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. Conclusion: 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.

Versão portuguesa do Hearing Handicap Inventory for Adults: dados preliminares

Perda auditiva: elevada prevalência da perda auditiva (14,1%), efeitos na comunicação, efeitos em aspectos psicossociais, cognitivos, da fala e linguagem. Avaliação da perda auditiva: baseia-se nos testes audiométricos psicoacústicos, base audiometria tonal, que permite estimar a sensibilidade auditiva (normal vs anormal), permite classificar o tipo e grau de perda auditiva, avaliação qualitativa com a utilização de escalas desenvolvidas para esse efeito, HHIA (Hearing Handicap Inventory for Adults), instrumento desenvolvido a partir do HHIE (Hearing Handicap Inventory for the Elderly), para ser usado com pacientes de idade inferior a 65 anos.