کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
6041178 | 1189269 | 2015 | 8 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Respiratory muscle dysfunction in facioscapulohumeral muscular dystrophy
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کلمات کلیدی
ERVSDBMIPMEPaHIPaCO2PCFSpO2FSHDPFT, Pulmonary function tests - آزمونهای عملکرد ریه، تست های عملکرذ ریویsleep-disordered breathing - اختلال تنفسی در خوابperipheral capillary oxygen saturation - اشباع اکسیژن مویرگی محیطیMechanical ventilation - تهویه مکانیکیPeak cough flow - جریان سرفه بالاExpiratory reserve volume - حجم ذخیره انبساطMaximal inspiratory pressure - حداکثر فشار القاییMaximal expiratory pressure - حداکثر فشار بیحسیFacioScapuloHumeral Muscular Dystrophy - دیستروفی عضلانی FacioScapuloHumeralapnea/hypopnea index - شاخص آپنه / هیپوپنیbody mass index - شاخص توده بدنBMI - شاخص توده بدنیinspiratory capacity - ظرفیت انجمادVital capacity - ظرفیت حیاتیRespiratory failure - نارسایی تنفسی
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
علوم اعصاب تکاملی
پیش نمایش صفحه اول مقاله
![عکس صفحه اول مقاله: Respiratory muscle dysfunction in facioscapulohumeral muscular dystrophy Respiratory muscle dysfunction in facioscapulohumeral muscular dystrophy](/preview/png/6041178.png)
چکیده انگلیسی
Respiratory insufficiency in facioscapulohumeral muscular dystrophy has rarely been studied. We compared two age- and sex-matched groups of 29 patients, with and without respiratory dysfunction. Tests in the 29 patients with respiratory dysfunction suggested predominant expiratory muscle dysfunction, leading to ineffective cough in 17 patients. Supine and upright vital capacities were not different (Pâ=â0.76), suggesting absence of diaphragmatic dysfunction. By stepwise regression, only expiratory reserve volume correlated with the Walton and Gardner-Medwin score (R2â=â0.503; Pâ=â0.001). Compared to controls, patients with respiratory dysfunction had higher values for the Walton and Gardner-Medwin score (6.1â±â1.9 vs. 3.2â±â1.2; Pâ<0.0001) and body mass index (26.9â±â6.0 vs. 22.9â±â4.0 kg/m2; Pâ=â0.003) and a smaller number of D4Z4 allele repeats (4.8â±â1.6 vs. 5.7â±â1.8; Pâ=â0.05). Mechanical ventilation was required eventually in 20 patients, including 14 who were wheelchair bound. Three patients had acute respiratory failure requiring mechanical ventilation; 16 patients had poor airway clearance, including 10 with sleep apnea syndrome, responsible in 7 for chronic hypercapnia. Two patients presented isolated severe sleep apnea syndrome. Respiratory dysfunction in facioscapulohumeral muscular dystrophy is predominantly related to expiratory muscle weakness. Respiratory function and cough effectiveness should especially be monitored in patients with severe motor impairment and high body mass index.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Neuromuscular Disorders - Volume 25, Issue 8, August 2015, Pages 632-639
Journal: Neuromuscular Disorders - Volume 25, Issue 8, August 2015, Pages 632-639
نویسندگان
Dante Brasil Santos, Ghilas Boussaid, Tanya Stojkovic, David Orlikowski, Nadege Letilly, Anthony Behin, Sandrine Butel, Frédéric Lofaso, Hélène Prigent,