Paediatric Respiratory Disease: Parenchymal Diseases

95 Pages·2011·5.814 MB·English

by  Andrew Bush, Jane C. Davies

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Website: www.clinicalpublishing.co.uk
IsBN: 978 1 84692 086 8

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Paediatric Respiratory_NEW_TPS_cover.indd 1 30/08/2011 09:50

An Atlas of Investigation and Management
PAEDIATRIC
RESPIRATORY
DISEASE
PARENCHYMAL DISEASES
Edited by
Andrew Bush, MB BS(Hons), MA, MD, FRCP, FRCPCH
Professor of Paediatric Respirology and Consultant Paediatric Chest Physician
Department of Paediatric Respiratory Medicine
Imperial College and Royal Brompton and Harefi eld NHS Foundation Trust
London, UK
Jane C. Davies, MB ChB, MRCP, MRCPCH, MD(Hons)
Reader and Honorary Consultant
Department of Paediatric Respiratory Medicine
Royal Brompton and Harefi eld NHS Foundation Trust and Imperial College
London, UK
CLINICAL PUBLISHING
OXFORD
A01_PAED_0868_PRELIMS-cpp.indd 3 25/08/2011 16:19

Clinical Publishing
an imprint of Atlas Medical Publishing Ltd
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© Atlas Medical Publishing Ltd 2011
First published 2011
All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted,
in any form or by any means, without the prior permission in writing of Clinical Publishing or Atlas Medical
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Although every effort has been made to ensure that all owners of copyright material have been acknowledged
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Clinical Publishing and Atlas Medical Publishing Ltd bear no responsibility for the persistence or accuracy of
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ISBN-13 978 1 84692 086 8
ISBN e-book 978 1 84692 628 0
The publisher makes no representation, express or implied, that the dosages in this book are correct.
Readers must therefore always check the product information and clinical procedures with the most
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A01_PAED_0868_PRELIMS-cpp.indd 4 25/08/2011 16:19
Contents
Contributors vi
Abbreviations viii
1. Congenital lung malformations 1
Chris Dewhurst, George K. Kokai, Gurdeep S. Mann, Nigel (Ben) Shaw
2. Neonatal lung disease 11
Anne Greenough, Caroline May
3. Neuromuscular and chest wall disease (including non-invasive ventilation) 25
Anita K. Simonds
4. Interstitial lung disease 37
Andrew Bush, Andrew G. Nicholson
5. Primary and secondary thoracic tumours 51
Catherine Wynne, N.J. Sebire, Kieran McHugh, Julia Chisholm
6. Rare lung diseases 69
Samatha Sonnappa, Robert Dinwiddie
Index 79
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vi
Contributors
Andrew Bush, MB BS(Hons), MA, MD, FRCP, FRCPCH Gurdeep S. Mann, MRCP, FRCR
Professor of Paediatric Respirology and Consultant Consultant Radiologist
Paediatric Chest Physician Department of Radiology
Department of Paediatric Respiratory Medicine Alder Hey Children’s Hospital NHS Foundation Trust
Imperial College and Royal Brompton and Harefield Liverpool, UK
NHS Foundation Trust
London, UK Caroline May, MB BS, BSc(Hons), MRCPCH
Specialist Registrar in Neonatal Medicine
Julia Chisholm, BMBCh, PhD, FRCPCH Elizabeth Ward Neonatal Unit and Neonatal Transfer
Deputy Head Service (NTS)
Children and Young People’s Unit The Royal London Hospital
Royal Marsden NHS Foundation Trust Whitechapel
Sutton, UK London, UK
Chris Dewhurst, MBChB, MRCPCH, PGCTLCP Kieran McHugh, FRCR, FRCPI, DCH
Consultant Neonatologist Consultant Paediatric Radiologist
Neonatal Intensive Care Unit Department of Radiology
Liverpool Women’s Hospital Great Ormond Street Hospital for Children
Liverpool, UK London, UK
Robert Dinwiddie, MB, FRCP, FRCPCH Andrew G. Nicholson, DM, FRCPath
Honorary Senior Lecturer Consultant Histopathologist and Professor of
Portex Unit, Respiratory Medicine Respiratory Pathology
UCL Institute of Child Health and Great Ormond Department of Histopathology
Street Hospital for Children NHS Trust Royal Brompton and Harefield Hospitals NHS
London, UK Foundation Trust
National Heart and Lung Institute
Anne Greenough, MD Imperial College
Professor of Neonatology and Clinical Respiratory London, UK
Physiology
Division of Asthma, Allergy and Lung Biology N. J. Sebire, MB BS, BClinSci, MD, DRCOG, FRCPath
MRC-Asthma UK Centre in Allergic Mechanisms of Professor of Paediatric Pathology
Asthma Institute of Child Health and Great Ormond Street Hospital
King’s College London, UK
London, UK
Nigel (Ben) Shaw, MB ChB, MRCPCH (UK), MD,
George K. Kokai, FRCPath MA (Clin Ed), FRCPCH
Consultant Paediatric Pathologist Consultant in Neonatal and Respiratory Paediatrics
Department of Paediatric Histopathology Liverpool Women’s Hospital and Royal Liverpool
