Controversies in Pediatric and Adolescent Hematology

Description

Hematological disorders in children and adolescents pose a wide range of management challenges and treatment dilemmas. In this book an internationally acclaimed panel of authors, each chosen for expertise in their field, have produced a state-of-the-art collection of review articles focusing on the very latest advances and controversies in the management of pediatric and adolescent hematological problems. The whole range of benign and malignant, congenital and acquired, acute and chronic conditions is discussed in detail. Individual chapters cover hematologic problems on the pediatric intensive care unit, treatments for iron deficiency and ITP; advances in stem cell transplantation, gene therapy, novel pharmaceutics and molecular diagnostics, as well as transition from child to adult are also explored. Providing an up-to-date look at both specific hematologic disorders in the pediatric and adolescent population and also hematologic problems that arise in association with systemic disease, this book is essential reading not only for pediatric and adult hematologists but also for pediatricians, pediatric or hematologic specialist nurse practitioners and pediatric pharmacologists.

Informations

• Publié par: S. Karger AG
• Date de parution: 29 novembre 2013
• Nombre de lectures: 2
• EAN13: 9783318024234
• Langue: English
• Poids de l'ouvrage: 1 Mo

Extrait

Controversies in Pediatric and Adolescent Hematology
Pediatric and Adolescent Medicine
Vol. 17
Series Editors
David Branski Jerusalem
Wieland Kiess Leipzig
Controversies in Pediatric and Adolescent Hematology
Volume Editors
Angela E. Thomas Edinburgh
Christina Halsey Glasgow
13 figures, and 19 tables, 2014
Pediatric and Adolescent Medicine Founded 1991 by D. Branski, Jerusalem
_______________________ Angela E. Thomas Consultant Pediatric Hematologist Royal Hospital for Sick Children Edinburgh EH9 1LF
_______________________ Christina Halsey Consultant Pediatric Hematologist and Scottish Senior Clinical Research Fellow Institute of Infection, Immunity and Inflammation College of Medical, Veterinary and Life Sciences University of Glasgow Glasgow G12 8TA
Library of Congress Cataloging-in-Publication Data
Controversies in pediatric and adolescent hematology / volume editors, Angela E. Thomas, Christina Halsey.
p. ; cm. –– (Pediatric and adolescent medicine, ISSN 1017-5989 ; vol. 17)
Includes bibliographical references and indexes.
ISBN 978-3-318-02422-7 (hard cover: alk. paper) –– ISBN (invalid) 978-3-318-02423-4 (electronic version)
I. Thomas, Angela E., editor of compilation. II. Halsey, Christina, editor of compilation. III. Series: Pediatric and adolescent medicine ; v. 17. 1017-5989
[DNLM: 1. Child. 2. Hematologic Diseases. 3. Adolescent. 4. Infant. W1 PE163HL v. 17 2014 / WS 300]
RJ269.5
618.92'15––dc23
2013034474
Bibliographic Indices. This publication is listed in bibliographic services, including Current Contents ® .
Disclaimer. The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publisher and the editor(s). The appearance of advertisements in the book is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
Drug Dosage. The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any change in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
© Copyright 2014 by S. Karger AG, P.O. Box, CH-4009 Basel (Switzerland)
www.karger.com
Printed in Germany on acid-free and non-aging paper (ISO 9706) by Kraft Druck GmbH, Ettlingen
ISSN 1017-5989
e-ISSN 1662-3886
ISBN 978-3-318-02422-7
e-ISBN 978-3-318-02423-4
Contents
Preface
Thomas, A.E. (Edinburgh); Halsey, C. (Glasgow)
Neonatal Thrombocytopenia
Chakravorty, S.; Roberts, I. (London)
Paediatric and Adolescent Immune Thrombocytopenia: Prevention of Bleeding versus Burden of Treatment
Cooper, N. (London)
Thrombosis in Paediatrics: Genetic versus Environmental Risk Factors and Implications for Management
Monagle, P. (Melbourne, Vic.)
Hematological Problems in Pediatric Intensive Care
Revel-Vilk, S. (Jerusalem); Cox, P. (Toronto, Ont.); Robitaille, N. (Montréal, Que.); Blanchette, V. (Toronto, Ont.)
New Advances in the Treatment of Children with Hemophilia
Lillicrap, D. (Kingston, Ont.)
Myelodysplastic and Myeloproliferative Diseases in Children: Current Concepts
Vyas, P. (Oxford)
Towards Personalised Medicine in Childhood Acute Lymphoblastic Leukaemia
Halsey, C. (Glasgow)
Reduced Intensity Conditioning in Paediatric Haematopoietic Cell Transplantation
Chiesa, R.; Veys, P. (London)
Can Iron Chelators Replace Stem Cell Transplantation in the Treatment of Thalassaemia Syndromes?
Darbyshire, P.J. (Birmingham)
Iron Deficiency: When and Why Oral Iron May Not Be Enough
Crary, S.E. (Little Rock, Ark.); Buchanan, G.R. (Dallas, Tex.)
Principles of Transitional Care in Haematology
Bolton-Maggs, P.H.B.; Choudhuri, S. (Manchester)
