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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 α and β 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.
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Hemoglobinopathy. --- Hemoglobinopathies --- Thalassemia --- Hemoglobinopathy. --- therapy. --- therapy.
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Hemoglobin --- Hemoglobin --- Gene expression --- Hemoglobinopathy
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Hemoglobin --- X-ray crystallography. --- Hemoglobinopathy. --- Structure.
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Hemoglobinopathies --- Hemoglobins, Abnormal --- Hemoglobinopathy --- Blood --- Hémoglobinopathie --- Sang --- laboratory manuals. --- Examination. --- Examen --- Hemoglobinopathy. --- Laboratory manuals.
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Hemoglobinopathy --- Hemoglobin --- Hemoglobinopathies --- Diagnosis --- Congresses --- Analysis --- Congresses --- diagnosis
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Paroxysmal Nocturnal Hemoglobinuria (PNH) has been recognized for over a century. This mysterious disease is now understood at the level of the gene and the protein. The pathophysiology is related to a class of cell surface proteins with distinctive biochemical and physical characteristics. Recently it has been acknowledged that PNH is not rare, and once sensitive assays--based on the chemistry of the proteins--can be applied to many patients.Written by international experts in the field, this book includes a number of distinctive characteristics, such as the clinical features of PNH, the
Hemoglobinopathy. --- Anemia. --- Abnormal hemoglobin --- Hemoglobin, Abnormal --- Anemia --- Hemoglobin polymorphisms --- Anaemia --- Blood --- Diseases
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Thalassemia. --- Cooley's anemia --- Erythroblastic anemia --- Mediterranean anemia --- Mediterranean disease --- Thalassanemia --- Hemoglobinopathy --- Hemolytic anemia
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Sickle cell anemia --- Drepanocytic anemia --- Meniscocytosis --- Sickle cell disease --- Blood hyperviscosity syndrome --- Hemoglobinopathy --- Hemolytic anemia --- Treatment.
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