Disease Recurrence After Liver Transplantation by Paul J. Thuluvath

Author:Paul J. Thuluvath
Language: eng
Format: epub
Publisher: Springer New York, New York, NY

9.3.1 Wilson Disease

Wilson’s disease is an inherited autosomal-recessive disorder of copper metabolism. The disease is characterized by excessive deposition of copper in the body with predominant involvement of the liver, brain, kidneys, and corneas. The disease mostly presents itself with neurological manifestations in adolescents and young adults with signs of progressive hepatic dysfunction. Remarkable improvements have been achieved with medical therapy with chelating agents in patients with resolution of symptoms, even in more advanced cases of the disease. In situations where medical therapy has failed or in new cases presenting with acute liver failure, liver transplantation has proven to be the accepted treatment. Improvement of neurological manifestations of the disease in the form of dementia, psychosis, extrapyramidal or cerebellar signs after liver transplantation has been a matter of controversy in reported series. In a report from the University of Pittsburgh on 45 patients with an age range of 8–52 years, 30 patients were transplanted for acute or subacute liver failure and the remaining 15 for chronic liver disease. One-, 5-, and 10-year patient survival was 73.3, 73.3, and 68.9 %. Of these patients, 17 had neurological manifestations at the time of transplant. Twelve of these patients survived showing improvement in nine patients [25]. In another multicenter study from France on 121 patients (adults and pediatrics), 89 % of patients survived at 1 year and 87 % at 5, 10, 15, and 20 years. Out of 19 patients with neurological manifestations at transplant, follow-up data were available for 11 patients. Of these 11 patients, 8 patients experienced partial or complete neurological improvement and three remained with stable condition [26].

The US transplant registry of 170 pediatric patients and 400 adults with Wilson’s disease showed excellent outcomes after liver transplantation. The overall 1- and 5-year survival was 90.1 and 89 % for children and 88 and 86 % for adults [27].

Liver transplantation is a cure for patients with Wilson disease who failed medial therapy or in those with late initial presentation with complications of the disease, including acute liver failure with excellent long-term outcomes. Over 50 % improvement has been reported in those transplanted with neurological manifestation of the disease.

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