title: Ventilatory support in facioscapulohumeral muscular dystrophy.
abstract: Respiratory insufficiency due to respiratory muscle weakness is a common complication of many neuromuscular diseases. The prevalence of respiratory failure in facioscapulohumeral muscular dystrophy (FSHD) is unknown. The authors identified 10 FSHD patients on nocturnal ventilatory support at home, representing approximately 1% of the Dutch FSHD population. Severe muscle disease, wheelchair dependency, and kyphoscoliosis appeared to be risk factors for respiratory failure.
authors.affiliation: Department of Neurology, University Medical Center Nijmegen, Reinier Postlaan 4, 6500 HB Nijmegen, The Netherlands. m.wohlgemuth@neuro.umcn.nl
authors.forename: M
authors.initials: M
authors.lastname: Wohlgemuth
authors.forename: E L
authors.initials: EL
authors.lastname: van der Kooi
authors.forename: R G
authors.initials: RG
authors.lastname: van Kesteren
authors.forename: S M
authors.initials: SM
authors.lastname: van der Maarel
authors.forename: G W
authors.initials: GW
authors.lastname: Padberg
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D005260:Female; D005660:Funnel Chest / Q000150:complications; D006701:Home Nursing; D006801:Humans; D007738:Kyphosis / Q000150:complications; D008297:Male; D008875:Middle Aged; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000150:complications / Q000453:epidemiology / Q000628:therapy*; D009426:Netherlands / Q000453:epidemiology; D012120:Respiration Disorders / Q000453:epidemiology / Q000209:etiology / Q000628:therapy*; D012121:Respiration, Artificial; D012132:Respiratory Muscles / Q000503:physiopathology; D012307:Risk Factors; D012600:Scoliosis / Q000150:complications; D014910:Wheelchairs