PubMed چکیده/رکورد

Evolution and Hospitalization Determinants of Homozygous Sickle Cell Disease in Semi-Rural Gabon.

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چکیده اصلی

BACKGROUND: HbSS causes high early mortality in sub-Saharan Africa, but longitudinal data from semi-rural Francophone Central Africa remain scarce. OBJECTIVE: To investigate clinical evolution and hospitalization determinants in homozygous sickle cell patients in semi-rural Gabon. METHODS: An ambispective cohort study of 286 HbSS patients (156 retrospective, 130 prospective) was conducted at Franceville University Hospital. Biological profiles were modeled via principal component analysis (PCA), and hospitalization predictors were identified using multivariate Firth's penalized logistic regression with multiple imputation (MICE). RESULTS: The predominantly pediatric cohort (median age: 9.5 years) had a 92.3% hospitalization rate. Vaso-occlusive crises (57.0%) and fever (58.9%) drove emergency admissions, alongside anemic shock (12.9%). PCA separated chronic hemolytic severity (PC1) from acute rheological adjustments (PC2). Hospitalization risk was driven exclusively by acute clinical complications, showing no association with socio educational indicators. A significant admission reduction occurred during 2021-2023 (OR ≈ 0.23; p = 0.013) due to optimized local protocols. CONCLUSIONS: Acute complications overrode static social determinants at admission ("clinical supremacy"). Public health priorities must incorporate universal neonatal screening, expanded hydroxyurea access, transcranial Doppler surveillance, and school-based individualized care plans.

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