儿童和成人Prader-Labhart-Willi综合征的管理,特别强调重组人生长激素的治疗。

Marta Drabik, Andrzej Lewiński, Renata Stawerska
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引用次数: 3

摘要

简介:普瑞德-威利综合征(PWS)是一种基因决定的疾病,表现为多种异常,其中包括下丘脑-垂体系统功能障碍。只有综合的、多学科的护理才能使患者有机会显著提高生活质量,并实现与一般人群没有差异的预期寿命。目的:本研究的目的是总结有关PWS患者管理的现有文献。结论:世界各地越来越多的基于临床试验的报告表明,rhGH治疗在儿童期和成长期结束后的PWS患者中具有不可否认的益处。它们包括改善身体成分,改善血脂,增加骨矿物质密度,改善精神状态和患者的生活质量。
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Management of Prader-Labhart-Willi syndrome in children and in adults, with particular emphasis on the treatment with recombinant human growth hormone.

Introduction: Prader-Willi syndrome (PWS) is a genetically determined disease that manifests itself in a number of abnormalities resulting, among others, from dysfunction of the hypothalamic-pituitary system. Only integrated, multidisciplinary care gives patients the chance to significantly improve the quality of life and achieve a life expectancy that does not differ from the general population.

Aim: The aim of the study was to summarize the available literature on the management of patients suffering from PWS.

Conclusions: More and more reports based on clinical trials conducted around the world indicate the undeniable benefits of rhGH therapy in patients with PWS in childhood and after the end of growth period. They consist in improving the body composition, improving the lipid profile, increasing bone mineral density and improving the mental state and patients' quality of life.

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来源期刊
Pediatric Endocrinology, Diabetes and Metabolism
Pediatric Endocrinology, Diabetes and Metabolism Medicine-Pediatrics, Perinatology and Child Health
CiteScore
2.00
自引率
0.00%
发文量
36
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