18.14.8 Pulmonary amyloidosis 4261 S.J. Bourke
18.14.8 Pulmonary amyloidosis 4261 S.J. Bourke
Contents xxvi 18.11 Diffuse parenchymal lung diseases 4166
18.11.1 Diffuse parenchymal lung disease: An introduction 4166 F. Teo and A.U. Wells
18.11.2 Idiopathic pulmonary fibrosis 4177 P.L. Molyneaux, A.G. Nicholson, N. Hirani, and A.U. Wells
18.11.3 Bronchiolitis obliterans and cryptogenic
organizing pneumonia 4185
Vasilis Kouranos and A.U. Wells
18.11.4 The lung in autoimmune rheumatic disorders 4191 M.A. Kokosi and A.U. Wells
18.11.5 The lung in vasculitis 4200 G.A. Margaritopoulos and A.U. Wells 18.12 Sarcoidosis 4208 Robert P. Baughman and Elyse E. Lower 18.13 Pneumoconioses 4219 P.T. Reid 18.14 Miscellaneous conditions 4235
18.14.1 Diffuse alveolar haemorrhage 4235 S.J. Bourke and G.P. Spickett
18.14.2 Eosinophilic pneumonia 4238 S.J. Bourke and G.P. Spickett
18.14.3 Lymphocytic infiltrations of the
lung 4241
S.J. Bourke
18.14.4 Hypersensitivity pneumonitis 4244 S.J. Bourke and G.P. Spickett
18.14.5 Pulmonary Langerhans’ cell
histiocytosis 4256
S.J. Bourke
18.14.6 Lymphangioleiomyomatosis 4257 S.J. Bourke
18.14.7 Pulmonary alveolar proteinosis 4259 S.J. Bourke
18.14.8 Pulmonary amyloidosis 4261 S.J. Bourke
18.14.9 Lipoid (lipid) pneumonia 4263 S.J. Bourke
18.14.10 Pulmonary alveolar microlithiasis 4265 S.J. Bourke
18.14.11 Toxic gases and aerosols 4267 Chris Stenton
18.14.12 Radiation pneumonitis 4271 S.J. Bourke
18.14.13 Drug-induced lung disease 4272 S.J. Bourke 18.15 Chronic respiratory failure 4282 Michael I. Polkey and P.M.A. Calverley 18.16 Lung transplantation 4292 P. Hopkins and A.J. Fisher 18.17 Pleural diseases 4305 D. de Fonseka, Y.C. Gary Lee, and N.A. Maskell 18.18 Disorders of the thoracic cage and diaphragm 4328 John M. Shneerson and Michael I. Polkey 18.19 Malignant diseases 4338
18.19.1 Lung cancer 4338 S.G. Spiro and N. Navani
18.19.2 Pulmonary metastases 4360 S.G. Spiro
18.19.3 Pleural tumours 4361 Y.C. Gary Lee
18.19.4 Mediastinal tumours and cysts 4368 Y.C. Gary Lee and Helen E. Davies SECTION 19 Rheumatological disorders Section editor: Richard A. Watts 19.1 Joints and connective tissue—structure and function 4379 Thomas Pap, Adelheid Korb-Pap, Christine Hartmann, and Jessica Bertrand 19.2 Clinical presentation and diagnosis of rheumatological disorders 4386 Christopher Deighton and Fiona Pearce 19.3 Clinical investigation 4395 Michael Doherty and Peter C. Lanyon 19.4 Back pain and regional disorders 4406 Carlo Ammendolia and Danielle Southerst 19.5 Rheumatoid arthritis 4415 Kenneth F. Baker and John D. Isaacs 19.6 Spondyloarthritis and related conditions 4441 Jürgen Braun and Joachim Sieper 19.7 Infection and arthritis 4457 Graham Raftery and Muddassir Shaikh 19.8 Reactive arthritis 4464 Carmel B. Stober and Hill Gaston 19.9 Osteoarthritis 4470 Andrew J. Barr and Philip G. Conaghan
Contents xxvii 19.10 Crystal-related arthropathies 4482 Edward Roddy and Michael Doherty 19.11 Autoimmune rheumatic disorders and vasculitides 4495
19.11.1 Introduction 4495 David A. Isenberg and Ian Giles
19.11.2 Systemic lupus erythematosus and related disorders 4499 Anisur Rahman and David A. Isenberg
19.11.3 Systemic sclerosis (scleroderma) 4513 Christopher P. Denton and Carol M. Black
19.11.4 Sjögren’s syndrome 4532 Wan-Fai Ng
