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 v...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.展开更多
文摘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.
