Disorders of hemoglobin: genetics, pathophysiology, and clinical management
Saved in:
| Format: | Book |
|---|---|
| Language: | English |
| Published: |
Cambridge
Cambridge Univ. Press
2001
|
| Edition: | 1. publ. |
| Subjects: | |
| Online Access: | Inhaltsverzeichnis |
| Physical Description: | XIV, 1268 S. Ill., graph. Darst. |
| ISBN: | 0521632668 |
Staff View
MARC
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| 245 | 1 | 0 | |a Disorders of hemoglobin |b genetics, pathophysiology, and clinical management |c ed. by Martin H. Steinberg ... |
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| 650 | 7 | |a Hemoglobine |2 gtt | |
| 650 | 4 | |a Anemia, Sickle Cell | |
| 650 | 4 | |a Hemoglobinopathies | |
| 650 | 4 | |a Hemoglobinopathy | |
| 650 | 4 | |a Thalassemia | |
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Record in the Search Index
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|---|---|
| adam_text | Contents
Contributors page ix
Foreword xj
H. Franklin Bunn
Introduction xiii
Martin H. Steinberg, Bernard G. Forget, Douglas R. Higgs,
and Ronald L. Nagel
1 Hemoglobin: A Historical Perspective 1
Helen M. Ranney
SECTION ONE: THE MOLECULAR, CELLULAR, AND GENETIC BASIS
OF HEMOGLOBIN DISORDERS 25
Bernard G. Forget and Douglas R. Higgs
2 A Developmental Approach to Hematopoiesis 27
Elaine Dzierzak
3 Erythropoiesis 52
Anna Rita Migliaccio and Thalia Papayannopoulou
4 Nuclear Factors that Regulate Erythropoiesis 72
Gerd A. Blobel and Mitchell). Weiss
5 Organization, Evolution, and Regulation of the Globin Genes 95
Ross Hardison
6 Molecular Genetics of the Human Globin Genes 117
Bernard G. Forget
7 Molecular and Cellular Basis of Hemoglobin Switching 131
George Stamatoyannopoulos
8 Posttranscriptional Factors Influencing the Hemoglobin Content
of the Red Cell 146
Shu Ching Huang and Edward ]. Benz, Jr.
9 Molecular Anatomy and Physiology of Hemoglobin 174
Max F. Perutz
10 Hemoglobins of the Embryo and Fetus and Minor Hemoglobins of Adults 197
Ronald L. Nagel and Martin H. Steinberg
vi CONTENTS
SECTION TWO: THE p THALASSEMIAS 231
Bernard G. Forget
11 Pathophysiology of P Thalassemia 233
Eliezer A. Rachmilewitz and Stanley L. Schrier
12 Molecular Mechanisms of P Thalassemia 252
Bernard G. Forget
13 Clinical Aspects of P Thalassemia 277
Nancy Olivieri and D. J. Weatherall
14 Strucutural Variants with a p Thalassemia Phenotype 342
Swee Lay Thein
15 Hereditary Persistence and Fetal Hemoglobin and 8p Thalassemia 356
W. G. Wood
SECTION THREE: a THALASSEMIA 389
Douglas R. Higgs
16 Pathophysiology of a Thalassemia 391
Steve A. Liebhaber and Stanley L. Schrier
17 Molecular Mechanisms of a Thalassemia 405
Douglas R. Higgs
18 Clinical and Laboratory Features of oc Thalassemia Syndromes 431
Douglas R. Higgs and Donald K. Bowden
19 The Alpha Thalassemia/Mental Retardation Syndrome 470
Richard J. Gibbons and Douglas R. Higgs
SECTION FOUR: SICKLE CELL DISEASE 489
Martin H. Steinberg and Ronald L. Nagel
20 General Pathophysiology of Sickle Cell Anemia 494
Ronald L. Nagel and Orah S. Platt
21 Cell Adhesion and Microheology in Sickle Cell Disease 527
Robert P. Hebbel and Narla Mohandas
22 Red Cell Membrane in Sickle Cell Disease 550
Carlo Brugnara
23 Polymer Structure and Polymerization of Deoxyhemoglobins 577
Frank Ferrone and Ronald L. Nagel
24 Clinical Aspects of Sickle Cell Anemia in Adults and Children 611
Kwaku Ohene Frempong and Martin H. Steinberg
25 Nature and Treatment of the Acute Painful Episode in Sickle Cell Disease 671
Lennette J. Benjamin
26 Genetics of the Ps Gene: Origins, Epidemiology, and Epistasis
in Sickle Cell Anemia 711
Ronald L. Nagel and Martin H. Steinberg
27 Hemoglobin SC Disease and HbC Disorders 756
Ronald L. Nagel and Martin H. Steinberg
28 Compound Heterozygous and Other Sickle Hemoglobinopathies 786
Martin H. Steinberg
29 Sickle Cell Trait 811
Martin H. Steinberg
SECTION FIVE: EPIDEMIOLOGY AND GENETIC SELECTION
OF HEMOGLOBINOPATHIES AND THALASSEMIA 831
Ronald L. Nagel
30 Malaria and Hemoglobinopathies 832
Ronald L. Nagel
CONTENTS
31 Worldwide Distribution of P Thalassemia 861
