Epidemiology, diagnosis, and progression of major sickle cell syndrome in children in the pediatric departments of Niamey National Hospital.
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Keywords

major sickle cell disease
pediatrics
HNN
Niamey
Niger

How to Cite

1.
Malam-Abdou B, Kouakou B, Maman Brah M, Djibrilla-Almoustapha A, Samaila A, Elhadji-Chefou M, et al. Epidemiology, diagnosis, and progression of major sickle cell syndrome in children in the pediatric departments of Niamey National Hospital. J Sah Sci Santé [Internet]. 2026 Jan. 30 [cited 2026 Oct. 14];6(1):1748-56. Available from: https://www.j3s-fss-uas.org/index.php/jsss/article/view/220

Abstract

Introduction :
Sickle cell disease is a genetic and hereditary disease that is found worldwide and is transmitted in an autosomal recessive pattern. The objective of this study was to describe the epidemiological, diagnostic, and evolutionary aspects of sickle cell disease in children in the pediatric departments of the National Hospital of Niamey in 2022.

Methodology :
This was a prospective six-month study conducted between July 2022 and December 2022 involving 35 cases of sickle cell disease in children aged 0 to 14 years.

Results :
During the study period, 1,522 patients were hospitalized in pediatric wards A and B. A total of 35 cases of major sickle cell syndrome were identified, 11 in pediatric ward A (0.7%) and 24 in pediatric ward B (1.6%), representing an overall frequency of 2.3%. The epidemiological profile was that of a child under 24 months of age (31.4%), with males predominating at 54.3% of cases (sex ratio = 1.18). Consanguineous marriage accounted for 48.6% of cases. The SS form was predominant at 91.4%, and the SC form at 8.6%. Pallor was the most common reason for consultation (74.3%), followed by fever (65.7%). Palmar-plantar pallor was the most prominent physical sign (80%). Infections were the most common acute complications, accounting for 97.1% of cases. Severe anemia was found in 51.4% of cases. Treatment was provided on an inpatient basis, with patients being discharged in 100% of cases.

Conclusion :
Sickle cell disease is a common condition in Niger. The SS sickle cell profile is the most common, and infectious complications are the most frequent.

PDF (French)
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Copyright (c) 2026 B Malam-abdou, B Kouakou, M Maman Brah, A Djibrilla-almoustapha, A Samaila, M Elhadji-chefou, M Moussaram Aïchatou