杜氏肌营养不良症中的肺功能与脊柱侧弯

Pulmonary function and scoliosis in Duchenne dystrophy.

作者信息

Miller F, Moseley C F, Koreska J, Levison H

机构信息

Shriner's Hospital for Crippled Children, Los Angeles, California.

出版信息

J Pediatr Orthop. 1988 Mar-Apr;8(2):133-7.

PMID:3350945

Abstract

Pulmonary function data were evaluated in 68 Duchenne muscular dystrophy patients to determine the rate of decline of the percentage of normal forced vital capacity (FVC). The percentage of normal FVC declined most rapidly during the adolescent growth spurt, which demonstrated a need for an accurate and reliable means for determining height in these patients. The age at 35% of normal FVC was 14.9 +/- 3.6 years (2 SD), and survival for the 28 patients who died was 3.2 (range 0.2-5.7) years. Pulmonary function tests were performed 10-74 months after spinal stabilization on 21 Duchenne dystrophy patients and compared with 46 nonfused scoliotic Duchenne patients. No difference was found in the rate of deterioration of the percentage of normal FVC.

摘要

对68例杜氏肌营养不良患者的肺功能数据进行了评估，以确定正常用力肺活量（FVC）百分比的下降速率。正常FVC百分比在青春期生长突增期间下降最为迅速，这表明需要一种准确可靠的方法来测定这些患者的身高。达到正常FVC 35%时的年龄为14.9±3.6岁（标准差为2），28例死亡患者的存活时间为3.2年（范围为0.2 - 5.7年）。对21例杜氏肌营养不良患者在脊柱稳定术后10 - 74个月进行了肺功能测试，并与46例未融合脊柱侧弯的杜氏患者进行了比较。未发现正常FVC百分比的恶化速率存在差异。