Statutory Premarital Haemoglobinopathy Screening, Marriage Decisions, and Affected Births: A Systematic Review

Statutory premarital screening for haemoglobinopathies has been implemented in several Middle Eastern countries and elsewhere to identify couples at reproductive risk, but its effectiveness in reducing affected births remains uncertain. We conducted a systematic review and meta-analysis of studies evaluating statutory premarital screening for any haemoglobinopathy, searching MEDLINE, Cochrane Central Register of Controlled Trials (CENTRAL), Scopus, and Web of Science from 2000 onward. The primary outcome was the proportion of at-risk couples who did not proceed with marriage; the secondary outcome was change in affected births following programme implementation. Of 7,479 records screened after deduplication, 11 studies evaluating 7 programmes in 7 countries or territories were included. Ten studies contributed to the primary outcome, representing 14,906 at-risk couples. The proportion of couples who did not proceed with marriage varied substantially, with a pooled estimate of 33.6% (95% confidence interval, 16.3-56.6%; I²=99.1%). Three programmes reported reductions of 75%-100% in affected births following implementation, although these uncontrolled comparisons had important methodological limitations. Overall certainty of evidence was very low. Statutory premarital haemoglobinopathy screening is associated with substantial variation in subsequent marriage decisions, while affected births declined in programmes reporting longitudinal data. However, marked heterogeneity and risk of bias preclude reliable estimates of programme effectiveness or causal attribution of reductions in affected births to screening.