oxford american handbook of endocrinology and diabetes

Oxford American Handbook of
Endocrinology and
Diabetes
Draznin_Prelims.indd i 4/2/2011 6:51:22 PM
About the Oxford American Handbooks in Medicine
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Oxford American
Handbook of
Endocrinology
and Diabetes
Edited by

B oris D raznin, MD, PhD
Director, Adult Diabetes Program
Professor of Medicine
Division of Endocrinology, Metabolism and Diabetes
University of Colorado School of Medicine
Aurora, Colorado
S ol E pstein, MD
Professor of Medicine and Geriatrics
Mount Sinai School of Medicine
New York, NY
with
H elen E. T urner
J ohn A.H. W ass
1
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3
Oxford University Press, Inc. publishes works that further
Oxford University’s objective of excellence
in research, scholarship and education.
Oxford New York
Auckland Cape Town Dar es Salaam Hong Kong Karachi
Kuala Lumpur Madrid Melbourne Mexico City Nairobi
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With offi ces in
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Guatemala Hungary Italy Japan Poland Portugal
Singapore South Korea Switzerland Thailand Turkey Ukraine Vietnam
Copyright © 2011 by Oxford University Press, Inc.
Published by Oxford University Press Inc.
198 Madison Avenue, New York, New York 10016

www.oup.com
Oxford is a registered trade mark of Oxford University Press
First published 2011
UK version published: 2009
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,
electronic, mechanical, photocopying, recording, or otherwise,
without the prior permission of Oxford University Press,
Library of Congress Cataloging-in-Publication Data
Oxford American handbook of endocrinology and diabetes / edited by
Boris Draznin … [et al.].
p. ; cm.
Handbook of endocrinology and diabetes
Includes bibliographical references and index.
ISBN 978-0-19-537428-5
1. Endocrinology—Handbooks, manuals, etc. 2. Diabetes—Handbooks, manuals,
etc. I. Draznin, Boris. II. Title: Handbook of endocrinology and diabetes.
[DNLM: 1. Endocrine System Diseases—Handbooks. 2. Diabetes
Mellitus—Handbooks. WK 39]
RC649.O94 2011
616.4—dc22
2010050977
10 9 8 7 6 5 4 3 2 1
Printed in China
on acid-free paper
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This material is not intended to be, and should not be considered, a sub-
stitute for medical or other professional advice. Treatment for the con-
ditions described in this material is highly dependent on the individual
circumstances. And, while this material is designed to offer accurate infor-

mation with respect to the subject matter covered and to be current as
of the time it was written, research and knowledge about medical and
health issues is constantly evolving and dose schedules for medications are
being revised continually, with new side effects recognized and accounted
for regularly. Readers must therefore always check the product informa-
tion and clinical procedures with the most up-to-date published prod-
uct information and data sheets provided by the manufacturers and the
most recent codes of conduct and safety regulation. Oxford University
Press and the authors make no representations or warranties to readers,
express or implied, as to the accuracy or completeness of this material,
including without limitation that they make no representation or warran-
ties as to the accuracy or effi cacy of the drug dosages mentioned in the
material. The authors and the publishers do not accept, and expressly
disclaim, any responsibility for any liability, loss, or risk that may be claimed
or incurred as a consequence of the use and/or application of any of the
contents of this material.

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vi
Preface
In this era of rapid electronic transfer of medical information, the ques-
tion is often posed: “Do we need textbooks anymore, as by the time they
are printed much of the information is outdated?” This question is best
answered by this publication, which offers the reader current, practical,
clinical evidence–based information that will benefi t patients with endo-
crine and metabolic disease. This handbook is not meant to be a fully
comprehensive tome but to act as a quick and easily accessible source of
information.
While we have endeavored to include material that is as current as pos-
sible, the many advances occurring in the fi eld of endocrinology and dia-

betes made this a daunting task. This meant including emerging advances in
translational research, molecular biology, genomics, diagnostic tests, and
therapies without sacrifi cing clinical presentations.
Our expert colleagues, to whom we as co-editors are eternally indebted
and who are too numerous to mention individually, have devoted a con-
siderable amount of time to ensuring the provision of the most relevant
information in a very readable fashion. They accepted their assignments
with grace and a willingness to participate, which made our task as editors
so much easier. We are certain that the reader will enjoy this U.S. version
of the Oxford Handbook of Endocrinology and Diabetes and apply this to
their clinical practice.
We thank the authors of the original British edition, Helen E. Turner and
John A.H. Wass. We also thank the editorial staff of the Oxford University
Press for their professional assistance.
Boris Draznin and Sol Epstein

