Objective Women with sickle cell disease (SCD) have adverse maternal and infant outcomes. Our aim was to determine whether the outcomes of SCD mothers and their infants differed from African or Caribbean women not affected by SCD and whether there were differences between SCD individuals with the haemoglobin SS (HbSS) or haemoglobin SC (HbSC) genotypes. Furthermore, we wished to determine if any differences related to deprivation. Design A matched cohort study. Setting Tertiary perinatal centre in London Patients 4964 African or Caribbean women without SCD and 148 with SCD. Main outcome measures Mode of delivery, maternal exchange transfusion, birthweight, neonatal unit admission, neonatal death and deprivation indices Results SCD women were more likely to be delivered by caesarean section (p<0.001) and had babies of lower birthweight (p<0.001). Their infants were no more likely to be admitted to neonatal intensive care unit or suffer a neonatal death. There were no significant differences between the SCD women and those without SCD in their deprivation index or deprivation decile. The women with the HbSS genotype compared to those with the HbSC genotype were more anaemic (p<0.02), required more exchange transfusions (p<0.001) and were more likely to be delivered by caesarean section (p=0.008). The infant outcomes did not differ significantly between the genotypes. Conclusions Although, the SCD women, particularly those with the HbSS genotype, had greater morbidity, infant morbidity, and mortality was similar in mothers with the HbSS or HbSC genotypes and those without SCD. Data are available upon reasonable request.

中文翻译：

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镰状细胞病女性的孕产妇和婴儿结局：一项匹配队列研究

目的 患有镰状细胞病 (SCD) 的女性会产生不良的母婴结局。我们的目的是确定 SCD 母亲及其婴儿的结局是否与未受 SCD 影响的非洲或加勒比妇女不同，以及血红蛋白 SS (HbSS) 或血红蛋白 SC (HbSC) 基因型的 SCD 个体之间是否存在差异。此外，我们希望确定是否存在与剥夺有关的任何差异。设计匹配队列研究。设置伦敦第三围产期中心 患者 4964 名没有 SCD 的非洲或加勒比妇女和 148 名患有 SCD 的妇女。主要结局指标 分娩方式、产妇换血、出生体重、新生儿病房入住、新生儿死亡和剥夺指数 结果 SCD 妇女更有可能通过剖腹产分娩 (p<0.001)，并且婴儿出生体重较低 (p<0.001) 。他们的婴儿不太可能被送入新生儿重症监护室或新生儿死亡。 SCD 女性与非 SCD 女性的剥夺指数或剥夺十分位数没有显着差异。与 HbSC 基因型的女性相比，具有 HbSS 基因型的女性更贫血 (p<0.02)，需要更多的换血 (p<0.001)，并且更有可能通过剖腹产分娩 (p=0.008)。不同基因型之间的婴儿结局没有显着差异。结论 尽管 SCD 女性，特别是具有 HbSS 基因型的女性，发病率较高，但具有 HbSS 或 HbSC 基因型的母亲与无 SCD 的母亲的婴儿发病率和死亡率相似。数据可根据合理要求提供。