基础与临床内分泌学 英文版PDF电子书下载

1．Introduction to Endocrinology John D.Baxter, MD, Ralff C.J.Ribeiro, MD,PhD 1

The Endocrine System 1

Paracrine Autocrine Actions 3

Chemical Composition of Hormones 3

Vitamins Hormones 4

Hormones Oncogenes 4

Hormones the Immune System 5

Eicosanoids: Prostaglandins, Thromboxanes, Leukotrienes, Related Compounds 5

Mechanisms of Hormone Action 6

Hormone Receptors 6

Regulation of Responsiveness to Hormones 7

Interactions Between Hormone Response Systems 8

Classes of Hormone Action 8

Use of Receptors for Classifying Types of Hormone Action 9

Classification of Hormones by the Types of Ligands: Agonists, Antagonists, Partial Agonists-Partial Antagonists, Inactive Compounds 10

Hypothalamic-Pituitary Relationships 11

Neurotransmitters Hormones 11

Endocrine Nervous System Relationships: Neuroendocrinology 11

Gene Expression in Endocrinology Metabolism: Molecular Basis for Function of the System Genetic Endocrine Disease 13

Evolution of the Endocrine System 14

Cellular Control Networks the Origins of Regulatory Chemicals 15

The Origins of Regulatory Proteins 15

The Origins of Hormones 15

Intracellular Communication 16

Evolution of Genes Involved in the Endocrine System 16

Integrative Networks 16

Evolution of the Endocrine Glands 16

Hormone Synthesis Release 17

Hormone Transport 17

Steroid Thyroid Hormones Vitamin D 18

Polypeptide Hormones 18

Catecholamine Eicosanoid Metabolism 19

Steroid Thyroid Hormones Vitamin D 19

Peptide Hormones 19

Metabolism Elimination of Hormones 19

Regulation of the Endocrine System 20

Actions of Hormones 20

Developmental Effects 20

Cell Growth Cancer 20

Central Nervous System Effects 21

Effects on Metabolism 21

Effects on Cardiovascular Renal Function 21

Effects on Mineral Water Metabolism 22

Effects on Skeletal Functions 22

Effects on Reproductive Function 22

Release of Other Hormones 22

Effects on Immunologic Functions 23

Disorders of the Endocrine System 23

Endocrine Hypofunction 23

Endocrine Hyperfunction 24

Defects in Sensitivity to Hormones 25

Syndromes of Hormone Excess Due to Administration of Exogenous Hormone or Medication 26

Disorders of the Endocrine Glands not Associated With Endocrine Disease 27

Approach to the Patient With Endocrine Disease 27

History Physical Examination 27

Laboratory Studies 28

Screening for Endocrine Diseases 32

Treatment of Endocrine Diseases 33

Uses of Hormones in Therapy of Nonendocrine Disease 34

2．Hormone Synthesls Release Vishwanath R.Lingappa, MD, PhD, Synthia H.Mellon, PhD 38

Overview of Hormone Biosynthesis 38

Membrane Vesicle-Mediated Hormone Export 39

Hormone Export not Mediated by Membrane VesicIes 49

Metabolism. Transport. Elimination. Regulation of Hormones 55

3．Mechanisms of Hormone Action David G.Gardner, MD 59

Receptors 59

Neurotransmitter Peptide Hormone Receptors 61

Seven-Transmembrane Receptors 62

G Protein Transducers 62

Effectors 63

G Protein-Linked Receptors Human Disease 66

Growth Factor Receptors 67

Cytokine Receptors 69

Guanylyl Cyclase-Linked Receptors 70

Nuclear Action of Peptide Hormones 71

Nuclear Receptors 72

Steroid Receptor Family 72

Thyroid Receptor Family 74

Nongenomic Effects of the Steroid Hormones 77

Steroid Thyroid Hormone Receptor Resistance Syndromes 77

4．Endocrine Autoimmunity Juan Carlos Jaume, MD 80

Immune Recognition Response 81

Basic Immune Components Mechanisms 81

Tolerance 85

Autoimmunity Is Multifactorial 90

Genetic Factors in Autoimmunity 90

Environmental Factors in Autoimmunity 90

Single Gland Autoimmune Syndromes 91

Autoimmune Aspects of Thyroid Disease 91

Autoimmune Aspects of Type I Diabetes 93

Autoimmune Aspects of Other Endocrinopathies 95

Autoimmune Polyglandular Syndromes 97

Autoimmune Polyglandular SyndromeⅠ(APS-Ⅰ) 97

Autoimmune Polyglandular SyndromeⅡ(APS-Ⅱ) 98

Management of Autoimmune Polyglandular Syndromes 98

5．Hypothalamus Pituitary David C.Aron, MS, MD, James W.Findling, MD, J.Blake Tyrrell, MD 100

