Drépanocytose chez l’enfant : Etude de 4 cas au niveau de l’Etablissement Publique Hospitalier de Ain Tedles (Mostaganem)

Abstract

Sickle cell disease is an autosomal recessive genetic disease that results from a point mutation of the sixth codon of the β globin gene, characterized by the synthesis of abnormal hemoglobin S which differs from normal hemoglobin A (HbA ) by forming polymers when it is deoxygenated, which induces sickling of red blood cells. Our study was carried out at the Public Hospital Establishment (EPH) of Ain Tedeles (wilaya of Mostaganem). We made a retrospective survey of the archives of patients with hemoglobinopathy, in the pediatric department of the establishment. Among the 39 files of hemoglobinopathies treated, we found 4 cases of sickle cell disease (prevalence = 10.25%). All cases were male and were aged 1 year (2 cases) and 6 years (2 cases). We also performed a complete blood count and smear of a sickle cell patient at the medical laboratory (Hematology section). The blood smear allowed us to observe characteristic sickle cells. The complete blood count of the 4 patients showed that the average red blood cell count was 4.4 109 / l, the average hemoglobin level of 11.07 g / dl, the average blood volume estimated at 92.25 fl. hematocrit at 34.75% and the mean corpuscular hemoglobin content at 28.25 pg.