HArrison Endocrinology

560 Pages·2010·20.07 MB·English

by  Jameson, J. Larry

Second Edition
HARRISON’S
Endocrinology
Derived from Harrison’s Principles of Internal Medicine, 17th Edition
Editors
ANTHONY S. FAUCI, EUGENE BRAUNWALD,
MD MD
Chief,Laboratory of Immunoregulation; Distinguished Hersey Professor of Medicine,
Director,National Institute of Allergy and Infectious Diseases, Harvard Medical School;Chairman,TIMI Study Group,
National Institutes of Health,Bethesda Brigham and Women’s Hospital,Boston
DENNIS L. KASPER, STEPHEN L. HAUSER,
MD MD
William Ellery Channing Professor of Medicine,Professor of Robert A.Fishman Distinguished Professor and Chairman,
Microbiology and Molecular Genetics,Harvard Medical School; Department of Neurology,University of California,San Francisco
Director,Channing Laboratory,Department of Medicine,
Brigham and Women’s Hospital,Boston
J. LARRY JAMESON, ,
MD PhD
Professor of Medicine;
DAN L. LONGO,
MD Vice President for Medical Affairs
Scientific Director,National Institute on Aging, and Lewis Landsberg Dean,
National Institutes of Health, Northwestern University Feinberg
Bethesda and Baltimore School of Medicine,Chicago
JOSEPH LOSCALZO, ,
MD PhD
Hersey Professor ofTheory and Practice of Medicine,
Harvard Medical School;Chairman,Department of Medicine;
Physician-in-Chief,Brigham and Women’s Hospital,Boston
Second Edition
HARRISON’S
Endocrinology
Editor
J. Larry Jameson, MD, PhD
Professor of Medicine;
Vice President for Medical Affairs and Lewis Landsberg Dean,
Northwestern University Feinberg School of Medicine,Chicago
New York Chicago San Francisco Lisbon London Madrid Mexico City
Milan New Delhi San Juan Seoul Singapore Sydney Toronto
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ISBN: 978-0-07-174147-7
MHID: 0-07-174147-X
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CONTENTS
Contributors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii 12 The Menopause Transition and
Postmenopausal Hormone Therapy . . . . . . . . . 207
Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ix JoAnn E.Manson,Shari S.Bassuk
1 Principles of Endocrinology . . . . . . . . . . . . . . . . 1
13 Hirsutism and Virilization . . . . . . . . . . . . . . . . 216
J.Larry Jameson
David A.Ehrmann
SECTION I 14 Gynecologic Malignancies. . . . . . . . . . . . . . . . 222
PITUITARY, THYROID, AND Robert C.Young
ADRENAL DISORDERS
15 Sexual Dysfunction. . . . . . . . . . . . . . . . . . . . . 232
2 Disorders of the Anterior Pituitary and Kevin T.McVary
Hypothalamus. . . . . . . . . . . . . . . . . . . . . . . . . . 16
Shlomo Melmed, J.Larry Jameson SECTION III
DIABETES MELLITUS, OBESITY,
3 Disorders of the Neurohypophysis . . . . . . . . . . . 50 LIPOPROTEIN METABOLISM
Gary L.Robertson
16 Biology of Obesity . . . . . . . . . . . . . . . . . . . . . 242
4 Disorders of the Thyroid Gland . . . . . . . . . . . . . 62
Jeffrey S.Flier,Eleftheria Maratos-Flier
J.Larry Jameson,Anthony P.Weetman
17 Evaluation and Management of Obesity . . . . . 251
5 Disorders of the Adrenal Cortex. . . . . . . . . . . . . 99
Robert F.Kushner
Gordon H.Williams,Robert G.Dluhy
18 The Metabolic Syndrome . . . . . . . . . . . . . . . . 259
6 Pheochromocytoma . . . . . . . . . . . . . . . . . . . . 133
Robert H.Eckel
Hartmut P.H.Neumann
19 Diabetes Mellitus. . . . . . . . . . . . . . . . . . . . . . . 267
Alvin C.Powers
SECTION II
REPRODUCTIVE ENDOCRINOLOGY 20 Hypoglycemia. . . . . . . . . . . . . . . . . . . . . . . . . 314
Philip E.Cryer
7 Disorders of Sex Development. . . . . . . . . . . . . 144
John C.Achermann,J.Larry Jameson 21 Disorders of Lipoprotein Metabolism. . . . . . . . 323
Daniel J.Rader,Helen H.Hobbs
8 Disorders of the Testes and Male
Reproductive System. . . . . . . . . . . . . . . . . . . . 156
Shalender Bhasin,J.Larry Jameson SECTION IV
DISORDERS AFFECTING MULTIPLE
9 Testicular Cancer. . . . . . . . . . . . . . . . . . . . . . . 180 ENDOCRINE SYSTEMS
Robert J.Motzer,George J.Bosl
22 Endocrine Tumors of the Gastrointestinal
