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Liver transplantation for patients with genetic liver conditions has high survival rate
Date:4/5/2013

reatment to correct genetic conditions. Second, the severity of genetic liver disease may not be as obvious. Since donated livers are allocated according to who needs them most, people with genetic liver diseases often have to be given extra consideration when weighing how long they can wait for a donor organ.

Although the genetic defect permeates every cell in the affected patient's body, it expresses mainly in the liver. However, certain disorders could also affect other organs. During a transplant, when the old liver is replaced with a new one "it's almost a form of gene therapy," explains Dr. Petrowsky, who is now vice chair of the department of visceral and transplant surgery at the University Hospital Zurich in Switzerland. "The genetic defect is still in every cell, but since it's mainly expressed in the liver, the genetic disorder is corrected by the transplanted liver which does not harbor the genetic defect."

Dr. Petrowsky's team wanted to evaluate the impact of the transplant procedure on patients' outcomes, especially because patients families go through so much before a transplantable liver can be procured. Therefore, the investigators looked at patient records from the UCLA liver transplant database for 74 children and 78 adults who had received liver transplants between 1984 and 2012 to correct genetic disorders. For 68 percent of those patients, the genetic disorder led to cirrhosis, or scaring of the liver, making liver transplantation their only hope for survival.

Five years later, 89 percent of children in the study were still alive, and 77 percent were still alive 20 years later. For adults, 73 percent were still alive after five years and 50 percent were still alive after 20 years.

"Without a transplant, the five-year survival rates are below five percent, depending on the severity of the liver disease," Dr. Petrowsky said.

Only one pediatric patient and one adult patient had a recurrence
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Contact: Sally Garneski
pressinquiry@facs.org
312-202-5409
American College of Surgeons
Source:Eurekalert

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