This book is a completely revised new edition of the definitive reference on disorders of hemoglobin. Authored by world-renowned experts, the book focuses on basic science aspects and clinical features of hemoglobinopathies, covering diagnosis, treatment, and future applications of current research. While the second edition continues to address the important molecular, cellular, and genetic components, coverage of clinical issues has been significantly expanded, and there is more practical emphasis on diagnosis and management throughout. The book opens with a review of the scientific underpinnings. Pathophysiology of common hemoglobin disorders is discussed next in an entirely new section devoted to vascular biology, the erythrocyte membrane, nitric oxide biology, and hemolysis. Four sections deal with Ip and I² thalassemia, sickle cell disease, and related conditions, followed by special topics. The second edition concludes with current and developing approaches to treatment, incorporating new agents for iron chelation, methods to induce fetal hemoglobin production, novel treatment approaches, stem cell transplantation, and progress in gene therapy.SECTION TWO PATHOPHYSIOLOGY OF HEMOGLOBIN AND ITS DISORDERS Martin H. Steinberg One convenient classification of hemoglobin ... and thalassemias can have similar pathophysiological effects, and result in similar clinical features, such generalizations do not apply to all hemoglobin disorders. ... The erythrocyte membrane is a complex dynamic structure with multiple regulated functions.
|Title||:||Disorders of Hemoglobin|
|Author||:||Martin H. Steinberg, Bernard G. Forget, Douglas R. Higgs, David J. Weatherall|
|Publisher||:||Cambridge University Press - 2009-08-17|