19.11.5 Inflammatory myopathies 4537
Ingrid E. Lundberg, Hector Chinoy, and
Robert Cooper
19.11.6 Large vessel vasculitis 4546 Raashid Luqmani and Cristina Ponte
19.11.7 ANCA-associated vasculitis 4556 David Jayne
19.11.8 Polyarteritis nodosa 4569 Loïc Guillevin
19.11.9 Small vessel vasculitis 4573 Richard A. Watts
19.11.10 Behçet’s syndrome 4579
Sebahattin Yurdakul, Izzet Fresko, and
Hasan Yazici
19.11.11 Polymyalgia rheumatica 4584 Bhaskar Dasgupta and Eric L. Matteson
19.11.12 Kawasaki disease 4590
Brian W. McCrindle
19.12 Miscellaneous conditions presenting to the
rheumatologist 4598
Stuart Carter, Lisa Dunkley, and Ade Adebajo
SECTION 20
Disorders of the skeleton
Section editor: Cyrus Cooper
20.1 Skeletal disorders—general approach and clinical
conditions 4615
B. Paul Wordsworth and M.K. Javaid
20.2 Inherited defects of connective tissue:
Ehlers–Danlos syndrome, Marfan’s syndrome,
and pseudoxanthoma elasticum 4670
N.P. Burrows
20.3 Osteomyelitis 4688
Martin A. McNally and Anthony R. Berendt
20.4 Osteoporosis 4696
Nicholas C. Harvey, Juliet Compston, and Cyrus Cooper
20.5 Osteonecrosis, osteochondrosis, and
osteochondritis dissecans 4703
Gavin Clunie
20.6 Bone cancer 4709
Helen Hatcher
SECTION 21
Disorders of the kidney and
urinary tract
Section editor: John D. Firth
21.1 Structure and function of the kidney 4717
Steve Harper and Robert Unwin
21.2 Electrolyte disorders 4729
21.2.1 Disorders of water and sodium homeostasis 4729 Michael L. Moritz and Juan Carlos Ayus
21.2.2 Disorders of potassium homeostasis 4748 John D. Firth 21.3 Clinical presentation of renal disease 4764 Richard E. Fielding and Ken Farrington 21.4 Clinical investigation of renal disease 4781 Andrew Davenport 21.5 Acute kidney injury 4807 John D. Firth 21.6 Chronic kidney disease 4830 Alastair Hutchison 21.7 Renal replacement therapy 4861
21.7.1 Haemodialysis 4861 Robert Mactier
21.7.2 Peritoneal dialysis 4874 Simon Davies
21.7.3 Renal transplantation 4879 Nicholas Torpey and John D. Firth 21.8 Glomerular diseases 4909
21.8.1 Immunoglobulin A nephropathy and IgA
vasculitis (HSP) 4909
Jonathan Barratt and John Feehally
21.8.2 Thin membrane nephropathy 4918 Peter Topham and John Feehally
Contents xxviii
21.8.3 Minimal-change nephropathy and focal segmental glomerulosclerosis 4919 Moin Saleem and Lisa Willcocks
21.8.4 Membranous nephropathy 4928 An S. De Vriese and Fernando C. Fervenza
21.8.5 Proliferative glomerulonephritis 4933 Alan D. Salama and Mark A. Little
21.8.6 Membranoproliferative glomerulonephritis 4937 Tabitha Turner-Stokes and Mark A. Little
21.8.7
Antiglomerular basement membrane
disease 4943
Mårten Segelmark and Thomas Hellmark
21.9
Tubulointerstitial diseases 4951
21.9.1 Acute interstitial nephritis 4951 Simon D. Roger
21.9.2
Chronic tubulointerstitial nephritis 4956
Marc E. De Broe, Channa Yamasumana,
Patrick C. D’Haese, Monique M. Elseviers, and
Benjamin Vervaet
21.10 The kidney in systemic disease 4975
21.10.1 Diabetes mellitus and the kidney 4975 Rudolf Bilous
21.10.2 The kidney in systemic vasculitis 4988 David Jayne
21.10.3 The kidney in rheumatological
disorders 5001
Liz Lightstone and Hannah Beckwith
21.10.4 The kidney in sarcoidosis 5012 Ingeborg Hilderson and Jan Donck
