An Introduction to Primary Immunodeficiency Diseases
Primary immunodeficiency diseases are a heterogeneous group of inherited disorders with defects in one or more components of the immune system. Primary immunodeficiency diseases are not as rare as once believed. Infections are the hallmark of immunodefici
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Nima Rezaei Asghar Aghamohammadi • Luigi D. Notarangelo (Eds.)
Primary Immunodeficiency Diseases Definition, Diagnosis, and Management
With 55 Figures, mostly in Color
Editor-in-Chief Nima Rezaei Immunology, Asthma and Allergy Research Institute Children’s Medical Center Hospital Tehran University of Medical Sciences Tehran 14194 Iran and Section of Infection, Inflammation and Immunity School of Medicine and Biomedical Sciences The University of Sheffield S10 2RX, Sheffield UK Editorial Consultants Asghar Aghamohammadi Division of Allergy and Clinical Immunology Children’s Medical Center Hospital Tehran University of Medical Sciences Tehran 14194 Iran Luigi D. Notarangelo Division of Immunology Children’s Hospital Harvard Medical School Boston, MA 02115 USA
ISBN 978-3-540-78537-8
e-ISBN 978-3-540-78936-9
Library of Congress Control Number: 2008932110 © 2008 Springer-Verlag Berlin Heidelberg This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilm or in any other way, and storage in data banks. Duplication of this publication or parts thereof is permitted only under the provisions of the German Copyright Law of September 9, 1965, in its current version, and permissions for use must always be obtained from Springer-Verlag. Violations are liable for prosecution under the German Copyright Law. The use of general descriptive names, registered names, trademarks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. Product liability: The publishers cannot guarantee the accuracy of any information about dosage and application contained in this book. In every individual case the user must check such information by consulting the relevant literature. Cover design: Frido Steinen, estudio Calamar, Spain Printed on acid-free paper 5 4 3 2 1 0 springer.com
Dedication
This book would not have been possible without the continuous encouragement by our parents and our wives, Maryam, Soheila and Evelina. We wish to dedicate it to our children, Ariana, Hamid Reza, Fatemeh, Claudio, Marco and Giulia, with the hope that progress in diagnosis and management of these diseases may result in improved survival and quality of life for the next generations, and at the same time that international collaboration in research will happen without barriers. Whatever we have learnt, comes from our mentors. This book is therefore also dedicated to all of them, but most importantly to our patients and their families whose continuous support has guided us during the years.
Foreword
This new text on Primary Immunodeficiency Diseases covers all aspects of these fascinating disorders. So much basic immunology has been learned from studies of these patients and so much more has still to be understood. In addition, we
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