The management of sickle-cell crisis in a pregnant patient by exchange transfusion is described, the procedure leading to immediate and dramatic improvement in the condition. Partial exchange transfusion in three other patients with sickle-cell anaemia, judged by episodes of crisis in previous pregnancies to be at particular risk, is also reported and the value of this method of management disc...

The infrequent occurrence of gout in patients with sickle cell anaemia contrast with the high incidence of hyperuricaemia and impaired renal function. This report records the third case of synovial membrane uric acid deposition and the first case of tophaceous deposits in haemoglobin SS patients. The limitations of a diagnosis of gout on the basis of hyperuricaemia and arthritis are confirmed. ...

BACKGROUND & OBJECTIVES Children with sickle cell disease require more frequent hospital care and younger children (<5 yr of age) are more vulnerable to mortality. There are limited data on the events leading to hospitalizations and death in younger children with sickle cell disease from India. This study was, therefore, undertaken to evaluate the morbidity pattern in hospitalized under five ch...

THE increase in the coloured population in Great Britain during the past 10 years has given an opportunity of observing a disease which has hitherto been reported mainly in the American literature and in papers submitted by clinicians in West Africa. According to the Home Office, there are approximately 115,000 West Indians and 25,000 West Africans in the United Kingdom. In 1950-53 the net inwa...

Sickle cell disease is a multisystem characterised by chronic haemolytic anaemia, painful vaso-occlusive crises and acute end-organ damage. It one of the most common serious inherited single gene conditions worldwide has major impact on health affected individuals. Peri-operative complications are higher in patients with sickle compared general population may be or non-sickle-related. Complicat...

INTRODUCTION Homozygous sickle cell carriers have an increased perioperative mortality. Some indications may justify an exchange blood transfusion to reduce the proportion of haemoglobin S. The advantages of general blood transfusion in a perioperative setting have not been proven and thus remain controversial. It is not clear whether reducing the proportion of haemoglobin S minimizes periopera...

The haemoglobinopathies are a heterogenous group of disorders which have highly variable clinical manifestations. At one end of the spectrum there is incompatibility with life and, at the other end, the patient under a stress, such as pregnancy, may experience some deterioration in her normal healthy state. Outside this wide range lie the symptomless carrier states which may demand obstetric ac...