21.10.5 Renal involvement in plasma cell dyscrasias, immunoglobulin-based amyloidoses, and fibrillary glomerulopathies, lymphomas, and leukaemias 5016 Pierre Ronco, Frank Bridoux, and Arnaud Jaccard
21.10.6 Haemolytic uraemic syndrome 5027 Edwin K.S. Wong and David Kavanagh
21.10.7 Sickle cell disease and the kidney 5032 Claire C. Sharpe
21.10.8 Infection-associated nephropathies 5034 A. Neil Turner
21.10.9 Malignancy-associated renal disease 5041 A. Neil Turner
21.10.10 Atherosclerotic renovascular disease 5044 Philip A. Kalra and Diana Vassallo 21.11 Renal diseases in the tropics 5049 Vivekanand Jha 21.12 Renal involvement in genetic disease 5065 D. Joly and J.P. Grünfeld 21.13 Urinary tract infection 5074 Charles Tomson and Neil Sheerin 21.14 Disorders of renal calcium handling, urinary stones, and nephrocalcinosis 5093 Christopher Pugh, Elaine M. Worcester, Andrew P. Evan, and Fredric L. Coe 21.15 The renal tubular acidoses 5104 John A. Sayer and Fiona E. Karet 21.16 Disorders of tubular electrolyte handling 5112 Nine V.A.M. Knoers and Elena N. Levtchenko 21.17 Urinary tract obstruction 5124 Muhammad M. Yaqoob and Kieran McCafferty 21.18 Malignant diseases of the urinary tract 5136 Tim Eisen, Freddie C. Hamdy, and Robert A. Huddart 21.19 Drugs and the kidney 5150 Aine Burns and Caroline Ashley Index Volume 4 List of abbreviations xxxv List of contributors xlv SECTION 22 Haematological disorders Section editors: Chris Hatton and Deborah Hay 22.1 Introduction to haematology 5169 Chris Hatton 22.2 Haematopoiesis 5172
22.2.1 Cellular and molecular basis of haematopoiesis 5172 Paresh Vyas and N. Asger Jakobsen
22.2.2 Diagnostic techniques in the assessment of haematological malignancies 5181 Wendy N. Erber
Contents xxix 22.3 Myeloid disease 5189
22.3.1 Granulocytes in health and disease 5189 Joseph Sinning and Nancy Berliner
22.3.2 Myelodysplastic syndromes 5197 Charlotte K. Brierley and David P. Steensma
22.3.3 Acute myeloid leukaemia 5205 Nigel Russell and Alan Burnett
22.3.4 Chronic myeloid leukaemia 5213 Mhairi Copland and Tessa L. Holyoake†
22.3.5 The polycythaemias 5227 Daniel Aruch and Ronald Hoffman
22.3.6 Thrombocytosis and essential thrombocythaemia 5239 Daniel Aruch and Ronald Hoffman
22.3.7 Primary myelofibrosis 5247 Evan M. Braunstein and Jerry L. Spivak
22.3.8 Eosinophilia 5254 Peter F. Weller
22.3.9 Histiocytosis 5259 Chris Hatton 22.4 Lymphoid disease 5263
22.4.1 Introduction to lymphopoiesis 5263 Caron A. Jacobson and Nancy Berliner
22.4.2 Acute lymphoblastic leukaemia 5269
H. Josef Vormoor, Tobias F. Menne, and
Anthony V. Moorman
22.4.3 Hodgkin lymphoma 5280 Vijaya Raj Bhatt and James O. Armitage
22.4.4 Non-Hodgkin lymphoma 5288 Vijaya Raj Bhatt and James O. Armitage
22.4.5 Chronic lymphocytic leukaemia 5302 Clive S. Zent and Aaron Polliack
22.4.6 Plasma cell myeloma and related monoclonal gammopathies 5310 S. Vincent Rajkumar and Robert A. Kyle 22.5 Bone marrow failure 5325
22.5.1 Inherited bone marrow failure syndromes 5325 Irene Roberts and Inderjeet S. Dokal
22.5.2 Acquired aplastic anaemia and pure red cell
aplasia 5336
Judith C.W. Marsh, Shreyans Gandhi,
and Ghulam J. Mufti
22.5.3 Paroxysmal nocturnal haemoglobinuria 5348 Lucio Luzzatto 22.6 Erythroid disorders 5354
22.6.1 Erythropoiesis 5354 Vijay G. Sankaran
22.6.2 Anaemia: pathophysiology, classification, and clinical features 5359 David J. Weatherall† and Chris Hatton