10 The Female Reproductive System: Tract and Pancreas. . . . . . . . . . . . . . . . . . . . . . 348
Infertility and Contraception . . . . . . . . . . . . . . 186 Robert T.Jensen
Janet E.Hall
23 Multiple Endocrine Neoplasias and
11 Menstrual Disorders and Pelvic Pain. . . . . . . . . 201 Autoimmune Endocrinopathies. . . . . . . . . . . . 367
Janet E.Hall Camilo Jimenez,Robert F.Gagel
v
vi Contents
24 Endocrine Paraneoplastic Syndromes . . . . . . . . 379 28 Osteoporosis. . . . . . . . . . . . . . . . . . . . . . . . . . 443
J.Larry Jameson Robert Lindsay,Felicia Cosman
SECTION V 29 Paget’s Disease and Other Dysplasias of Bone . . 462
DISORDERS OF BONE AND CALCIUM Murray J.Favus,Tamara J.Vokes
METABOLISM
Appendix
Laboratory Values of Clinical Importance . . . . . 475
25 Bone and Mineral Metabolism in
Alexander Kratz,Michael A.Pesce,Daniel J.Fink
Health and Disease . . . . . . . . . . . . . . . . . . . . . 388
F.Richard Bringhurst,Marie B.Demay,
Stephen M.Krane,Henry M.Kronenberg Review and Self-Assessment. . . . . . . . . . . . . . . 491
Charles Wiener,Gerald Bloomfield,
26 Approach to Hypercalcemia and Cynthia D.Brown,Joshua Schiffer,Adam Spivak
Hypocalcemia. . . . . . . . . . . . . . . . . . . . . . . . . 406
Sundeep Khosla Index. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 525
27 Diseases of the Parathyroid Gland. . . . . . . . . . . 411
John T.Potts,Jr.
CONTRIBUTORS
Numbers in brackets refer to the chapter(s) written or co-written by the contributor.
JOHN C.ACHERMANN,MD MURRAY J.FAVUS,MD
Lecturer in Endocrinology,UCL Institute of Child Health, Professor of Medicine,Interim Head,Endocrine Section;Director,
University College,London,United Kingdom [7] Bone Section,University of Chicago Pritzker School of Medicine,
Chicago [29]
SHARI S.BASSUK,ScD
Epidemiologist,Division of Preventive Medicine, DANIEL J.FINK,†MD,MPH
Brigham and Women’s Hospital,Boston [12] Associate Professor of Clinical Pathology,College of Physicians and
Surgeons,Columbia University,New York [Appendix]
SHALENDER BHASIN,MD
Chief and Professor,Department of Endocrinology, JEFFREY S.FLIER,MD
Diabetes,& Nutrition,Boston University,Boston [8] Caroline Shields Walker Professor of Medicine,
Harvard Medical School;Dean of the Faculty of Medicine,
GERALD BLOOMFIELD,MD,MPH Harvard School of Medicine,Boston [16]
Department of Internal Medicine,The Johns Hopkins University
School of Medicine,Baltimore [Review and Self-Assessment] ROBERT F.GAGEL,MD
Professor of Medicine and Head,Division of Internal Medicine,
GEORGE J.BOSL,MD University of Texas MD Anderson Cancer Center,Houston [23]
Chairman,Department of Medicine,Memorial Sloan-Kettering
Cancer Center;Professor of Medicine,Joan and Sanford I.Weill JANET E.HALL,MD
Medical College of Cornell University,New York [9] Associate Professor of Medicine,Harvard Medical School;Associate
Physician,Massachusetts General Hospital,Boston [10,11]
F.RICHARD BRINGHURST,MD
Senior Vice President for Medicine and Research Management, HELEN H.HOBBS,MD
Massachusetts General Hospital;Associate Professor of Medicine, Investigator,Howard Hughes Medical Institute;Professor of Internal
Harvard Medical School,Boston [25] Medicine and Molecular Genetics,University of Texas Southwestern
Medical Center,Dallas [21]
CYNTHIA D.BROWN,MD
Department of Internal Medicine,The Johns Hopkins University J.LARRY JAMESON,MD,PhD
School of Medicine,Baltimore [Review and Self-Assessment] Professor of Medicine;Vice President for Medical Affairs and Lewis
Landsberg Dean,Northwestern University Feinberg School of
FELICIA COSMAN,MD Medicine,Chicago [1,2,4,7,8,24]
Associate Professor of Clinical Medicine,Columbia University College
of Physicians and Surgeons;Medical Director,Clinical Research ROBERT T.JENSEN,MD
Center,Helen Hayes Hospital,West Haverstraw,New York [28] Chief,Digestive Diseases Branch,National Institute of Diabetes,
Digestive and Kidney Diseases,National Institutes of Health,
PHILIP E.CRYER,MD Bethesda [22]
Irene E.and Michael M.Karl Professor of Endocrinology and
Metabolism in Medicine,Washington University,St.Louis [20] CAMILO JIMENEZ,MD