Hypothalamic Hormones 105

Anterior Pituitary Hormones 110

ACTH Related Peptides 110

Growth Hormone 112

Prolactin 114

Thyrotropin 116

Gonadotropins:Luteinizing Hormone Follicle-Stimulating Hormone 117

Endocrinologic Evaluation of the Hypothalamic-Pituitary Axis 119

Evaluation of ACTH 120

Evaluation of Growth Hormone 121

Evaluation of Prolactin 122

Evaluation of TSH 122

Evaluation of LH FSH 122

Problems in Evaluation of the Hypothalamic-Pituitary Axis 122

Effects of Pharmacologic Agents on Hypothalamic-Pituitary Function 123

Endocrine Tests of Hypothalamic-Pituitary Function 123

Neuroradiologic Evaluation 123

Pituitary Hypothalamic Disorders 126

Empty Sella Syndrome 128

Hypothalamic Dysfunction 129

Hypopituitarism 130

Pituitary Adenomas 136

Posterior Pituitary 151

Antidiuretic Hormone(ADH:Vasopressin) 151

Oxytocin 152

Control of Water Balance 152

Diabetes Insipidus 154

Syndrome of Inappropriate Secretion of Antidiuretic Hormone(SIADH) 158

6．Growth Dennis Styne, MD 163

Normal Growth 163

Intrauterine Growth 163

The Placenta 164

Classic Hormones of Growth Fetal Growth 164

Growth Factors Oncogenes in Fetal Growth 164

Insulin-like Growth Factors, Receptors, Binding Proteins 164

Insulin 165

Epidermal Growth Factor 165

Postnatal Growth 166

Fetal Origins of Adult Disease 166

Measurement of Growth 176

Skeletal (Bone)Age 178

Disorders of Growth 178

Short Stature Due to Nonendocrine Causes 178

Short Stature Due to Endocrine Disorders 184

The Diagnosis of Short Stature 195

Tall Stature Due to Nonendocrine Causes 196

Tall Stature Due to Endocrine Disorders 197

7．The Thyroid Gland Francis S.Greenspan, MD, FACP 201

Anatomy Histology 202

Physiology 204

Structure of Thyroid Hormones 204

Iodine Metabolism 204

Thyroid Hormone Synthesis Secretion 206

Thyroid Hormone Transport 210

Abnormalities in Thyroid Hormone Synthesis Release 210

Metabolism of Thyroid Hormones 213

Control of Thyroid Function 217

The Action of Thyroid Hormones 222

Physiologic Changes in Thyroid Function 225

Thyroid Autoimmunity 226

Tests of Thyroid Function 227

Tests of Thyroid Hormones in Blood 227

Evaluation of the Hypothalamic-Pituitary-Thyroid Axis 228

Iodine Metabolism Biosynthetic Activity 229

Thyroid Imaging 231

Thyroid Ultrasonography or Magnetic Resonance Imaging 231

Thyroid Biopsy 232

Effects of Thyroid Hormones on Peripheral Tissues 232

Measurement of Thyroid Autoantibodies 232

Summary: Clinical Use of Thyroid Function Tests 233

Disorders of the Thyroid 233

Hypothyroidism 235

Hyperthyroidism Thyrotoxicosis 242

Thyroid Hormone Resistance Syndromes 253

Nontoxic Goiter 254

Thyroiditis 256

Effects of Ionizing Radiation on the Thyroid Gland 258

Thyroid Nodules Thyroid Cancer 260

8．Mineral Metabolism Metabolic Bone Disease Dolores Shoback, MD, Robert Marcus, MD, Daniel Bikle, MD, PhD, Gordon Strewler, MD 273

