Sickle Cell Disease

Preface. Basic concepts; distribution of sickle cell disease; nomenclature and genetics of sickle cell disease; diagnosis of sickle cell disease; pathophysiology of sickle cell disease; historical aspects; the bone marrow; the blood; the liver; the spleen; the immune system; the gut; cardiovascular system; pulmonary system; leg ulceration; bone and joint lesions; the painful crisis; renal manifestations; priapism; the nervous system; the eyes; physical and sexual development; pregnancy and contraception; homozygous sickle cell disease; sickle cell - haemoglobin C disease; sickle cell - beta thalassaemia; other forms of sickle cell disease; sickle cell - hereditary persistence of fetal haemoglobin; the sickle cell trait; age and patterns of clinical involvement; causes of mortality; general supportive measures; blood transfusion; therapeutic attempts to inhibit sickling; surgery and anaesthesia; screening and sickle cell clinics; the epidemiology of sickle cell disease. References. Index.