Dimitris Loukopoulos and Panagoula Kollia
32 Geographic Distribution of a Thalassemia 878
Luigi E Bernini
33 Geographic Heterogeneity of Sickle Cell Disease 895
Graham Serjeant
SECTION SIX: DIAGNOSIS AND SPECIAL TREATMENTS FOR
SICKLE CELL DISEASE AND P THALASSEMIA 907
Martin H. Steinberg and Ronald L. Nagel
34 Laboratory Diagnosis of Hemoglobin Disorders and Animal Models
for Their Study 910
Mary E. Fabry
35 DNA Based Diagnosis of Hemoglobin Disorders 941
/. M. Old
36 Prenatal Diagnosis and Screening for Thalassemia and Sickle Cell Disease 958
Antonio Cao, Maria Cristina Rosatelli, and James R. Eckman
37 Transfusion and Iron Chelation Therapy in Thalassemia
and Sickle Cell Anemia 979
Alan R. Cohen and John B. Porter
38 Pharmacologic Treatment of Sickle Cell Disease and Thalassemia:
The Augmentation of Fetal Hemoglobin 1028
Griffin P. Rodgers and Martin H. Steinberg
39 Bone Marrow Transplantation in P Thalassemia 1052
Emanuele Angelucci and Guido Lucarelli
40 Bone Marrow Transplantation in Sickle Cell Anemia 1073
Christiane Vermylen
41 Prospects for Gene Therapy of Sickle Cell Disease and Thalassemia 1084
Brian P. Sorrentino and Arthur W. Nienhuis
42 Experimental Therapies for Sickle Cell Anemia and P Thalassemia 1119
Yves Beuzard and Lucia De Franceschi
SECTION SEVEN: OTHER INHERITED DISORDERS OF HEMOGLOBIN 1137
Ronald L. Nagel
43 Hemoglobin E Disorders 1139
Suthat Fucharoen
44 Disorders of Hemoglobin Function and Stability 1155
Ronald L. Nagel
45 Native and Recombinant Mutant Hemoglobins of Biological Interest 1195
Martin H. Steinberg and Ronald L. Nagel
SECTION EIGHT: ACQUIRED DISORDERS OF HEMOGLOBIN 1213
Ronald L. Nagel
46 CO , NO , Met , and Sulf Hemoglobinemias: The Dyshemoglobins 1214
Ronald L. Nagel and Ernst R. Jaffe
Index 1235
|
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| illustrated | Illustrated |
| indexdate | 2024-07-09T18:43:22Z |
| institution | BVB |
| isbn | 0521632668 |
| language | English |
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| physical | XIV, 1268 S. Ill., graph. Darst. |
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| spelling | Disorders of hemoglobin genetics, pathophysiology, and clinical management ed. by Martin H. Steinberg ... 1. publ. Cambridge Cambridge Univ. Press 2001 XIV, 1268 S. Ill., graph. Darst. txt rdacontent n rdamedia nc rdacarrier Hemoglobine gtt Anemia, Sickle Cell Hemoglobinopathies Hemoglobinopathy Thalassemia Hämoglobinopathie (DE-588)4022815-0 gnd rswk-swf Hämoglobinopathie (DE-588)4022815-0 s DE-604 Steinberg, Martin H. Sonstige oth HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=009067346&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
| spellingShingle | Disorders of hemoglobin genetics, pathophysiology, and clinical management Hemoglobine gtt Anemia, Sickle Cell Hemoglobinopathies Hemoglobinopathy Thalassemia Hämoglobinopathie (DE-588)4022815-0 gnd |
| subject_GND | (DE-588)4022815-0 |
| title | Disorders of hemoglobin genetics, pathophysiology, and clinical management |
| title_auth | Disorders of hemoglobin genetics, pathophysiology, and clinical management |
| title_exact_search | Disorders of hemoglobin genetics, pathophysiology, and clinical management |
| title_full | Disorders of hemoglobin genetics, pathophysiology, and clinical management ed. by Martin H. Steinberg ... |
| title_fullStr | Disorders of hemoglobin genetics, pathophysiology, and clinical management ed. by Martin H. Steinberg ... |
| title_full_unstemmed | Disorders of hemoglobin genetics, pathophysiology, and clinical management ed. by Martin H. Steinberg ... |
| title_short | Disorders of hemoglobin |
| title_sort | disorders of hemoglobin genetics pathophysiology and clinical management |
| title_sub | genetics, pathophysiology, and clinical management |
| topic | Hemoglobine gtt Anemia, Sickle Cell Hemoglobinopathies Hemoglobinopathy Thalassemia Hämoglobinopathie (DE-588)4022815-0 gnd |
| topic_facet | Hemoglobine Anemia, Sickle Cell Hemoglobinopathies Hemoglobinopathy Thalassemia Hämoglobinopathie |
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