Department of Pediatric Haematology and Oncology, Erasmus MC - Sophia Children's Hospital, NC-825, Wytemaweg 80, 3015 CN, Rotterdam, The Netherlands.
Department of Pediatric Radiology, Erasmus MC - Sophia Children's Hospital, Rotterdam, The Netherlands.
BMC Med. 2020 Dec 22;18(1):393. doi: 10.1186/s12916-020-01864-8.
Silent cerebral infarcts (SCIs) are the most common neurological complication in children and adults with sickle cell disease (SCD). In this systematic review, we provide an overview of studies that have detected SCIs in patients with SCD by cerebral magnetic resonance imaging (MRI). We focus on the frequency of SCIs, the risk factors involved in their development and their clinical consequences.
The databases of Embase, MEDLINE ALL via Ovid, Web of Science Core Collection, Cochrane Central Register of Trials via Wiley and Google Scholar were searched from inception to June 1, 2019.
The search yielded 651 results of which 69 studies met the eligibility criteria. The prevalence of SCIs in patients with SCD ranges from 5.6 to 80.6% with most studies reported in the 20 to 50% range. The pooled prevalence of SCIs in HbSS and HbSβ SCD patients is 29.5%. SCIs occur more often in patients with the HbSS and HbSβ genotype in comparison with other SCD genotypes, as SCIs are found in 9.2% of HbSC and HbSβ patients. Control subjects showed a mean pooled prevalence of SCIs of 9.8%. Data from included studies showed a statistically significant association between increasing mean age of the study population and mean SCI prevalence. Thirty-three studies examined the risk factors for SCIs. The majority of the risk factors show no clear association with prevalence, since more or less equal numbers of studies give evidence for and against the causal association.
This systematic review and meta-analysis shows SCIs are common in patients with SCD. No clear risk factors for their development were identified. Larger, prospective and controlled clinical, neuropsychological and neuroimaging studies are needed to understand how SCD and SCIs affect cognition.
无症状性脑梗死（SCI）是镰状细胞病（SCD）患儿和成年患者最常见的神经系统并发症。在本系统评价中，我们对通过脑磁共振成像（MRI）检测 SCD 患者 SCI 的研究进行了概述。我们重点关注 SCI 的发生率、其发生的相关危险因素及其临床后果。
我们检索了 Embase、MEDLINE ALL（通过 Ovid）、Web of Science 核心合集、Cochrane 临床试验中心注册库（通过 Wiley）和 Google Scholar 数据库，检索时间从建库至 2019 年 6 月 1 日。
搜索结果有 651 项，其中 69 项研究符合纳入标准。SCD 患者 SCI 的发生率范围为 5.6%80.6%，大多数研究报道的发生率在 20%50%之间。HbSS 和 HbSβ SCD 患者 SCI 的总发生率为 29.5%。与其他 SCD 基因型相比，HbSS 和 HbSβ 基因型的患者 SCI 更常见，9.2%的 HbSC 和 HbSβ 患者存在 SCI。对照组 SCI 的平均总发生率为 9.8%。纳入研究的数据显示，研究人群的平均年龄增加与平均 SCI 发生率之间存在统计学显著关联。有 33 项研究探讨了 SCI 的危险因素。大多数危险因素与发生率之间没有明确的关联，因为或多或少有同样数量的研究提供了支持和反对因果关联的证据。
本系统评价和荟萃分析显示，SCD 患者 SCI 较为常见。目前尚未确定其发生的明确危险因素。需要开展更大规模、前瞻性、对照的临床、神经心理学和神经影像学研究，以了解 SCD 和 SCI 如何影响认知。
