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OXFORD MEDICAL PUBLICATIONS

Paediatric
Rheumatology
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Oxford Specialist
Handbooks in
Paediatrics
Paediatric
Rheumatology
Edited by

Helen Foster
MBBS(Hons), MD, Cert Med Ed, DCH, FRCP, FRCPCH
Professor Paediatric Rheumatology
Newcastle University
Honorary Consultant in Paediatric Rheumatology,
Great North Children's Hospital, Newcastle Hospitals
NHS Foundation Trust,
Newcastle upon Tyne, UK

Paul A. Brogan
BSC(Hon), MBChB(Hon), MSC, PhD, MRCPCH, FRCPCH
Senior Lecturer in Paediatric Vasculitis and Honorary
Consultant in Paediatric Rheumatology
UCL Institute of Child Health, and Great Ormond
St Hospital for Children NHS Trust
London, UK

1
1
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It furthers the University’s objective of excellence in research, scholarship,
and education by publishing worldwide. Oxford is a registered trade mark of
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© Oxford University Press 2012
The moral rights of the authors have been asserted
First Edition published in 2012
Impression: 1
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a retrieval system, or transmitted, in any form or by any means, without the
prior permission in writing of Oxford University Press, or as expressly permitted
by law, by licence or under terms agreed with the appropriate reprographics
rights organization. Enquiries concerning reproduction outside the scope of the
above should be sent to the Rights Department, Oxford University Press, at the
address above
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and you must impose this same condition on any acquirer
British Library Cataloguing in Publication Data
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Data available
ISBN 978–0–19–959263–0
Printed in Great Britain by
Ashford Colour Press Ltd, Gosport, Hampshire
Oxford University Press makes no representation, express or implied, that the
drug dosages in this book are correct. Readers must therefore always check
the product information and clinical procedures with the most up-to-date
published product information and data sheets provided by the manufacturers
and the most recent codes of conduct and safety regulations. The authors and
the publishers do not accept responsibility or legal liability for any errors in the
text or for the misuse or misapplication of material in this work. Except where
otherwise stated, drug dosages and recommendations are for the non-pregnant
adult who is not breast-feeding
Links to third party websites are provided by Oxford in good faith and
for information only. Oxford disclaims any responsibility for the materials
contained in any third party website referenced in this work.
 v

Preface

The purpose of this book

This handbook aims to help you understand and manage virtually any
paediatric rheumatology condition that may present to you in clinical
practice; it is pocket-sized with a system of bullet points to allow easy
reference to up-to-date evidence-based (where possible) and consensus-
derived opinion. This first edition of the handbook is written amidst a
revolution in clinical genetics and novel therapeutics in the field of mul-
tisystemic inflammatory disease, and captures state-of-the-art clinical
care and best current practice in this context. Essential further reading,
including website links, direct you to sources where more information
can be obtained. The handbook is divided into sections to cover essential
elements of the clinical assessment, the approach to investigations and
management, along with important key facts of knowledge and practical
advice. It includes advice on the care of acute emergencies, chronic disor-
ders, and the spectrum of common conditions seen by all paediatricians
to those seen rarely and usually cared for in specialized paediatric rheu-
matology centres, in collaboration with local units. Many of the chapters
will be applicable to the day-to-day care of patients on a general paediatric
ward or in the outpatient clinic. Acute emergencies that can occur in multi-
system disease and issues arising from the use of potent immunosuppressive
treatments are dealt with in detail.
This handbook will provide guidance for trainees, the general paediatrician,
and the multidisciplinary team specialists (doctors, nurses, physiotherapists,
occupational therapists, podiatrists, and psychologists) who care for chil-
dren with rheumatological conditions. We bring together for the first time
practical guidance for the use of outcome measures, clinical scores of
disease activity, and drug prescribing relevant to paediatric rheumatology.
‘State-of-the-art treatment’ of paediatric rheumatology conditions includes
an ever increasing number of biologic and novel therapies—we provide
practical guidance for the use of such treatments with guidelines, algo-
rithms, and emphasis of the role of the multidisciplinary team to deliver
optimal clinical care.
We are very proud of our extensive authorship, including doctors and
allied health professionals, many of whom are international leaders in
their field. We have successfully engaged with the breadth of the British
Society for Paediatric and Adolescent Rheumatology (BSPAR) community,
with representation from the majority of centres across the UK and have
contributions from all current UK trainees in paediatric rheumatology
working in partnership with consultant supervisors. We also have input
from parents of children with rheumatic disease to emphasize their role
in working together with healthcare professionals—much of what we
do in clinical practice lacks a robust evidence base and we emphasize the
importance of input from consumers on how to involve and fully engage
children and their families with clinical decision-making and research
opportunities to facilitate growth of evidence to inform best practice.
vi PREFACE

This book is a measure of the growing collaboration of paediatric rheuma-

tology both within the UK and with our colleagues further afield to develop
and share knowledge, promote highest quality clinical care, education,
and training to facilitate access to optimal care for all children with rheu-
matic disease.
All profits from the sales of the handbook will be donated to BSPAR.
Helen Foster
Paul A. Brogan
 vii

Foreword 1

UK’s Paediatric Rheumatology Clinical Studies Group

The importance of supporting the very best clinical research to improve
the care of children with rheumatic and musculoskeletal health has been
exemplified by the establishment by the National Institute of Health
Research (NIHR) Medicines for Children Research Network (MCRN)1
and Arthritis Research UK of the MCRN/Arthritis Research UK Paediatric
Rheumatology Clinical Studies Group (CSG).2 This partnership of exper-
tise has supported a major opportunity and development in recent years
of taking forward a comprehensive and long lasting, national clinical
research programme for Paediatric Rheumatology.
Following a detailed, widespread consultation and consensus process,
involving all relevant stakeholders including consumers (see Foreword 2
for comment on consumer input), the CSG’s primary task was to define
the key strategic priorities in paediatric rheumatology with respect to:
‘What are the key clinical research priorities that will change clinical practice
in paediatric rheumatology?’
To achieve this, the CSG:
• Looks to address the gaps in the evidence base in the management
of children and young people with rheumatic or musculoskeletal
conditions.
• Works with all interested parties towards developing a comprehensive
portfolio of key research clinical trials and related studies covering the
entire spectrum of major disease areas in paediatric rheumatology in
the UK.
• Has a strong multidisciplinary membership including: 4 consumer
representatives, clinicians, clinical academics, basic scientists, allied
health professionals, and a pharmacist expert in paediatric formulations.
The CSG’s ‘goal’ is that:
• ‘All children and young people in the UK with a rheumatological
condition may be given the opportunity to be enrolled in a clinical trial
or well-conducted clinical study from point of diagnosis onwards, and
in so doing improve the care and outcome of them and patients with
similar conditions in the future;
• That all children have the option of contributing towards a related, fully
informed and consented Biobank (e.g. DNA and serum) for subsequent
investigation into the cause of their condition.’
To facilitate this process, the CSG has developed (to date) 9 ‘topic specific
groups’ (TSGs) to foster collaboration and support multidisciplinary efforts
to address the key priority areas within paediatric rheumatology, as well as
organizing ad hoc meetings to focus on specific projects.
• Each TSG has members of the CSG as ‘link-persons’ to support
communication and integration of the activities of the TSGs within the
wider CSG research agenda and support structures.
viii FOREWORD 1

• The 9 TSGs are: Auto-inflammatory diseases; Bone Health; Juvenile

Dermatomyositis; Juvenile Idiopathic Arthritis and associated Uveitis;
Juvenile-onset Systemic Lupus Erythematosus; Childhood Scleroderma;
Non-inflammatory, musculoskeletal disorders; Childhood Vasculitides.
In addition, the CSG supports investigators in the following way:
• Welcoming and publicizing expressions of interest in working towards
these priority studies, calling for submission of protocols and proposals
to address these priorities.
• Working with stakeholders and funding bodies and to identify
barriers to research participation and identifying strategies to facilitate
engagement and collaboration in clinical research.
• Supporting protocols where relevant can also include pilot and
feasibility studies to provide proof of concept for definitive studies in
terms of recruitment, data acquisition.
• Encouraging consideration of research ‘add-ons’ to bring value-added
benefit to the children participating in these studies, e.g. pharmacogenetic
and qualitative studies.
• Being advisory to investigators, the MCRN on studies submitted for
NIHR Portfolio adoption, including commercial trials, and to the
Arthritis Research UK and assist with their development.
• Working in close collaboration with other MCRN and Arthritis
Research UK CSGs, as well as other topic specific research networks in
all areas of mutual interest.
References
1 M http://www.mcrn.org.uk.
2 M http://www.arthritisresearchuk.org/research/clinical_study_groups/csg_-_mcrn_paediatric_
rheumato.aspx.
 ix

