Handbook of Immunological Investigations in Children. Handbooks of Investigation in Children PDF
Preview Handbook of Immunological Investigations in Children. Handbooks of Investigation in Children
Handbooks of Investigation in Children This is a series of unique guides to the appropriate tests to be carried out in children with suspected disorders. Instructions for the performance and evaluation of tests are clearly explained. Each title is based on the authors' personal experience in the respective field and is devoted to the investigation of children only. Careful reference to these titles in clinical practical will help both to eliminate inadequate testing and to ensure that the practitioner will obtain the maximum information from the investigations carried out. To amplify the explicit text, case histories helpfully illustrate how the authors have used and interpreted investigations. These pocket-sized books are essential tools for all those involved in the diagnosis and management of childhood disorders. Other titles Handbook of Endocrine Investigations in Children I. A. Hughes Handbook of Haematological Investigations in Children R. F. Stevens Handbook of Neurological Investigations in Children John B. P. Stephenson and Mary D. King Handbook of Renal Investigations in Children CM. Taylor and S. Chapman Handbook of Immunological Investigations in Children J. Graham Watson, MD, BSC, FRCPE, FRCP, DCH Late Consultant Paediatrician, Newcastle General Hospital, Newcastle Upon Tyne A. Graham Bird, MD, MRCPath, FRCP Director, AIDS Immunology Unit, Edinburgh Senior Lecturer in Immunology, Royal Infirmary, Edinburgh WRIGHT London Boston Singapore Sydney Toronto Wellington Wright is an imprint of Butterworth Scientific (^ PART OF REED INTERNATIONAL RL.C. All rights reserved. No part of this publication may be reproduced in any material form (including photocopying or storing it in any medium by electronic means and whether or not transiently or incidentally to some other use of this publication) without the written permission of the copyright owner except in accordance with the provisions of the Copyright, Designs and Patents Act 1988 or under the terms of a licence issued by the Copyright Licensing Agency Ltd, 33-34 Alfred Place, London, England WCIE 7DP. Applications for the copyright owner's written permission to reproduce any part of this publication should be addressed to the Publishers. Warning: The doing of an unauthorised act in relation to a copyright work may result in both a civil claim for damages and criminal prosecution This book is sold subject to the Standard Conditions of Sale of Net Books and may not be re-sold in the UK below the net price given by the Publishers in their current price list. First published 1990 © Butterworth & Co. (Publishers) Ltd, 1990 British Library Cataloguing in Publication Data Watson, J. Graham Handbook of immunological investigations in children. 1. Children. Diseases. Immunological aspects. Diagnosis I. Title II. Bird, A. Graham III. Series 618.920079 ISBN0-7236-0973-X Library of Congress Cataloging-in-Publication Data Watson, J. Graham. Handbook of immunological investigations in children/J. Graham Watson, A. Graham Bird. p. cm. — (Handbooks of investigation in children) Includes bibliographical references. ISBN0-7236-0973-X 1. Immunological diseases in children—Handbooks, manuals, etc. 1. Bird, Angus Graham. II. Title. III. Series. [DNLM: 1. Immunologie Diseases—diagnosis—handbooks. 2. Immunologie Diseases-in infancy & childhood—handbooks. WD 301 W339h] RJ385.W38 1990 618.92'97—dc20 DNLM/DLC 90-12005 for Library of Congress CIP Composition by Genesis Typesetting, Borough Green, Sevenoaks, Kent Printed and bound in England by Page Bros. Ltd., Norwich, Norfolk Foreword The two authors of this book bring together the special skills involved in their own personal practices. The text combines the pragmatic approach of an excellent general paediatrician; Dr Graham Watson, whose special interest was in clinical immunology, with the broad laboratory expertise of Dr Graham Bird, an adult clinical immunologist. Clinical immunology, a subject so poorly taught in medical schools, covers so much of modern day medical practice, yet few books on the subject provide the reader with a practical approach to the clinical problems. This book, however, is of immense practical value because it stresses which immunological investigations are necessary for correct diagnosis in the different clinical settings and it also provides the normal values and ranges so necessary for correct interpretation. It is extremely sad that Dr Graham Watson did not see the final published result. His untimely death in late 1989 prevented him developing the clinical practice of paediatric immunology in Newcastle into a major referral and teaching centre for the North of England. He had become interested in immunology through his training in London in bone marrow transplantation, but