Quality of life in patients with familial amyloidotic polyneuropathy long-term after liver transplantation

Autor: Elizabeth B. Haagsma, Gerda Drent, Bouke P. C. Hazenberg, Cornelia W. Graveland
Přispěvatelé: Translational Immunology Groningen (TRIGR)
Jazyk: angličtina
Rok vydání: 2009
Předmět:
Zdroj: Amyloid: Journal of protein folding disorders, 16(3), 133-141. Taylor & Francis Ltd
ISSN: 1350-6129
Popis: Liver transplantation aims to halt the progression of the disease in patients with familial amyloidotic polyneuropathy (FAP) caused by hereditary transthyretin-related (ATTR) amyloidosis. Insight in health-related quality of life of these transplanted FAP-patients can be of help to optimize health care delivery. The aim of this cross-sectional study was to assess the health-related quality of life of patients with FAP long-term after transplantation.Nine patients with a post-transplant follow-up of 4 years or more were included in the study. During the annual checks, health-related quality of life was measured with the Short Form-36 (SF-36). Data were compared with non-FAP transplanted patients with the same duration of follow-up and with the normal Dutch population. Pre-transplant, all patients had signs of mild to moderate peripheral polyneuropathy.The results showed that in patients with FAP health-related quality of life was stable in the first 4 years after transplantation. The domain of physical well-being at 4 years after transplantation was significantly lower compared to non-FAP transplanted patients and control Dutch population. The domain of emotional well-being was comparable with non-FAP controls. However, on most health areas patients with FAP scored lower than the non-FAP transplanted patients and the Dutch controls. After four years, the three patients with FAP with longest follow-up (9-12 years) deteriorated in all health domains, except in self-perceived mental health.This study, including only a small number of patients with FAP, shows a relatively low health-related quality of life after liver transplantation, which may deteriorate further with longer follow-up.
Databáze: OpenAIRE
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