DUCHENNE MUSCULAR-DYSTROPHY - PATTERNS OF CLINICAL PROGRESSION AND EFFECTS OF SUPPORTIVE THERAPY
DUCHENNE MUSCULAR-DYSTROPHY - PATTERNS OF CLINICAL PROGRESSION AND EFFECTS OF SUPPORTIVE THERAPY
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DOI：
10.1212/wnl.39.4.475
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发表时间：
1989-04-01
期刊：
影响因子：
9.9
通讯作者：
ARFKEN, C
中科院分区：
文献类型：
--
作者：
BROOKE, MH;FENICHEL, GM;ARFKEN, C
283名患有杜兴营养不良的男孩和10名患有贝克尔营养不良的男孩接受了长达10年的跟踪调查，该方案准确地测量了他们的功能、力量、痉挛和背曲。临床异质性是值得注意的。肌肉越强壮的患者死于心肌病的可能性越大。病情较弱的患者死于呼吸衰竭。定义了一系列里程碑，这对于跟踪单个患者的疾病是有用的。这种方法允许建立一个评分系统，允许在单个男孩身上定义疾病的严重程度。对物理治疗和手术干预的评估表明，夜间夹板和脊柱侧弯手术是有效的治疗形式。
Two-hundred eighty-three boys with Duchenne dystrophy and 10 with Becker dystrophy have been followed for up to 10 years in a protocol that accurately measured their function, strength, contractures, and back curvature. Clinical heterogeneity is noted. Patients whose muscles were stronger were more likely to die from a cardiomyopathy. Weaker patients died from respiratory failure. A series of milestones is defined, which is of use in following the illness in an individual patient. This approach permits a scoring system that allows the severity of the disease to be defined in an individual boy. Evaluation of physical therapy and surgical intervention shows that night splints and scoliosis surgery are effective forms of treatment.