22.6.3 Anaemia as a challenge to world health 5366 David J. Roberts and David J. Weatherall†
22.6.4 Iron metabolism and its disorders 5371 Timothy M. Cox and John B. Porter
22.6.5 Anaemia of inflammation 5402 Sant-Rayn Pasricha and Hal Drakesmith
22.6.6 Megaloblastic anaemia and miscellaneous deficiency anaemias 5407 A.V. Hoffbrand
22.6.7 Disorders of the synthesis or function of haemoglobin 5426 Deborah Hay and David J. Weatherall†
22.6.8
Anaemias resulting from defective maturation
of red cells 5450
Stephen J. Fuller and James S. Wiley
22.6.9 Disorders of the red cell membrane 5456 Patrick G. Gallagher
22.6.10 Erythrocyte enzymopathies 5463 Alberto Zanella and Paola Bianchi
22.6.11 Glucose-6-phosphate dehydrogenase deficiency 5472 Lucio Luzzatto
22.6.12 Acquired haemolytic anaemia 5479 Amy Powers and Leslie Silberstein 22.7 Haemostasis 5490
22.7.1
The biology of haemostasis and
thrombosis 5490
Gilbert C. White, II, Harold R. Roberts,
and Nigel S. Key
22.7.2 Evaluation of the patient with a bleeding tendency 5509 Trevor Baglin
22.7.3 Thrombocytopenia and disorders of platelet function 5520 Nicola Curry and Susie Shapiro
22.7.4 Genetic disorders of coagulation 5532 Eleanor S. Pollak and Katherine A. High
22.7.5 Acquired coagulation disorders 5546 T.E. Warkentin † It is with great regret that we report that Tessa L. Holyoake died on 30 August, 2017. † It is with great regret that we report that David J. Weatherall died on 8 December, 2018.
Contents xxx 22.8 Transfusion and transplantation 5563
22.8.1 Blood transfusion 5563 D.S. Giovanniello and E.L. Snyder
22.8.2 Haemopoietic stem cell transplantation 5579
E.C. Gordon-Smith and Emma C. Morris
SECTION 23
Disorders of the skin
Section editor: Roderick J. Hay
23.1
Structure and function of skin 5591
John A. McGrath
23.2
Clinical approach to the diagnosis of skin
disease 5596
Vanessa Venning
23.3
Inherited skin disease 5602
Thiviyani Maruthappu and David P. Kelsell
23.4
Autoimmune bullous diseases 5612
Kathy Taghipour and Fenella Wojnarowska
23.5
Papulosquamous disease 5621
Christopher E.M. Griffiths
23.6
Dermatitis/eczema 5630
Peter S. Friedmann, Michael J. Arden-Jones, and Roderick J. Hay
23.7
Cutaneous vasculitis, connective tissue
diseases, and urticaria 5639
Volha Shpadaruk and Karen E. Harman
23.8
Disorders of pigmentation 5677
Eugene Healy
23.9
Photosensitivity 5688
Hiva Fassihi and Jane McGregor
23.10 Infections of the skin 5695
Roderick J. Hay
23.11 Sebaceous and sweat gland disorders 5699
Alison M. Layton
23.12 Blood and lymphatic vessel disorders 5709
Peter S. Mortimer and Roderick J. Hay
23.13 Hair and nail disorders 5724
David de Berker
23.14 Tumours of the skin 5732
Edel O’Toole
23.15 Skin and systemic diseases 5743
Clive B. Archer and Charles M.G. Archer
23.16 Cutaneous reactions to drugs 5752
Sarah Walsh, Daniel Creamer, and Haur Yueh Lee
23.17 Management of skin disease 5761
Rod Sinclair
SECTION 24
Neurological disorders
Section editor: Christopher Kennard
24.1 Introduction and approach to the patient with
neurological disease 5775
Alastair Compston and Christopher Kennard
24.2 Mind and brain: Building bridges
between neurology, psychiatry, and
psychology 5778
Adam Zeman
24.3 Clinical investigation of neurological disease 5781