following his appointment to Newcastle he developed the wider aspects of paediatric immunology as a thriving subspecialty. At the time of his death, he had become an acknowledged expert in the field. Fortunately, this valuable knowledge was not entirely lost as the easily-read pages of this book demonstrate. RJ Levinsky Hugh Greenwood Professor of Immunology March, 1990 Preface Immunology is often presented as an obscure and complex basic science with little direct application to clinical medicine. Nevertheless in the last 20 years a large number of observations and investigations derived from basic understanding have greatly contributed to understanding the pathogenesis of a wide range of diseases. Clinical immunology has become an established investigational speciality and laboratory services are now established in most hospital regions. The increasing complexity and sensitivity of laboratory investigations, particularly in cellular immunology and the use of monoclonal antibodies, has allowed ever more specific cellular immunohistology, the definition of selective abnormalities of cell membranes, and even smaller quantities of free or tissue-fixed antibody and antigen to be identified. It is inevitable, with the emergence of more effective immunosuppressive agents and the development of cloned cytokines with potent influences on immune responses, that immunology will enter a new and exciting therapeutic era. This handbook is aimed at registrars in training, although we hope medical students, established practitioners of all grades, and laboratory staff will find it of value. It is biased towards the diseases of childhood. Primary immunodeficiency therefore has a greater place than it would in an adult immunology text. Necessarily the book reflects the practice and opinion of the authors. Our intention is to bring laboratory immunological investigations in- to the ward and out-patient clinic. Basic immunology is dealt with only briefly and we have no wish to supplant standard texts describing the organization and function of the immune system. We intend that users of this handbook will find the appropriate investigations easily when confronted by a clinical problem or diagnosis with immunological implications. Sections are arranged under organ- or disease-based headings and the relevant immunological investigations for specified individual diseases are described. We have aimed to provide a clear commentary on the appropriateness of individual tests, their indica- tions, limitations, and interpretation in a clinical context. In immunology more than other investigational specialities, the Vll viii Preface dividing line between routine investigations and research procedures is blurred. Infectious disease and classical immunology are inextricably linked. We have included investigations that seem relevant to the clinician without being confined by the traditions of hospital pathology departments. Treatment is described only in primary immunodeficien- cy, although immunologically based treatments are mentioned where appropriate. We thank our colleagues who have read sections of the manuscript and contributed many useful thoughts. The skilful secretarial assistance of Mrs Jennifer Watson has been much appreciated and is gratefully acknowledged, as is the editorial supervision of Sylvia Hull of Butterworths. Abbreviations ABPA allergic bronchopulmonary aspergillosis ACA adrenal cortical antibodies ADA adenosine deaminase ADCC antibody-dependent cellular cytotoxicity AGM antiglomerular basement membrane AIDS acquired immunodeficiency syndrome AIHA autoimmune haemolytic anaemia ALL acute lymphocytic leukaemia AMA antimicrosomal antibody AML acute myeloid leukaemia ANA antinuclear antibody ANCA antineutrophil cytoplasmic antibody AP haemolytic activity of the alternative pathway of complement 50 AT ataxia-telangiectasia ATA antithyroglobulin antibody BM basement membrane BMT bone marrow transplant BMZ basement membrane zone BSA bovine serum albumin Clinh Cl esterase inhibitor C3 complement factor 3 C3nef C3 nephritic factor CAH chronic active hepatitis CD cluster of differentiation CH haemolytic activity of the classical pathway of complement 50 CID combined immunodeficiency CIE countercurrentimmunoelectrophoresis CFT complement fixation test CMC chronic mucocutaneous candidiasis CMI cell-mediated immunity CMP cows milk precipitins CMV cytomegalovirus Con A concanavalin A CPEN cytopathic effect neutralization CR1 complement receptor 1 CREST syndrome of calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly and telangiectasis CRP C-reactive protein DCT direct Coombs test DI dermopathy-associatedimmunoglobulin DIF direct immunofluorescence X Abbreviations xi DNA deoxynucleic acid DNCB dinitrochlorobenzene DNP deoxynucleoprotein ds double stranded DTH delayed type hypersensitivity DTP diphtheria, tetanus and pertussis immunization DXR radiotherapy EA early antigen (of EBV) EBNA