Abstract：
Becker muscular dystrophy (BMD) and Duchenne muscular dystrophy (DMD) are associated with intelligence quotients (IQs) lower than the normative values, and it is suggested that IQ is negatively correlated with the number of affected isoforms (i.e., Dp427, Dp140 and Dp71). Therefore, the objective of this meta-analysis was to estimate the IQ, and the IQ-genotype association according to the altered dystrophin isoforms, in the population with BMD or DMD.
A systematic search in Medline, Web of Science, Scopus and the Cochrane Library was conducted from inception to March 2023. Observational studies that determined the IQ and/or the IQ by genotype in the population with BMD or DMD were included. Meta-analyses of IQ, IQ by genotype and IQ-genotype association by comparing IQ according to the genotype were conducted. The results are shown as the mean/mean differences and 95% confidence intervals.
Fifty-one studies were included. The IQ in BMD was 89.92 (85.84, 94.01) and in DMD was 84.61 (82.97, 86.26). Moreover, the IQ for Dp427-/Dp140+/Dp71+ and Dp427-/Dp140-/Dp71+ was 90.62 (86.72, 94.53) and 80.73 (67.49, 93.98) in BMD, while the IQ for Dp427-/Dp140+/Dp71+, Dp427-/Dp140-/Dp71+ and Dp427-/Dp140-/Dp71- was 93.05 (89.42, 96.67), 81.78 (77.23, 86.32) and 49.19 (40.47, 57.90) in DMD. Finally, in DMD, Dp427-/Dp140-/Dp71+ vs Dp427-/Dp140+/Dp71+ and Dp427-/Dp140-/Dp71- vs Dp427-/Dp140-/Dp71+ were associated with -10.73 (-14.66, -6.81) and -36.14 (-48.87, -23.41) points, respectively.
The IQ in BMD and DMD was lower than the normative values. Moreover, in DMD, there is a synergistic association between the number of affected isoforms and IQ.
A systematic search in Medline, Web of Science, Scopus and the Cochrane Library was conducted from inception to March 2023. Observational studies that determined the IQ and/or the IQ by genotype in the population with BMD or DMD were included. Meta-analyses of IQ, IQ by genotype and IQ-genotype association by comparing IQ according to the genotype were conducted. The results are shown as the mean/mean differences and 95% confidence intervals.
Fifty-one studies were included. The IQ in BMD was 89.92 (85.84, 94.01) and in DMD was 84.61 (82.97, 86.26). Moreover, the IQ for Dp427-/Dp140+/Dp71+ and Dp427-/Dp140-/Dp71+ was 90.62 (86.72, 94.53) and 80.73 (67.49, 93.98) in BMD, while the IQ for Dp427-/Dp140+/Dp71+, Dp427-/Dp140-/Dp71+ and Dp427-/Dp140-/Dp71- was 93.05 (89.42, 96.67), 81.78 (77.23, 86.32) and 49.19 (40.47, 57.90) in DMD. Finally, in DMD, Dp427-/Dp140-/Dp71+ vs Dp427-/Dp140+/Dp71+ and Dp427-/Dp140-/Dp71- vs Dp427-/Dp140-/Dp71+ were associated with -10.73 (-14.66, -6.81) and -36.14 (-48.87, -23.41) points, respectively.
The IQ in BMD and DMD was lower than the normative values. Moreover, in DMD, there is a synergistic association between the number of affected isoforms and IQ.
摘要：
背景：Becker和Duchenne肌营养不良（BMD和DMD）与智商（IQ）低于标准值有关，并且建议IQ与受影响的同工型的数量呈负相关(即，Dp427、Dp140、Dp71)。因此,这项荟萃分析的目的是估计智商，和根据改变的肌营养不良蛋白亚型的智商基因型关联，在BMD或DMD人群中。
方法：Medline中的系统搜索，WebofScience,Scopus和Cochrane图书馆从成立到2022年3月进行。包括在具有BMD或DMD的群体中通过基因型确定IQ和/或IQ的观察性研究。智商的荟萃分析，智商按基因型，并通过根据基因型比较IQ进行IQ-基因型关联。结果显示为平均值/平均值差异和95%置信区间。
结果：纳入51项研究。BMD的IQ为89.92（85.84，94.01），DMD的IQ为84.61（82.97，86.26）。此外，在BMD中,Dp427-/Dp140+/Dp71+和Dp427-/Dp140-/Dp71+的IQ分别为90.62(86.72,94.53)和80.73(67.49,93.98),而IQ为Dp427-/Dp140+/Dp71+，Dp427-/Dp140-/Dp71+,和Dp427-/Dp140-/Dp71-为93.05（89.42，96.67），DMD中的81.78（77.23，86.32）和49.19（40.47，57.90）。最后,在DMD中，Dp427-/Dp140-/Dp71+与Dp427-/Dp140+/Dp71+和Dp427-/Dp140-/Dp71-vs.Dp427-/Dp140-/Dp71+与-10.73（-14.66，-6.81）和-36.14（-48.87，-23.41）点相关，分别。
结论：BMD和DMD的智商低于标准值。此外，在DMD中，受影响的同工型数量与智商之间存在协同关联。
方法：Medline中的系统搜索，WebofScience,Scopus和Cochrane图书馆从成立到2022年3月进行。包括在具有BMD或DMD的群体中通过基因型确定IQ和/或IQ的观察性研究。智商的荟萃分析，智商按基因型，并通过根据基因型比较IQ进行IQ-基因型关联。结果显示为平均值/平均值差异和95%置信区间。
结果：纳入51项研究。BMD的IQ为89.92（85.84，94.01），DMD的IQ为84.61（82.97，86.26）。此外，在BMD中,Dp427-/Dp140+/Dp71+和Dp427-/Dp140-/Dp71+的IQ分别为90.62(86.72,94.53)和80.73(67.49,93.98),而IQ为Dp427-/Dp140+/Dp71+，Dp427-/Dp140-/Dp71+,和Dp427-/Dp140-/Dp71-为93.05（89.42，96.67），DMD中的81.78（77.23，86.32）和49.19（40.47，57.90）。最后,在DMD中，Dp427-/Dp140-/Dp71+与Dp427-/Dp140+/Dp71+和Dp427-/Dp140-/Dp71-vs.Dp427-/Dp140-/Dp71+与-10.73（-14.66，-6.81）和-36.14（-48.87，-23.41）点相关，分别。
结论：BMD和DMD的智商低于标准值。此外，在DMD中，受影响的同工型数量与智商之间存在协同关联。
