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The angiotensin-converting enzyme (ACE) insertion/deletion (I/D) gene polymorphism has been inconsistently reported to be a risk factor for Childhood immunoglobulin A vasculitis (IgAV) nephritis. We comprehensively searched electronic databases as of Jan 2020. Nineteen studies with 1104 cases and 1589 controls were included. Sensitivity analyses based on different subgroups were performed. Further analyses were conducted for association of ACE polymorphism with disease severity and prognosis. Significant associations were found between AC