Foreword 2

Parent and young person involvement in research—a

‘consumer’s’ perspective
The social and psychological aspects of living with any chronic condition
can create a different type of expertise to that of clinicians and researchers.
Consumer involvement is concerned with creating a forum where the
experience, knowledge, and expertise of both healthcare professionals
and the public can come together in an equal partnership to benefit
healthcare research. High-quality research, optimal accrual to research
projects, and improved patient outcomes are dependent on listening to
the voices of children and young people, their families and carers, and
taking account of their experiences, priorities, and perspectives. Engaged
effectively, consumers can contribute to all aspects of clinical research and
clinical service development and including:
• Identifying and prioritizing research ideas that are relevant to clinical
practice.
• Commissioning research to highlight topic areas of concern to patients.
• Designing research studies and associated documentation to enhance
the feasibility of the project by identifying potential barriers to study
participation.
• The management of studies (e.g. as members of steering group).
• Analysis or interpretation of research results to address key issues
relevant to patients.
• Dissemination of research results throughout the lay community.
Key principles for the productive involvement of patients, parents, young
people and families, (referred to as patient and public involvement or PPI),
in partnership for research and clinical service development are discussed
in detail. In summary these are:
• All members of the clinical research group should understand the
benefits of PPI perspective.
• Participation of new PPI members will be facilitated if they are formally
introduced and effort is made to find out about their background/
experiences.
• In advance of meetings, it is important to consider the PPI angle on all
agenda items and identify those which may be of particular interest to
PPI members so they may prepare for discussions.
• Include introductions at the start of a meeting and clarify the meeting
purpose, use plain English, and minimize jargon.
• During the meetings, encourage questions and invite the PPI members
to comment on all issues.
• Consumers’ contributions are often based on their own experiences of
an illness, and wherever possible they should be representative of the
broader community.
• After a meeting it is important to minute contributions made by PPI
members with a follow-up process for feedback.
x FOREWORD 2

Further reading
TwoCan Associates for the UKCRC and NCRI. Patient and public involvement in research
groups – Guidance for Chairs. TwoCan Associates, 2010. M http://www.twocanassociates.
co.uk/pubs.php.
 xi

Foreword 3

I am delighted as the current Convenor of The British Society for

Paediatric and Adolescent Rheumatology (BSPAR) to provide a Foreword
for this Oxford Handbook of Paediatric Rheumatology.
BSPAR has evolved over several decades from the multidisciplinary British
Paediatric Rheumatology Group to achieve its new charitable status, and
is determined and committed to encourage and support clinical care,
research, education, and training in paediatric rheumatology in the UK.
It was in order to help deliver these goals that in 2010 BSPAR achieved
charitable status and I am truly proud of the commitment made by all the
contributing authors of this book to donate any fees due and profits made
to BSPAR.
This handbook has been produced by a dedicated editorial team and with
contributions from a large and truly multidisciplinary authorship. This
highlights the fundamental principle that underpins the ethos of BSPAR—
namely the sense of teamwork that helps individual teams deliver day-to-day
healthcare to children and young people with rheumatologic disorders
and also drives us towards our goal of high-quality research continually
improving the delivery of clinical care.
Throughout our activities it is our patients and their families who directly
benefit from the improved training and dissemination of information that
BSPAR provides to the medical and allied health carers with whom they
come into direct contact. Through education and training, all healthcare
professionals improve the service which they provide to their patients,
and I am sure that this handbook will prove invaluable to many working
in this field.
Dr Gavin Cleary
Convenor
xii

Foreword 4

Paediatric rheumatology training in the UK

In the UK, training in paediatric rheumatology follows completion of
general paediatric training and is overseen by the Royal College of
Paediatrics and Child Health (RCPCH) with a Competency Based
Framework and a process of assessment. Training in paediatric rheuma-
tology is through the national training programme for the training of spe-
cialists in paediatrics (called the ‘Grid’) with competitive entry organized
by RCPCH. This programme of training is a minimum of 2 years, recom-
mended to be in more than one accredited centre, and trainees complete
a programme of formative and summative assessments which are com-
petency-based. Ultimately trainees acquire a Certificate of Completion
of Training to be eligible to be appointed to Consultant posts working
in the National Health Service. Training for general paediatricians with
an interest in rheumatology is also encouraged and such trainees do not
need to be appointed to the Grid but are recommended to have 1 year’s
training in a recognized paediatric rheumatology centre. The Competency
Frameworks for Speciality Paediatrics form the basis of the curriculum
for training and there is a Framework for the Specialist in Paediatric
Rheumatology and also for the general paediatrician with an interest in
rheumatology. Further details are given on the format of training and the
Competency Frameworks are given on the British Society of Paediatric
and Adolescent Rheumatology website (M http://www.bspar.org.uk/
pages/trainees_area_members.asp) and the RCPCH website (M http://
www.rcpch.ac.uk/Training/Competency-Framework).
Increasingly throughout the UK and elsewhere, there are networks of
clinical services working together to deliver paediatric rheumatology care,
often across large geographical areas and encompassing various models of
care with specialist expertise linking with other, and often smaller units.
The role of adult rheumatologists is still very important as part of these
clinical networks, although in many areas the focus of the adult rheuma-
tology team is increasingly towards transitional care. There is a great need
to raise awareness of paediatric rheumatology and the importance of early
and prompt access to appropriate specialist care. There is a shortage of
specialist paediatric rheumatology multidisciplinary teams in the UK and
further afield. The role of shared care between specialist services with
local general paediatric and adult rheumatology teams is increasingly
important so that high-quality clinical care can be delivered and equi-
tably. Such clinical networks require personnel with training and support,
highlighting the need for training, training positions, and a mechanism for
support and teaching resources. This content of this handbook is largely
based on the content of the Competency Frameworks for Paediatric
Rheumatology and aims to address learning needs of paediatric rheuma-
tology trainees, general paediatricians with an interest in rheumatology,
as well as other healthcare professionals working within the specialty and
clinical networks. To date there are no competency frameworks or curricula
 FOREWORD 4 xiii
for allied health professionals working in paediatric rheumatology but
through involvement of nurses and therapists as contributors to the hand-
book, we aim to have addressed some of the learning needs.
Helen Foster
Chair of RCPCH Specialist Advisory Committee for Rheumatology
This page intentionally left blank
 xv

Contents

Detailed contents xvii

Symbols and abbreviations xxiii
Contributors xxix

1 Clinical skills and assessment 1

2 Common and important clinical problems 53
3 Juvenile idiopathic arthritis 129
4 Systemic diseases 167
5 Bone diseases, skeletal dysplasias, disorders
of collagen 329
6 Infection and immunization 355
7 The multidisciplinary approach to management 377
8 Specialized therapeutic approaches 389
9 British Society of Paediatric and Adolescent
Rheumatology clinical guidelines and protocols 409
10 Rashes in paediatric rheumatology 447

Index 457
This page intentionally left blank
 xvii

Detailed contents

Symbols and abbreviations xxiii

Contributors xxix

1 Clinical skills and assessment 1

Epidemiology of paediatric musculoskeletal conditions 2
History taking, physical examination, and approaches to
investigation 4
Normal variants of lower limb development 9
The gait cycle and abnormal gait patterns 11
Normal gait and musculoskeletal development 15
pGALS: paediatric gait, arms, legs, spine
musculoskeletal screening examination 18
pREMS: paediatric regional examination of the
musculoskeletal system 23
Musculoskeletal ultrasound in juvenile idiopathic arthritis 28
Autoantibodies 32
Thermography in rheumatic disease 35
Nailfold capillaroscopy in rheumatic disease 37
Outcome measures in paediatric rheumatology 41
The Child Health Assessment Questionnaire (CHAQ) 45

2 Common and important clinical problems 53

Musculoskeletal presentations and non-accidental injury 54
Malignancy and musculoskeletal presentations 56
Bone tumours 60
Bone and joint infections 64
Infections in the immunocompromised 70
Fractures in children and adolescents 76
The limping child 80
Pain syndromes and the assessment of pain 87
Growing pains 92
Pyrexia of unknown origin 94
Back pain in children and adolescents 100
Scoliosis 103
xviii DETAILED CONTENTS

Hip pain and hip problems in children and adolescents 106

Knee pain in children and adolescents 117
Foot and ankle problems 121
Joint hypermobility 124

3 Juvenile idiopathic arthritis 129

Genetics and JIA: HLA and non-HLA/MHC associations with
subtypes of JIA 130
Immunology and aetiology of JIA 137
Classification of JIA 139
JIA subtypes and their clinical presentations 141
Prognostic indicators in JIA 146
Uveitis screening in JIA: the approach to screening and guidelines 148
Surgery in the young adult with JIA: practical issues 152
Treatment approaches in JIA 155
Treatment pathways in JIA 159
Disease activity scores in rheumatoid arthritis and JIA 165

