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Liver transplantation for familial amyloidotic polyneuropathy (FAP): a single-center experience over 16 years
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Surgical Sciences, Transplantation Surgery.
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2007 (English)In: American Journal of Transplantation, ISSN 1600-6135, E-ISSN 1600-6143, Vol. 7, no 11, 2597-2604 p.Article in journal (Refereed) Published
Abstract [en]

Orthotopic liver transplantation (LTx) is currently the only available treatment that has been proven to halt the progress of familial amyloidotic polyneuropathy (FAP). The aim of this study was to assess mortality and symptomatic response to LTx for FAP. All 86 FAP patients transplanted at our hospital between April 1990 and November 2005 were included in the study. Five patients underwent retransplantation. The 1-, 3- and 5-year patient survival rates in patients transplanted during 1996-2005 were 94.6%, 92.3% and 92.3%, respectively, a significant difference from the rates of 76.7%, 66.7% and 66.7%, respectively, during 1990-1995 (p = 0.0003). Multivariate analysis revealed that the age at the time of LTx (>or=40 years), duration of the disease (>or=7 years) and modified body mass index (mBMI) (<600) were independent prognostic factors for patient survival. A halt in the progress of symptoms was noted in most patients, but only a minority experienced an improvement after LTx. To optimize the posttransplant prognosis, LTx should be performed in the early stages of the disease, and close post-LTx monitoring of heart function by echocardiography and of heart arrhythmia by Holter ECG is mandatory.

Place, publisher, year, edition, pages
2007. Vol. 7, no 11, 2597-2604 p.
National Category
Medical and Health Sciences
URN: urn:nbn:se:uu:diva-143627DOI: 10.1111/j.1600-6143.2007.01969.xPubMedID: 17868062OAI: oai:DiVA.org:uu-143627DiVA: diva2:390778
Available from: 2011-01-24 Created: 2011-01-24 Last updated: 2011-11-29Bibliographically approved

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