Sickle Cell Disease: Basic Principles and Clinical Practice

Written by 80 of the world's foremost basic scientists and clinicians, this volume is the first comprehensive reference on sickle cell disease. Because this disease has diverse manifestations and involves many medical specialties, the contributors were chosen for their expertise in specific areas. Their discussions cover virtually every aspect of the disease - its molecular and cellular biology, pathophysiology, diagnosis, organ-specific complications, and clinical management. Coverage begins with recent laboratory findings on sickle hemoglobin and sickle red cell pathology. Subsequent sections examine the pathophysiology and natural history of sickle cell disease and describe diagnostic methods, including prenatal diagnosis and neonatal Screening. A major section focuses on strategies for management of the organ-specific complications and psychosocial aspects of the disease. Coverage of therapy includes antibiotics, transfusion, surgery, patient control, nursing concerns, and future prospects for cure of the disease by gene therapy, bone marrow transplantation, and drugs that induce fetal haemoglobin synthesis.