24.3.1 Lumbar puncture 5781 R. Rhys Davies and Andrew J. Larner
24.3.2 Electrophysiology of the central and peripheral nervous systems 5785 Christian Krarup
24.3.3 Imaging in neurological diseases 5802
Andrew J. Molyneux, Shelley Renowden, and
Marcus Bradley
24.3.4 Investigation of central motor pathways:
Magnetic brain stimulation 5817
K.R. Mills
24.4 Higher cerebral function 5821
24.4.1 Disturbances of higher cerebral function 5821 Peter J. Nestor
24.4.2 Alzheimer’s disease and other dementias 5830 Jonathan M. Schott 24.5 Epilepsy and disorders of consciousness 5860
24.5.1 Epilepsy in later childhood and adulthood 5860 Arjune Sen and M.R. Johnson
24.5.2 Narcolepsy 5882 Matthew C. Walker
24.5.3 Sleep disorders 5886 Paul J. Reading
24.5.4 Syncope 5896 Andrew J. Larner
24.5.5 The unconscious patient 5901 David Bates
Contents xxxi
24.5.6 Brainstem death and prolonged disorders of consciousness 5908 Ari Ercole, Peter J. Hutchinson, and John D. Pickard 24.6 Disorders of the special senses 5913
24.6.1 Visual pathways 5913 Sara Ajina and Christopher Kennard
24.6.2 Eye movements and balance 5922 Michael Strupp and Thomas Brandt
24.6.3 Hearing loss 5931 Linda Luxon 24.7 Disorders of movement 5937
24.7.1 Subcortical structures: The cerebellum, basal ganglia, and thalamus 5937 Mark J. Edwards and Penelope Talelli
24.7.2 Parkinsonism and other extrapyramidal diseases 5946 Elisaveta Sokolov, Vinod K. Metta, and K. Ray Chaudhuri
24.7.3 Movement disorders other than Parkinson’s disease 5956 Bettina Balint and Kailash Bhatia
24.7.4 Ataxic disorders 5976 Nicholas Wood 24.8 Headache 5987 Peter J. Goadsby 24.9 Brainstem syndromes 6006 David Bates 24.10 Specific conditions affecting the central nervous system 6010
24.10.1 Stroke: Cerebrovascular disease 6010 J. van Gijn (revised by Peter M. Rothwell)
24.10.2 Demyelinating disorders of the central nervous system 6026 Alasdair Coles and Siddharthan Chandran
24.10.3 Traumatic brain injury 6042 Tim Lawrence and Laurence Watkins
24.10.4 Intracranial tumours 6048 Jeremy Rees
24.10.5 Idiopathic intracranial hypertension 6054 Alexandra Sinclair 24.11 Infections of the central nervous system 6060
24.11.1 Bacterial infections 6060 Diederik van de Beek and Guy E. Thwaites
24.11.3 Intracranial abscesses 6097 Tim Lawrence and Richard S.C. Kerr
24.11.4 Neurosyphilis and neuro-AIDS 6100 Hadi Manji
24.11.5 Human prion diseases 6109 Simon Mead and R.G. Will 24.12 Disorders of cranial nerves 6120 Robert D.M. Hadden 24.13 Disorders of the spinal cord 6127
24.13.1 Diseases of the spinal cord 6127 Anu Jacob and Andrew J. Larner
24.13.2 Spinal cord injury and its management 6135 Wagih El Masri(y) and Michael Barnes 24.14 Diseases of the autonomic nervous system 6150 Christopher J. Mathias and David A. Low 24.15 The motor neuron diseases 6166 Tom Jenkins, Alice Brockington, and Pamela J. Shaw 24.16 Diseases of the peripheral nerves 6176 Robert D.M. Hadden 24.17 Inherited neurodegenerative diseases 6197 Swati Sathe 24.18 Disorders of the neuromuscular junction 6295 David Hilton-Jones and Jacqueline Palace 24.19 Disorders of muscle 6304
24.19.1 Structure and function of muscle 6304 Michael G. Hanna and Enrico Bugiardini
24.19.2 Muscular dystrophy 6310 Kate Bushby and Chiara Marini-Bettolo
24.19.3 Myotonia 6328 David Hilton-Jones
