Background: Molecular genetic factors regulating hemoglobin F (Hb F) expression are important modifiers of the severity of sickle cell anemia (SS). Methods: The prevalence of XmnI polymorphic site, the Gg:Ag ratio and the Hb F level were determined using PCR-RFLP procedure, HPLC and alkaline denaturation method, respectively, in various haplotypes of 52 patients with SS, 18 patients with sickle...

OBJECTIVE To characterize the deaths of 193 children with sickle cell disease screened by a neonatal program from 1998 to 2012 and contrast the initial years with the final years. METHODS Deaths were identified by active surveillance of children absent to scheduled appointments in Blood Bank Clinical Centers (Hemominas). Clinical and epidemiological data came from death certificates, neonatal...

background sickle hemoglobin is the most common abnormal hemoglobin in the united states. hemoglobin s arises as a result of a single amino acid substitution (glutamic acid to valin at position 6 of the β-globine chain). the presence of fetal hemoglobin (hbf) plays a relatively protective role since a significant amount of hbf interferes with hbs polymerization, the pathogenesis mechanism of th...

background sickle cell patients suffer from many physical, psychological, and social problems that can affect their quality of life. to deal with this chronic condition and manage their disease and prevent complications associated with the disease, they must learn skills and behaviours. the aim of this study was to determine the effectiveness of self-management programs on quality of life in th...

INTRODUCTION Sickle cell disease (SCD) is the most common inherited disorder of haemoglobin worldwide. This study evaluated the chromatographic patterns and red blood cell indices of sickle cell patients to determine the co-inheritance of other haemoglobin(Hb) variants and β-thalassaemia trait. METHODS Red cell indices, blood film, sickle solubility test, Hb electrophoresis using alkaline cel...

We report the general phenotype severity and the hematological presentation in a cohort of 125 sickle cell anemia (SCA) patients with identical homozygous HbS/S genotype and categorized by identical β(S) haplotype, both with and without alpha thalassemia. No clear general phenotype correlation was found when patients were compared regardless of the haplotype but overall, patients with homozygou...

I NTEREST has been focused recemitly umpomi the hemoglobinopathies amid their elucidation by means of electrophoresis. Included among these are thalassemia, sickle cell anemia, hemoglobimi C, D, amid E disease, amid their combimiatiomis, all of which share fri common a number of hematologic ahnormalities. Dameshek has stated that the diagnosis of thalassemia is made omi a number of more or less...

There are about 50,000 people in the United States who are homozygous for the sickle hemoglobin gene and thus have sickle cell anemia. Sickle cell anemia is primarily seen in persons of African heritage, about 1 in 14 of whom is an asymptomatic carrier (a heterozygote). One in 700 newborns of African heritage is affected.1 Although it is the most severe of the common sickle cell diseases (which...

We found the article by Koshy et al,’ entitled “Leg Ulcers in Patients With Sickle Cell Disease” very interesting. However, we want to make some comments mainly addressed to the factors implicated in the development of leg ulcers in sickle cell disease that, in our opinion, bring insights into the pathogenic mechanism of this complication. Among the contributing factors, the authors refer to ‘‘...

We examined the extent to which the intracellular polymerization of sickle hemoglobin (HbS) can account for the severity of anemia and of vaso-occlusive manifestations in the various sickling syndromes. Polymer formation in sickle cell disease depends principally on the intraerythrocytic hemoglobin composition and concentration. In our studies, the polymer fraction in sickle red cells was deter...