نتایج جستجو برای: homozygous sickle cell disease
تعداد نتایج: 2943221 فیلتر نتایج به سال:
BACKGROUND In sickle cell disease, the quantification of Hb A2 is important for the differential diagnosis between sickle cell anemia (Hb SS) and Hb S/β(0)-thalassemia. OBJECTIVE To determine Hb A2 levels as quantified by high performance liquid chromatography in patients with sickle cell anemia (Hb SS) and with the SC hemoglobinopathy, with or without concomitant alpha thalassemia. METHODS...
INTRODUCTION Total splenectomy in sickle cell disease is related to a high risk of fulminant sepsis and increased incidence of other events, which have not been reported in patients with partial splenectomy. In this study we examined the patients with sickle cell disease and partial splenectomy and compared the clinical and laboratory results with non-splenectomized patients. MATERIAL AND MET...
Peripheral vascular response to inspiratory breath hold in paediatric homozygous sickle cell disease
Introduction: Sickle cell disease, the most frequent hemoglobinopathy, is one of many causes psychological repercussions. Objectives: To determine prevalence disorders in children/adolescents living with sickle disease and to identify associated factors. Patients Method: An analytical cross-sectional study was conducted from June September 2019 at national center mother-child consultation Unive...
A homozygous mutation in the gene for (HbA), is the proximate cause of sickle cell disease (SCD). Sickle hemoglobin (HbS) shows peculiar biochemical properties, which lead to polymerizing when deoxygenated. HbS polymerization is associated with a reduc increased red cell density which further accelerate HbS polymerization. Dense, dehydrated erythrocytes are likely to undergo instant polymerizat...
A homozygous mutation in the gene for β globin, a subunit of adult hemoglobin A (HbA), is the proximate cause of sickle cell disease (SCD). Sickle hemoglobin (HbS) shows peculiar biochemical properties, which lead to polymerizing when deoxygenated. HbS polymerization is associated with a reduction in cell ion and water content (cell dehydration), increased red cell density which further acceler...
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