background: iran is a country located on the thalassemic belt of the globe. investigating such issues is of a greater significance in southern provinces of the country where the high prevalence of thalassemia has imposed high costs and mental pressure on families and the healthcare system. methods: in this cross-sectional study conducted in 2011 as a census, the data related to patients or dise...

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...

objective:  the aim of this study was to assess clinical laboratory funding for differential diagnosis of sickle cell disease (scd) and other associated disorders for better understanding of clinical types and prevention of sickling events. material and methods:  this is a descriptive crossed-sectional study that analyzed the peripheral blood film, sickle cell preparation, hemoglobin electroph...

potent induction of fetal hemoglobin (hbf) production results in alleviating the complications of β-thalassemia and sickle cell disease (scd). hbf inducer agents can trigger several molecular signaling pathways critical for erythropoiesis. janus kinase/signal transducer and activator of transcription (jak/stat), mitogen activated protein kinas (mapk) and phosphoinositide 3-kinase (pi3k) are con...

Routine hematological tests were performed in a family which was at risk for sickle cell disease. Cellulose acetate electrophoresis and Triton PAGE were employed to differentiate between various variants of hemoglobin. Based on the data a pedigree was constructed which indicated that few members of the respectively had received the S gene, some of them were sickle cell disease while few were si...

BACKGROUND Sickle cell disease is a genetic disorder that leads to abnormally high levels of hemoglobin sickling in erythrocytes. Patients suffer debilitating and severe complications that affect multiple organs, though mainly the liver, gallbladder, spleen, bones, and kidneys. It has a significant impact on morbidity and mortality rates and is associated with substantial health care costs. M...

Uvaria chamae is used in the treatment of sickle cell crisis Benin. The aim this work to determine safety roots aqueous extract wistar rats. was obtained by maceration. tests for Acute Oral Toxicity (AOT) and Sub-Chronic (SCT) respectively forced gavage a single dose 2000 mg / Kg 200 body weight 28 days were performed on female Wistar animals, serum creatinine, transaminases number white blood ...

Background: There are many parameters that modulate the severity of sickle cell anemia. Fetal hemoglobin (Hb F) is one of these major variables. However, its effect is clinically inconsistent. We conducted a descriptive study to assess the influence of Hb F on clinical events and hematological variables in patients with sickle cell anemia. Methods: 151 patients with sickle cell anemia with a st...

Recent studies have recognised the importance of pulmonary hypertension (PH) in sickle cell disease (SCD). The aim of this study was to determine the prevalence and prognostic impact of PH and its features in patients with SCD. 80 patients with SCD underwent baseline clinical evaluation, laboratory testing, 6-min walk tests (6MWTs) and echocardiography. Patients with a peak tricuspid regurgitan...

We report a 20-year-old female with sickle cell anaemia and with an HbF concentration of 15.8%. The patient was not using hydroxyurea and was not receiving regular blood transfusions. The patient never had chronic manifestations of sickle cell anaemia, only pain crises of a mild intensity. After laboratory tests, we found that she was homozygous for HbS with the Bantu/atypical haplotype, and wa...