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杜氏肌营养不良症患者从儿童期向成年期的过渡。

Transition from Childhood to Adulthood in Patients with Duchenne Muscular Dystrophy.

机构信息

Department of Allergology and Pulmonology, Medical University of Gdańsk, 80-211 Gdańsk, Poland.

Department of Clinical Nutrition, Medical University of Gdansk, 80-211 Gdańsk, Poland.

出版信息

Medicina (Kaunas). 2020 Aug 24;56(9):426. doi: 10.3390/medicina56090426.

Abstract

Recently, progress has been observed in the knowledge about Duchenne Muscular Dystrophy (DMD), which is a severe and commonly diagnosed genetic myopathy in childhood, historically resulting in early death. Currently, there are a lot of methods available to improve the clinical course of DMD and extend patients' life expectancy to more than 30 years of age. The key issue for DMD patients is the period between 16-18 years of age, which is described as a transition from pediatric- to adult-oriented healthcare. Adolescents and adults with DMD have highly complex healthcare needs associated with long-term steroid usage, orthopedic, ventilation, cardiac, and gastrointestinal problems. The current paper provides a comprehensive overview of special healthcare needs related to the transfer of a patient with DMD from child-oriented to adult-oriented care. Additionally, the need to organize effective care for adults with DMD is presented.

摘要

最近,人们对杜氏肌营养不良症(DMD)的认识取得了进展,DMD 是一种严重且常见的儿童期遗传性肌肉疾病,历史上导致患者早亡。目前,有很多方法可以改善 DMD 的临床病程,并将患者的预期寿命延长至 30 岁以上。DMD 患者的关键问题是 16-18 岁之间的时期,这一时期被描述为从儿科到成人医疗保健的过渡。患有 DMD 的青少年和成年人有高度复杂的医疗保健需求,与长期使用类固醇、骨科、通气、心脏和胃肠道问题有关。本文全面概述了与将 DMD 患者从儿科护理转向成人护理相关的特殊医疗保健需求,并提出了为 DMD 成年人组织有效护理的必要性。

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Performance of Upper Limb module for Duchenne muscular dystrophy.上肢模块在杜氏肌营养不良症中的表现。
Dev Med Child Neurol. 2020 May;62(5):633-639. doi: 10.1111/dmcn.14361. Epub 2019 Sep 19.
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Duchenne muscular dystrophy and caregiver burden: a systematic review.杜氏肌营养不良症和照顾者负担:系统评价。
Dev Med Child Neurol. 2018 Oct;60(10):987-996. doi: 10.1111/dmcn.13934. Epub 2018 Jun 14.

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