Author Index
Subject Index
Preface
The care of infants, children and young people with hematological disorders provides a fascinating and ever changing spectrum of diagnoses, management challenges and treatment dilemmas. In infancy not only does the blood system undergo physiological changes in adaptation to the extra-uterine environment but it is also the time when congenital disease will present often for the first time within a family. During early childhood periods of rapid growth put great nutritional demands on the body and iron deficiency anemia is very common. Throughout childhood, the establishment of an immune repertoire with rapid lymphocyte proliferation in response to infectious challenge may also predispose to autoimmune disorders such as ITP or malignancies such as acute lymphoblastic leukemia. However, it is not only young children that provide challenges - adolescence is a time of great upheaval and there is increasing recognition of the unique needs of patients with lifelong conditions during their transition from pediatric to adult-centered models of care. In this volume, we focus on recent advances in the understanding and treatment of many of these neonatal, childhood and adolescent disorders. We are also delighted to include a chapter on the management of problems that may arise in the intensive care unit - either due to life-threatening presentations of primary hematological disorders or due to hematological responses secondary to significant systemic illness.
Drug therapy for pediatric disorders is particularly challenging, many drugs are unlicensed in children and it is often difficult to extrapolate optimal treatment schedules from adult data. In addition, although children often tolerate toxic treatment for conditions such as leukemia much better than adults, the impact of late effects of treatment can be much greater due to the long life expectancy. This leads to great debate and controversy: How aggressive does the treatment need to be? What are the long-term effects of treatment? Can we tailor treatment more specifically to individuals to maximize benefit while minimizing risk? These questions are addressed in this volume for a spectrum of diseases with some of the controversies moving towards resolution, for the time being at least, and translating into advances in clinical practise.
We are very grateful to the authors for their expertise and enthusiasm in helping us complete this project in a timely fashion and to the publishers for their help and encouragement with the volume. We hope that you both enjoy the debate and discussion in the book and find it a useful tool for your clinical practise.
Angela E. Thomas, Edinburgh Christina Halsey, Glasgow
Thomas AE, Halsey C (eds): Controversies in Pediatric and Adolescent Hematology. Pediatr Adolesc Med. Basel, Karger, 2014, vol 17, pp 1-15 (DOI: 10.1159/000350343)
______________________
Neonatal Thrombocytopenia
Subarna Chakravorty Irene Roberts
Centre for Haematology, Imperial College London and Department of Paediatrics, St Mary's Hospital, Imperial College Healthcare NHS Trust, London, UK
______________________
Abstract
Thrombocytopenia is the commonest haematological abnormality in neonates where the advice of a haematologist is sought and specialist management may be necessary to prevent associated mortality or long-term disability. The majority of neonates who present with thrombocytopenia within 72 h of birth are preterm and born to mothers with placental dysfunction where chronic fetal hypoxia perturbs normal platelet production. In such cases, thrombocytopenia is usually mild to moderate (platelets 50-150 × 10 9 /l) and resolves within 10 days of birth. Neonatal thrombocytopenia presenting after 72 h of age is usually due to late-onset sepsis or necrotising enterocolitis and is frequently severe and treated with multiple platelet transfusions. In term neonates, where thrombocytopenia is much less common, the clinically most important cause is neonatal alloimmune thrombocytopenia (NAIT). Around 10% of children with NAIT develop intracranial haemorrhage, which may occur prior to birth. Prompt diagnosis and transfusion of HPA-compatible platelets is key to successful management of NAIT. Recent studies indicate that many neonates with severe thrombocytopenia receive platelet transfusions based on consensus national or local guidelines despite little evidence of benefit. Controlled trials of platelet transfusion for neonatal thrombocytopenia, currently in progress, are needed to improve the management of this common complication of neonatal medicine.
Copyright © 2014 S. Karger AG, Basel
Neonatal thrombocytopenia, whilst rare in the general population, is frequently encountered in neonatal intensive care units, particularly among very low birth weight or sick neonates, up to 80% of which may be affected [