Newborn screening for sickle cell disease commenced in 1992 in Sao Paulo State and by the end of 2000, the programme covered 78 institutions in 36 municipalities with the screening of 281,884 babies. Initially based on liquid cord blood samples, these are being replaced by dried filter paper capillary samples to ease handling and avoid diagnostic confusion from maternal contamination. The preva...

Sickle cell disease is the result of altered genetic make up due to hereditary encounter and its form as homozygous sickle cell anemia is the most common and severe. The disease is characterized by chronic anemia, recurrent pain crises and vascular occlusion. Neurologically, there is a high incidence of stroke in childhood, as well as cognitive dysfunction. Newborn screening programmes and prev...

BACKGROUND Sickle cell disease causes chronic and recurrent hemolysis which is a recognized risk factor for cholelithiasis. This complication occurs in 50% of adults with sickle cell disease. Surgery is the consensual therapy for symptomatic patients, but the surgical approach is still controversial in asymptomatic individuals. AIMS To determine the frequency and to describe and discuss the o...

Sickle cell anemia is an inherited defect that affects the structure and synthesis of hemoglobin. In sickle cell trait, the affected individuals carry one gene for the abnormal hemoglobin (HbS). Sickle cell disease, however, is the homozygous state in which the abnormal hemoglobin is predominant in red blood cells, leading to devastating multisystem problems. Complications of the disease in chi...

Sickle cell haemoglobin (HbS) results from an autosomal recessively inherited mutation in which the 17th nucleotide of the beta globin gene is changed from thymine to adenine and the amino acid glutamic acid is replaced by valine at position 6 in the beta globin chain. [1, 2]Sickle cells have a reduced deformability and are easily destroyed, causing occlusion of the microcirculation and a chron...

BACKGROUND Little is known about the significance of haemoglobin genotype in dengue fever severity. This study was undertaken to determine the case fatality ratio and the impact of genotype in patients with sickle cell disease and confirmed dengue fever. METHODS This retrospective analysis included 40 patients with confirmed dengue and sickle cell disease, during the study period (2010-2012)....

Background. Hemoglobin SC disease is one of the most frequent hemoglobinopathy. Surprisingly, few studies were dedicated to this disease, currently considered as a mild variant of homozygous sickle cell disease. The aim of this study was to update our knowledge about hemoglobin SC disease. Design and Methods. We conducted this study in a monocentric series of 179 patients. Clinical and biologic...

The sickle cell disease is a genetically inherited commonly encountered hematological disorder that causes high degree of morbidity, mortality and fetal wastage. The suspected cases of hemoglobinopathies suffering from anemia are routinely referred from different peripheral Primary Health Centres and Hospitals in the state of Orissa (India) to our Centre for detailed investigations and genetic/...