cold could cause him to have another potentially fatal crisis.

Because MSUD does not originate in the liver and is caused by a lack of enzymes throughout the body, a patient with MSUD who receives a liver transplant is able to make enough of the enzymes in the liver to overcome the shortage elsewhere in the body. In turn, an MSUD patient's liver can be transplanted into someone else without passing on the disease because that patient's cells still makes the enzyme lacking in the liver.

Johnathan's liver was transplanted into Ali Al-Garni, a young man suffering from familial cholestasis, a genetic disease that can cause liver failure. Both transplants were successful, and Johnathan and Ali are recovering.

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