Identification of sleep hypoventilation in young individuals with Becker muscular dystrophy: A pilot study

Autor: Yuko Nakamura, Chika Hosoda, Norika Kubota, Yoshiaki Saito, Takako Ohno, Yoko Nishimura, Yasuko Tokita, Akiko Tamasaki-Kondo, Wataru Matsumura, Yoshihide Sunada, Yoshihiro Maegaki, Masafumi Matsuo, Masuyuki Fukada
Rok vydání: 2017
Předmět:
musculoskeletal diseases
Adult
Male
medicine.medical_specialty
Vital capacity
Adolescent
Vital Capacity
Pilot Projects
Ventricular Function
Left
Pulmonary function testing
Dystrophin
03 medical and health sciences
FEV1/FVC ratio
Young Adult
0302 clinical medicine
Sleep Apnea Syndromes
Developmental Neuroscience
030225 pediatrics
Internal medicine
medicine
Humans
Respiratory function
Muscular dystrophy
Creatine Kinase
Retrospective Studies
Ejection fraction
business.industry
Retrospective cohort study
General Medicine
Hypoventilation
respiratory system
Carbon Dioxide
medicine.disease
respiratory tract diseases
Muscular Dystrophy
Duchenne
Pediatrics
Perinatology and Child Health
Cardiology
Neurology (clinical)
medicine.symptom
business
Sleep
Hypercapnia
030217 neurology & neurosurgery
circulatory and respiratory physiology
Follow-Up Studies
Zdroj: Braindevelopment. 40(7)
ISSN: 1872-7131
Popis: Aim To report on sleep hypercapnia in Becker muscular dystrophy (BMD) at earlier stages than ever recognized. Subjects and methods This 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. Results In 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. Conclusion Sleep 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.
Databáze: OpenAIRE
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