Sickle cell disease was recognized to have a molecular basis more than 50 years ago, since when knowledge of the pathophysiology of the disease has paralleled the developments of molecular biology. Characterized by a striking red cell deformity, a great deal has been learnt about the mechanisms involved and animal models developed over the last 10 years have contributed to these studies. Despite greater understanding of the molecular mechanisms, this knowledge has had little impact in improving management at the bedside. It is the authors' belief that much of importance remains to be learnt from clinical studies which ultimately may define the questions most relevant for our molecular colleagues to answer. Jamaica has provided a superb setting for clinical studies, which initially documented the extremely variable clinical course and then proceeded to seek the determinants in a Cohort Study based on newborn diagnosis and follow-up which has continued for the past 27 years. This new edition of Sickle Cell Disease reviews the history and many of the recent developments in laboratory studies but retains the perspective of a practising clinician. A critical appraisal of the available observations and evidence has sought to define the best practice in its management.
Readership: Haematologists and all those involved in the study of management of sickle cell disease.
"" . . . this remains an excellent overview of sickle cell disease. It is well written and easy to read . . . I would consider this an excellent purchase for those with an interest in sickle cell disease."" - ACP News
Part 1 General aspects 1: Basic concepts 2: Distribution of sickle cell disease 3: Nomenclature and genetics of sickle cell disease 4: Diagnosis of sickle cell disease 5: Pathophysiology of sickle cell disease 6: Historical aspects Part 2 System review 7: The bone marrow 8: The blood 9: The liver 10: The spleen 11: The immune system 12: The gut and abdomen 13: Cardiovascular system 14: Pulmonary system 15: Leg ulceration 16: Bone and joint lesions 17: The painful crisis 18: Renal manifestations 19: Priapism 20: The nervous system 21: The eyes 22: Physical and sexual development 23: Pregnancy and contraception 24: Endocrine system, metabolism, and nutrition Part 3 Comparison of sickle cell syndromes 25: Homozygous sickle cell disease 26: Sickle cell-haemoglobin C disease 27: Sickle cell-beta thalassaemia 28: Other forms of sickle cell disease 29: Sickle cell - hereditary persistence of fetal haemoglobin 30: The sickle cell trait Part 4 Management 31: General supportive measures 32: Transfusion 33: Attempts to inhibit sickling 34: Surgery and anaesthesia 35: Screening and sickle cell clinics Part 5 Epidemiology 36: Age and patterns of clinical involvement 37: Causes of death 38: Natural history References Index