Cellular Extracellular Calcium Metabolism 273

Parathyroid Hormone 274

Calcitonin 279

Vitamin D 281

Integrated Control of Mineral Homeostasis 288

Medullary Thyroid Carcinoma 288

Hypercalcemia 288

Hypocalcemia 300

Bone Anatomy Remodeling 306

Osteoporosis 310

Osteomalacia Rickets 320

Paget s Disease of Bone (Osteitis Deformans) 326

Renal Osteodystrophy 329

9．Glucoctorioids Adrenal Androgens David C.Aron, MD, MS, James W.Findling, MD, J.Blake Tyrrell, MD 334

Embryology Anatomy 334

Biosynthesis of Cortisol Adrenal Androgens 337

Circulation of Cortisol Adrenal Androgens 342

Metabolism of Cortisol Adrenal Androgens 342

Biologic Effects 343

Glucocorticoids 343

Adrenal Androgens 347

Laboratory Evaluation 347

Disorders of Adrenocortical Insufficiency 351

Primary Adrenocortical Insufficiency (Addison s Disease) 351

Secondary Adrenocortical Insufficiency 355

Diagnosis of Adrenocortical Insufficiency 355

Treatment of Adrenocortical Insufficiency 358

Prognosis of Adrenocortical Insufficiency 360

Cushing s Syndrome 360

Hirsutism Virilism 371

Incidental Adrenal Mass 371

Glucocorticoid Therapy for Nonendocrine Disorders 373

10．Endocrine Hypertension Burl R.Don, MD, Morris Schambelan, MD 377

Hypertension of Adrenal Origin 377

Hypertension of Renal Origin 389

Other Hormone Systems Hypertension 395

11．Adrenal Medulla Alan Goldfien, MD 399

Anatomy 399

Hormones of the Adrenal Medulla 400

Catecholamines 400

Other Hormones 409

Adrenal Medullary Hyperfunction 410

Adrenal Medullary Hypofunction 410

Disorders of Adrenal Medullary Function 410

Pheochromocytoma 411

12．Testes Glenn D.Braunstein, MD 422

Anatomy Structure-Function Relationships 422

Testes 422

Accessory Structures 424

Physiology of the Male Reproductive System 424

Gonadal Steroids 424

Control of Testicular Function 426

Evaluation of Male Gonadal Function 428

Clinical Evaluation 428

Laboratory Tests of Testicular Function 428

Pharmacology of Drugs Used to Treat Male Gonadal Disorders 431

Androgens 431

Gonadotropins 433

Clinical Male Gonadal Disorders 434

Klinefelter s Syndrome 434

Gonadotropin-Releasing Hormone 434

Bilateral Anorchia 436

Leydig Cell Aplasia 437

Cryptorchidism 437

Noonan s Syndrome (Male Turner s Syndrome) 439

Myotonic Dystrophy 440

Adult Seminiferous Tubule Failure 440

Adult Leydig Cell Failure (Andropause) 441

Erectile Dysfunction (Impotence) 442

Male Infertility 444

Gynecomastia 446

Testicular Tumors 448

13．Ovaries Alan Goldfien, MD 453

Anatomy of the Ovaries 453

Hormones of the Ovary 454

Steroid Hormones 454

Other Ovarian Hormones Regulatory Substances 460

Relaxin 460

The Menstrual Cycle 461

Hormonal Profiles During the Menstrual Cycle 461

The Ovarian Cycle 463

Hormone Interaction Regulation During the Menstrual Cycle 466

Cyclic Changes in the Female Reproductive Tract 467

Histology of the Endometrium Throughout the Menstrual Cycle 467

Cervical Mucus 469

Vaginal Epithelium 470

Extragenital Symptoms Associated With Normal Menstrual Function 471

Premenstrual Dysmorphic Disorder (Premenstrual Syndrome) 471

Dysmenorrhea 472

Disorders of Ovarian Menstrual Function 473

Amenorrhea in the Absence of Sexual Maturation 474

Amenorrhea in Patients With Normal Secondary Sex Characteristics 474

Disorders of Androgen Metabolism 480

Hirsutism 483

Clomiphene Citrate 488