4 Systemic diseases 167

Vasculitis
The classification of paediatric vasculitis 168
The epidemiology of paediatric vasculitis 171
The investigation of primary systemic vasculitis 172
The standard treatment of childhood vasculitis 174
Henoch–Schönlein purpura 179
Kawasaki disease 183
The anti-neutrophil cytoplasmic antibody (ANCA)-associated
vasculitides 188
Polyarteritis nodosa (PAN) 192
Cutaneous polyarteritis nodosa (cPAN) 198
Takayasu arteritis 199
Behçet’s disease 205
Central nervous system vasculitis in children 209
Other vasculitides 214
Vasculitis mimics: non-inflammatory vasculopathies 217
Juvenile systemic lupus erythematosus
JSLE: epidemiology and aetiology 223
JSLE: clinical features and diagnostic criteria 226
 DETAILED CONTENTS xix
Assessment of the child with JSLE and monitoring
of disease activity 229
JSLE: approach to management 232
JSLE: renal involvement 236
Neonatal lupus syndrome 240
B-cell-targeted therapies for systemic lupus erythematosus 241
British Isles Lupus Assessment Group (BILAG) 2004 Index 248
SLEDAI 2000 Disease Activity Index 256
SLICC/ACR Damage Index (paediatric) 258
Connective tissue diseases
Scleroderma 260
Juvenile dermatomyositis 266
Overlap syndromes 275
Antiphospholipid syndrome (APS) 279
Paediatric uveitis 283
Autoinflammatory diseases and immunodeficiency
Periodic fever syndromes/autoinflammatory disease 288
Chronic recurrent multifocal osteomyelitis 297
Sarcoidosis 300
Macrophage activation syndrome 305
Primary immunodeficiency and rheumatological disease 309
Other systemic diseases
Mucopolysaccharidoses (MPS) and mucolipidoses (ML) 312
Chromosomal abnormalities and associated
musculoskeletal morbidity 318
Arthritis and other musculoskeletal features associated
with cystic fibrosis 323
Inflammatory bowel disease and musculoskeletal features 326

5 Bone diseases, skeletal dysplasias, disorders of collagen 329

Metabolic bone diseases 330
Skeletal dysplasias 333
The osteochondroses 343
Heritable disorders of connective tissue 346

6 Infection and immunization 355

Tuberculosis and mycobacterial disease 356
Rheumatic fever 363
xx DETAILED CONTENTS

Lyme disease 368

Varicella zoster infections 371
Immunization schedules and the immunocompromised 374

7 The multidisciplinary approach to management 377

The multidisciplinary team 378
The role of the clinical nurse specialist 380
The role of the physiotherapist 382
The role of the occupational therapist 384
The role of the podiatrist 385
Transitional care 387

8 Specialized therapeutic approaches 389

Corticosteroid intra-articular injections 390
Biologic therapies for paediatric rheumatological diseases 393
Approvals for use of biologic therapies 399
Medicines for children and paediatric rheumatology 401
Haematopoietic stem cell transplantation 405

9 British Society of Paediatric and Adolescent

Rheumatology clinical guidelines and protocols 409
BSPAR standards of care for children and young people with JIA 410
BSPAR drug information leaflets for parents and families 412
BSPAR guidelines for treatments used in paediatric
rheumatology 415
Non-steroidal anti-inflammatory drugs (NSAIDs) 416
Disease-modifying anti-rheumatic drugs (DMARDs) 418
Azathioprine 423
Ciclosporin 425
Intravenous cyclophosphamide 427
Pamidronate 431
Epoprostenol/iloprost 433
Etanercept (Enbrel®) 434
Other anti-TNF-A: adalimumab (Humira®) and
infliximab (Remicade®) 436
Anti-IL-1 treatments: anakinra (Kineret®), rilonacept
(Regeneron®), and canakinumab (Ilaris®) 437
Abatacept (Orencia®) 439
 DETAILED CONTENTS xxi
®
Anti-IL-6 treatment: tocilizumab (Ro-Actemra ) 440
Anti-B-cell therapies: rituximab (Mabthera®) 441
Systemic corticosteroids (oral, intramuscular, and intravenous) 443
Intra-articular corticosteroid use in JIA 445

10 Rashes in paediatric rheumatology 447

Systemic JIA 448
Palmar psoriasis in a patient with psoriatic JIA 448
Nail pits and psoriasis in a patient with psoriatic JIA 449
Psoriatic JIA (elbows) 449
Psoriatic JIA (hands) 450
HSP with bilateral ankle swelling 450
Juvenile dermatomyositis with periorbital oedema 451
Severe juvenile dermatomyositis with anterior chest wall
vasculitis—‘shawl sign’ 451
Extensor surface vasculitis in juvenile dermatomyositis 452
Subtle Gottron’s papules in a patient with severe
juvenile dermatomyositis 452
Periungual erythema in undifferentiated connective
tissue disease 453
Systemic vasculitis with pyrexia, livido reticularis, vasculitic rash with
skin necrosis 453
Maculo-papular rash of Epstein–Barr virus associated with
haemophagocytic lymphohistiocytosis (HLH) 454
Erythema nodosum 454
Generalized peeling in Kawasaki disease 455
Purpura in Wegener’s granulomatosis 455
SLE associated with congenital C1q deficiency 456

Index 457
This page intentionally left blank
 xxiii

Symbols and abbreviations

b cross-reference
i increase/d
d decrease/d
4 male
5 female
7 approximately
± plus/minus
2D two-dimensional
3D three-dimensional
AAV ANCA-associated vasculitides
ACE angiotensin-converting enzyme
ACL anticardiolipin
ACR American College of Rheumatology
ALL acute lymphoblastic leukaemia
ALP alkaline phosphatase
ALPS autoimmune lymphoproliferative syndrome
ANA antinuclear antibody
ANCA anti-neutrophil cytoplasmic antibody
anti-CCP anti-cyclic citrullinated protein
AP anteroposterior
aPL antiphospholipid [antibody]
APS antiphospholipid syndrome
APTT activated partial thromboplastin time
ARF acute rheumatic fever
ASIC anterior superior iliac crest
ASOT anti-streptolysin O titre
AZA azathioprine
BD Behçet’s disease
BJHS benign joint hypermobility syndrome
BNFC British National Formulary for Children
BP blood pressure
BPRG British Paediatric Rheumatology Group
BSA body surface area
BSPAR British Society of Paediatric and Adolescent
Rheumatology
CAA coronary artery abnormality
xxiv SYMBOLS AND ABBREVIATIONS

cANCA cytoplasmic anti-neutrophil cytoplasmic antibody

CAPS catastrophic antiphospholid syndrome or cryopyrin
associated periodic syndrome
CAU chronic anterior uveitis
CCP cyclic citrullinated protein
CD Crohn’s disease
CF cystic fibrosis
CHAQ Child Health Assessment Questionnaire
CINCA chronic infantile neurologic cutaneous and articular
syndrome
CNS central nervous system or clinical nurse specialist
cPACNS primary angiitis of the central nervous system in children
cPAN cutaneous polyarteritis nodosa
CRMO chronic recurrent multifocal osteomyelitis
CRP C-reactive protein
CRPS complex regional pain syndrome
CSF cerebrospinal fluid
CSS Churg–Strauss syndrome
CT computed tomography
CTA computed tomography angiography
CTD connective tissue disease
CXR chest x-ray
CYC cyclophosphamide
DAS Disease Activity Score
DD Degos disease
DDH developmental dysplasia of the hip
DIRA deficiency of IL-1 receptor antagonist
DMARD disease-modifying antirheumatic drug
DXA dual-emission X-ray absorptiometry
ECG electrocardiogram
EEG electroencephalogram
EMG electromyography
ENA extractable nuclear antigen
EOS early-onset sarcoidosis
ERA enthesitis-related arthritis
ERT enzyme replacement therapy
ESR erythrocyte sedimentation rate
EULAR European League Against Rheumatism
FBC full blood count
FCAS familial cold autoinflammatory syndrome
FII factitious or induced illness
 SYMBOLS AND ABBREVIATIONS xxv
FMD fibromuscular dysplasia
FMF familial Mediterranean fever
G6PD glucose-6-phosphate dehydrogenase
GAG glycosaminoglycan
GBM glomerular basement membrane
GFR glomerular filtration rate
GI gastrointestinal
GN glomerulonephritis
GP General practitioner
GvHD graft-versus-host disease
GWAS genome-wide association study
HA healthy adults
HC healthy children
HIV human immunodeficiency virus
HLA human leucocyte association
HLH haemophagocytic lymphohistiocytosis
HSCT haematopoietic stem cell transplantation
HSN Henoch–Schönlein nephritis
HSP Henoch–Schönlein purpura
HUVS hypocomplementaemic urticarial vasculitic syndrome
IA intra-articular
IAC intra-articular corticosteroid
IBD inflammatory bowel disease
IC indeterminate colitis
Ig immunoglobulin
ILAR International League of Associations for Rheumatology
IM intramuscular
INR international normalized ratio
IV intravenous
IVIG intravenous immunoglobulin
IVMP intravenous methylprednisolone
JDM juvenile dermatomyositis
JIA juvenile idiopathic arthritis
JSLE juvenile-onset systemic lupus erythematosus
jSSc juvenile systemic sclerosis
KD Kawasaki disease
kg kilogramme/s
L litre/s
LDH lactate dehydrogenase
LFT liver function test
xxvi SYMBOLS AND ABBREVIATIONS