24.19.4 Metabolic and endocrine disorders 6334 David Hilton-Jones and Richard Edwards
24.19.5 Mitochondrial disease 6343 Patrick F. Chinnery and D.M. Turnbull 24.20 Developmental abnormalities of the central nervous system 6350 Chris M. Verity, Jane A. Hurst, and Helen V. Firth 24.21 Acquired metabolic disorders and the nervous system 6368 Neil Scolding 24.22 Neurological complications of systemic disease 6376 Neil Scolding
Contents xxxii 24.23 Paraneoplastic neurological syndromes 6384 Jeremy Rees 24.24 Autoimmune encephalitis and Morvan’s syndrome 6393 Camilla Buckley and Angela Vincent SECTION 25 Disorders of the eye Section editor: Christopher P. Conlon 25.1 The eye in general medicine 6399 Tasanee Braithwaite, Richard W.J. Lee, and Peng T. Khaw SECTION 26 Psychiatric and drug-related disorders Section editor: Michael Sharpe 26.1 General introduction 6445 Michael Sharpe 26.2 The psychiatric assessment of the medical patient 6447 Jane Walker, Roger Smyth, and Michael Sharpe 26.3 Common psychiatric presentations in medical patients 6454
26.3.1 Confusion 6454 Bart Sheehan and Thomas Jackson
26.3.2 Self-harm 6457 Kate E.A. Saunders and Keith Hawton
26.3.3 Medically unexplained symptoms 6460 Michael Sharpe
26.3.4 Low mood 6462 Jane Walker 26.4 Psychiatric treatments in the medically ill 6465
26.4.1 Psychopharmacology in medical practice 6465 Philip J. Cowen
26.4.2 Psychological treatments 6470 Michael Sharpe and Simon Wessely 26.5 Specific psychiatric disorders 6475
26.5.1 Delirium 6475 Bart Sheehan
26.5.2 Dementia 6478 Bart Sheehan
26.5.3 Organic psychoses 6482 Curtis McKnight and Jason Caplan
26.5.4 Alcohol misuse 6486 Jonathan Wood
26.5.5 Substance misuse 6490 Stephen Potts
26.5.6 Depressive disorder 6493 Joseph Cerimele and Lydia Chwastiak
26.5.7 Bipolar disorder 6498 Kate E.A. Saunders and John Geddes
26.5.8 Anxiety disorders 6501 Ted Liao and Steve Epstein
26.5.9
Acute stress disorder, adjustment disorders,
and post-traumatic stress disorder 6506
Jonathan I. Bisson
26.5.10 Eating disorders 6509 Christopher G. Fairburn
26.5.11 Schizophrenia 6513 Stephen M. Lawrie
26.5.12 Somatic symptom and related
disorders 6517
Michael Sharpe
26.5.13 Personality disorders 6520 Iain Jordan 26.6 Changing unhealthy behaviours 6524
26.6.1 Brief interventions for excessive alcohol consumption 6524 Amy O’Donnell, Eileen Kaner, and Nick Heather
26.6.2 Obesity and weight management 6529 Susan Jebb and Paul Aveyard
26.6.3
Smoking cessation 6533
Paul Aveyard
26.7
Psychiatry, liaison psychiatry, and
psychological medicine 6536
Michael Sharpe
SECTION 27
Forensic medicine
Section editor: John D. Firth
27.1 Forensic and legal medicine 6541
Jason Payne-James, Paul Marks, Ralph Bouhaidar, and
Steven B. Karch
Contents
xxxiii
SECTION 28
Sport and exercise medicine
Section editor: John D. Firth
28.1 Sport and exercise medicine 6565
Cathy Speed
SECTION 29
Biochemistry in medicine
Section editor: Christopher P. Conlon
29.1 The use of biochemical analysis for diagnosis
and management 6577
Brian Shine and Nishan Guha
SECTION 30
Acute medicine
Section editor: John D. Firth
30.1 Acute medical presentations 6591
Sian Coggle, Elaine Jolly, and John D. Firth
30.2 Practical procedures 6644
Elaine Jolly, Sian Coggle, and John D. Firth
Index
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