TITLE:
Major Sickle Cell Syndromes in Maradi, Niger: Epidemiological, Clinical, Biological and Therapeutic Aspects
AUTHORS:
Moustapha Elhadji-Chefou, Badé Malam-Abdou, Amadou Djibrilla-Almoustapha, Maman Brah Moustapha, Djamila Mahamadou Ali, Oumou Kimso, Ibrahim Timi Liman Elhadji Ali, Aminatou Seydou Yacouba, Abdou Alzouma
KEYWORDS:
Major Sickle Cell Syndromes, Epidemiological, Clinical, Biological, Therapeutic Aspects Maradi/Niger
JOURNAL NAME:
Open Journal of Blood Diseases,
Vol.14 No.4,
November
4,
2024
ABSTRACT: Introduction: Sickle cell disease is the most widespread genetic disorder in the world. This study aimed to investigate the epidemiological, clinical, biological, and therapeutic aspects of sickle cell anemia in Maradi, Niger. Methodology: This was a retrospective descriptive study carried out at the Centre Hospitalier Régional (CHR) of Maradi over 12 months, from September 2021 to August 2022. Results: This study included 246 patients with sickle cell anemia. The average age was 7.9 years, ranging from 0 to 16 years, and a sex ratio of 1.2. We observed kinship in 54.9% of the parents of sickle cell patients. Among the fathers, 62.6% were primarily blue-collar workers, while 87.4% of the mothers were housewives. Clinically, we observed pallor in 78.5% of the patients, jaundice in 43.5%, splenomegaly in 12.6%, and hepatomegaly in 11.8%. The most common vaso-occlusive crises involved osteoarticular pain (34.6%), followed by hand-foot syndrome (26.4%) and abdominal pain (20.3%). The SS form predominated biologically, representing 93.5% of cases, while double heterozygotes SC represented 6.5%. Most patients (56.5%) presented with severe anemia, with moderate anemia observed in 38.6% of cases. All patients received folic acid. Level I and II analgesics were used in 82.6% and 46.7% of patients, respectively, and patients received non-steroidal anti-inflammatory drugs in 72.3% of cases. A small proportion of patients (7.8%) were on background treatment with Hydrea. Conclusion: The results of our study are similar to those often described in sickle cell anemia.