简介
Summary:
Publisher Summary 1
New edition of a text on thalassemias that summarizes the evidence suggesting that the clinical picture of this disorder may result from several different inherited defects of globin synthesis similar in type to the other inherited hemoglobinopathies. The 16 chapters reflect the vast amount of new information obtained about the genetic control of hemoglobin in general and the thalassaemias in particular, which are likely to pose an increasingly serious health problem in this century. Discussion includes historical background; biology, clinical features, and diagnosis and management of thalassaemia; and the future. Written and edited by D. J. Weatherall and J.B. Clegg (both of the molecular haematology unit, Institute of Molecular Medicine, U. of Oxford) with additional contributions by R. Gibbons, J.M. Old, Swee Lay Thein, and W.G. Wood (all associated with the U. of Oxford), and Nancy F. Oliveri (Hospital for Sick Children and the Toronto General Hospital). Annotation c. Book News, Inc., Portland, OR (booknews.com)
Publisher Summary 2
In the new edition of this successful and authoritative book, the thalassaemias are reviewed in detail with respect to their clinical features, cellular pathology, molecular genetics, prevention and treatment. It is aimed at specialists in haematology in the laboratory or clinical setting, particularly in areas where thalassaemia is common either in the native population or in immigrant communities.
The fourth edition has been both updated and re-organized. Three new chapters have been added on the link between alpha-thalassaemia and mental retardation, on avoidance and population control and on global epidemiology. Considerable emphasis is placed on molecular pathology reflecting the huge burst of information to have come out of this field in the last few years.
目录
Table Of Contents:
Authors and contributors vi
Preface to the fourth edition vii
Preface to the first edition ix
Acknowledgements x
Part 1 Historical background
Historical perspectives: the many and diverse routes to our current understanding of the thalassaemias 3(62)
Part 2 The biology of the thalassaemias
D.R. Higgs
Swee Lay Thein
W.G. Wood
Human haemoglobin 65(56)
Thalassaemia: classification, genetics and relationship to other inherited disorders of haemoglobin 121(12)
The molecular pathology of the thalassaemias 133(59)
The pathophysiology of the thalassaemias 192(45)
Distribution and population genetics of the thalassaemias 237(50)
Part 3 Clinical features of the thalassaemias
R. Gibbons
D.R. Higgs
Nancy F. Olivieri
W.G. Wood
The β thalassaemias 287(70)
The δβ and related thalassaemias 357(36)
The β and δβ thalassaemias in association with structural haemoglobin variants 393(57)
Hereditary persistence of fetal haemoglobin 450(34)
The α thalassaemias and their interaction with structural haemoglobin variants 484(42)
α Thalassaemia with mental retardation or myelodysplasia 526(24)
Thalassaemia intermedia 550(47)
Part 4 Diagnosis and management of thalassaemia
J.M. Old
Nancy F. Olivieri
Swee Lay Thein
Avoidance and population control 597(33)
Management and prognosis 630(56)
The laboratory diagnosis of the thalassaemias 686(47)
Part 5
The future 727(6)
References 733(90)
Appendix: Addresses of patient support organizations 823(4)
Index 827
Authors and contributors vi
Preface to the fourth edition vii
Preface to the first edition ix
Acknowledgements x
Part 1 Historical background
Historical perspectives: the many and diverse routes to our current understanding of the thalassaemias 3(62)
Part 2 The biology of the thalassaemias
D.R. Higgs
Swee Lay Thein
W.G. Wood
Human haemoglobin 65(56)
Thalassaemia: classification, genetics and relationship to other inherited disorders of haemoglobin 121(12)
The molecular pathology of the thalassaemias 133(59)
The pathophysiology of the thalassaemias 192(45)
Distribution and population genetics of the thalassaemias 237(50)
Part 3 Clinical features of the thalassaemias
R. Gibbons
D.R. Higgs
Nancy F. Olivieri
W.G. Wood
The β thalassaemias 287(70)
The δβ and related thalassaemias 357(36)
The β and δβ thalassaemias in association with structural haemoglobin variants 393(57)
Hereditary persistence of fetal haemoglobin 450(34)
The α thalassaemias and their interaction with structural haemoglobin variants 484(42)
α Thalassaemia with mental retardation or myelodysplasia 526(24)
Thalassaemia intermedia 550(47)
Part 4 Diagnosis and management of thalassaemia
J.M. Old
Nancy F. Olivieri
Swee Lay Thein
Avoidance and population control 597(33)
Management and prognosis 630(56)
The laboratory diagnosis of the thalassaemias 686(47)
Part 5
The future 727(6)
References 733(90)
Appendix: Addresses of patient support organizations 823(4)
Index 827
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