title: Sleep-disordered breathing in Duchenne muscular dystrophy: a preliminary study of the role of portable monitoring.
abstract: Although Duchenne muscular dystrophy (DMD) is often associated with sleep disordered breathing (SDB), it is not standard clinical practice to routinely test this population for SDB, and the optimal timing and methodology for such testing has not been established. Our objectives were: 1) to examine the concordance between laboratory polysomnography (PSG) and two portable monitoring systems, and 2) to identify clinical factors associated with the onset of SDB. We performed a cross-sectional pilot study of patients with DMD who were 6 years of age or older, and who were registered at the Alberta Children's and Calgary General Hospitals. Patient symptom and functional rating scores were calculated, and pulmonary function tests, awake oxygen saturation, and capillary blood gases were obtained. PSG was performed according to standard methods, and results were compared with Snoresat(R) (Saga Tech Electronics, Inc.) and EdenTec(R) (Nellcor Puritan Bennett) portable home monitors. Eleven boys were studied. Ten of 11 subjects had normal awake oxygen saturation and capillary blood gases. Median forced vital capacity (FVC) was 70% of predicted values (15-104%). PSG identified 3 boys with severe hypoventilation occurring throughout REM sleep. Reported symptom severity did not predict the patients with significant SDB. All 3 boys with SDB had a severe functional disability and severely reduced FVCs. Portable monitoring in the home identified all patients with abnormal PSG. One additional patient was falsely identified by the EdenTec(R) monitor. We conclude that initial results using Snoresat(R) or EdenTec(R) monitoring equipment for the identification of SDB are promising, but further validation of portable home monitoring is required in this group of patients.
authors.affiliation: Division of Respirology, Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada. valkirk@crha-health.ab.ca
authors.forename: V G
authors.initials: VG
authors.lastname: Kirk
authors.forename: W W
authors.initials: WW
authors.lastname: Flemons
authors.forename: C
authors.initials: C
authors.lastname: Adams
authors.forename: K P
authors.initials: KP
authors.lastname: Rimmer
authors.forename: M D
authors.initials: MD
authors.lastname: Montgomery
mesh_terms: D000293:Adolescent; D000328:Adult; D002245:Carbon Dioxide / Q000097:blood; D002648:Child; D003430:Cross-Sectional Studies; D018575:Home Care Services, Hospital-Based; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000097:blood / Q000150:complications* / Q000503:physiopathology; D010100:Oxygen / Q000097:blood; D010865:Pilot Projects; D017286:Polysomnography; D012891:Sleep Apnea Syndromes / Q000175:diagnosis* / Q000209:etiology; D014797:Vital Capacity
chemical_list: D002245:Carbon Dioxide; D010100:Oxygen