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vii
Contents
Contributors viii
Detailed contents ix
Symbols and abbreviations xxviii
Part 1 Thyroid
1

Part 2 Pituitary
81

Part 3 Adrenals
163

Part 4 Reproductive endocrinology
221

Part 5 Endocrine disorders of pregnancy
345

Part 6 Calcium and bone metabolism
365

Part 7 Pediatric endocrinology
429

Part 8 Neuroendocrine disorders
463

Part 9 Inherited endocrine syndromes
and multiple endocrine

neoplasia (MEN)
485

Part 10 Miscellaneous endocrinology
513

Part 11 Diabetes
585

Part 12 Lipids and hyperlipidemia
659

Part 13 Laboratory endocrinology
687

Index 699

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viii
Contributors
Marc-Andre Cornier, MD
Associate Professor of Medicine
Division of Endocrinology,
Metabolism and Diabetes
University of Colorado School of
Medicine
Aurora, CO
Part : Miscellaneous
Endocrinology
Emily Gallagher, MB,
BCh, BAO
Division of Endocrinology,
Diabetes, and Bone Disease
Mount Sinai School of Medicine
New York, NY
Part : Neuroendocrine Disorders
Dina Green, MD
Assistant Professor of Medicine
Division of Endocrinology,
Diabetes, and Bone Disease
Mount Sinai School of Medicine

New York, NY
Parts : Thyroid, Neuroendocrine
Disorders, Lipids and
Hyperlipidemia
Janice M. Kerr, MD
Assistant Professor
Division of Endocrinology,
Metabolism and Diabetes
University of Colorado Denver/
Anschutz Medical Campus
Aurora, CO
Part : Pituitary
Caroline Messer, MD
Division of Endocrinology,
Diabetes, and Bone Disease
Mount Sinai School of Medicine
New York, NY
Part : Thyroid
Kristen Nadeau, MS, MD
Assistant Professor of Pediatrics
Division of Pediatric
Endocrinology
University of Colorado School
of Medicine
Aurora, CO
Part : Pediatric Endocrinology
Rachel Pessah, MD
Division of Endocrinology,
Diabetes, and Bone Disease
Mount Sinai School of Medicine

New York, NY
Part : Thyroid
E. Chester Ridgway, MD
Professor of Medicine
Senior Associate Dean for
Academic Affairs
Vice Chairman: Department
of Medicine
University of Colorado School
of Medicine
Aurora, CO
Part : Laboratory Endocrinology
Barrie Weinstein, MD
Division of Endocrinology,
Diabetes, and Bone Disease
Mount Sinai School of Medicine
New York, NY
Part : Lipids and Hyperlipidemia
Margaret E. Wierman, MD
Chief of Endocrinology, Denver
VAMC
Professor of Medicine, Physiology
and Biophysics
University of Colorado School
of Medicine
Aurora, CO
Part : Reproductive Endocrinology
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ix
Detailed contents

Part 1 Thyroid
1 Anatomy and physiology of the thyroid 2
Anatomy 2
Physiology 4
2 Thyroid investigations 7
Tests of hormone concentration 7
Tests of homeostatic control 7
Antibody screen 10
Scintiscanning 11
Ultrasound (US) scanning 13
Fine needle aspiration (FNAC) cytology 14
Computed tomography (CT) 14
Additional laboratory investigations 15
Sick euthyroid syndrome (non-thyroidal illness syndrome) 15
Atypical clinical situations 16
3 Thyrotoxicosis 17
Etiology 17
Manifestations of hyperthyroidism 19
Treatment 21
Thyroid crisis (storm) 28
Thyrotoxicosis in pregnancy
31
Hyperthyroidism in children 35
Secondary hyperthyroidism 36
4 Graves’ ophthalmopathy, dermopathy, and acropachy 39
Graves’ ophthalmopathy 39
Graves’ dermopathy 43
Thyroid acropachy 43
5 Multinodular goiter and solitary adenomas 44
Background 44

