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Sickle Cell Disease and adverse Maternal and Perinatal Outcomes: a Systematic Review and Meta-Analysis of Observational Studies

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Sickle Cell Disease and Adverse Maternal and Perinatal Outcomes: a Systematic Review and Meta-analysis of Observational Studies


Sickle cell disease (SCD) is defined as an autosomal recessive haemoglobinopathy which includes sickle cell anaemia and various compound heterozygous genotypes. SCD is characterised by chronic haemolytic anaemia and vaso-occlusive complications. It is the commonest genetic disorder globally, with more than 300,000 children born with the condition each year. The disease is associated with high lifetime morbidity and premature mortality.

Better quality of care for individuals with SCD has improved survival and thus, there are increasing numbers of women reaching reproductive age. Studies consistently show that pregnant women with SCD are at increased risk of maternal and foetal adverse outcomes, however these risks vary considerably between studies.

The global maternal mortality rate for SCD is 0.07-9.2%. The UN Millennium Developmental Goal 5 aims to lower 75% of maternal mortality globally by 2015. SCD may hinder some countries accomplishing this.

It is reasonable to assume that differences in the quality of health service provision and accessibility to healthcare resources are likely to influence outcomes of women with SCD in pregnancy.  These factors, combined with the heterogeneity inherent in the pathophysiology of SCD, lead to uncertainty in estimating the degree of risk associated with SCD pregnancy.RESEARCH PURPOSE

 To date, most studies reporting SCD pregnancy-related events have been limited by small sample sizes (often single institution based) and vary in their methods and robustness of  ascertainment of maternal and perinatal outcomes. We therefore sought to summarise and improve the accuracy and quality of information relating to outcomes of SCD in pregnancy by conducting a systematic review and meta-analysis.


To investigate and quantify the association between SCD in pregnancy and adverse maternal and perinatal outcomes, compared to a comparator group with normal haemoglobin structure (HbAA).

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