کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8681208 | 1579634 | 2018 | 4 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Successful use of non-invasive positive pressure ventilation in a patient with the severe form of X-linked myotubular myopathy
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کلمات کلیدی
EPAPIPAPNPPVXLMTMNon-invasive positive pressure ventilation - تهویه فشار مثبت غیر تهاجمیPectus excavatum - سینه قیفی شکلinspiratory positive airway pressure - فشار هوای مثبت فشاریexpiratory positive airway pressure - فشار هوای مثبت هوایی بیحسیX-linked myotubular myopathy - میوپاتی میوتوبولی مرتبط با X مرتبط استSpinal muscular atrophy type 1 - نوع آتروفی عضلانی نخاعی 1PiP - پیپ
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
علوم اعصاب تکاملی
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چکیده انگلیسی
The severity of X-linked myotubular myopathy (XLMTM) ranges from mild to severe, depending on the level of ventilatory support required. Patients with the severe form of XLMTM usually die within the first year of life due to respiratory failure. Most survivors need tracheostomies, and there has only been one report about the use of non-invasive positive pressure ventilation (NPPV) in patients with the severe form of XLMTM because of the severity of the associated respiratory failure. We successfully applied NPPV with high-span positive inspiratory pressure (PIP) in a patient with the severe form of XLMTM, who also had secondary pectus excavatum. About a year after the initiation of NPPV with high-span PIP, the patient's pectus excavatum had improved. As the patient's pectus excavatum improved, his respiratory disturbance was ameliorated, and the frequency of respiratory infections gradually decreased. NPPV might be the first-choice respiratory management strategy for patients with XLMTM.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Brain and Development - Volume 40, Issue 5, May 2018, Pages 421-424
Journal: Brain and Development - Volume 40, Issue 5, May 2018, Pages 421-424
نویسندگان
Kenji Inoue, Tomohiro Kumada, Ikuko Hiejima, Tatsuya Fujii,