Clinical evaluation 45
Studies 46
Treatment 47
Pathology 48
Thyroid nodules in pregnant mothers 48
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6 Thyroiditis 49
Background 49
Chronic autoimmune thyroiditis 50
Other types of thyroiditis 51
7 Hypothyroidism 53
Background 53
Clinical picture 55
Treatment of hypothyroidism 56
Treatment of subclinical hypothyroidism 57
Management of myxedema coma 58
Congenital hypothyroidism 60
8 Amiodarone and thyroid function 62
Background 62
Pathogenesis 63
Diagnosis and treatment 64
9 Thyroid cancer 66
Epidemiology 66
Etiology 67
Papillary thyroid carcinoma 68

Staging of DTC 69
Surgery 70

Postoperative thyroid hormone therapy 70
Radioactive iodine therapy 71
Management of recurrent disease 74
Follicular thyroid carcinoma (FTC) 75
Follow-up of papillary and follicular thyroid carcinoma 76
Medullary thyroid carcinoma (MTC) 78
Anaplastic (undifferentiated) thyroid cancer 79
Lymphoma 79
Part 2 Pituitary
10 Pituitary gland anatomy and physiology 82
Embryology and anatomy 82
Anterior gland physiology 83
Posterior gland physiology 85
11 Imaging 86
Background 86
MRI appearances 86
Pituitary adenomas 87
Craniopharyngiomas/Rathke’s cleft cyst 88
DETAILED CONTENTS
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12 Pituitary function testing 89
Background 89
Lactotropes 89
Thyrotropes 89
Gonadotropes 90
Corticotropes 91
Somatotropes 93
13 Hypopituitarism 94

Defi nition 94
Etiologies of hypopituitarism 94
Features 95
Investigations 97
Treatment 97
14 Anterior pituitary hormone replacement 98
Background 98
Glucocorticoids replacement 98
Growth hormone replacement 99
Thyroid hormone replacement 100
Sex hormone replacement 100
15 Pituitary tumor basics
101
Epidemiology 101
Classifi cation 101
Pathophysiology 102
Investigations 102
Management and natural history 103
16 Prolactinomas 104
Epidemiology 104
Etiology and pathogenesis 104
Clinical features 104
Investigations 106
Hyperprolactinemia and drugs 106
Idiopathic hyperprolactinemia 106
Treatment 107
Management of prolactinomas in pregnancy 109
17 Acromegaly 110
Defi nition 110
Epidemiology 110

Etiologies of acromegaly 110
Pathophysiology 110
Clinical features 110
Investigations 112
Management 114
Mortality data 115
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18 Cushing’s disease 116
Defi nition 116
Epidemiology 116
Differential diagnosis 116
Etiologies of CS 116
Clinical features of CS 117
Investigations 118
Treatment 122
Follow-up 124
19 Gonadotropinomas 125
Epidemiology 125
Pathology 125
Clinical features 125
Investigations 126
Management 126
Follow-up and prognosis 126
20 Thyrotropinomas 128
Epidemiology 128
Clinical features 128
Investigations
128

Management 128
21 Craniopharyngiomas and Rathke’s cleft cysts 130
Craniopharyngiomas 130
Epidemiology 130
Pathology 130
Clinical features 130
Investigations 131
Management 131
Follow-up 131
Rathke’s cleft cysts (RCC) 132
Epidemiology 132
Pathology 132
Clinical features 132
Investigations 132
Management 132
Follow-up 133
22 Parasellar tumors 134
Meningiomas 134
Germ cell tumor (GCT) 134

Gangliocytomas 134
Chordomas/condrosarcomas 135
Hamartomas 135
Metastatic tumors to the pituitary 135
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23 Infl ammatory and infi ltrative pituitary diseases 136
Background 136
Investigations 137

Lymphocytic hypophysitis 137
Sarcoidosis 138
Langerhans’ cell histiocytosis 139
Hemochromatosis 139
24 Surgical treatment of pituitary tumours 140
Transsphenoidal surgery (TSS) 140
Transfrontal craniotomy 143
25 Medical treatment of pituitary tumors 144
Dopamine agonists (DA) 144
Somatostatin analogs 145
Growth hormone receptor antagonists 146
26 Pituitary radiotherapy 147
Background 147
Techniques 147
Effi cacy 148
Complications
149
27 Diabetes insipidus (DI) 151
Defi nition 151
Classifi cation and etiologies 151
Features 152
Investigations 153
Treatment 154
Caveats for water deprivation study 155
28 Hyponatremia 156
Incidence 156
Etiologies 156
Neurosurgical hyponatremia 157
Classifi cation and clinical features 157
Investigations 158

