Impairment of respiratory muscle strength in Berardinelli-Seip congenital lipodystrophy subjects

Autor: Jorge Luiz Dantas de Medeiros, Bruno Carneiro Bezerra, Thiago Anderson Brito de Araújo, Aquiles Sales Craveiro Sarmento, Lázaro Batista de Azevedo Medeiros, Lucien Peroni Gualdi, Maria do Socorro Luna Cruz, Thaiza Teixeira Xavier Nobre, Josivan Gomes Lima, Julliane Tamara Araújo de Melo Campos
Jazyk: angličtina
Rok vydání: 2018
Předmět:
Zdroj: Respiratory Research, Vol 19, Iss 1, Pp 1-11 (2018)
Druh dokumentu: article
ISSN: 1465-993X
DOI: 10.1186/s12931-018-0879-8
Popis: Abstract Background Berardinelli-Seip Congenital Generalized Lipodystrophy (BSCL) is an ultra-rare metabolic disease characterized by hypertriglyceridemia, hyperinsulinemia, hyperglycemia, hypoleptinemia, and diabetes mellitus. Although cardiovascular disturbances have been observed in BSCL patients, there are no studies regarding the Respiratory Muscle Strength (RMS) in this type of lipodystrophy. This study aimed to evaluate RMS in BSCL subjects compared with healthy subjects. Methods Eleven individuals with BSCL and 11 healthy subjects matched for age and gender were included in this study. The Maximum Inspiratory Pressure (MIP), Maximum Expiratory Pressure (MEP), and Peripheral Muscle Strength (PMS) were measured for three consecutive years. BSCL subjects were compared to healthy individuals for MIP, MEP, and PMS. Correlations between PMS and MIP were also analyzed. The genetic diagnosis was performed, and sociodemographic and anthropometric data were also collected. Results BSCL subjects showed significantly lower values for MIP and MEP (p
Databáze: Directory of Open Access Journals
Externí odkaz:
Nepřihlášeným uživatelům se plný text nezobrazuje