Alder Hey Children’s Hospital NHS Foundation Trust Children’s Hospital Alder Hey
Liverpool, UK Liverpool, UK
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Contributors vii
Anita K. Simonds, MD, FRCP Catherine Wynne, BSc (Hons), MBChB, MRCPCH
Consultant in Respiratory Medicine Consultant Paediatrician
National Heart and Lung Institute Department of Paediatrics
Royal Brompton and Harefi eld NHS Foundation Trust Royal Alexandra Children’s Hospital
London, UK Brighton, UK
Samantha Sonnappa, MBBS, MD, DCH, MRCP,
FRCPCH, PhD
Clinician Scientist and Honorary Consultant in
Respiratory Medicine
UCL Institute of Child Health and Great Ormond
Street Hospital for Children NHS Trust
London, UK
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viii
Abbreviations
ALL acute lymphoblastic leukaemia MRI magnetic resonance imaging
AML acute myeloid leukaemia MYCN N-myc proto-oncogene protein
BAL bronchoalveolar lavage NEHI neuroendocrine cell hyperplasia in infancy
b-HCG beta-human chorionic gonadotropin NHL Non-Hodgkin’s lymphoma
BPD bronchopulmonary dysplasia NICU neonatal intensive care unit
CCAM congenital cystic adenomatoid malformation NIV non-invasive ventilation
chILD interstitial lung disease in all age children PAP pulmonary alveolar proteinosis
CT computed tomography PAS periodic acid-Schiff [stain]
CXR chest radiograph PIE pulmonary interstitial emphysema
DMD Duchenne muscular dystrophy PL pulmonary lymphangiectasia
FEV1 forced expiratory volume in 1 second PPB pleuropulmonary blastoma
FVC forced vital capacity pPNET peripheral primitive neuroectodermal tumour
GBS Group B streptococcus (or Streptococcus RDS respiratory distress syndrome
agalactiae) REM rapid eye movement
GM-CSF granulocyte–macrophage colony-stimulat- SFTP surfactant protein (SP- prefix also commonly
ing factor used)
HRCT high-resolution computed tomographic scan SMA spinal muscular atrophy
ILD interstitial lung disease SVC superior vena cava
LCH Langerhans cell histiocytosis T-ALL T-cell acute lymphoblastic leukaemia
LDH lactate dehydrogenase T-IPPV tracheostomy intermittent positive pressure
LPD lymphoproliferative disease ventilation
MD muscular dystrophy UIP usual interstitial pneumonia
MIBG metaiodobenzylguanidine scan VC vital capacity
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Chapter 1
1
Congenital lung
malformations
Chris Dewhurst, George K. Kokai, Gurdeep S. Mann, Nigel (Ben) Shaw
Introduction Management is mainly symptomatic, although the
rudimentary bronchus may act as a reservoir for infection
Congenital lung malformations comprise a rare but and surgery is occasionally required.
important group of disorders that may present at any age, Pulmonary hypoplasia (1.2) is more common, and
from the previable fetus through to adulthood. They can is usually secondary to intrauterine factors affecting
range in severity from asymptomatic to incompatible with pulmonary development (Table 1.1). The degree of
life. This collection of disorders can present in similar underdevelopment of the lung(s) determines presentation,
ways and may have a similar aetiology. In recent years which ranges from apparent health through to severe,
more advanced radiological imaging has led to an increase life-threatening respiratory distress. The lungs are often
in the number of lesions detected both antenatally and
postnatally in asymptomatic individuals. The clinical
dilemma is how best to manage this latter group.
Pulmonary agenesis, aplasia and
hypoplasia
Pulmonary agenesis and aplasia are rare; they occur when
the pulmonary bud fails to develop beyond the carina.
This leads to complete absence of lung parenchyma,
which in 30% of cases is bilateral and incompatible with
life. If unilateral, the unaffected lung is usually normal
but overinfl ates to fi ll the contralateral empty hemithorax.
Antenatal ultrasound reveals increased echogenicity
(‘bright lung’) on the side of the abnormal pulmonary
tissue (1.1). Children may present with respiratory
distress shortly after birth or in later childhood with
recurrent chest infections, wheeze or breathlessness; some
remain asymptomatic and are diagnosed incidentally. The 1.1 Antenatal ultrasound image of ‘bright lung’ (between
prognosis depends on the presence of other associated callipers). This is a non-specifi c marker and may indicate the
presence of any of the congenital lung abnormalities. Serial
congenital anomalies (cardiac (14%), gastrointestinal
ultrasound scans may also show regression of the lesion
(14%), skeletal (12%), vascular (9%) and genitourinary
with a normal healthy lung being present after birth. The
(9%)), which are more common with right-sided defects. arrow indicates the heart.
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