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BACKGROUND The prognostic implications of P-wave dispersion in patients with a variety of cardiac disease conditions are increasingly being recognised. The relationship between P-wave dispersion and left ventricular function in sickle cell anaemia is unknown. OBJECTIVE This study was aimed at evaluating the relationship between P-wave dispersion and left ventricular function in adult Nigerian...

The levels of glycated haemoglobin, fetal haemoglobin and methaemoglobin in 618 Saudi subjects were determined. A statistically significant decrease in the percentage of glycated haemoglobin was observed in all haemoglobinopathic groups studied in comparison to normal controls. However, there was no significant difference in the percentage of glycated haemoglobin in patients with sickle cell an...

Erythrocytes from 14 patients with homozygous sickle cell anaemia were treated with the calcium ionophore A23187 to induce loss of cellular potassium and water. The dehydrated cells showed a decrease in filterability (loss of deformability) through pores of 5 micron diameter. Oxpentifylline and cetiedil citrate, which preserve erythrocyte cation and water content, had a significant (p less than...

A Ghanaian family is described in which a sickle-cell haemoglobin C man married to a sickle-cell thalassaemia woman produced 12 children (eight alive). Four children have sickle-cell anaemia, two sickle-cell haemoglobin C disease, one has sickle-cell thalassaemia, and one is asymptomatic haemoglobin C thalassaemia.It is emphasized that the contribution that adult sickle-cell disease patients ma...

Sickle cell disease (HbS) is a haemolytic anaemia characterized by the formation of abnormal haemoglobin. In patients with sickle cell disease, high rates of erythrocyte generation, degradation, and hyperbilirubinemia increase the risk for cholelithiasis. Previous studies have found that the incidence of cholelithiasis is 70% in adult patients. In sickle cell disease, decreased oxygen concentra...

The kidney of patients with sickle cell disease (SCD) is affected by both haemodynamic changes of chronic anaemia and by the consequences of vaso-occlusion which are especially marked within the renal medulla. There are many abnormalities in renal structure and function as a result of these changes. Functional changes occur with increasing age in subjects with sickle cell disease. Proteinuria, ...

Genetic disorders of haemoglobin (haemoglobinopathies), including the thalassaemias and sickle cell anaemia, abound in historically malarious regions, due to the protection they provide against death from severe malaria. Despite the overall spatial correlation between malaria and these disorders, inter-population differences exist in the precise combinations of haemoglobinopathies observed. Gre...

A young adult with homozygous sickle cell anaemia (Hb SS) suffered a fatal sickle cell crisis complicated by the sickle chest syndrome. At necropsy multiple large infarcts of the lung, bone marrow, and pituitary gland were found. The large majority of pulmonary infarcts were not associated with either gross or microscopic vaso-occlusion. These findings are discussed and correlated with past and...

BACKGROUND The aim of the study was to calculate the cost to the UK National Health Service of providing treatment services for patients with sickle cell disorders. The rates of differential morbidity and mortality, in the first 10 years of life, between screen-detected early diagnosed and clinically presenting late diagnosed cohorts of sickle cell disorder patients are also estimated. METHOD...