生长激素治疗普瑞德-威利综合征。

4区 医学 Q2 Medicine
Maïthé Tauber, Gwenaelle Diene, Catherine Molinas
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引用次数: 9

摘要

重组人生长激素(rhGH)用于患有Prader-Willi综合征的儿童的欧洲上市许可是第一个针对儿童代谢和身体成分影响的适应症。在美国,它被用于与PWS相关的身材矮小。重组生长激素是针对PWS人群的第一种治疗方法,通过促进处方生长激素的医生(主要是儿科内分泌学家)的接触,并管理多学科护理的组织,从根本上改变了这些儿童的护理。重组生长激素治疗不仅在儿童而且在年轻人中改善了线性生长、身体组成和社会化。包括生长激素在内的综合激素缺乏症的病理生理学开始被揭示。我们现在必须把重点放在rhGH治疗不能改变的合并症上,如进食障碍和行为问题,以真正改变患者的生活。从青少年到年轻人的护理过渡也仍然是一个挑战。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Growth Hormone Treatment for Prader-Willi Syndrome.

The European Marketing Authorization for recombinant human growth hormone (rhGH) in children with Prader-Willi syndrome was the first indication for metabolic and body composition effects in children. In the US it is indicated for short stature associated with PWS. Recombinant hGH is the first treatment for the PWS population and radically changed the care of these children by facilitating access to physicians who prescribe rhGH, mainly paediatric endocrinologists, and manage the organization of multidisciplinary care. Recombinant hGH treatment improved linear growth, body composition, and socialization not only in children but also in young adults. The pathophysiology of combined hormonal deficiencies including GH is starting to be unravelled. We now have to focus on co-morbidities that are not modified by rhGH treatment, such as feeding disorders and behaviour problems, to truly change the life of patients. The transition of care from adolescents to young adults also remains a challenge.

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来源期刊
Pediatric endocrinology reviews : PER
Pediatric endocrinology reviews : PER Medicine-Endocrinology, Diabetes and Metabolism
自引率
0.00%
发文量
1
期刊介绍: PEDIATRIC ENDOCRINOLOGY REVIEWS (PER) publishes scholarly review articles in all areas of clinical and experimental Endocrinology, Diabetes, Nutrition and Metabolism. PER is intended for practicing pediatricians, pediatric endocrinologists, pediatric diabetologists, pediatric gastroenterologists, neonatologists, pediatric gynecologists, nutritionists, sport physicians and pediatricians-in-training. PER will also publish topics on specific subjects or as proceedings of scientific meetings in the above fields of interest. All articles, whether invited or direct contributions, are peer-reviewed. PER publishes correspondence, book reviews, a meeting calendar and meeting reports.
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