High early mortality among Ugandan children with sickle cell anemia and congenital HIV identified by newborn screening

Résumé fourni par la source

The Uganda Sickle Surveillance Study documented sickle cell anemia (SCA) throughout the country. An important corollary was the identification of infants born with SCA and vertically transmitted HIV. The consequences of SCA and HIV together are poorly understood, although both likely contribute to morbidity and early mortality. Sickle Evaluation and Assessment of Risks: Comorbidity with HIV (SEARCH) was a retrospective case-control study designed to evaluate the effects of concurrent SCA and HIV, comparing children with both conditions vs controls (children with one or neither condition). A database algorithm identified 3 to 6 age-matched controls from each category within the closest geographical region to improve the likelihood of successful contact. Among 258 690 screened infants in ∼6 years, a total of 206 714 were eligible and 103 children with both SCA and HIV were identified, plus appropriate controls. Personal contact was attempted for 41 children with both conditions identified within ∼150 km of Kampala, Uganda along with 41 controls from each category. Family contact was successful in 26 cases. Mortality was compared between children with both conditions and pooled controls using a 2-sided Fisher exact test, with an incident rate ratio of 3.952 (95% confidence interval, 1.983-7.875). Clinical morbidity was also documented in children living with SCA and HIV. Using national screening data, SEARCH confirmed poor clinical outcomes and high mortality in Ugandan children born with SCA and HIV. SEARCH documents the need for guidelines and strategies for children with both conditions. The expansion of care for SCA, including structured education and wider access to hydroxyurea, represents major steps toward improving outcomes for these children. This trial was registered at www.clinicaltrials.gov as #NCT06301893.

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