LMWH low-molecular-weight heparin

LN lupus nephritis
LS localized scleroderma
LTBI latent tuberculosis infection
LV livedoid vasculopathy
MAS macrophage activation syndrome
MC&S microscopy, culture and sensitivity
MCPJ metacarpophalangeal joint
MCTD mixed connective tissue disease
MDT multidisciplinary team
MED multiple epiphyseal dysplasia
mg milligramme/s
MHC major histocompatibility complex
min minute/s
MKD mevalonate kinase deficiency
ML mucolipidoses
mL millilitre/s
mm millimetre/s
MMF mycophenolate mofetil
MPO myeloperoxidase
MPS mucopolysaccharidoses
MRA magnetic resonance angiography
MRI magnetic resonance imaging
MSK musculoskeletal
MSUS musculoskeletal ultrasound
MTPJ metatarsophalangeal joint
MTX methotrexate
MWS Muckle–Wells syndrome
NAI non-accidental injury
NBO non-bacterial osteitis
NFC nailfold capillary
NICE National Institute for Health and Clinical Excellence
NOMID neonatal onset multisystem inflammatory disease
NTI narrow therapeutic index
OCD osteochondritis dissecans
OI osteogenesis imperfecta
OT occupational therapist
PA posterior–anterior
PACNS primary angiitis of the central nervous system
PAH pulmonary arterial hypertension
 SYMBOLS AND ABBREVIATIONS xxvii
PAN polyarteritis nodosa
pANCA perinuclear anti-neutrophil cytoplasmic antibody
PAPA pyogenic sterile arthritis, pyoderma gangrenosum, and
acne [syndrome]
PCR polymerase chain reaction
PET positron emission tomography
pGALS paediatric gait, arms, legs, spine musculoskeletal screening
examination
PIPJ proximal interphalangeal joint
PK pharmacokinetic
PO per os (orally)
PR3 proteinase 3
pREMS paediatric regional examination of the musculoskeletal
system
PRT paediatric rheumatology team
PTH parathyroid hormone
PUO pyrexia of unknown origin
PVL Panton–Valentine leukocidin
PXE pseudoxanthoma elasticum
QoL quality of life
RA rheumatoid arthritis
RCT randomized control trial
RD Raynaud’s disease
RF rheumatoid factor
RICE Rest, ice, compress, elevate
RP Raynaud’s phenomenon
sec second/s
SAPHO synovitis acne pustulosis hyperostosis osteitis [syndrome]
SEDC spondyloepiphyseal dysplasia congenita
SIADH syndrome of inappropriate anti-diuretic hormone
secretion
sJIA systemic juvenile idiopathic arthritis
SLE systemic lupus erythematosus
SmPC summary of product characteristics
SNP single nucleotide polymorphism
SSc systemic sclerosis
SSZ sulphasalazine
TA triamcinolone acetonide
TB tuberculosis
TGFB transforming growth factor-beta
TH triamcinolone hexacetonide
xxviii SYMBOLS AND ABBREVIATIONS

TMJ temporomandibular joint

TNF tumour necrosis factor
TPMT thiopurine methyltransferase
TRAPS TNF receptor-associated period syndrome
TRM transplant-related mortality
TST tuberculin skin test
U&E urea and electrolyte
UC ulcerative colitis
UK United Kingdom
US ultrasound
VAS visual analogue score
VZIG varicella-zoster immunoglobulin
VZV varicella zoster virus
WCC white cell count
yr year/s
 xxix

Contributors

Dr Mario Abinun clinical presentations; Treatment

Consultant Paediatric approaches in JIA
Immunologist Chapter 7: The multidisciplinary
Great North Children’s Hospital, team
Newcastle upon Tyne NHS Chapter 9: Disease-modifying anti-
Hospitals Foundation Trust, rheumatic drugs (DMARDs):
Newcastle upon Tyne, UK Hydroxychloroquine
Chapter 2: Infections in the Dr Kate Armon
immunocompromised
Chapter 4: Autoinflammatory Consultant Paediatric
diseases and immunodeficiency: Rheumatologist and Honorary
Primary immunodeficiency and Senior Lecturer at the University
rheumatological disease of East Anglia
Chapter 6: Immunization schedules Jenny Lind Children’s Hospital,
and the Immunocompromised Norfolk and Norwich University
Chapter 8: Haematopoietic stem Hospital, UK
cell transplantation Chapter 2: Joint hypermobility

Mr Alwyn Abraham Dr Eileen Baildam

Consultant, Trauma Orthopaedics Consultant Paediatric
and Paediatric Orthopaedics Rheumatologist and Honorary
Leicester Royal Infirmary, UK Senior Lecturer
Chapter 1: Normal variants of lower Alder Hey Children’s Foundation
limb development; Normal gait NHS Trust, Liverpool, UK
and musculoskeletal development; Chapter 4: Connective tissue
The gait cycle and abnormal gait diseases: Scleroderma
patterns Dr Kathy Bailey
Chapter 2: Fractures in children and Consultant Paediatric
adolescents Rheumatologist
Dr Beverley Almeida George Eliot Hospital,
Specialist Registrar, Paediatric Nuneaton, UK
Rheumatology Chapter 4: Autoinflammatory
Great Ormond Street Hospital for diseases and immunodeficiency:
Children NHS Foundation Trust, Inflammatory bowel disease and
London, UK musculoskeletal features
Chapter 1: Thermography in Ms Catrin Barker
rheumatic disease; Nailfold Acting Deputy Chief Pharmacist
capillaroscopy in rheumatic disease Pharmacy Department, Alder Hey
Dr Tania Amin Children’s NHS Foundation Trust,
Specialist Registrar, Paediatrics Liverpool, UK
Royal Hospital for Sick Children, Chapter 8: Medicines for children
Edinburgh, UK and paediatric rheumatology
Chapter 3: JIA subtypes and their
xxx CONTRIBUTORS

