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[The importance of sickle cell anemia in a pediatric environment in Gabon]
1Service de pédiatrie B, Centre Hospitalier de Libreville, Gabon.
Insights
Sickle-cell disease is a significant pediatric health issue in Gabon, ranking third for admissions and causing high mortality, especially in children without regular medical follow-up. Early detection and consistent care are crucial for better outcomes.
Area of Science:
- Pediatric Hematology
- Public Health
- Genetics
Context:
- Sickle-cell disease (SCD) poses a significant health burden in children in Gabon.
- Studies were conducted over four years (1992-1995) at the Pediatric Unit of Libreville Hospital.
- Understanding SCD's prevalence, features, and management is critical for improving child health outcomes in the region.
Purpose:
- To determine the importance and features of sickle-cell disease in Gabonese children.
- To evaluate the effectiveness and modes of regular medical follow-up for children with SCD.
- To analyze hospitalization and management data for pediatric SCD patients.
Summary:
- Sickle-cell disease is the third leading cause of pediatric admissions (13%) and contributes to 8.4% of total mortality.
- Main hospitalization causes include acute anemia, painful crises, and bacterial infections. Half of affected children lack medical follow-up, and 80% of deaths occurred in those without care.
- Regular ambulatory care for 210 children revealed complications like splenomegaly (33%), hepatomegaly (70%), and icterus (40%). Growth, puberty, and school delays were noted as social handicaps. HIV and HBsAg prevalence were also assessed.
Impact:
- Findings highlight the critical need for improved medical follow-up and management strategies for pediatric sickle-cell disease in Gabon.
- The study suggests that a dedicated sickle-cell disease management unit, integrated with a Pediatric Unit, offers a cost-effective solution in the local context.
- Comparative studies are recommended to explore genetic and environmental factors influencing SCD outcomes.
Abstract:
According studies run during four years (1992-1995) in the Pediatric Unit of Libreville Hospital, to determine importance and features of sickle-cell disease in children in Gabon, profit and mode of regular follow-up, data of hospitalisation and management of children with sickle-cell disease were found as follow: Sickle-cell disease is third rank of admission motivations (13% of total admissions), and first after 4 years. Mortality is 8.4% of total mortality. Main causes of hospitalisation were acute anemia, painful crisis, and bacterial infections. Half of children had no medical follow-up, third was regularly checked, the remaining very irregularly, 80% of died children had no medical follow-up. A survey with an ambulatory taking-care which concerned 210 kids regularly checked, shows theses outcomes: the social families background was either medium or disavow. More than half of children were detected before one year, mainly by complication. Third had splenomegaly, 70% hepatomegaly and 40% were icteric. Based on 103 children tested for HIV, only 2 were found positive, but 22% were positive for HBs Ag. Growth, puberty and school retardation is a supplementary social handicap. A comparative with other cohorts must headlight genetic and personal environing matters. Unit of management of sickle-cell disease, annexed to a Pediatric Unit, provided that official acknowledgement, seems to be the best solution, as regards cost/efficiency, in the local context.