Tag Archives: liver transplant

Lysosomal Acid Lipase Dificiency (LAL-D) in adults: Epidemiologic Multi-Center Study Underway

A multi-center, investigator-initiated epidemiologic study is underway [clinical trial: NCT01633489] aiming to determine the proportion of adults affected by late-onset lysosomal acid lipase deficiency (LAL-D) using definitive genetic testing.

LAL-D is an autosomal recessive disease caused by mutations in the Iipase gene that lead to decreased or absent enzyme activity. Lack of such activity causes lysosomal accumulation of cholesteryl ester in various organs, including the liver, spleen and adrenals, which leads to morbidity and mortality.1 Transplant hepatologist Karen L. Krok, M.D., Penn State Hershey Gastroenterology and Hepatology, explains, “At Penn State Hershey and other study centers in Pennsylvania, adults with cryptogenic cirrhosis or nonalcoholic steatohepatitis [NASH] who are awaiting liver transplant will have the opportunity to be tested for LAL-D. The signs and symptoms of LAL-D are similar to other common conditions; however, 10 to 15 percent of patients have no comorbid condition like hepatitis, obesity, diabetes or alcohol use to explain the disease. (Learn more about clinical features of LAL-D in adults.) Continue reading

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Keys to a Successful Live Donor Adult Liver Transplant Program

Transplant patient and donor at press conference

Surgeons at Penn State Hershey Medical Center, the only Medicare-approved center for liver transplantation in central Pennsylvania, performed the region’s first adult living donor liver transplant on Jestine Reider and John Kreider, brother and sister from Elizabethtown, Pa. in July 2008.

One of the greatest challenges facing patients who require a liver transplant is surviving the wait for a donor organ. Each year, nearly 16,000 patients in the United States are on the liver transplant waiting list, according to UNOS; yet only between 5,000 and 6,000 receive a transplant from a deceased donor.1 Zakiyah Kadry, M.D., said “Although liver transplant patients are stratified based on MELD scores, some die while waiting. To decrease wait times and associated mortality, some patients can receive grafts from live donors.”

In 2011, only 247 live donor liver transplants were performed in the United States, according to HRSA/ OPTN statistics.2 “Live donor programs must be UNOS-certified and require at least two surgeons trained in hepato-biliary surgery, as well as transplantation,” notes Kadry. Continue reading

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