杜氏肌营养不良症的管理——脊柱手术和家庭夜间通气对提高生存率的叠加效应。

Managing Duchenne muscular dystrophy--the additive effect of spinal surgery and home nocturnal ventilation in improving survival.

作者信息

Eagle Michelle, Bourke John, Bullock Robert, Gibson Mike, Mehta Jwalant, Giddings Dave, Straub Volker, Bushby Kate

机构信息

University of Newcastle and Newcastle upon Tyne Hospitals Trust, New Castle Muscle Centre, Institute of Genetics, Center for Life, Central Parkway, Newcastle, NE13BZ, UK.

出版信息

Neuromuscul Disord. 2007 Jun;17(6):470-5. doi: 10.1016/j.nmd.2007.03.002. Epub 2007 May 8.

DOI:10.1016/j.nmd.2007.03.002

PMID:17490881

Abstract

OBJECTIVES

To determine the long term survival in patients with Duchenne muscular dystrophy (DMD) following spinal surgery and nocturnal ventilation.

STUDY DESIGN

A retrospective review of 100 consecutive patients born between 1970 and 1990 was conducted.

RESULTS

Forty-seven patients had surgical spinal fusion, 27 were subsequently ventilated. Fourteen patients received ventilation only. Thirty-nine patients received neither intervention. The age at which ventilation was required correlated with the age at which ambulation was lost. Those who walked for longer were less likely to require spinal surgery. Mean vital capacity dropped from 1.4 to 1.13 L 1 year post-operatively. Patients having both spinal surgery and ventilation had a median survival of 30 years whereas those who were only ventilated survived to 22.2 years.

CONCLUSION

Nocturnal ventilation improves survival in DMD. Spinal surgery does not increase forced vital capacity but in combination with nocturnal ventilation further improves median survival to 30 years.

摘要

目的

确定杜氏肌营养不良症（DMD）患者在接受脊柱手术和夜间通气后的长期生存率。

研究设计

对1970年至1990年间出生的100例连续患者进行回顾性研究。

结果

47例患者接受了脊柱融合手术，其中27例随后接受了通气治疗。14例患者仅接受了通气治疗。39例患者未接受任何干预。需要通气的年龄与失去行走能力的年龄相关。行走时间较长的患者接受脊柱手术的可能性较小。术后1年，平均肺活量从1.4升降至1.13升。接受脊柱手术和通气治疗的患者中位生存期为30年，而仅接受通气治疗的患者生存期为22.2年。