Several studies indicate that prognosis for survival in Duchenne muscular dystrophy (DMD) has improved in recent decades. However, published evidence is inconclusive and some estimates may be obsolete due to improvements in standards of care, in particular the routine use of mechanical ventilatory support in advanced stages of the disease. In this systematic review and meta-analysis (PROSPERO identifier: CRD42019121800), we searched MEDLINE (through PubMed), CINAHL, Embase, PsycINFO, and Web of Science for studies published from inception up until December 31, 2018, reporting results of life expectancy in DMD. We pooled median survival estimates from individual studies using the median of medians, and weighted median of medians, methods. Risk of bias was established with the Newcastle–Ottawa Scale. Results were stratified by ventilatory support and risk of bias. We identified 15 publications involving 2662 patients from 12 countries from all inhabited continents except Africa. Median life expectancy without ventilatory support ranged between 14.4 and 27.0 years (pooled median: 19.0 years, 95% CI 18.0–20.9; weighted pooled median: 19.4 years, 18.2–20.1). Median life expectancy with ventilatory support, introduced in most settings in the 1990s, ranged between 21.0 and 39.6 years (pooled median: 29.9 years, 26.5–30.8; weighted pooled median: 31.8 years, 29.3–36.2). Risk of bias had little impact on pooled results. In conclusion, median life expectancy at birth in DMD seems to have improved considerably during the last decades. With current standards of care, many patients with DMD can now expect to live into their fourth decade of life.

几项研究表明，在最近几十年中，杜兴氏肌营养不良症（DMD）的生存预后得到了改善。但是，已发表的证据尚无定论，由于改善了护理标准，尤其是在疾病的晚期阶段常规使用机械通气支持，一些估计可能已过时。在这项系统的回顾和荟萃分析（PROSPERO标识符：CRD42019121800）中，我们搜索了MEDLINE（通过PubMed），CINAHL，Embase，PsycINFO和Web of Science，研究从开始到2018年12月31日之间发表的研究，报告了预期寿命在DMD中。我们使用中位数的中位数和中位数的加权中位数，汇总了各个研究的中位数生存估计值。偏倚风险是通过纽卡斯尔-渥太华量表确定的。结果根据通气支持和偏倚风险进行分层。我们确定了15个出版物，涉及来自非洲以外所有居住大洲12个国家的2662名患者。在没有通气支持的情况下，预期寿命的中位数为14.4至27.0岁（合并中位数：19.0岁，95％CI 18.0-20.9；加权合并中位数：19.4岁，18.2-20.1）。在1990年代的大多数环境中，通气支持的预期寿命中位数为21.0至39.6岁（合并中位数：29.9岁，26.5-30.8；加权合并中位数：31.8岁，29.3-36.2）。偏见风险对合并结果影响很小。总之，在过去的几十年中，DMD出生时的平均预期寿命似乎已大大提高。按照目前的护理标准，许多DMD患者现在可以活到他们的第四个十年。