Ovulation Induction 488

Anovulatory Bleeding 488

Human Menopausal Gonadotropins (Menotropins) 489

Gonadotropin-Releasing Hormone 490

Bromocriptine 490

Therapeutic Use of Ovarian Hormones Their Synthetic Analogs 490

Treatment of Primary Hypogonadism 490

Ovarian Suppression 490

Threatened Abortion 490

Inadequate Luteal Phase 491

Diagnostic Uses 491

Inhibitors of Ovarian Function 491

Gonadotropin-Releasing Hormone Analogs Antagonists 491

Sclective Estrogen Receptor Modulators Antiestrogens 491

Antiprogestins 492

Antiandrogens 492

Danazol 492

Menopause 493

Hormonal Changes 493

Clinical Manifestations of Menopause 494

Management of Menopause 495

Hormonal Contraception (Oral Contraceptives) 498

Pharmacologic Effects of Estrogens Synthetic Progestins 498

Clinical Uses of Oral Contraceptives 499

Adverse Effects of Oral Contraceptives 500

Noncontraceptive Advantages of Hormonal Contraception 502

Contraindications Cautions 502

Drug Interactions With Oral Contraceptives 502

Contraception With Progestins 503

Postcoital Contraceptives Emergency Contraception 503

Infertility 503

Semen Abnormalities 503

Infertility in the Presence of Ovulation 504

Ovulatory Disorders 504

In Vitro Fertilization 505

Gamete Intrafollicular Transfer(GIFT) 505

14．Abnormalities of Sexual Determination Differentiation Felix A.Conte, MD, Melvin M.Grumbach, MD 509

Normal Sex Differentiation 509

Testicular Ovarian Differentiation 515

Psychosexual Differentiation 517

Abnormal Sex Differentiation 519

Seminiferous Tubule Dysgenesis: Chromatin-Positive Klinefelter s Syndrome Its Variants 520

Syndrome of Gonadal Dysgenesis: Turner s Syndrome Its Variants 522

46.XX 46.XY Gonadal Dysgenesis 524

True Hermaphroditism 526

Female Pseudohermaphroditism 527

P450 Aromatase Deficiency 531

Maternal Androgens Progestogens 531

Male Pseudohermaphroditism 531

Unclassified Forms of Abnormal Sexual Development in Males 540

Unclassified Forms of Abnormal Sexual Development in Females 542

Management of Patients With Intersex Problems 542

15．Puberty Dennis Styne, MD 547

Physiology of Puberty 547

Delayed Puberty or Absent Puberty (Sexual Infantilism) 556

Precocious Puberty (Sexual Precocity) 566

16．The Endocrinology of Pregnancy Robert N.Taylor, MD, PhD, Dan I.Lebovic, MD, Mary C.Martin-Cadieux, MD 575

Conception Implantation 575

Fetal-Placental-Decidual Unit 576

Polypeptide Hormones 577

Steroid Hormones 580

Maternal Adaptation to Pregnancy 581

Fetal Endocrinology 584

Endocrine Control of Parturition 586

Endocrinology of the Puerperium 587

Endocrine Disorders Pregnancy 589

Pregnancy Pituitary Pathology 589

Pregnancy Breast Cancer 590

Hypertensive Disorders of Pregnancy 591

Hyperthyroidism in Pregnancy 592

Hypothyroidism in Pregnancy 593

Diabetes Mellitus Pregnancy (by John L.Kitzmiller, MD) 593

17．Regulatory Peptides of the Gut Sean J.Mulvihill, MD, Haile T.Debas, MD 603

Gastrin 607

Cholecystokinin 608

Somatostatin 609

Secretin 609

Bombesin Gastrin-Releasing Peptide 610

Calcitonin Gene-Related Peptidc 611

Gastric Inhibitory Peptide 611

Vasoactive Intestinal Peptide 611

Galanin 612

Substance P 612

Enkephalins 612

Motilin 613

Neurotensin 613

Pancreatic Polypeptide Family 614

Enteroglucagon 614

Abnormalities of Regulatory Peptides in Diseases of the Gastrointestinal Tract 614

