Epidemiological and clinical characteristics of 66 Tunisian Sickle cell syndrome patients.

Autor: Sahli A; Laboratory of Biochemistry and Molecular Biology Children's Hospital Bechir Hamza (LR00SP03)., Ouali F; Laboratory of Biochemistry and Molecular Biology Children's Hospital Bechir Hamza (LR00SP03)., Dabboubi R; Laboratory of Biochemistry and Molecular Biology Children's Hospital Bechir Hamza (LR00SP03)., Fredj SH; Laboratory of Biochemistry and Molecular Biology Children's Hospital Bechir Hamza (LR00SP03)., Meddeb N; Laboratory of Biochemistry and Molecular Biology Children's Hospital Bechir Hamza (LR00SP03)., Mzoughi N; Laboratory of Biochemistry and Molecular Biology Children's Hospital Bechir Hamza (LR00SP03)., Messaoud T; Laboratory of Biochemistry and Molecular Biology Children's Hospital Bechir Hamza (LR00SP03).
Jazyk: angličtina
Zdroj: African health sciences [Afr Health Sci] 2023 Sep; Vol. 23 (3), pp. 213-222.
DOI: 10.4314/ahs.v23i3.26
Abstrakt: Introduction: Sickle cell syndrome (SCS) represent a real health problem. In this work, we propose to study the epidemiological and clinical features of 66 patients with SCS.
Methods: This is a retrospective descriptive cross-sectional study carried out on a population of 66 patients with SCS, (36 S/S, 18 S/β-thalassemia, seven S/C and five S/O Arab ), over a period of two years.
Results: The average age of our population is 15.5 years ± 8.4. 36 patients (55%) were born to a consanguineous marriage and 35 (53%) had siblings with SCS. The average baseline hemoglobin in our patients is 9.1g/dL±1.51. S/C patients have significantly higher baseline hemoglobin than S/S, S/β-thalassemia and S/OArab with p <0.05. Jaundice, mucosal skin pallor and hepatomegaly have been observed only in S/S, S/β-thalassemia and S/ OArab patients. The persistence of splenomegaly is more frequent in S/C than in S/S, and in S/-thalassemia than in S/S. The most common acute complications were vaso-occlusive attacks (69.7%) and worsening of anemia (54.54%). The most common chronic complication was cholelithiasis (36.36%).
Conclusion: S/C patients present the best tolerated form and were the least affected by chronic complications and therefore can lead an almost normal life.
Competing Interests: The authors stated that they had no conflicts of interest.
(© 2023 Sahli A et al.)
Databáze: MEDLINE
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