PSU29 French Survey on the Management of Anemia and Iron Supplementation in Patients in Elective Orthopedic Surgery

Abstract

OBJECTIVES: Familial amyloid polyneuropathy (FAP) is an autosomal dominant disorder, caused by mutant transthyretin protein (TTR). Because most TTR is produced in hepatocytes, liver transplantation (LTx) has been a treatment option to prevent long term disease progression. This study aims to report demographic and clinical characteristics of FAP patients submitted to LTx in Brazil compared to FAP World Transplant Registry (FAPWTR). METHODS: A literature review was performed by May 2012 through Cochrane Collaboration, Medline, EMBASE, and Lilacs databases. A retrospective analysis of epidemiological data available from FAPWTR was developed from 1995 to 2010. RESULTS: From 1995 to 2010, 1,893 LTx were recorded in 73 centers of 19 countries by FAPWTR. Of these, 5% were performed in 4 Brazilian centers. In FAPWRT cohort, 56% were male, median age at time of transplantation was 38 years (21-72), and the median disease duration before LTx was 3 years (0-30). The main causes of death were cardiac complications (22%), sepsis (22%), liver related complications (14%) and perioperative complications (3%). The 5-year survival rate was 82%. In a cohort from São Paulo University-Brazil, 24 patients underwent LTx. Patients characteristics were similar to FAPWRT (66.6% male; median age 36 years), except for disease duration before transplantation (median: 8 years [2-17]). Six deaths were registered and the main causes were sepsis and hepatic artery thrombosis. Cardiac related deaths was also observed but in one case. The overall 5-year survival rate was 58%. Data from 59 Brazilian subjects enrolled in Transthyretin Amyloidosis Outcomes Survey (THAOS) indicated 81% of symptomatic patients and LTx performed in 37.5%. CONCLUSIONS: When compared to FAPWTR, Brazilian cohort showed longer disease duration before LTx, and a shorter 5-year survival rate after the procedure. This might be indicative of a need for better diagnose and management of FAP patients in Brazil.