Duodenal Ulcer 614

Motility Disorders of the Gastrointestinal Tract 615

Intlammatory Bowel Disease 615

Neuroendocrine Tumors of the Gut 616

Multiple Endocrine Neoplasia Syndromes 616

Zollinger-Ellison Syndrome 616

VIPoma 617

Glucagonoma 617

Carcinoid Tumors Carcinoid Syndrome 617

Miscellaneous Tumors 618

Minimally Invasive Approaches to Gut Endocrine Tumors 619

Clinical Uses of Gut Peptides 619

Anatomy Histology 623

18．Pancreatic Hormones Diabetes Mellitus Umesh Masharani, MRCP(UK), John H.Karam, MD 623

The Endocrine Pancreas 623

Hormones of the Endocrine Pancreas 624

Diabetes Mellitus 633

Classification 633

Type 1 Diabetes Mellitus 633

Type 2 Diabetes 636

Other Specific Types of Diabetes 638

Clinical Features of Diabetes Mellitus 641

Type 1 Diabetes 641

Type 2 Diabetes 642

Laboratory Findings in Diabetes Mellitus 643

Urialysis 643

Blood Glucose Testing 644

Serum Ketone Determinations 645

Glycosylated Hemoglobin Assays 645

Lipoproteins in Diabetes 646

Capillary Morphometry 646

Diagnosis of Diabetes Mellitus 647

Oral Glucose Tolerance Test 647

Insulin Levels 648

Intravenous Glucose Tolerance Test 648

Treatment of Diabetes Mellitus 648

Diet 651

Oral Agents for the Treatment of Hyperglycemia 653

Insulin 661

Steps in the Management of the Diabetic Patient 666

Acute Complications of Diabetes Mellitus 675

Hypoglycemia 675

Coma 678

Chronic Complications of Diabctes Mellitus 686

Specific Chronic Conplications of Diabetes Mellitus 688

Diabetics Taking Insulin 694

Diabetics Taking Oral Hypoglycemic Agents 694

Surgery in the Diabetic Patient 694

Diabetics Regulated by Diet Alone 694

Prognosis for Patients With Diabetes Mellitus 695

19．Hypoglycemic Disorders John H.Karam, MD 699

Pathophysiology of the Counterregulatory Response to Neuroglycopenia 699

Classification of Hypoglycemic Disorders 703

Clinical Presentation of Hypoglycemia 704

Specific Hypoglycemic Disorders 706

Symptomatic Fasting Hypoglycemia With Hyperinsulinism 706

Symptomatic Fasting Hypoglycemia Without Hyperinsulinism 712

Nonfasting Hypoglycemia (Reactive Hypoglycemia) 713

20．Disorders of Lipoprotein Metabolism Mary J.Malloy, MD, John P.Kane, MD,PhD 716

Atherosclerosis 716

Overview of Lipid Transport 717

Differentiation of Disorders of Lipoprotein Metabolism 722

The Hypertriglyceridemias 724

Clinical Descriptions of Primary Secondary Disorders of Lipoprotein Metabolism 724