Hyponatremia treatment 158
29 Syndrome of inappropriate ADH (SIADH) 159
Defi nition 159
Features 159
Criteria for diagnosis 159

Types of SIADH 159
Treatment 160
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30 Pineal gland 161
Physiology 161
Pineal and intracranial germ cell tumors 161
Features 161
Investigations and staging 161
Management 162
Prognosis 162
Part 3 Adrenals
31 Anatomy and physiology 164
Anatomy 164
Physiology 166
32 Imaging 168
Computed tomography (CT) scanning 168
Magnetic resonance imaging (MRI) 168
Ultrasound (US) imaging 168
Normal adrenal 169
Radionucleotide imaging 169
Positron emission tomography (PET) 169
Venous sampling 169

33 Mineralocorticoid excess 170
Defi nitions 170
34 Primary aldosteronism 171
Epidemiology 171
Pathophysiology 171
Clinical features 171
Conn’s syndrome—aldosterone-producing adenoma 171
Bilateral adrenal hyperplasia (bilateral idiopathic
hyperaldosteronism) 173

Glucocorticoid-remedial aldosteronism (GRA) 173
Aldosterone-producing carcinoma 173
Screening 174
Confi rmation of diagnosis 176
Localization and confi rmation of differential diagnosis 179
Treatment 180
35 Excess other mineralocorticoids 182
Epidemiology 182
Apparent mineralocorticoid excess 182
Licorice ingestion 183
Ectopic ACTH syndrome 183
Deoxycorticosterone (DOC) excess 183
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36 Adrenal Cushing’s syndrome 184
Defi nition and epidemiology 184
Pathophysiology 184
Clinical features 185
Investigations 185

Treatment 186
Prognosis 186
Subclinical Cushing’s syndrome 187
37 Adrenal surgery 188
Adrenalectomy 188
38 Renal tubular abnormalities 190
Background 190
Liddle’s syndrome 190
Bartter’s syndrome 191
39 Mineralocorticoid defi ciency 192
Epidemiology 192
Causes 192
Treatment 192
40 Adrenal insuffi ciency
193
Defi nition 193
Epidemiology 194
Pathophysiology 195
41 Addison’s disease 196
Causes of primary adrenal insuffi ciency 196
Autoimmune adrenalitis 197
Clinical features 198
Autoimmune polyglandular syndrome (APS) type 1 198
APS types 2–4 199
Investigation of primary adrenal insuffi ciency 200
Treatment 203
42 Long-term glucocorticoid administration 206
Short-term steroids 206
Steroid cover 206
Long-term steroids 207

Cushing’s syndrome 207
43 Adrenal incidentalomas 208
Defi nition and epidemiology 208

Importance 208
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Differential diagnosis of an incidentally detected adrenal
nodule 208
Investigations 209
Management 209
44 Pheochromocytomas and paragangliomas 210
Defi nition 210
Incidence 210
Epidemiology 210
Pathophysiology 212
Clinical features 213
Complications 213
Investigations 214
Management 218
Part 4 Reproductive endocrinology
45 Hirsutism 222
Defi nition 222
Physiology of hair growth 222
Androgen production in women 223
Evaluation 224
46 Polycystic ovary syndrome (PCOS) 227
Defi nition 227
Epidemiology 227

Other causes of hyperandrogenic anovulation 228
Features 230
Laboratory evaluation 232
Management 234
47 Congenital adrenal hyperplasia (CAH) in adults 239
Defi nition 239
Epidemiology 239
Pathogenesis 239
Biochemistry 240
Clinical presentation 241
Laboratory evaluation 242

Management 244
Monitoring of treatment 246
Prognosis 247
48 Androgen-secreting tumors 249
Defi nition 249
Epidemiology and pathology 249
Clinical features 250
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Laboratory evaluation 250
Management 251
Prognosis 251
49 Menstrual function disorders 252
Defi nitions 252
Clinical evaluation 253
Laboratory evaluation 255
Management 256

