کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
6213309 1605985 2016 6 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Tracheobronchial stents in mucopolysaccharidosis
ترجمه فارسی عنوان
استنت های تراکئو برونشیل در موکوپلیساکاریدوز
کلمات کلیدی
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی بیماری های گوش و جراحی پلاستیک صورت
چکیده انگلیسی

IntroductionThe mucopolysaccharidoses are a group of hereditary disorders pathologically characterized by tissue accumulation of glycosaminoglycans due to deficient lysosomal metabolism which often leads to progressive airway stenosis and respiratory insufficiency. Relentless and treatment-refractory narrowing of the lower airways with ensuing severe limitation of quality of life is a challenging problem in mucopolysaccharidoses.Case reportsWe report 2 cases of MPS (Hunter's and Maroteaux-Lamy's syndrome resp.) in whom tracheal stents were placed to relieve severe tracheal obstruction. The first patient could be weaned from mechanical ventilation after stent placement but showed significant long-term stent-related morbidity. The second patient suffered a severe procedure-related complication due to positioning problems typical for MPS.ConclusionsVery good short-term success can be achieved with airway stent placement in patients with MPS and severe lower airway stenosis but a high risk of severe complications and important long-term morbidity have to be weighed against potential individual benefit.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: International Journal of Pediatric Otorhinolaryngology - Volume 83, April 2016, Pages 187-192
نویسندگان
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