nuclear antigen (of EBV) EBV Epstein-Barr virus EEG electroencephalogram El exophthalmogenic immunoglobulin EIA enzyme immunoassay ELISA enzyme-linked immunosorbent assay EM erythema marginatum or electron microscopy EN erythema nodosum FACS fluorescence activated cell sorter Fab antigen-binding fragment of Ig Fc crystallizable fragment of Ig (gives class specificity and biological activity) FDC follicular dendritic cell G a heavy-chain allotype of IgG m GBM glomerular basement membrane GVHD graft-versus-host disease Gy gray HES hypereosinophilic syndrome HI haemagglutination inhibition HIE hyperimmunoglobulin E syndrome HIb Haemophilus influenzae group b HIV human immunodeficiency virus HLA human leukocyte antigen HSV herpes simplex virus la immune associated IBD inflammatory bowel disease ICA islet-cell antibody ICAM1 intercellular adhesion molecule 1 IDDM insulin-dependent diabetes mellitus IEL intraepithelial lymphocytes IFN interferon Ig immunoglobulin IgGi immunoglobulin G subclass one IAA indirect haemagglutination IL interleukin ITP idiopathic thrombocytopenic purpura JAS juvenile ankylosing spondylitis JCA juvenile chronic arthritis JDMS juvenile dermatomyositis JRA juvenile rheumatoid arthritis K killer K a heavy chain allotype of IgG m KLH keyhole limpet haemocyanin La a cytoplasmic protein antigen xii Abbreviations LATS long-acting thyroid stimulator LFA leukocyte function antigen LIP lymphoid interstitial pneumonitis LKM liver and kidney microsomes MAF macrophage activating factor MBP myelin basic protein MHC major histocompatibility complex MIF migration inhibition factor MMR measles, mumps, and rubella immunization MPGN membranoproliferativeglomerulonephritis NBT nitroblue tetrazolium NK natural killer NP nucleotide phosphorylase nps nasopharyngeal secretions PA pernicious anaemia PAIgG platelet-associated immunoglobulin PBC primary biliary cirrhosis PCA parietal-cell antibody PCNA proliferating cell nuclear antibody PE pulmonary eosinophilia PH pulmonary haemosiderosis PHA phytohaemagglutinin PWM pokeweed mitogen RAST radio allergosorbent test RF rheumatoid factor RFLP restriction fragment length polymorphism RIA radioimmunoassay RNA ribonucleic acid RNP ribonucleoprotein Ro a cytoplasmic protein antigen SCID severe combined immunodeficiency Scl70 scleroderma 70 - a nuclear protein antigen SIDS sudden infant death syndrome SLE systemic lupus erythematosus Sm Smith (a ribonucleoprotein antigen) SM smooth muscle ss single-stranded SSA Sjögrens syndrome A - identical to Ro SSB Sjögrens syndrome B - identical to La TBII thyroid-binding inhibiting immunoglobulin TGII thyroid growth inhibiting immunoglobulin TGSI thyroid growth-stimulating immunoglobulin TPHA Treponema pallidum haemagglutination TSH thyroid-stimulating hormone TSI thyroid-stimulating immunoglobulin VCA virus capsid antigen (of EB V) VDRL venereal disease reference laboratory VZ varicella zoster WAS Wiskott-Aldrich syndrome WR Wassermann reaction XLA X-linked hypogammaglobulinaemia XLPS X-linked lymphoproliferative syndrome Chapter 1 Basic immunology This chapter does not attempt to replace the excellent texts available that provide detailed description of the organization and generation of immune responses. The intention of this introduction is merely to provide a simple background to the practical knowledge necessary to allow the reader to use the subsequent text. Functional organization of the immune response The immune response has evolved to provide a comprehensive system of defence against microbiological attack. In vertebrates, the system of specific recognition of antigens provided by lymphocyte responses has been added to the non-specific phagocytic and serum effector mechanisms present in invertebrates. A key feature of lymphocyte- based responses is that of memory which allows the previous experience of the individual to produce rapid, more effective subsequent protection and also allows the transfer of temporary protection to an offspring. Another key feature is that antibody and T-cell responses can be predictive. The random generation of specificities that result from DNA rearrangement as lymphocytes develop results in a broad repertoire of processes for antigen recognition which is not based on previous experience of the species but is capable of predicting and reacting to the emergence of new bacterial or viral species and thus ensuring the survival of a proportion of each generation. Humoral and cellular immunity Immune responses can be divided into two major functional compart- ments, both of which have recognition provided by the lymphocyte population and its products. These are linked to activation of the effector pathways responsible for antigen or micro-organism removal. Humoral immunity Antibody responses are generated by B lymphocytes and result in the appearance of immunoglobulin classes each of which bears identical 1
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