50 Premature ovarian insuffi ciency (POI) 257
Defi nition 257
Epidemiology 257
Pathogenesis 259
Clinical presentation 260
Laboratory evaluation 261
Management 262
Prognosis 263
51 Menopause 264
Defi nition 264
Long-term consequences
266
Clinical presentation 266
Evaluation (if HRT is being considered) 267
Hormone replacement therapy (HT) 268
52 Oral contraceptive pill (OCP) 275
Introduction 275
Benefi ts 276
Risks 277
Practical issues 279
53 Testicular physiology 280
Anatomy 280
Regulation of testicular function 281
Physiology 283
54 Male hypogonadism 284
Defi nition 284
Epidemiology 284
Secondary hypogonadism
(hypogonadotropic hypogonadism) 285
Primary hypogonadism

(hypergonadotropic hypogonadism) 289
Clinical assessment 293
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55 Androgen therapy 296
Treatment goals 296
Indications for treatment 297
Risks and side effects 298
56 Gynecomastia 301
Defi nition 301
Evaluation 303
Management 305
57 Erectile dysfunction 306
Defi nition 306
Physiology of male sexual function 306
Pathophysiology 307
Evaluation 308
Management 311
58 Testicular tumors 314
Epidemiology 314
Risk factors 314
Prognosis 315
59 Infertility
316
Defi nition 316
Causes 317
Evaluation 319
Management 324
Ovulation induction 328

60 Disorders of sexual differentiation 332
Clinical presentation 332
Evaluation 333
Androgen insensitivity syndrome 335
True hermaphroditism 336
General principles of management 337
61 Transsexualism 339
Defi nition 339
Epidemiology 339
Etiology 339
Management 340
Part 5 Endocrine disorders of pregnancy
62 Thyroid disorders 346
Normal physiology 346
Maternal hyperthyroidism 347
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Maternal hypothyroidism 349
Postpartum thyroid dysfunction 350
Thyroid cancer in pregnancy 351
63 Pituitary disorders 352
Normal anatomical changes during pregnancy 352
Normal physiology during pregnancy 353
Prolactinoma in pregnancy 354
Cushing’s syndrome 356
Acromegaly 357
Hypopituitarism in pregnancy 358
64 Adrenal disorders during pregnancy 361
Normal changes during pregnancy 361

Addison’s disease in pregnancy 362
Congenital adrenal hyperplasia 363
Pheochromocytoma 364
Part 6 Calcium and bone metabolism
65 Calcium and bone physiology 366
Bone turnover 366
Bone mass during life 367
Calcium 368
66 Investigation of bone 369
Bone turnover markers 369
Bone imaging 370
Bone mass measurements 371
Bone biopsy 372
67 Investigation of calcium, phosphate, and magnesium 373
Blood concentration 373
Urine excretion 374
Calcium-regulating hormones 375
68 Hypercalcemia 376
Epidemiology 376
Causes 376
Clinical features 377
Hyperparathyroidism 379
Treatment 382
Complications of parathyroidectomy
384
Other causes of hypercalcemia 385
Familial hypocalciuric hypercalcemia (FHH) 387
Vitamin D intoxication 387
Sarcoidosis 388
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69 Hypocalcemia 389
Causes 389
Clinical features 391
Investigation 393
Treatment 394
70 Rickets and osteomalacia 396
Defi nitions 396
Clinical features 396
Diagnosis 397
Vitamin D defi ciency 399
Hypophosphatemia 401
71 Hypomagnesemia 403
Introduction 403
Treatment 404
72 Osteoporosis 405
Introduction 405
Pathology 406
Epidemiology 408
Presentation 409
Investigation 410
Treatment 413
Complications of therapy 417
73 Paget’s disease 421
Pathology 421
Epidemiology 422
Clinical features 422
Investigation 423
Complications 424

