کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
5719916 1607678 2017 8 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
ReviewRespiratory Complications in Children with Prader Willi Syndrome
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی پریناتولوژی (پزشکی مادر و جنین)، طب اطفال و بهداشت کودک
پیش نمایش صفحه اول مقاله
ReviewRespiratory Complications in Children with Prader Willi Syndrome
چکیده انگلیسی

SummaryPrader Willi syndrome, resulting from the partial deletion or lack of expression of a region of genes on the paternal chromosome 15, has a number of phenotypic features which predispose affected patients to ventilatory problems. These include generalised hypotonia, abnormal arousal and ventilatory responses to hypoxia and hypercapnia, scoliosis and frequently, obesity. The spectrum of the resulting respiratory complications thus runs from sleep disordered breathing, to aspiration and respiratory functional impairment. While the use of growth hormone, in conjunction with multidisciplinary clinical management, is currently the cornerstone of clinical care of these patients, concerns have been raised following reports of sudden death shortly after growth hormone initiation. This review summarizes the respiratory complications commonly seen and draws together the published literature on the impact of growth hormone in relation to various respiratory parameters, aiming to provide the reader with the necessary information to manage these patients as safely as possible.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Paediatric Respiratory Reviews - Volume 22, March 2017, Pages 52-59
نویسندگان
, ,