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Emergency Management of Thalassaemia

Emergency Management of Thalassaemia
Author: John Porter (Hematologist)
Publisher:
Total Pages:
Release: 2012
Genre:
ISBN: 9789963717002

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The multi-organ pathology of the thalassaemias means that the most successful patient treatment programmes involve a number of medical staff, led by paediatricians and haematologists, but including other specialists in a multidisciplinary approach. However, this means that medical professionals not involved in such multidisciplinary groups, including primary care physicians and the staff of Accident and Emergency departments, rarely treat patients with thalassaemia. As a result, their knowledge of how to address acute complications in such patients is extremely limited. This handbook aims to provide a brief evaluation of the situations in which a thalassaemia patient may seek help beyond the specialized environment he/she is used to. The layout of the handbook is intended to enable the medical professional in a busy clinical setting to draw essential information at a glance, supporting rapid, accurate as possible, decisions on how to proceed.


The Obstetric Hematology Manual

The Obstetric Hematology Manual
Author: Sue Pavord
Publisher: Cambridge University Press
Total Pages: 362
Release: 2018-02-08
Genre: Medical
ISBN: 1108548377

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Understand the rapidly growing complexities of obstetric hematology and high-risk pregnancy management, with experts in the field. Now in its second edition, this comprehensive and essential guide focuses on providing the best support for patients and clinical staff, to prevent serious complications in pregnancy and the post-partum period for both mother and baby. Wide-ranging and detailed, the guide offers discussions on basic principles of best care, through to tackling lesser-known hematological conditions, such as cytopenias and hemoglobinopathies. Updated with color illustrations, cutting-edge research, accurate blood film reproductions, and practical case studies, the revised edition places invaluable advice into everyday context. This unique resource is essential reading for trainees and practitioners in obstetrics, anesthesia, and hematology, as well as midwives, nurses, and laboratory staff. Clarifying difficult procedures for disease prevention, the guide ensures safety when the stakes are high. Reflecting current evidence-based guidelines, the updated volume is key to improving pregnancy outcomes worldwide.


Iron Chelation Therapy

Iron Chelation Therapy
Author: Chaim Hershko
Publisher: Springer Science & Business Media
Total Pages: 275
Release: 2012-12-06
Genre: Science
ISBN: 1461505933

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Within the last few years, iron research has yielded exciting new insights into the under standing of normal iron homeostasis. However, normal iron physiology offers little protec tion from the toxic effects of pathological iron accumulation, because nature did not equip us with effective mechanisms of iron excretion. Excess iron may be effectively removed by phlebotomy in hereditary hemochromatosis, but this method cannot be applied to chronic anemias associated with iron overload. In these diseases, iron chelating therapy is the only method available for preventing early death caused mainly by myocardial and hepatic iron toxicity. Iron chelating therapy has changed the quality of life and life expectancy of thalassemic patients. However, the high cost and rigorous requirements of deferoxamine therapy, and the significant toxicity of deferiprone underline the need for the continued development of new and improved orally effective iron chelators. Such development, and the evolution of improved strategies of iron chelating therapy require better understanding of the pathophysiology of iron toxicity and the mechanism of action of iron chelating drugs. The timeliness of the present volume is underlined by several significant develop ments in recent years. New insights have been gained into the molecular basis of aberrant iron handling in hereditary disorders and the pathophysiology of iron overload (Chapters 1-5).


Prevention of Thalassaemias and Other Haemoglobin Disorders

Prevention of Thalassaemias and Other Haemoglobin Disorders
Author: Galanello Renzo
Publisher:
Total Pages: 190
Release: 2003
Genre:
ISBN: 9789963623396

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Volume 1 of the Prevention Book presents the principles of a programme for the prevention of the thalassaemia and other haemoglobin disorders, including a description of the various types of disorders requiring prenatal diagnosis, the strategies used for carrier screening, and a number of annexes listing upto date epidemiological and mutation data on thalassaemia. This book was written for use in combination with Volume 2, which describes many of the laboratory protocols in great detail.


The Thalassemias

The Thalassemias
Author: D. J. Weatherall
Publisher:
Total Pages: 186
Release: 1983
Genre: Medical
ISBN:

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Gene and Cell Therapies for Beta-Globinopathies

Gene and Cell Therapies for Beta-Globinopathies
Author: Punam Malik
Publisher: Springer
Total Pages: 254
Release: 2017-11-09
Genre: Medical
ISBN: 1493972995

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Hemoglobin defects, specifically sickle cell disease & thalassemia, combined, constitute the most common monogenic disorders in the world. In fact, nearly 2% of the world’s population carries a globin gene mutation. The transfer of the corrective globin gene through the HSC compartment by allogeneic HSC transplantation (HSCT) has already proven curative in both SCD and thalassemia patients, and provides the proof of concept that genetic manipulation of the defective organ might be equally therapeutic. However, procedural toxicities and the requirement of an HLA-matched sibling donor limit this approach to a fraction of affected individuals. The editors review the progress & the state of the field in HSCT for hemoglobinopathies & shed light on the major changes expected in the next decade. Although allogeneic HSCT is a curative option, it is limited by the availability of matched donors, which are often available only to 15-20% of patients. An alternative to allogeneic HS CT is genetic correction of autologous HSCs, to overcome donor availability & immune side effects. This Book reviews the progress made on additive gene therapy approaches & the current state of the field. Finally, targeted genetic correction is emerging as a novel therapeutic strategy in the hemoglobinopathies. Although ideal, the inefficiency of targeted correction was rate limiting for translation of this technology to the clinic. With advancements in zinc finger nucleases and TALE endonuclease mediated targeted correction, correction frequencies in hematopoietic stem cells is now reaching levels that may become clinically relevant. Furthermore, the ability to generate autologous embryonic stem cell like cells from primary somatic cells (skin fibroblasts or hematopoietic cells) of the affected individual has allowed for the potential application of genetic correction strategies.This Book reviews upcoming genetic strategies to reactivate fetal hemoglobin production and research advances.


Williams Hematology, 9E

Williams Hematology, 9E
Author: Kenneth Kaushansky
Publisher: McGraw Hill Professional
Total Pages: 2528
Release: 2015-12-23
Genre: Medical
ISBN: 0071833013

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Publisher's Note: Products purchased from Third Party sellers are not guaranteed by the publisher for quality, authenticity, or access to any online entitlements included with the product. The world's most highly regarded reference text on the mechanisms and clinical management of blood diseases A Doody's Core Title for 2019! Edition after edition, Williams Hematology has guided generations of clinicians, biomedical researchers, and trainees in many disciplines through the origins, pathophysiological mechanisms, and management of benign and malignant disorders of blood cells and coagulation proteins. It is acknowledged worldwide as the leading hematology resource, with editors who are internationally regarded for their research and clinical achievements and authors who are luminaries in their fields. The Ninth Edition of Williams Hematology is extensively revised to reflect the latest advancements in basic science, translational pathophysiology, and clinical practice. In addition to completely new chapters, it features a full-color presentation that includes 700 photographs, 300 of which are new to this edition, and 475 illustrations. Recognizing that blood and marrow cell morphology is at the heart of diagnostic hematology, informative color images of the relevant disease topics are conveniently integrated into each chapter, allowing easy access to illustrations of cell morphology important to diagnosis. Comprehensive in its depth and breath, this go-to textbook begins with the evaluation of the patient and progresses to the molecular and cellular underpinnings of normal and pathological hematology. Subsequent sections present disorders of the erythrocyte, granulocytes and monocytes, lymphocytes and plasma cells, malignant myeloid and lymphoid diseases, hemostasis and thrombosis, and transfusion medicine.