Assistant Professor,Department of Endocrine Neoplasia & Hormonal
MARIE B.DEMAY,MD Disorders,The University of Texas MD Cancer Center,Houston [23]
Associate Professor of Medicine,Harvard Medical School;
Associate Physician,Massachusetts General Hospital,Boston [25] SUNDEEP KHOSLA,MD
Professor of Medicine and Physiology,Mayo Clinic College of
ROBERT G.DLUHY,MD Medicine,Rochester [26]
Program Director,Fellowship in Endocrinology;Professor of
Medicine,Brigham and Women’s Hospital,Harvard Medical School; STEPHEN M.KRANE,MD
Associate Editor,New England Journal of Medicine,Boston [5] Persis,Cyrus and Marlow B.Harrison Distinguished Professor of
Medicine,Harvard Medical School,Massachusetts General Hospital,
ROBERT H.ECKEL,MD Boston [25]
Professor of Medicine,Division of Endocrinology,Metabolism and
Diabetes,Division of Cardiology;Professor of Physiology and ALEXANDER KRATZ,MD,PhD,MPH
Biophysics;Charles A.Boettcher II Chair in Atherosclerosis; Assistant Professor of Clinical Pathology,Columbia University
Program Director,Adult General Clinical Research Center, College of Physicians and Surgeons;Associate Director,
University of Colorado at Denver and Health Sciences Center; Core Laboratory,Columbia University Medical Center,
Director,Lipid Clinic,University Hospital,Aurora [18] New York-Presbyterian Hospital;Director,
Allen Pavilion Laboratory,New York [Appendix]
DAVID A.EHRMANN,MD
Professor of Medicine;Associate Director,University of Chicago HENRY M.KRONENBERG,MD
General Clinical Research Center,Chicago [13] Chief,Endocrine Unit,Massachusetts General Hospital;
Professor of Medicine,Harvard Medical School,Boston [25]
†Deceased.
vii
viii Contributors
ROBERT F.KUSHNER,MD ALVIN C.POWERS,MD
Professor of Medicine,Northwestern University Feinberg Joe C.Davis Chair in Biomedical Science;Professor of Medicine,
School of Medicine,Chicago [17] Molecular Physiology and Biophysics;Director,Vanderbilt Diabetes
Research and Training Center;Director,Vanderbilt Diabetes Center,
ROBERT LINDSAY,MD,PhD Nashville [19]
Professor of Clinical Medicine,Columbia University College of
Physicians and Surgeons;Chief,Internal Medicine,Helen Hayes DANIEL J.RADER,MD
Hospital,West Havershaw,New York [28] Cooper-McClure Professor of Medicine,University of Pennsylvania
School of Medicine,Philadelphia [21]
JOANN E.MANSON,MD,DrPH
Professor of Medicine and the Elizabeth Fay Brigham Professor of GARY L.ROBERTSON,MD
Women’s Health,Harvard Medical School;Chief,Division of Emeritus Professor of Medicine,Northwestern University Feinberg
Preventive Medicine,Brigham and Women’s Hospital,Boston [12] School of Medicine,Chicago [3]
ELEFTHERIA MARATOS-FLIER,MD JOSHUA SCHIFFER,MD
Associate Professor of Medicine,Harvard Medical School; Department of Internal Medicine,The Johns Hopkins University
Chief,Obesity Section,Joslin Diabetes Center,Boston [16] School of Medicine,Baltimore [Review and Self-Assessment]
KEVIN T.MCVARY,MD ADAM SPIVAK,MD
Associate Professor of Urology,Northwestern University Feinberg Department of Internal Medicine,The Johns Hopkins University
School of Medicine,Chicago [15] School of Medicine,Baltimore [Review and Self-Assessment]
SHLOMO MELMED,MD TAMARA J.VOKES,MD
Senior Vice President,Academic Affairs;Associate Dean, Associate Professor,Section of Endocrinology,University of
Cedars Sinai Medical Center,David Geffen School of Chicago,Chicago [29]
Medicine at UCLA,Los Angeles [2]
ANTHONY P.WEETMAN,MD,DSc
ROBERT J.MOTZER,MD Professor of Medicine and Dean of the School of Medicine and
Attending Physician,Department of Medicine,Memorial Biomedical Sciences,University of Sheffield,Sheffield,United
Sloan-Kettering Cancer Center;Professor of Medicine, Kingdom [4]
Weill Medical College of Cornell University,New York [9]
CHARLES WIENER,MD
HARTMUT P.H.NEUMANN,MD Professor of Medicine and Physiology;Vice Chair,Department of
Head,Section Preventative Medicine,Department of Nephrology Medicine;Director,Osler Medical Training Program,The Johns
