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[Current aspects of sickle cell disease in children in Gabon]
V Thuilliez1, V Ditsambou, J R Mba
1Service de pédiatrie B, Centre hospitalier de Libreville, Gabon.
Insights
Sickle-cell anemia in Gabonese children presents with common complications like anemia and painful crises. Factors influencing prognosis in sickle-cell disease require further investigation.
Area of Science:
- Pediatrics
- Hematology
- Global Health
Context:
- Sickle-cell anemia (SCA) exhibits variable clinical manifestations and prognosis.
- Factors influencing SCA prognosis, particularly geographic influences, remain understudied.
- Understanding regional variations in SCA is crucial for tailored management.
Purpose:
- To describe the clinical manifestations and management of SCA in children treated at Libreville Hospital.
- To identify common causes of hospitalization and outpatient presentations in pediatric SCA patients.
- To compare SCA characteristics in Gabon with those reported in other populations.
Summary:
- A two-year study (1992-1993) analyzed 205 hospital admissions and 131 outpatient cases of SCA in children.
- Key hospitalization causes included acute anemia, painful crises (increasing with age), and infections (pulmonary and bone).
- Outpatients frequently presented with fever, anemia, hand-foot syndrome, hepatomegaly, splenomegaly, jaundice, and growth disorders, with a mean hemoglobin of 7 g/dL.
Impact:
- Findings suggest SCA in Gabonese children shares similarities with those in Congolese cohorts.
- The study highlights potential genetic and environmental factors contributing to differences in SCA presentation across geographic regions.
- This research contributes to understanding the epidemiology and clinical spectrum of SCA in Sub-Saharan Africa.
Background:
Clinical manifestations and course of sickle-cell anemia are variable. Knowledge about the factors, possibly geographic, that influence prognosis are still scanty.
Population And Methods:
Data of hospitalization and management of children with sickle-cell disease were studied during two years (1992-1993) in the Pediatric Unit of Libreville Hospital. They concerned 205 admissions of 171 children and 131 outpatients.
Results:
The main causes of hospitalization were: acute anemia (36 cases before the age of 5 years); painful crisis whose frequency increased with age (23% before 5 years, 35% between 5 and 10, 42% after 10 years); infections, essentially pulmonary occurring early, and bone infections at any age. Eight children died (because a complication of their disease). Among the 131 outpatients, half were detected because pyrexia, anemia and/or more often "hand-foot syndrome". More than 60% had hepatomegaly, one third still had splenomegaly after five years of age and more than one third was icteric. More than half children older than ten years had growth disorders. Mean hemoglobin level was 7 g/dL. 21 of the 83 tested children for HBsAg were positive and only one out of 79 was positive for HIV.
Conclusions:
Clinical manifestations and course of sickle-cell anemia in our patients are similar to those reported in Congolese children. Genetic and environmental factors may be responsible for differences with children from other, in particular French, cohorts.