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27 May 2017
The Sickle Cell Disease Patient - Contents

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Book Comments

No. Item Page
  Foreword ix
  Preface X
  Acknowledgements xii
  Introduction xv
Section One: Historical survey and review of the literature 1
1 Historical study and first observations 3
2 Sickle cell disease in successive Ghanaian generations for three centuries 6
3 Some general observations on the genetic expression of sickle cell disease 21
4 Sickle cell disease as a haemoglobinopathy 26
5 The molecular pathology of sickle cell haemoglobin and other haemoglobin variants 37
6 Genetic control of haemoglobin synthesis 49
Section Two: Clinical features: 1 73
7 The history and geographical distribution of sickle cell disease 75
8 Balanced polymorphism and other factors relating to hereditary qualitative and quantitative erythrocyte defects 91
9 The pathogenesis of sickle cell disease 109
10 The crisis in sickle cell disease 115
Section Three: Clinical features: 2 123
11 Patients and methods 125
12 'Rheumatism', dactylitis and seasonal epidemiology of sickle cell crises in West Africa 162
13 Pallor and jaundice in sickle cell disease 171
14 Gnathopathy, bossing of skull bones, and finger clubbing in sickle cell disease patients 178
15 Body habitus and growth in sickle cell disease 189
16 Sickle cell dermatopathy in Ghana 198
17 Epistaxis and haematurla: two bleeding tendencies in sickle cell states 205
18 Sickle cell ophthalmopathy 212
19 Sickle cell priapism

226

20 Articular bone necrosis in sickle cell disease 236
21 Skeletal involvement in sickle cell disease 243
22 Abdominal pain in sickle cell disease 252
23 The liver in sickle cell disease 258
24 The spleen and lymph nodes in sickle cell disease 277
25 Cardio-respiratory involvement in sickle cell disease 292
26 Sickle cell disease and the central nervous system 306
27 Some quantitative aspects of pregnancy in sickle cell disease 318
28 Notes on some miscellaneous clinical features of sickle cell disease, clinic attendance, hospital admissions and mortality 325
Section Four: Some laboratory and other findings in sickle cell disease 339
29 Haematological profile in sickle cell disease 341
30 Percentage values of hacmoglobins S F A2 C A in various sickle cell phenotypes, and consideration of the sickle cell trait 349
31 Some aspects of renal function in sickle cell disease 372
32 Some radioactive isotope patterns of sickle cell disease in Accra 387
Section Five: Management and Conclusions 407
33 An approach to the management of the sickle cell disease patient 409
34 The prognostic and public health aspects of sickle cell disease in Ghana 428
35 Some miscellaneous aspects of sickle cell 445
36 Conclusion - The whole person 458
  Appendices 467
A Illustrative case histories 469
B Recommendations for abnormal haemoglobin standard 518
C Emergency treatment in sickle cell crisis 531
D Various public information forms 533
E African oriented education and research in clinical genetics 536
F Where to get help for sickle cell disease patients 540
  General references 547
  Index 635

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