Abstract
Sickle cell anemia is an autosomal recessive genetic disease by mutation of the β globin gene. This mutation induces the synthesis of abnormal hemoglobin (Hb) HbS, Mainly responsible for all clinical manifestations vaso-occlusives and chronic hemolysis with variable anemia. The objective of this study is the determination profile of haematological parameters of sickle cell children of the province of Kenitra (Morocco). In order to know the usual values and the particularities which are its own, we have observed that 50% of sickle cell children in our case study have severe anemia and 84% in patients aged 5 to 11 years. In addition, there is no significant difference among gender. The cases of hemoglobinosis S are divided into: Homozygous hemoglobinosis S (43%), Heterozygous sickle cell disease (17%), Hemoglobinosis S associated with alpha-thalassemia (1%), Hemoglobinosis S composite heterozygosity S/beta-thalassemia (5%), S/PHHF composite heterozygosity (6%), eventually composite heterozygosity S/beta+ thalassemia (28%). Homozygous sickle cell children have a long hospital stay, the highest number of hospitalizations and very severe sickle cell syndromes compared to the other phenotypic status of our population.
Highlights
The cases of hemoglobinosis S are divided into: Homozygous hemoglobinosis S (43%), Heterozygous sickle cell disease (17%), Hemoglobinosis S associated with alpha-thalassemia (1%), Hemoglobinosis S composite heterozygosity S/beta-thalassemia (5%), S/PHHF composite heterozygosity (6%), eventually composite heterozygosity S/beta+ thalassemia (28%)
Our results prove the existence of different types of phenotypes, among 86 patients who were identified in this heterozygous study, 37 homozygotes, 1 with associated α-thalassemia sickle cell disease, 24 with sickle cell disease associated with β+ -thalassemia, 4 with associated βthalassemia disease and 5 with S/PHHF composite heterozygosis
Sickle cell anemia is a hereditary blood disorder that can lead to life-long pain [14]-[19], Homozygous patients have more severe complications associated with increased morbidity and mortality, especially in older adolescents Platt, Powars and Raphael [14] [20] [21]
Summary
A. Belala et al 202 worldwide are born with sickle cell disease [1]. Major sickle cell syndromes encompass three major genetic forms: homozygoties S/S, Composite heterozygosities S/C and S/β or S/β+ thalassemia. The most severe forms are S/S homozygosities as well as S/β thalassemia. These syndromes mainly affect populations in sub-Saharan Africa, the Caribbean and North Africa. Confirmation of the diagnosis of sickle cell disease is based on the study of hemoglobin. This should be done at a distance from a transfusion. It confirms the presence of HbS (90% in homozygotes). The residual HbF level has an impact on the frequency of crises
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