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杜氏或贝克型肌营养不良男性患者的继发疾病

Secondary Conditions Among Males With Duchenne or Becker Muscular Dystrophy.

作者信息

Latimer Rebecca, Street Natalie, Conway Kristin Caspers, James Kathy, Cunniff Christopher, Oleszek Joyce, Fox Deborah, Ciafaloni Emma, Westfield Christina, Paramsothy Pangaja

机构信息

1 Cardiogenetic Testing Services, GeneDx, Gaithersburg, MD, USA.

2 Division of Human Development and Disability, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA, USA.

出版信息

J Child Neurol. 2017 Jun;32(7):663-670. doi: 10.1177/0883073817701368. Epub 2017 Apr 9.

Abstract

Duchenne and Becker muscular dystrophy are X-linked neuromuscular disorders characterized by progressive muscle degeneration. Despite the involvement of multiple systems, secondary conditions among affected males have not been comprehensively described. Two hundred nine caregivers of affected males (aged 3-31 years) identified by the Muscular Dystrophy Surveillance, Tracking, and Research Network completed a mailed survey that included questions about secondary conditions impacting multiple body functions. The 5 most commonly reported conditions in males with Duchenne were cognitive deficits (38.4%), constipation (31.7%), anxiety (29.3%), depression (27.4%), and obesity (19.5%). Higher frequencies of anxiety, depression, and kidney stones were found among nonambulatory males compared to ambulatory males. Attention-deficit hyperactivity disorder (ADHD) was more common in ambulatory than nonambulatory males. These data support clinical care recommendations for monitoring of patients with Duchenne or Becker muscular dystrophy by a multidisciplinary team to prevent and treat conditions that may be secondary to the diagnosis.

摘要

杜兴氏和贝克氏肌营养不良症是X连锁神经肌肉疾病,其特征为进行性肌肉退化。尽管涉及多个系统,但尚未对受影响男性的继发性疾病进行全面描述。由肌营养不良症监测、跟踪和研究网络确定的209名受影响男性(年龄在3至31岁之间)的照料者完成了一项邮寄调查,其中包括有关影响多种身体功能的继发性疾病的问题。杜兴氏男性患者中最常报告的5种疾病是认知缺陷(38.4%)、便秘(31.7%)、焦虑(29.3%)、抑郁(27.4%)和肥胖(19.5%)。与能行走的男性相比,不能行走的男性中焦虑、抑郁和肾结石的发生率更高。注意缺陷多动障碍(ADHD)在能行走的男性中比不能行走的男性更常见。这些数据支持多学科团队对杜兴氏或贝克氏肌营养不良症患者进行监测以预防和治疗可能继发于诊断的疾病的临床护理建议。

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