title: Liver transplantation for familial amyloidotic polyneuropathy (FAP): a single-center experience over 16 years.
abstract: 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.
authors.affiliation: Division of Transplantation Surgery, Department of Clinical Science, Intervention and Technology, Karolinska Institute, Karolinska University Hospital, Huddinge, Stockholm, Sweden. Shinji.Yamamoto@ki.se
authors.forename: S
authors.initials: S
authors.lastname: Yamamoto
authors.forename: H E
authors.initials: HE
authors.lastname: Wilczek
authors.forename: G
authors.initials: G
authors.lastname: Nowak
authors.forename: M
authors.initials: M
authors.lastname: Larsson
authors.forename: A
authors.initials: A
authors.lastname: Oksanen
authors.forename: T
authors.initials: T
authors.lastname: Iwata
authors.forename: H
authors.initials: H
authors.lastname: Gjertsen
authors.forename: G
authors.initials: G
authors.lastname: Söderdahl
authors.forename: L
authors.initials: L
authors.lastname: Wikström
authors.forename: Y
authors.initials: Y
authors.lastname: Ando
authors.forename: O B
authors.initials: OB
authors.lastname: Suhr
authors.forename: B-G
authors.initials: BG
authors.lastname: Ericzon
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000235:genetics / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival; D006801:Humans; D016031:Liver Transplantation / Q000401:mortality / Q000502:physiology*; D008297:Male; D008875:Middle Aged; D009752:Nutritional Status; D012189:Retrospective Studies; D016019:Survival Analysis