Mr Tom Beckingsale UCL Institute of Child Health and

Specialist Registrar, Trauma and Great Ormond St Hospital for
Orthopaedics Children NHS Foundation Trust,
Newcastle upon Tyne NHS London, UK
Hospitals Foundation Trust, Preface: The purpose of this book
Newcastle upon Tyne, UK Chapter 4: Vasculitis: The
Chapter 2: Bone tumours classification of paediatric vasculitis;
The epidemiology of paediatric
Prof Michael Beresford vasculitis; The investigation of
Professor, Child Health, and primary systemic vasculitis; The
Honorary Consultant standard treatment of childhood
Paediatric Rheumatology vasculitis; Henoch-Schönlein
Department of Women’s and purpura; Kawasaki disease;
Children’s Health, Institute of The anti-neutrophil cytoplasmic
Translational Medicine, University antibody (ANCA)-associated
of Liverpool; and Alder Hey Vasculitides; Polyarteritis nodosa
Children’s NHS Foundation Trust, (PAN); Cutaneous polyarteritis
Liverpool, UK nodosa (cPAN); Takayasu arteritis;
Foreword 1: UK’s Paediatric Behçet’s Disease; Central nervous
Rheumatology Clinical Studies Group system vasculitis in children; Other
Chapter 4: Juvenile systemic lupus vasculitides; Vasculitis mimics:
erythematosus: JSLE: non-inflammatory vasculopathies
epidemiology and aetiology; JSLE: Chapter 4: Juvenile systemic
clinical features and diagnostic lupus erythematosus: JSLE: renal
criteria; Assessment of the child involvement; B-cell-targeted
with JSLE and monitoring of therapies for systemic lupus
disease activity; JSLE: approach erythematosus
to management; Neonatal lupus Chapter 4: Connective tissue
syndrome; British Isles Lupus diseases: Antiphospholipid
Assessment Group (BILAG) 2004 syndrome (APS)
Index; SLEDAI 2000 Disease Activity Chapter 4: Autoinflammatory
Index; SLICC/ACR Damage Index diseases and immunodeficiency:
(paediatric) Sarcoidosis
Chapter 9: Intravenous
Prof Nick Bishop cyclophosphamide
Professor of Paediatrics and
Honorary Consultant Dr Richard Brough
Sheffield Children’s Hospital, UK Consultant Paediatrician
Chapter 5: Metabolic bone diseases Shrewsbury and Telford
NHS Trust, Royal Shrewsbury
Dr Emily Boulter Hospital, UK
Specialist Trainee Paediatrics Chapter 2: Foot and ankle
Great Ormond Street Hospital, problems
London, UK
Chapter 4: Vasculitis: Behçet’s Dr Jenny Campbell
Disease Specialist Registrar, Clinical
Genetics
Dr Paul A. Brogan Northern Genetics Service,
Senior Lecturer, Paediatric Institute of Genetic Medicine,
Vasculitis and Honorary International Centre for Life,
Consultant in Newcastle upon Tyne, UK
Paediatric Rheumatology Chapter 5: Skeletal dysplasias
 CONTRIBUTORS xxxi
Dr Amparo Cavalle Bristol Royal Hospital for
Specialist Registrar Children, Bristol and Royal
Department of Paediatric National Hospital for Rheumatic
Rheumatology, Birmingham Diseases, UK
Children’s Hospital, UK Chapter 2: Pain syndromes and the
Chapter 7: Transitional care assessment of pain
Chapter 9: Disease-modifying
Dr Tariq Chaudry anti-rheumatic drugs (DMARDs):
Clinical Fellow, Paediatric Sulphasalazine; Azathioprine
Rheumatology
Great Ormond Street Hospital for Dr Hannah Connell
Children NHS Foundation Trust, Consultant Clinical Psychologist
London, UK Bristol Royal Hospital for
Chapter 1: Autoantibodies Children, Bristol and Royal
National Hospital for Rheumatic
Dr Alice Chieng Diseases, Bristol, UK
Consultant, Paediatric Chapter 2: Pain syndromes and the
Rheumatology assessment of pain
Royal Manchester Children’s
Hospital, UK Dr Mary Cruikshank
Chapter 4: Other systemic diseases: Specialist Registrar, Paediatric
Mucopolysaccharidoses (MPS) and Rheumatology
mucolipidoses (ML) Royal Hospital for Sick Children,
Glasgow, UK
Dr Julia Clark Chapter 3: Classification of JIA;
Consultant, Paediatric Immunology Prognostic indicators in JIA
and Infectious Diseases, Chapter 4: Other systemic diseases:
Honorary Senior Clinical Chromosomal abnormalities
Lecturer and associated musculoskeletal
Great North Children’s Hospital, morbidity
Newcastle upon Tyne NHS Chapter 9: Intra-articular
Hospitals Foundation Trust, corticosteroid use in JIA
Newcastle upon Tyne, UK
Chapter 6: Tuberculosis and Dr Joyce Davidson
mycobacterial infections Consultant Paediatric
Rheumatologist
Dr Gavin Cleary Royal Hospital for Sick Children,
Consultant Paediatric Glasgow, UK
Rheumatologist Chapter 3: JIA subtypes and their
Department of Rheumatology, clinical presentations;
Alder Hey Children’s NHS Prognostic indicators in JIA
Foundation Trust, Liverpool, UK Chapter 9: Intra-articular
Foreword 3 corticosteroid use in JIA
Chapter 4: Autoinflammatory
diseases and immunodeficiency: Dr Karen Davies
Chronic recurrent multifocal Consultant Paediatrician and
osteomyelitis Paediatric Rheumatologist
Chapter 10 and Colour plate section New Cross Hospital,
Wolverhampton, UK
Dr Jacqui Clinch Chapter 9: BSPAR standards of
Consultant, Paediatric care for children and young people
Rheumatology and Childhood Pain with JIA
xxxii CONTRIBUTORS

Dr Penny Davis NHS Foundation Trust and UCL

Consultant Paediatric Institute of Child Health,
Rheumatologist London, UK
Department of Rheumatology, Chapter 4: Vasculitis: The standard
Birmingham Children’s treatment of childhood
Hospital, UK vasculitis; Kawasaki disease; The
Chapter 2: Hip pain and hip anti-neutrophil cytoplasmic antibody
problems in children and (ANCA)-associated Vasculitides;
adolescents Central nervous system vasculitis
in children; Other vasculitides;
Prof Michael Dillon Vasculitis mimics: non-inflammatory
Emeritus Professor vasculopathies
Department of Nephrology, Great Chapter 4: Connective Tissue
Ormond Street Hospital for Diseases: Antiphospholipid
Children NHS Foundation Trust Syndrome (APS)
and UCL Institute of Child Health,
London, UK Ms Jill Ferrari
Chapter 4: Vasculitis: Polyarteritis Senior Lecturer
nodosa (PAN); Cutaneous Department of Health and
polyarteritis nodosa (cPAN) Bioscience, University of East
London, and Great Ormond
Ms Sharon Douglas Street Hospital for Children,
Consumer volunteer on the London, UK NHS Foundation Trust
MCRN/ARUK Paediatric Chapter 7: The role of the podiatrist
Rheumatology Clinical Study
Group, Consumer representative, Dr Daniel Fishman
Longniddry, Scotland, UK Consultant Rheumatologist
Foreword 2: Parent and young Luton and Dunstable NHS
person involvement in research—a Foundation Trust, UK
‘consumer’s’ perspective Chapter 3: Disease activity scores in
rheumatoid arthritis and JIA
Ms Deborah Eastwood
Consultant Orthopaedic Surgeon Prof Helen Foster
Great Ormond Street Hospital for Professor Paediatric
Children NHS Foundation Trust, Rheumatology, Newcastle
London, UK University and Honorary
Chapter 5: The osteochondroses Consultant
Great North Children’s Hospital,
Mr Clive Edelsten Newcastle upon Tyne NHS
Consultant, Paediatric Hospitals Foundation Trust,
Ophthalmology Newcastle upon Tyne, UK
Medical Ophthalmology Preface: The purpose of this book
Department, Great Ormond Foreword 4: Paediatric rheumatology
Street Hospital for Children NHS training in the UK
Foundation Trust, London, UK Chapter 1: History taking, physical
Chapter 4: Connective tissue examination, and approaches to
diseases: Paediatric uveitis investigation; Normal variants
Dr Despina Eleftheriou of lower limb development;
Normal gait and musculoskeletal
Clinical Research Fellow and development; The gait cycle and
Honorary Consultant abnormal gait patterns; pGALS:
Great Ormond Street Hospital
 CONTRIBUTORS xxxiii
paediatric gait, arms, legs, Mr Craig Gerrand
spine musculoskeletal screening Consultant Orthopaedic Surgeon
examination; pREMS: paediatric Newcastle upon Tyne NHS
regional examination of the Hospitals Foundation Trust,
musculoskeletal system Newcastle Upon Tyne, UK
Chapter 2: The limping child; Chapter 1: Bone tumours
Growing pains
Dr Sophie Hambleton
Dr Mark Friswell Clinical Senior Lecturer and
Consultant Paediatric Honorary Consultant
Rheumatologist Newcastle University and the
Great North Children’s Hospital, Great North Children’s Hospital,
Newcastle upon Tyne NHS Newcastle upon Tyne NHS
Hospitals Foundation Trust, Hospitals Foundation Trust,
Newcastle upon Tyne, UK Newcastle upon Tyne, UK
Chapter 3: Treatment Chapter 6: Varicella zoster infections
pathways in JIA
Chapter 9: BSPAR guidelines for Ms Liz Hardy
treatments used in paediatric Specialist Physiotherapist,
rheumatology; Disease-modifying Paediatric Rheumatology
anti-rheumatic drugs (DMARDs): Great North Children’s Hospital,
Mycophenolate mofetil (MMF); Newcastle upon Tyne NHS
Intravenous cyclophosphamide; Hospitals Foundation Trust,
Pamidronate; Epoprostenol/iloprost, Newcastle upon Tyne, UK
Etanercept (Enbrel®); Other Chapter 1: Normal variants of lower
anti-TNF-α: adalimumab limb development
(Humira®) and infliximab Chapter 2: Knee pain in children
(Remicade®); Anti-IL-1 treatments: and adolescents
anakinra (Kineret®), rilonacept
(Regeneron®), and canakinumab Dr Kirsty Haslam
(Ilaris®), Abatacept (Orencia®); Consultant Paediatrician with
Systemic corticosteroids (oral, special interest in Rheumatology
intramuscular, and intravenous) Bradford Teaching Hospitals
NHS Foundation Trust,
Dr Paul Galea Bradford, UK
Honorary Clinical Senior Lecturer Chapter 2: Back pain in children
University of Glasgow and adolescents; Scoliosis
Royal Hospital for Sick Children,
Glasgow, UK Dr Nathan Hasson
Chapter 3: Classification of JIA Consultant, Paediatric
Rheumatology
Dr Janet Gardner-Medwin The Portland Hospital, London, UK
Senior Lecturer, Paediatric Chapter 5: Heritable disorders of
Rheumatology connective tissue
University of Glasgow, UK
Chapter 3: Uveitis screening in JIA: Dr Dan Hawley
the approach to screening Consultant Paediatric
and guidelines Rheumatologist
Chapter 4: Other systemic diseases: Department of Paediatric
Chromosomal abnormalities and Rheumatology, Sheffield Children’s
associated musculoskeletal NHS Foundation Trust,
morbidity Sheffield, UK
xxxiv CONTRIBUTORS

