Fetal haemoglobin (HbF) is a major ameliorating factor in sickle cell disease. We investigated if a quantitative trait locus on chromosome 6q23 was significantly associated with HbF and F cell levels in individuals of African descent. Single nucleotide polymorphisms (SNPs) in a 24-kb intergenic region, 33-kb upstream of the HBS1L gene and 80-kb upstream of the MYB gene, were typed in 177 health...

This study was prompted by the previous finding of amino-aciduria (Choremis, Zannos, and Basti, 1957) and qualitative and quantitative changes of serum proteins in thalassaemia and sickle-cell anaemia (Allamanis, 1955; Afentaki, 1949; Minnich, Na-Nakorn, Chongchareonsuk, and Kochaseni, 1954). The electrophoretic pattern shows a fall in albumin, a moderate fall in the a, a29 and I globulin fract...

OBJECTIVE The aim of this study was to determine, using a combination of measures, the prevalence of iron deficiency anaemia (IDA) in children under five years-of-age who have sickle cell disease (SCD) and attend the Sickle Cell Clinic (SCU) of the Tropical Medicine Research Institute. MATERIALS AND METHODS Children with homozygous sickle cell anaemia (Hb SS) or doubly heterozygous for Hb S a...

Sickle cell/haemoglobin D-Punjab disease is a disorder with similar clinical features to sickle cell anaemia. This report describes the case of an 11 year old boy with this disease who was treated with regular transfusions from infancy. He underwent splenectomy at the age of 10 years for hypersplenism. Histology of the spleen revealed a striking pattern of heavy sinusoidal endothelial iron load...

Sixteen subjects, with sickle-cell anaemia, all Iranians (ages 3 to 56 years), with very mild symptomatology are reported. Some of the subjects had been totally asymptomatic. Splenomegaly was noted in 9 cases. There was an increase in the mean level of fetal haemoglobin (18%); this is the probable explanation for the mild phenotype. In 29 subjects with sickle-cell trait, the level of HbF was al...

OBJECTIVE To examine the haematological profiles of patients with sickle cell anaemia above the age of 30 years. DESIGN Prospective cross sectional study. SETTING Department of Haematology University College Hospital, Ibadan, Nigeria. SUBJECTS 98 patients with sickle cell anaemia above the age of 30 years. MAIN OUTCOME MEASURES Clinical and haematological profiles of the patients. RES...

BACKGROUND South Africa has a low incidence of sickle cell disease (SCD). However, its demographics are changing because of immigration from sub-Saharan African countries where SCD is prevalent. OBJECTIVES We aimed to determine the frequency of SCD presenting to the Haematology/Oncology Service at Red Cross War Memorial Children's Hospital in Cape Town and to measure the associated disease bu...

BACKGROUND Anaemia is a major public health problem in developing countries, contributing significantly to morbidity and mortality amongst children under-five years of age. About 43 % of under-fives are anaemic worldwide, and two-thirds reside in sub-Saharan Africa. Even where blood transfusion is available for treatment there is still a significant case fatality rate ranging between 6 and 18 %...

Sickle cell anaemia (SS) and sickle cell-haemoglobin C disease (SC) patients exhibit severe red blood cell (RBC) rheological alterations involved in the development of several complications. The contribution of oxidative stress in these haemorheological abnormalities is still unknown. We compared RBC reactive oxygen species (ROS) and glutathione (GSH) content, and the haemorheological profile o...