Identification of sleep hypoventilation in young individuals with Becker muscular dystrophy: A pilot study |
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Authors: | Yuko Nakamura Yoshiaki Saito Norika Kubota Wataru Matsumura Chika Hosoda Akiko Tamasaki-Kondo Yoko Nishimura Yoshihide Sunada Masuyuki Fukada Takako Ohno Yoshihiro Maegaki Masafumi Matsuo Yasuko Tokita |
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Affiliation: | 1. Department of Pediatrics, Matsue Medical Center, 5-8-31 Agenogi, Matsue 690-8556, Japan;2. Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, 36-1 Nishi-cho, Yonago 683-8504, Japan;3. Department of Pediatrics, Tottori Rehabilitation Center for Children with Disabilities, 7-13-3 Kamifukubara, Yonago 683-0004, Japan;4. Department of Pediatrics, Shimane Prefectural Central Hospital, 4-1-1 Himebara, Izumo 693-8555, Japan;5. Department of Neurology, Kawasaki Medical School, 577 Matsushima, Kurashiki 701-0192, Japan;6. Fukada Clinic, 53-1 Takaoka-cho, Izumo 693-0066, Japan;g. Western Shimane Medical and Welfare Center for the Disabled, 1926 Watazu, Gotsu 695-0001, Japan;h. Department of Medical Rehabilitation, Faculty of Rehabilitation, Kobe Gakuin University, 518 Arise, Ikawadani-cho, Nishi-ku, Kobe 651-2180, Japan |
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Abstract: | AimTo report on sleep hypercapnia in Becker muscular dystrophy (BMD) at earlier stages than ever recognized.Subjects and methodsThis retrospective study examined nocturnal hypercapnia in six young Becker muscular dystrophy (BMD) patients with deletions of one or more exons of DMD gene. Clinical information, consecutive data on forced vital capacity (FVC%), forced expiratory volume in one second (FEV1%), peak expiratory flow (PEF%), peak cough flow (PCF), average PCO2 in all-night monitoring, and left ventricular ejection fraction (LVEF) were reviewed.ResultsIn five BMD patients, including three who were still ambulant, nocturnal average PCO2 was elevated to >45?mmHg at 12–31?years of age. Noninvasive positive pressure ventilation was initiated in four patients. Gradual declines in FVC% and PEF% were evident in one BMD patient with exon 3–7 deletion, whereas these functions did not change in the remaining BMD patients. PCF, FEV1%, and LVEF were less informative for the assessment of respiratory function in this patient series.ConclusionSleep hypercapnia was present in certain BMD patients, which was unexpected from the routine pulmonary function tests. Individualized assessment of nocturnal PCO2, partly based on the deletion types, should be further explored in the clinical practice of BMD patients. |
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Keywords: | Becker muscular dystrophy Noninvasive positive pressure ventilation Respiratory function Sleep disordered breathing Hypercapnia |
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