BACKGROUND Little is known about the effects of blood rheology on the occurrence of acute chest syndrome and painful vaso-occlusive crises in children with sickle cell anemia and hemoglobin SC disease. DESIGN AND METHODS To address this issue, steady-state hemorheological profiles (blood viscosity, red blood cell deformability, aggregation properties) and hematologic parameters were assessed ...

BACKGROUND Fetal hemoglobin level is a heritable complex trait that strongly correlates swith the clinical severity of sickle cell disease. Only few genetic loci have been identified as robustly associated with fetal hemoglobin in patients with sickle cell disease, primarily adults. The sole approved pharmacologic therapy for this disease is hydroxyurea, with effects largely attributable to ind...

OBJECTIVE The aim of the present study was to characterize sickle cell disease retinopathy in children and teenagers from Bahia, the state in northeastern Brazil with the highest incidence and prevalence of sickle cell disease. METHODS A group of 51 sickle cell disease patients (36 hemoglobin SS and 15 hemoglobin SC) with ages ranging from 4 to 18 years was studied. Ophthalmological examinati...

a case of sickle cell hb d disease is reported in  young iranian male. the father of whom carried an as sickle cell trait and the mother an ad trait. this disease was diagnosed by hb electrophoresis. agar gel electrophoresis sickling and solubility tests.this is the first case of sickle cell hb d disease reported in iran.

objective: the use of fetal hemoglobin (hbf) inducer drugs is considered as a novel approach in treatment of β-hemoglobinopathies, especially β- thalassemia and sickle cell disease. hbf inducers including hydroxyurea, histone deacetylase (hdac) inhibitor agents such as sodium butyrate, azacitidine, decitabine and new immunomodulator drugs like pomalidomide, lenalidomide and thalidomide can redu...

V. NEEL, M.D., PH.D. T HIS REPORT deals with a new inherited abnormality of hemoglobin encountered in American Negroes. The existence of this traitcame to light ill the course of extensive clinical and genetic studies u’egarding the sickling pheuiomenon. Among the individuals examined thei’e were anumber of patients ‘it-h a hemolytic syndrome associated with erythrocytic sickling in whom neithe...

H syndrome is an autosomal recessive genodermatosis with reports dating back to the last decade. This syndrome is caused by mutations in the SCL29A3 gene. The clinical characteristics of this syndrome consist of dermatological manifestations, including hyperpigmented, hypertrichotic, and indurated patches and plaques. It affects various systems by causing heart anomalies, hepatosplenomegaly, hy...

Background: Sickle cell disease is a genetic disorder of hemoglobin causing myriad of pathology including anemia. The purpose of this study was to evaluate the baseline values of steady state hemoglobin and packed cell volume as a guide to managing the early recognition of hemolytic crises in sickle cell anemia. Methods: A cross-sectional study was conducted among the sickle cell patients atte...