青少年肌肉萎缩症的Duchenne和Becker:当前观点。

IF 1.7 Q2 PEDIATRICS Adolescent Health Medicine and Therapeutics Pub Date : 2018-03-15 eCollection Date: 2018-01-01 DOI:10.2147/AHMT.S125739
Jennifer G Andrews, Richard A Wahl
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引用次数: 39

摘要

杜氏肌营养不良症(DMD)和贝克尔肌营养不良症(BMD)是一种限制生命的进行性神经肌肉疾病,具有显著的合并症,其中许多在青春期表现出来。与DMD相比,BMD是一种较轻的病症表现,患病率也低得多,因此在文献中较少出现,或者BMD患者的影响更严重,可能被纳入DMD人群。关于DMD的临床管理已经发表了许多共识文件,其中最新的是在2010年发布的。这些临床管理共识文件的出现,特别是呼吸护理,大大增加了这些人的寿命,青少年时期现在是一个过渡到成年生活的点,而不是生命的终点。本文综述了青少年期DMD和BMD的相关文献,重点介绍了青少年期的临床表现、慢性疾病对青少年的影响以及青少年对其慢性疾病的影响。此外,我们描述的角色,姑息治疗专家可以在改善这些人的结果。患有DMD和BMD的人活到成年的比例越来越大,这表明需要对青春期的干预措施和困难进行更多的研究,以改善他们生活的社会方面。
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Duchenne and Becker muscular dystrophy in adolescents: current perspectives.

Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are life-limiting and progressive neuromuscular conditions with significant comorbidities, many of which manifest during adolescence. BMD is a milder presentation of the condition and much less prevalent than DMD, making it less represented in the literature, or more severely affected individuals with BMD may be subsumed into the DMD population using clinical cutoffs. Numerous consensus documents have been published on the clinical management of DMD, the most recent of which was released in 2010. The advent of these clinical management consensus papers, particularly respiratory care, has significantly increased the life span for these individuals, and the adolescent years are now a point of transition into adult lives, rather than a period of end of life. This review outlines the literature on DMD and BMD during adolescence, focusing on clinical presentation during adolescence, impact of living with a chronic illness on adolescents, and the effect that adolescents have on their chronic illness. In addition, we describe the role that palliative-care specialists could have in improving outcomes for these individuals. The increasing proportion of individuals with DMD and BMD living into adulthood underscores the need for more research into interventions and intracacies of adolescence that can improve the social aspects of their lives.

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来源期刊
自引率
0.00%
发文量
13
审稿时长
16 weeks
期刊介绍: Adolescent Health, Medicine and Therapeutics is an international, peer reviewed, open access journal focusing on health, pathology, and treatment issues specific to the adolescent age group, including health issues affecting young people with cancer. Original research, reports, editorials, reviews, commentaries and adolescent-focused clinical trial design are welcomed. All aspects of health maintenance, preventative measures, disease treatment interventions, studies investigating the poor outcomes for some treatments in this group of patients, and the challenges when transitioning from adolescent to adult care are addressed within the journal. Practitioners from all disciplines are invited to submit their work as well as health care researchers and patient support groups. Areas covered include: Physical and mental development in the adolescent period, Behavioral issues, Pathologies and treatment interventions specific to this age group, Prevalence and incidence studies, Diet and nutrition, Specific drug handling, efficacy, and safety issues, Drug development programs, Outcome studies, patient satisfaction, compliance, and adherence, Patient and health education programs and studies.
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