title: Quality of life in patients with familial amyloidotic polyneuropathy long-term after liver transplantation.
abstract: 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.
authors.affiliation: Department of Gastroenterology and Hepatology, University Medical Center Groningen, University of Groningen, The Netherlands. g.drent@int.umcg.nl <g.drent@int.umcg.nl>
authors.forename: Gerda
authors.initials: G
authors.lastname: Drent
authors.forename: Cornelia W
authors.initials: CW
authors.lastname: Graveland
authors.forename: Bouke P C
authors.initials: BP
authors.lastname: Hazenberg
authors.forename: Elizabeth B
authors.initials: EB
authors.lastname: Haagsma
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000601:surgery*; D018450:Disease Progression; D005260:Female; D006304:Health Status; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D011115:Polyneuropathies / Q000601:surgery*; D011788:Quality of Life; D011795:Surveys and Questionnaires; D016896:Treatment Outcome; D055815:Young Adult