Treatment 425
Monitoring therapy 426
74 Inherited disorders of bone 427
Osteogenesis imperfecta 427
Part 7 Pediatric endocrinology
75 Growth and stature 430
Growth 430
Short stature 434
Genetic short stature 436
Constitutional delay of growth and puberty 436
Growth hormone defi ciency 437
Tall stature and rapid growth 445
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76 Puberty 447
Normal puberty 447
Precocious puberty 449
Delayed or absent puberty 453
77 Sexual differentiation 455
Normal sexual differentiation 455
Assessment of ambiguous genitalia 456
Disorders of sex development (DSD) 457
Congenital adrenal hyperplasia 459
Part 8 Neuroendocrine disorders
78 The neuroendocrine system 464
Introduction 464
79 Carcinoid tumors 465
Defi nition and classifi cation 465
Incidence 465

Carcinoid syndrome 466
Diagnostic investigations 467
Tumor localization and staging 468
Treatment 469
Prognosis 471
80 Insulinomas 472
Defi nition 472
Incidence 472
Clinical presentation 472
Biochemical investigations 472
Tumor localization 473
Treatment 474
Prognosis 474
81 Gastrinomas 475
Defi nition 475
Prevalence 475
Clinical presentation 475
Biochemical investigations 476
Treatment 477
82 Glucagonomas 478
Defi nition 478
Incidence 478
Clinical presentation 478
Biochemical investigations 479
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Tumour localization 479
Treatment 480
83 VIPomas 481

Defi nition 481
Clinical presentation 481
Biochemical investigations 482
Tumor localization 482
Treatment 482
84 Somatostatinomas 483
Defi nition 483
Clinical features 483
Biochemical investigations 484
Tumor localization 484
Treatment 484
Part 9 Inherited endocrine syndromes and
multiple endocrine neoplasia (MEN)
85 McCune–Albright syndrome 486
Defi nition 486
Genetics 486
Clinical features 487
Prognosis 488
86 Neurofi bromatosis 489
Defi nition 489
NF1 490
87 Von Hippel–Lindau disease 492
Defi nition 492
Genetics 493
Clinical features 494
Prognosis 495
88 Carney complex 496
Defi nition 496
Genetics 496
Clinical presentation 496

89 Cowden syndrome 497

Incidence 497
Genetics 497
Clinical presentation 497
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90 POEMS syndrome 498
Defi nition 498
Clinical presentation 498
Treatment 498
91 MEN type 1 499
Defi nition 499
Genetics 499
Clinical features 499
Management 501
Mutational analysis 501
Screening 502
92 MEN type 2 503
Defi nition 503
Genetics 503
Clinical features—MEN2a 504
Clinical features—MEN2b 505
Management 505
Mutational analysis 506
Screening 506

93 Inherited primary hyperparathyroidism 507
Epidemiology 507

Causes 507
HPT-JT 507
FIHP 508
94 Inherited renal calculi 509
Defi nition 509
Genetics 510
Management 511
Part 10 Miscellaneous endocrinology
95 Hypoglycemia 514
Defi nition (Whipple’s triad) 514
Epidemiology 515
Pathophysiology 516
Clinical features 517
Management 519
Postprandial reactive hypoglycemia (PRH) 520
96 Obesity 522
Defi nition 522
Epidemiology 523
Etiology 524
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Pathophysiology 526
Consequences of obesity 527
Evaluation of an obese patient 528
Investigations 529
Management 530
97 Endocrinology and aging 534
Introduction 534
Fluid and electrolyte homeostasis in the elderly 535

Bone disease 537
GH and IGF-1 in the elderly 538
Gonadal function in the elderly 539
Adrenal function in the elderly 541
Thyroid disease 542
98 Endocrinology of critical illness 544
Endocrine dysfunction and AIDS 544
Cancer 549
Syndromes of ectopic hormone production 550
Liver disease 554
Renal disease 556
Endocrinology in the critically ill
558
99 Perioperative management of endocrine patients 561
Transsphenoidal surgery/craniotomy 561
Thyroidectomy 564
Parathyroidectomy 565
Pheochromocytoma 567
100 Syndromes of hormone resistance 569
Defi nition 569
Thyroid hormone resistance 569
Androgen resistance 569
Glucocorticoid resistance 569
ACTH resistance 570
Mineralocorticoid resistance 570
Differential diagnosis of possible manifestations of
endocrine disorders 571
101 Stress and the endocrine system 575
Defi nition 575
Endocrine effects of stress 576

Metabolism 576
102 Alternative or complementary therapy
and endocrinology 577

Introduction 577
Alternative therapy used in patients with diabetes
mellitus 578
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