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杜氏肌营养不良症患者心、肺功能下降的自然病史。

The natural history of cardiac and pulmonary function decline in patients with duchenne muscular dystrophy.

机构信息

Department of Orthopedics, University of California, Davis, CA, USA.

出版信息

Spine (Phila Pa 1976). 2011 Jul 1;36(15):E1009-17. doi: 10.1097/BRS.0b013e3181fea1ed.

DOI:10.1097/BRS.0b013e3181fea1ed
PMID:21289561
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3120906/
Abstract

STUDY DESIGN

Retrospective review of scoliosis progression, pulmonary and cardiac function in a series of patients with Duchenne Muscular Dystrophy (DMD).

OBJECTIVE

To determine whether operative treatment of scoliosis decreases the rate of pulmonary function loss in patients with DMD.

SUMMARY OF BACKGROUND DATA

It is generally accepted that surgical intervention should be undertaken in DMD scoliosis once curve sizes reach 35° to allow intervention before critical respiratory decline has occurred. There are conflicting reports, however, regarding the effect of scoliosis stabilization on the rate of pulmonary function decline when compared to nonoperative cohorts.

METHODS

We reviewed spinal radiographs, echocardiograms, and spirometry, hospital, and operative records of all patients seen at our tertiary referral center from July 1, 1992 to June 1, 2007. Data were recorded to Microsoft Excel (Microsoft, Redmond, WA) and analyzed with SAS (SAS Institute, Cary, NC) and R statistical processing software (www.r-project.org).

RESULTS

The percent predicted forced vital capacity (PPFVC) decreased 5% per year before operation. The mean PPFVC was 54% (SD = 21%) before operation with a mean postoperative PPFVC of 43% (SD = 14%). Surgical treatment was associated with a 12% decline in PPFVC independent of other treatment variables. PPFVC after operation declined at a rate of 1% per year and while this rate was lower, it was not significantly different than the rate of decline present before operation (P = 0.18). Cardiac function as measured by left ventricular fractional shortening declined at a rate of 1% per year with most individuals exhibiting a left ventricular fractional shortening rate of more than 30 before operation.

CONCLUSION

Operative treatment of scoliosis in DMD using the Luque Galveston method was associated with a reduction of forced vital capacity related to operation. The rate of pulmonary function decline after operation was not significantly reduced when compared with the rate of preoperative forced vital capacity decline.

摘要

研究设计

回顾性研究一系列杜氏肌营养不良症(DMD)患者脊柱侧凸进展、肺和心脏功能的情况。

目的

确定 DMD 患者脊柱侧凸手术治疗是否会降低肺功能丧失的速度。

背景资料概要

通常认为,一旦 DMD 脊柱侧凸的曲线大小达到 35°,就应该进行手术干预,以便在出现临界呼吸下降之前进行干预。然而,关于脊柱侧凸稳定化对肺功能下降率的影响,与非手术队列相比,存在相互矛盾的报告。

方法

我们回顾了 1992 年 7 月 1 日至 2007 年 6 月 1 日期间在我们的三级转诊中心就诊的所有患者的脊柱 X 光片、超声心动图和肺活量计、住院和手术记录。数据记录到 Microsoft Excel(Microsoft,Redmond,WA)中,并使用 SAS(SAS Institute,Cary,NC)和 R 统计处理软件(www.r-project.org)进行分析。

结果

手术前,预计用力肺活量的百分比(PPFVC)每年下降 5%。手术前平均 PPFVC 为 54%(SD=21%),术后平均 PPFVC 为 43%(SD=14%)。手术治疗与 PPFVC 下降 12%独立相关,与其他治疗变量无关。手术后 PPFVC 以每年 1%的速度下降,虽然下降速度较慢,但与手术前的下降速度没有显著差异(P=0.18)。左心室缩短分数(LVFS)作为心脏功能的衡量标准,每年下降 1%,大多数患者在手术前的 LVFS 率超过 30。

结论

使用 Luque Galveston 方法对 DMD 脊柱侧凸进行手术治疗与与手术相关的用力肺活量减少有关。与术前用力肺活量下降率相比,术后肺功能下降率没有显著降低。

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