Chapter 4: Connective tissue Dr Sharmila Jandial

diseases: Scleroderma Consultant Paediatric
Chapter 9: Disease-modifying Rheumatologist
anti-rheumatic drugs (DMARDs) Great North Children’s Hospital,
Dr Anne Hinks Newcastle NHS Hospitals
Foundation Trust, Newcastle upon
Lecturer Tyne, UK
Arthritis Research UK Chapter 1: History taking, physical
Epidemiology Unit, University examination, and approaches to
of Manchester, Manchester investigation; pGALS: paediatric gait,
Academic Health Science Centre, arms, legs, spine musculoskeletal
Manchester, UK screening examination
Chapter 3: Genetics and JIA: HLA
and non-HLA/MHC associations Dr Akhila Kavirayani
with subtypes of JIA Specialist Registrar, Paediatric
Dr Richard Hull Rheumatology
Bristol Royal Hospital for
Consultant Adult and Paediatric Children, Bristol, UK
Rheumatologist Chapter 2: Hip pain and hip problems
Queen Alexandra Hospital, in children and adolescents
Portsmouth, UK Chapter 9: Disease-modifying
Chapter 3: Surgery in the young anti-rheumatic drugs (DMARDs):
adult with JIA: practical issues Sulphasalazine
Dr Kimme Hyrich Dr Alison Kelly
Senior Lecturer, Rheumatic Specialist Registrar, Paediatric
Disease Epidemiology Rheumatology
Arthritis Research UK Bristol Royal Hospital for Children,
Epidemiology Unit, University of University Hospitals Bristol NHS
Manchester, UK Foundation Trust, Bristol, UK
Chapter 1: Epidemiology of Chapter 4: Connective tissue
musculoskeletal conditions diseases: Paediatric uveitis
Mr Talal Ibrahim Chapter 4: Autoinflammatory
Clinical Fellow, Paediatric diseases and immunodeficiency:
Orthopaedic Surgery Macrophage activation syndrome
Hospital for Sick Children, Ms Jane Kelly
Toronto, Canada Clinical Nurse Specialist, Paediatric
Chapter 1: Normal gait and Rheumatology
musculoskeletal development; Alder Hey Children’s Foundation
The gait cycle and abnormal gait Trust, Liverpool, UK
patterns Chapter 8: Biologic therapies for
Chapter 2: Fractures in children and paediatric rheumatological diseases
adolescents
Mr Chris Kershaw
Ms Gill Jackson
Consultant Orthopaedic Surgeon
Clinical Nurse Specialist, Paediatric Leicester Royal Infirmary,
Rheumatology Leicester, UK
Leeds Teaching Hospitals NHS Chapter 1: The gait cycle and
Trust, The General Infirmary, abnormal gait patterns
Leeds, UK
Chapter 7: The role of the clinical Dr Khulood Khawaja
nurse specialist Consultant Paediatrician,
 CONTRIBUTORS xxxv
Paediatric Rheumatologist Dr Clodagh Lowry
Bradford Teaching Hospitals NHS Consultant Paediatric
Foundation Trust, Bradford, UK Rheumatologist
Chapter 4: Autoinflammatory The Children’s University Hospital,
diseases and immunodeficiency: Dublin, Ireland
Arthritis and other musculoskeletal Chapter 4: Connective tissue
features associated with cystic diseases: Juvenile
fibrosis dermatomyositis
Dr Orla Killeen Ms Sue Maillard
Consultant Paediatric Physiotherapist
Rheumatologist Great Ormond Street Hospital for
Our Lady’s Children’s Children NHS Foundation Trust,
Hospital Crumlin, Dublin, London, UK
Ireland Chapter 4: Connective tissue
Chapter 2: Malignancy and diseases: Juvenile dermatomyositis
musculoskeletal presentations Chapter 7: The role of the
Dr Sophia King physiotherapist
Specialist Registrar, Paediatrics Dr Despoina Maritsi
Yorkhill Hospital, Glasgow, UK Clinical Fellow in Paediatric
Chapter 9: Non-steroidal Rheumatology
anti-inflammatory drugs Great Ormond Street Hospital for
(NSAIDs); Methylprednisolone Children NHS Foundation Trust,
Dr Helen Lachmann London, UK
Chapter 4: Vasculitis: Takayasu
Consultant Physician arteritis
UK National Amyloidosis
Centre, University College Dr Stephen Marks
London Medical School, Consultant Paediatric Nephrologist
London, UK Department of Paediatric
Chapter 4: Autoinflammatory Nephrology, Great Ormond
diseases and immunodeficiency: Street Hospital for Children
Periodic fever syndromes/ NHS Foundation Trust,
autoinflammatory disease London, UK
Chapter 4: Vasculitis: Henoch–
Dr Alice Leahy
Schönlein purpura
Consultant Paediatric Chapter 4: Juvenile systemic
Rheumatologist lupus erythematosus: JSLE: renal
Southampton General Hospital, involvement
Southampton, UK
Chapter 6: Lyme disease Dr Kate Martin
Consultant Paediatrician
Dr Valentina Leone Cheltenham General Hospital,
Specialist Registrar, Paediatric Cheltenham, UK
Rheumatology Chapter 2: Musculoskeletal
Great North Children’s Hospital, presentations and non-accidental
Newcastle upon Tyne NHS injury
Hospitals Foundation Trust,
Newcastle upon Tyne, UK Dr Neil Martin
Chapter 6: Tuberculosis and Specialist Registrar, Paediatric
mycobacterial disease Rheumatology
xxxvi CONTRIBUTORS

