The work presented in this book is an attempt at a comprehensive statement of the natural history of the sickle cell disease patient and is based upon more than 1500 consecutive patients personally observed and followed up by the author for more than five years. Comments and discussions are, however, based on experience acquired from active supervision of a total of over 3,000 sickle cell disease patients in Ghana between 1965 and 1977, giving a rough experience value of close to 30,000 patient-years. Sickle cell disease is defined and the various genotypes and phenotypes comprising the disease are clarified. More than 70 years of actively expanding literature has been reviewed, including new information on the molecular pathology and pathophysiology of these haemoglobinopathies. The epidemiology of sickle cell disease, together with a catalogue of clinical features and complications, as witnessed by the author in Accra, have, with the aid of computers, been stated in quantitative terms. The work shows that sickle cell disease is a problem of great magnitude, deserving of national and international attention.
An approach to treatment is given, with greater emphasis on patient management than is usual, paying attention to aggravating factors such as infections within poor socio-economic conditions and stressing the importance of genetic counselling and family planning for patients.
Sprache
Verlagsort
Zielgruppe
Für höhere Schule und Studium
Für Beruf und Forschung
Maße
Höhe: 246 mm
Breite: 189 mm
Dicke: 35 mm
Gewicht
ISBN-13
978-0-333-39239-3 (9780333392393)
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Schweitzer Klassifikation
Part 1 Historical survey and review of the literature: historical observations; sickle cell disease in successive Ghanaian generations for 3 centuries; some general observations on the genetic expression of sickle cell disease; sickle cell disease as an haemoglobinopathy; the molecular pathology of sickle cell haemoglobin and other haemoglobin variants; genetic control of haemoglobin synthesis. Part 2 Clinical features 1: history of sickle cell disease in the world; balanced polymorphism and other factors relating to hereditary qualitative and quantitative erythrocyte defects; the pathgenesis of sickle cell disease; the crisis in sickle cell disease. Part 3 Clinical features 2: patients and methods, the sickle cell/haemoglobinopathy clinic of Korle Bu teaching hospital; rheumatism, dactylitis and seasonal epidemiology of sickle cell crises in Accra; pallor and jaundice in sickle cell disease; gnathopathy, bossing of skull bones and finger clubbing in sickle cell disease; body habitus and growth in sickle cell disease; sickle cell dermathopathy in Accra; epistaxis and haematuria - two bleeding tendencies in sickle cell states; sickle cell ophthalmopathy; sickle cell priapism; articular bone neurosis in scikle cell disease; skeletal involvement in sickle cell disease; abdominal pain in sickle cell disease; the liver in sickle cell disease; the spleen and lymphnodes in sickle cell disease; cardio-respiratory involvement in sickle cell disease; sickle cell disease and the central nervous system; some quantitative aspects of pregnancy in sickle cell disease in Accra; notes on some miscellaneous clinical features of sickle cell disease. Part 4 Some laboratory and other findings in sickle cell disease: haemotological profile in sickle cell disease; percentage values of haemoglobins S,F,A2,C and A is various sickle cell phenotypes and the position of the sickle cell trait; some aspects of renal function in sickle cell disease; some radio-active isotope patterns of sickle cell disease in Accra. Part 5 Management and conclusions: an approach to the treatment of sickle cell disease; the prognostic and public health aspects of sickle cell disease in Accra; the socio-politics of "Sickle Cell"; conclusions - the whole person. Appendices: Case histories; some laboratory investigations; Korle Bu hospital schedule for emergency treatment of sickle cell crisis; list of some sickle cell disease patient orientated centres in the world.