Primary Hypertriglyceridemia 725

Secondary Hypertriglyceridemia 727

The Primary Hypercholesterolemias 731

Familial Hypercholesterolemia 731

Familial Combined Hyperlipidemia 732

LP(a) Hyperlipoproteinemia 732

Familial Ligand-Defective Apo B 732

Secondary Hypercholesterolemia 732

Hypothyroidism 732

Nephrosis 733

Immunoglobulin Disorders 733

Anorexia Nervosa 733

Cholestasis 733

The Primary Hypolipidemias 733

Primary Hypolipidemia Due to Deficiency of High-Density Lipoproteins 734

Primary Hypolipidemia Due to Deficiency of Apo B-Containing Lipoproteins 735

Other Disorders of Lipoprotein Metabolism 736

The Lipodystrophies 736

Secondary Hypolipidemia 736

Rare Disorders 737

Treatment of Hyperlipidemia 737

Dietary Factors in the Management of Lipoprotein Disorders 738

Drugs Used in Treatment of Hyperlipoproteinemia 739

Bile Acid Sequestrants 739

Niacin (Nicotinic Acid) 740

Fibric Acid Derivatives 741

HMGA-CoA Reductase Inhibitors 741

Combined Drug Therapy 742

Possible Untoward Consequences of Lipid-Lowering Therapy 743

21．Obesity Overweight Marc K.Hellerstein, MD, PhD, Elizabeth J.Parks, PhD 745

Controversies Uncertainties Regarding the Pathogenesis of Obesity 754

Dietary Therapies 755

Survey of Treatment Approaches Their Efficacy 755

Surgical Treatment of Obesity 756

Pharmacologic Therapies 756

Other Conditions Affected by Treatment of Obesity or Overweight 759

Type 2 Diabetes Mellitus 759

Hyperlipidemia 759

Polycystic Ovarian Syndrome 759

Hypertension 759

Gallstones 759

Osteoarthritis Other Weight-Sensitive States 759

A Systematic Approach to the Treatment of Obesity 759

22．Hormones Cancer Debasish Tripathy, MD, Christopher C.Benz, MD 762

Hormonal Effects on Tumors 762

Growth Promotion Malignant Transformation 762

Tumor Growth Mediated by Autocrine Paracrine Factors 764

Nonsecretory Tumors 765

Secretory Tumors 765

Tumors Affecting Endocrine Status 765

Steroid-Dependent Tumors 765

Treatment-Induced Endocrinopathy 766

Endocrine Therapy for Cancer 767

Steroid Receptors Treatment 767

Primary Modalities of Endocrine Intervention 768

Combination Endocrine Therapy 769

Breast Cancer in Women 770

Clinical Problems 770

Chemo-Endocrine Therapy 770

Breast Cancer in Men 774

Endometrial Cancer 774

Prostatic Cancer 775

Miscellaneous Tumors 776

23．Humoral Manifestatlons of Mallgnancy Dolores Shoback, MD, Janet Funk, MD 778

Ectopic Hormone Receptor Syndromes 778

Hypercalcemia of Malignancy 779

Ectopic Cushing s Syndrome 783

Syndrome of Inappropriate ADH(SIADH) Secretion 785

Non-Islet Cell Tumors Hypoglycemia 786

Other Hormones Secreted by Tumors 787

Oncogenic Osteomalacia 788

Gut Hormones 789

Erythropoietin 789

Renin 789

Multiple Endocrine Neoplasia Type 1 (MEN 1) 792

24．Multiple Endocrine Neoplasia David G.Gardner, MD 792

Multiple Endocrine Neoplasia Type 2 (MEN 2) 797

25．Geriatric Endocrinology Susan L.Greenspan, MD, Neil M.Resnick, MD 802

Thyroid Function Disease 802

Disorders of the Thyroid Gland 803

Carbohydrate Intolerance Diabetes Mellitus 805

Aging the Physiology of Carbohydrate Intolerance 805

Diabetes Mellitus 806

Osteoporosis 808

Nonketotic Hyperosmolar Coma 808

Osteoporosis Calcium Homeostasis 808

Hyperparathyroidism 815

Changes in Water Balance 816

Hypernatremia 816

Hyponatremia 816

Hyporeninemic Hypoaldosteronism 816

Glucocorticoids Stress 817

Disorders of the Hypothalamid-Pituitary-Adrenal Axis 817

Changes in Reproductive Function in Men 818

Benign Prostatic Hyperplasia 819

26．Endocrine Emergencies David G.Gardner, MD, Francis S.Greenspan, MD 823

Myxedema Coma 823

Thyroid Storm 824

Thyrotoxic Periodic Paralysis 825

Amiodarone Induced Thyrotoxicosis 826

Acute Adrenal Insufficiency 827

Pituitary Apoplexy 828

Diabetic Ketoacidosis 829

Nonketotic Hyperosmolar Coma 832

Hypercalcemic Crisis 835

Acute Hypocalcemia 837

Hyponatremia 839

Diabetes Insipidus 841

27．AIDS Endocrinopathies Deborah E.Sellmeyer, MD, Carl Grunfeld, MD, PhD 846

Thyroid Disorders 846

Adrenal Disorders 847

Bone Mineral Disorders 849

Gonadal Disorders 849

Pituitary Disorders 850

Pancreatic Disorders 850

Lipid Disorders 851

Conclusion 851

Appendix: Table of Normal Hormone Reference Ranges 853

Index 867