Great Ormond Street Hospital for Dr Sabrina McHale

Children NHS Foundation Trust, Specialist Registrar
London, UK Rheumatology Department, Our
Chapter 4: Autoinflammatory Lady’s Hospital for Sick Children,
diseases and immunodeficiency: Dublin, Ireland
Sarcoidosis Chapter 1: Epidemiology of
Chapter 5: Heritable disorders of paediatric musculoskeletal
connective tissue conditions
Chapter 9: Methylprednisolone
Dr Anne-Marie McMahon
Dr Joanne May Consultant, Paediatric
Specialist Registrar, Paediatric Rheumatology
Rheumatology Sheffield Children’s Hospital,
Bristol Children’s Hospital, Sheffield, UK
Bristol, UK Chapter 2: Bone and joint infections
Chapter 2: Pain syndromes and the
assessment of pain Ms Carolyn McTimoney
Chapter 9: Azathioprine Paediatric Rheumatology
Occupational Therapist
Dr Liza McCann Great North Children’s Hospital,
Consultant Paediatric Newcastle upon Tyne NHS
Rheumatologist Hospitals Foundation Trust,
Alder Hey Children’s NHS Newcastle upon Tyne, UK
Foundation Trust, Liverpool, UK Chapter 7: The role of the
Chapter 8: Biologic therapies for occupational therapist
paediatric rheumatological diseases
Chapter 9: Anti-B-cell therapies: Dr Andrea Myers
rituximab (Mabthera®) Consultant Rheumatologist,
Honorary Senior Lecturer
Dr Janet McDonagh Northumbria Healthcare NHS
Clinical Senior Lecturer and Trust, Tyne and Wear, UK
Honorary Consultant Chapter 2: Knee pain in children
Birmingham Children’s Hospital and adolescents
NHS Foundation Trust and
University of Birmingham, Dr Kiran Nistala
Birmingham, UK Arthritis Research UK Career
Chapter 7: Transitional care Progression Fellow, Honorary
Paediatric Rheumatology
Dr Flora McErlane Consultant
Specialist Registrar, Paediatric Rheumatology Unit, UCL Institute
Rheumatology of Child Health, London, UK
Alder Hey Children’s Hospital Chapter 3: Immunology and
NHS Trust, Liverpool, UK aetiology of JIA
Chapter 3: Disease activity scores in
rheumatoid arthritis and JIA Dr Susan O’Connell
Chapter 4: Autoinflammatory Consultant Physician
diseases and immunodeficiency: Lyme Borreliosis Unit, Health
Chronic recurrent multifocal Protection Agency laboratory,
osteomyelitis Southampton, UK
Chapter 9: Anti-B-cell therapies: Chapter 6: Lyme disease
rituximab (Mabthera®)
 CONTRIBUTORS xxxvii
Dr Clare Pain Rheumatologist
Specialist Registrar, Paediatric Great Ormond Street Hospital for
Rheumatology Children NHS Foundation Trust,
Alder Hey Children’s NHS London, UK
Foundation Trust, Liverpool, UK Chapter 4: Connective tissue
Chapter 4: Juvenile systemic lupus diseases: Juvenile dermatomyositis
erythematosus: JSLE: epidemiology Dr Athimalaipet Ramanan
and aetiology; JSLE: clinical features
and diagnostic criteria; Assessment Consultant, Paediatric
of the child with JSLE and monitoring Rheumatology
of disease activity; JSLE: approach Bristol Royal Hospital for Children
to management; Neonatal lupus and Royal National Hospital
syndrome; British Isles Lupus for Rheumatic Diseases, Bristol,
Assessment Group (BILAG) 2004 and Honorary Reader, Bristol
Index; SLEDAI 2000 Disease Activity University, Bristol, UK
Index; SLICC/ACR Damage Index Chapter 4: Connective tissue
(paediatric) diseases: Paediatric uveitis
Chapter 8: Biologic therapies for Chapter 4: Autoinflammatory
paediatric rheumatological diseases diseases and immunodeficiency:
Macrophage activation syndrome
Dr Anand Patel Chapter 9: Ciclosporin
Specialist Registrar
Dermatology Department, Dr Tim Rapley
Nottingham University Social Scientist
Hospitals NHS Trust, Institute of Health and Society,
Nottingham, UK Newcastle University, Newcastle
Chapter 6: Rheumatic fever upon Tyne, UK
Chapter 1: pREMS: paediatric
Dr Sanjay Patel regional examination of the
Consultant Paediatrician musculoskeletal system
Department of Paediatric
Infectious Diseases, St Mary’s Dr Phil Riley
Hospital, London, UK Consultant Paediatric
Chapter 2: Infections in the Rheumatologist
immunocompromised Royal Manchester Children’s
Chapter 4: Autoinflammatory Hospital, Manchester, UK
diseases and immunodeficiency: Chapter 4: Other systemic diseases:
Primary immunodeficiency and Mucopolysaccharidoses (MPS) and
rheumatological disease mucolipidoses (ML)
Chapter 8: Haematopoietic stem
cell transplantation Dr Madeleine Rooney
Senior Lecturer and Consultant in
Dr James Peters Paediatric Rheumatology
Specialist Registrar Centre for Infection and Immunity,
Department of Rheumatology, Queen’s University Belfast, Ireland
University College Hospital Chapter 1: Musculoskeletal
London, UK ultrasound and inflammatory arthritis
Chapter 4: Connective tissue Chapter 5: Metabolic bone diseases
diseases: Overlap syndromes
Dr Clive Ryder
Dr Clarissa Pilkington Consultant Paediatric
Consultant Paediatric Rheumatologist
xxxviii CONTRIBUTORS

Birmingham Children’s Hospital, Prof Taunton Southwood

Birmingham, UK Professor and Honorary
Chapter 8: Corticosteroid Consultant Paediatric
intra-articular injections Rheumatologist
Dr Rangaraj Satyapal School of Infection and Immunity,
College of Medical and Dental
Consultant Paediatric and Sciences, University of
Adolescent Rheumatologist Birmingham, UK
Nottingham Children’s Hospital; Chapter 1: Capillaroscopy in
Queen’s Medical Centre Campus, rheumatic disease
Nottingham University Hospitals
NHS Trust, Nottingham, UK Ms Liz Stretton
Chapter 6: Rheumatic fever Clinical Nurse Specialist, Paediatric
Chapter 9: Methotrexate Rheumatology
Dr Debajit Sen Nottingham Children’s Hospital;
Queen’s Medical Centre Campus,
Consultant, Paediatric and Nottingham University Hospitals
Adolescent Rheumatology NHS Trust, Nottingham, UK
University College London Chapter 8: Approvals for use of
Hospital, and Great Ormond biologic therapies
Street Hospital for Children NHS
Foundation Trust, London, UK Ms Helen Strike
Chapter 4: Connective tissue Clinical Nurse Specialist, Paediatric
diseases: Overlap syndromes Rheumatology
Dr Ethan Sen Paediatric and Adolescent
Rheumatology, Bristol Royal
Specialist Registrar, Paediatric Hospital for Children, Bristol, UK
Rheumatology Chapter 9: BSPAR drug information
Bristol Royal Hospital for leaflets for parents and families
Children, Bristol, UK
Chapter 9: Ciclosporin Dr Alexandra Tabor
Dr Utpal Shah Consultant Paediatrician
University Hospital of North
Formulation Research Fellow Staffordshire, Stoke-on-Trent,
Cheshire, Merseyside and North Staffordshire, UK
Wales Local Research Network; Chapter 1: Autoantibodies and
Medicines for Children Research rheumatic disease
Network; Alder Hey Children’s Chapter 4: Vasculitis: The
NHS Foundation Trust, classification of paediatric vasculitis;
Liverpool, UK The epidemiology of paediatric
Chapter 8: Medicines for children vasculitis
and paediatric rheumatology
Prof Wendy Thomson
Dr Eve Smith
Professor of Genetic Epidemiology
Specialist Trainee Paediatrics Arthritis Research UK
Great North Children’s Hospital, Epidemiology Unit, School
Newcastle upon Tyne NHS of Translational Medicine,
Hospitals Foundation Trust, Manchester Academy of Health
Newcastle upon Tyne, UK Sciences, The University of
Chapter 2: The limping child Manchester, UK
 CONTRIBUTORS xxxix
Chapter 3: Genetics and JIA: HLA Birmingham, UK
and non-HLA/MHC associations Chapter 9: BSPAR drug information
with subtypes of JIA leaflets for parents and families
Dr Helen Venning Dr Nick Wilkinson
Consultant Paediatric and Consultant Paediatric
Adolescent Rheumatologist Rheumatologist
Nottingham Children’s Hospital; Nuffield Orthopaedic Centre NHS
Queen’s Medical Centre Campus, Trust, Oxford, UK
Nottingham University Hospitals Chapter 1: Outcome measures
NHS Trust, Nottingham, UK in paediatric rheumatology; The
Chapter 8: Approvals for use of Childhood Health Assessment Score
biologic therapies
Prof Patricia Woo
Ms Katherine Venter Emeritus Professor
Lecturer in Sociology University College London, UK
University of Leicester, UK Chapter 4: Autoinflammatory
Foreword 2: Parent and young diseases and immunodeficiency:
person involvement in research—a Periodic fever syndromes/
‘consumer’s’ perspective autoinflammatory disease
Dr Joanna Walsh Dr Mark Wood
Consultant Paediatric Consultant Paediatric and
Rheumatologist Adolescent Rheumatologist
Royal Hospital for Sick Children, Leeds General Infirmary,
Edinburgh, UK Leeds, UK
Chapter 3: Treatment approaches Chapter 2: Pyrexia of unknown
in JIA origin
Chapter 7: The multidisciplinary team
Chapter 9: Hydroxychloroquine Dr Joanna Worsfold
Consumer volunteer on the
Dr Kishore Warrier MCRN/ARUK Paediatric
Specialist Trainee in Paediatrics Rheumatology Clinical Study
Great North Children’s Hospital, Group
Newcastle upon Tyne NHS Foreword 2: Parent and young
Hospitals Foundation Trust, person involvement in research—a
Newcastle upon Tyne, UK ‘consumer’s’ perspective
Chapter 6: Immunization schedules
and the immunocompromised Prof Edmond Wraith
Consultant Paediatrician and
Prof Lucy Wedderburn Honorary Professor
Professor of Paediatric St Mary’s Hospital, Manchester,
Rheumatology UK
Rheumatology Unit, UCL Institute Chapter 4: Other systemic diseases
of Child Health, London, UK Mucopolysaccharidoses (MPS) and
Chapter 3: Immunology and mucolipidoses (ML)
aetiology of JIA
Dr Michael Wright
Ms Pamela Whitworth Consultant Clinical Genetics
Clinical Nurse Specialist Northern Genetics Service,
Birmingham Children’s Hospital, Newcastle upon Tyne Hospitals
xl CONTRIBUTORS

NHS Foundation Trust, Newcastle Newcastle upon Tyne NHS

upon Tyne, UK Hospitals Foundation Trust,
Chapter 5: Skeletal dysplasias Newcastle upon Tyne, UK
Chapter 7: The role of the clinical
Dr Sue Wyatt nurse specialist
Consultant Paediatric
Rheumatologist Ms Karen Wynne
Leeds General Infirmary, Clinical Nurse Specialist, Paediatric
Leeds, UK Rheumatology
Chapter 2: Back pain in children Great Ormond Street Hospital for
and adolescents; Scoliosis Children NHS Foundation Trust,
London, UK
Ms Ruth Wyllie Chapter 4: Vasculitis: The
Clinical Nurse Specialist, Paediatric investigation of primary systemic
Rheumatology vasculitis
Great North Children’s Hospital,
 Chapter 1 1

