Platelet function was measured on 29 occasions in 16 adult patients in the asymptomatic steady state of sickle-cell anaemia. There was a significant increase in platelet number and micro-aggregate formation, and a lower aggregation threshold with adenosine diphosphate, compared with 23 healthy controls. Similar changes were found, however, in 12 splenectomised patients without sickle-cell disea...

BACKGROUND The retinopathy is an uncommon complication in individuals with sickle cell trait except for the cases of sickle cell trait associated with systemic arterial hypertension, diabetes mellitus, syphilis, tuberculosis and sarcoidosis. CASE PRESENTATION A retinopathy in a 16 year-old child with no history of consanguinity in the parents revealed a sickle S trait associated to heterozygo...

results sickle cells were observed among 20% of subjects with the dilution of 1:50 and 70% of subjects with the dilution of 1:100. mean percentage of sickle red cells with dilutions of 1:50, 1:100 and 1:200 were 3.75%, 25.75% and 43.0%, respectively. conclusions it is suggested that c. fistula may protect rbc against sickling, in hypoxic conditions, in individuals with hbs gene defect. objectiv...

BACKGROUND Iron is vital for all the living organisms. However, excess iron is hazardous because it produces free radical formation. Therefore, the iron absorption is carefully regulated to maintain an equilibrium between the absorption and the body loss of iron. Considering the lack of specific excretory pathways for iron in humans, an iron overload in the tissues is frequently encountered. It...

The high frequency of the sickle-cell haemoglobin (HbS) gene in malaria endemic regions is believed to be due to a heterozygote (HbAS) advantage against fatal malaria. Data to prospectively confirm the protection associated with HbAS against mortality are lacking. We show that HbAS provides significant protection against all-cause mortality, severe malarial anaemia, and high-density parasitaemi...

INTRODUCTION Haematuria is one of the clinical manifestations of sickle cell nephropathy. Although dipstick urinalysis detects haemoglobin and by extension haematuria; it does not confirm haematuria. Urine sediment microscopy confirms haematuria and constitutes a non-invasive "renal biopsy". The need to correlate dipstick urinalysis and urine sediment microscopy findings becomes important becau...

AIMS Patients with sickle cell disease have significant morbidity and mortality. Pulmonary hypertension is suggested to be an important contributor but its nature and severity in these patients and how best to non-invasively assess it are controversial. We hypothesised that a high-output state rather than primary pulmonary vascular pathology may be the major abnormality in sickle cell disease. ...

Sickel cell disease is common in urban areas of Britain and it is estimated that in London alone there are nearly 2000 patients. One hundred and eighty four patients with sickle cell disease are known to the Central Middlesex Hospital, and 155 of those attend the sickle cell clinic regularly. The commonest cause for admission to hospital is acute painful or vaso-occlusive crisis, which accounts...

The blood picture of children with sickle-cell anaemia was found to change with age. The changes were most marked in the first year but the mean level of haemoglobin, haemoglobin F, and target cells fell until adolescence, and irreversibly sickled cells rose. Reticulocytes, Howell Jolly bodies, and normablasts altered little after one year. The fall in haemoglobin F suggested a delayed changeov...