title: Body composition, muscle strength, functional capacity, and physical disability risk in liver transplanted familial amyloidotic polyneuropathy patients.
abstract: BACKGROUND
  Familial amyloidotic polyneuropathy (FAP) is a neurodegenerative disease leading to sensory and motor polyneuropathies, and functional limitations. Liver transplantation is the only treatment for FAP, requiring medication that negatively affects bone and muscle metabolism. The aim of this study was to compare body composition, levels of specific strength, level of physical disability risk, and functional capacity of transplanted FAP patients (FAPTx) with a group of healthy individuals (CON).
  
  
  METHODS
  A group of patients with 48 FAPTx (28 men, 20 women) was compared with 24 CON individuals (14 men, 10 women). Body composition was assessed by dual-energy X-ray absorptiometry, and total skeletal muscle mass (TBSMM) and skeletal muscle index (SMI) were calculated. Handgrip strength was measured for both hands as was isometric strength of quadriceps. Muscle quality (MQ) was ascertained by the ratio of strength to muscle mass. Functional capacity was assessed by the six-minute walk test.
  
  
  RESULTS
  Patients with FAPTx had significantly lower functional capacity, weight, body mass index, total fat mass, TBSMM, SMI, lean mass, muscle strength, MQ, and bone mineral density.
  
  
  CONCLUSIONS
  Patients with FAPTx appear to be at particularly high risk of functional disability, suggesting an important role for an early and appropriately designed rehabilitation program.
authors.affiliation: Exercise and Health Laboratory, Faculty of Human Kinetics, Technical University of Lisbon, Portugal. mttomas@fmh.utl.pt
authors.forename: Maria T
authors.initials: MT
authors.lastname: Tomás
authors.forename: Maria H
authors.initials: MH
authors.lastname: Santa-Clara
authors.forename: Estela
authors.initials: E
authors.lastname: Monteiro
authors.forename: Tracy
authors.initials: T
authors.lastname: Baynard
authors.forename: Elvis Á
authors.initials: EÁ
authors.lastname: Carnero
authors.forename: Paula M
authors.initials: PM
authors.lastname: Bruno
authors.forename: Eduardo
authors.initials: E
authors.lastname: Barroso
authors.forename: Luís B
authors.initials: LB
authors.lastname: Sardinha
authors.forename: Bo
authors.initials: B
authors.lastname: Fernhall
mesh_terms: D015502:Absorptiometry, Photon; D000203:Activities of Daily Living; D000328:Adult; D028227:Amyloid Neuropathies, Familial / Q000628:therapy*; D001823:Body Composition; D016022:Case-Control Studies; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D004185:Disability Evaluation; D005260:Female; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D053580:Muscle Strength / Q000502:physiology*; D018482:Muscle, Skeletal / Q000503:physiopathology*; D011379:Prognosis; D012307:Risk Factors; D055815:Young Adult