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杜氏肌营养不良症患者身高与临床病程的关系。

Relation between height and clinical course in Duchenne muscular dystrophy.

作者信息

Zatz M, Rapaport D, Vainzof M, Rocha J M, Pavanello R de C, Colletto G M, Peres C A

机构信息

Departamento de Biologia, Universidade de São Paulo, Brazil.

出版信息

Am J Med Genet. 1988 Feb;29(2):405-10. doi: 10.1002/ajmg.1320290223.

Abstract

We have evaluated the relation between height and rate of clinical progression in boys with Duchenne muscular dystrophy (DMD). In all, 111 DMD patients with age ranging from 2 to 23 years (mean 8.2 +/- 3.4 years) were assessed; of these patients, 92 had their height measured. Clinical course was determined through Vignos scale of functional disability, motor ability, and timed functional tests. All patients had grossly elevated serum creatine-kinase (CK) and pyruvate-kinase (PK) levels. When height was adjusted for patients' age, a statistically significant correlation was found between height and clinical course (positive with Vignos scale and negative with motor ability), suggesting that smaller boys have a better clinical course than taller patients of comparable age. These results support our previous hypothesis and suggest that growth inhibition seems to be effective in diminishing the progression of DMD.

摘要

我们评估了杜氏肌营养不良症(DMD)男孩的身高与临床进展速度之间的关系。总共评估了111例年龄在2至23岁(平均8.2±3.4岁)的DMD患者;其中92例患者测量了身高。通过功能残疾、运动能力和定时功能测试的维格诺斯量表来确定临床病程。所有患者的血清肌酸激酶(CK)和丙酮酸激酶(PK)水平均显著升高。在对患者年龄进行校正后,发现身高与临床病程之间存在统计学上的显著相关性(与维格诺斯量表呈正相关,与运动能力呈负相关),这表明年龄相仿的情况下,体型较小的男孩临床病程比体型较高的患者更好。这些结果支持了我们之前的假设,并表明生长抑制似乎对减缓DMD的进展有效。

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