Epidemiological and clinical characteristics of 66 Tunisian Sickle cell syndrome patients

Abstract

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/OArab), 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.