Clinical skills and

assessment

Epidemiology of paediatric musculoskeletal conditions 2

History taking, physical examination, and approaches to
investigation 4
Normal variants of lower limb development 9
The gait cycle and abnormal gait patterns 11
Normal gait and musculoskeletal development 15
pGALS: paediatric gait, arms, legs, spine musculoskeletal
screening examination 18
pREMS: paediatric regional examination of the
musculoskeletal system 23
Musculoskeletal ultrasound in juvenile idiopathic arthritis 28
Autoantibodies 32
Thermography in rheumatic disease 35
Nailfold capillaroscopy in rheumatic disease 37
Outcome measures in paediatric rheumatology 41
The Child Health Assessment Questionnaire (CHAQ) 45
2 CHAPTER 1 Clinical skills and assessment

Epidemiology of paediatric
musculoskeletal conditions
Inflammatory musculoskeletal conditions
• Juvenile idiopathic arthritis (JIA), the most common chronic rheumatic
disease of childhood, is a clinically heterogeneous group of disorders
characterized by chronic inflammatory arthritis. JIA is considered to
be an autoimmune disease, which is thought to result of an immune
reaction caused or triggered by environmental factors such as
infectious agents in a genetically susceptible host, although the exact
aetiology has yet to be determined.
• Determining the incidence and prevalence, both within the UK and
internationally, has been challenged by changing classification criteria
(American College of Rheumatology [ACR] versus European League
Against Rheumatism [EULAR] versus League of Associations for
Rheumatology [ILAR]) as well as differences in case ascertainment.
• The earliest epidemiological studies of JIA performed in populations
from Western Europe and North America showed an incidence
varying between 1.3 per 100,000 person-years in France and 22.6 per
100,000 person-years in Norway. The prevalence of JIA was reported
as between 7 and 148 per 100,000 person-years, in France and Norway
respectively. However, comparison of these studies is difficult, because
different diagnostic criteria, e.g. EULAR vs. ACR, and different study
designs were used.
• More recent population-based studies data have shown a higher
prevalence of JIA, between 167 and 400 per 100,000 person-years;
this difference is likely due to undiagnosed cases being identified
in ‘healthy’ children when they underwent examination by trained
paediatric rheumatologists. A recent population-based multicentre
study performed in southeastern Norway found the annual incidence
of childhood arthritis was 71 per 100,000 children; however this study
included transient arthritis, postinfectious arthritis, and infectious
arthritis as well as JIA.
• A diagnostic register was established in the UK in 1989 with the
objectives of describing the demography and diagnostic classification
of children referred to paediatric rheumatology clinics and also to
estimate the incidence of juvenile arthritis in the UK. The annual
incidence rate for juvenile arthritis from 2 centres in 1996 was
10 per 100,000, and for all rheumatic disorders was 32–42 per
100,000 children under age 16.
• Ethnicity has also been found to play a role. Children of European
ancestry have been found to be at the highest risk of developing all
types of JIA compared to other ethnic groups, except rheumatoid
factor (RF)+ve polyarticular JIA. Also, native North American children
are at a higher risk than children of European descent of developing
a polyarticular course of disease, such as extended oligoarticular JIA,
RF−ve polyarticular JIA, and RF+ve polyarticular JIA.
 EPIDEMIOLOGY OF PAEDIATRIC MUSCULOSKELETAL CONDITIONS 3

Juvenile-onset systemic lupus erythematosus

• Childhood-onset juvenile systemic lupus erythematosus (SLE) was
found to account for <1% of patients in paediatric rheumatology clinics
in a UK Registry, compared to 1.5–3% in Canada and 4.5% in the USA.
In a Canadian study from a national Registry, the mean annual incidence
of SLE was estimated at 0.36 per 100,000 children.
Juvenile dermatomyositis
• The incidence of juvenile dermatomyositis in the UK and Ireland is
reported at a rate of 0.19 per 100,000 children. This compares with
rates of 0.25–0.41 per 100,000 for children in a US registry.
Juvenile scleroderma
• Scleroderma in childhood remains very rare. A recent UK study has
estimated the annual incidence to be 3.4 per million children for
localized scleroderma; and 0.27 per million for systemic disease.
Non-inflammatory musculoskeletal conditions
Hypermobility
• The prevalence of hypermobility in children as a phenomenon, as
opposed to benign joint hypermobility syndrome (BJHS), has been
measured in a number of studies previously and, depending on the age
or ethnicity of the study population or the inclusion criteria, has been
reported to be between 2.3% and 30%.
• However, the presence of hypermobility in children does not equate
to having BJHS. The latter is defined as the presence of a degree of
joint hypermobility measured by a prearranged and validated scoring
system, associated with musculoskeletal symptoms and signs, and other
connective tissue problems likely to be attributable to it.
• There is wide divergence in the joint laxity observed amongst
different ethnic groups. The Europeans are most stiff; the Bantu
tribes of intermediate joint laxity; and those originally from the Indian
subcontinent of most marked joint laxity.
• Considerable overlap between benign hypermobility and mild variants
of Ehlers–Danlos syndrome, Marfan syndrome, and osteogenesis
imperfect has been reported.
Complex regional pain syndrome
• The incidence of complex regional pain syndrome (CRPS) in children
is unknown. In a population study in the USA, the overall risk of CRPS
was 5.46 per 100,000 person years. However, this mainly reflected
adult CRPS, as the median age at diagnosis was 46yr.
• In large paediatric series CRPS is more common in girls (70%), and the
mean age at diagnosis is 13yr of age (range 5–17yr).
Further reading
Bowyer S, Roettcher P, and the members of the Pediatric Rheumatology Database Research
Group. Pediatric rheumatology clinic populations in the United States: results of a 3 year
survey. J Rheumatol 1996; 23:1968–74.
Malleson PN, Fung MY, Rosenberg AM. The incidence of pediatric rheumatic diseases: results from
the Canadian Pediatric Rheumatology Association Disease Registry. J Rheumatol 1996; 23:1981–7.
Symmons DP, Jones M, Osborne J, et al. Pediatric rheumatology in the United Kingdom: data from the
British Pediatric Rheumatology Group National Diagnostic Register. J Rheumatol 1996; 23:1975–80.
4 CHAPTER 1 Clinical skills and assessment

History taking, physical examination,

and approaches to investigation
• The differential diagnosis of musculoskeletal pain in children is broad
(Box 1.1). Taking a history and performing a physical examination
(general and musculoskeletal) are integral to making an accurate
diagnosis.
• In history taking it is worth asking open questions (Table 1.1), probing
for features that may suggest mechanical or inflammatory pathology
(Table 1.2), seeking any suggestion of systemic disease and ‘red flags’
(Box 1.2) to warrant urgent concern or clues in the history to suggest
inflammatory musculoskeletal disease (Box 1.3).
• Appropriate clinical assessment requires knowledge of normal
development, normal variants (see b Normal variants of lower limb
development, p 9), normal and abnormal gait patterns (see b The
gait cycle and abnormal gait patterns, p 11), clinical presentations at
different ages (e.g. the hip, see b Hip pain and hip problems in children
and adolescents, p 106), and an approach to initial investigations.
Further details are given in chapters relating to individual diseases.
Key points to note in the clinical assessment:
• A common pitfall in making a diagnosis is to inappropriately attribute
a child’s problem to trauma (which is a common event in the life of all
ambulant children).
• The history (often given by the parent or carer), may be based on
observations and interpretation of events made by others (such as
teachers or friends), and may be rather vague and ill-defined with
non-specific complaints such as ‘my child is limping’ or ‘my child is not
walking quite right’.
• Consider the age and development of the child and modify the
questioning and consultation style accordingly.
• The young child may deny having pain when asked directly, and this
should not be taken to mean that the child does not have any pain.
• Physical examination needs to be comprehensive:
• Assessment of pain is important (see b Pain syndromes and the
assessment of pain, p 87) and can result in physical examination
being challenging for everyone present.
• A musculoskeletal screening examination (e.g. pGALS—see b
pGALS, p 18) will identify areas of significant joint abnormality
which may not be apparent from the history alone. Some aspects
of pGALS, such as gait observation may be modified or need to be
postponed in the acutely unwell child.
• A more detailed regional musculoskeletal examination (see b
pREMS, p 23) is an adjunct to the screening examination and the
possibility of referred pain needs to be considered.
• Indolent presentations of chronic musculoskeletal disease can
impact on growth (either localized or generalized). It is important to
assess height and weight, review growth charts in the Parent Held
Record where available, and look for evidence of disproportionate
growth (e.g. asymmetrical leg length) or muscle wasting.
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