and General Medicine,Albert-Ludwigs-University of Freiburg, Hopkins University School of Medicine,Baltimore [Review and
Germany [6] Self-Assessment]
MICHAEL A.PESCE,PhD GORDON H.WILLIAMS,MD
Clinical Professor of Pathology,Columbia University College of Professor of Medicine,Harvard Medical School;Chief,
Physicians and Surgeons;Director of Specialty Laboratory,New York Cardiovascular Endocrinology Section,Brigham and Women’s
Presbyterian Hospital,Columbia University Medical Center, Hospital,Boston [5]
New York [Appendix]
ROBERT C.YOUNG,MD
JOHN T.POTTS,JR.,MD Chancellor,Fox Chase Cancer Center,Philadelphia [14]
Jackson Distinguished Professor of Clinical Medicine,Harvard
Medical School;Director of Research and Physician-in-Chief
Emeritus,Massachusetts General Hospital,Charlestown [27]
PREFACE
The editors of Harrison’s Principles of Internal Medicine refer Endocrine Systems;and (V) Disorders of Bone and Cal-
to it as the “mother book,” a description that confers cium Metabolism.
respect but also acknowledges its size and its ancestral sta- While Harrison’s Endocrinology is classic in its organiza-
tus among the growing list of Harrison’s products,which tion,readers will sense the impact of the scientific renais-
now include Harrison’s Manual of Medicine, Harrison’s sance as they explore the individual chapters in each section.
Online,and Harrison’s Practice,an online highly structured In addition to the dramatic advances emanating from
reference for point-of-care use and continuing educa- genetics and molecular biology, the introduction of an
tion.This book, Harrison’s Endocrinology, second edition, unprecedented number of new drugs,particularly for the
is a compilation of chapters related to the specialty of management of diabetes and osteoporosis,is transforming
endocrinology. the field of endocrinology. Numerous recent clinical
Our readers consistently note the sophistication of studies involving common diseases like diabetes, obesity,
the material in the specialty sections of Harrison’s. Our hypothyroidism, osteoporosis, and polycystic ovarian
goal was to bring this information to readers in a more syndrome provide powerful evidence for medical decision-
compact and usable form. Because the topic is more making and treatment.These rapid changes in endocrinology
focused, it was possible to increase the presentation of are exciting for new students of medicine and underscore
the material by enlarging the text and the tables.We the need for practicing physicians to continuously update
have also included a Review and Self-Assessment section their knowledge base and clinical skills.
that includes questions and answers to provoke reflec- Our access to information through web-based jour-
tion and to provide additional teaching points. nals and databases is remarkably efficient.While these
The clinical manifestations of endocrine disorders can sources of information are invaluable,the daunting body
usually be explained by considering the physiologic role of data creates an even greater need for synthesis and for
of hormones, which are either deficient or excessive. highlighting important facts. Thus, the preparation of
Thus,a thorough understanding of hormone action and these chapters is a special craft that requires the ability
principles of hormone feedback arms the clinician with to distill core information from the ever-expanding
a logical diagnostic approach and a conceptual frame- knowledge base.The editors are therefore indebted to our
work for treatment approaches.The first chapter of the authors, a group of internationally recognized authori-
book,Principles of Endocrinology,provides this type of ties who are masters at providing a comprehensive
“systems”overview.Using numerous examples of trans- overview while being able to distill a topic into a concise
lational research, this introduction links genetics, cell and interesting chapter.We are grateful to Emily Cowan
biology,and physiology with pathophysiology and treat- for assisting with research and preparation of this book.
ment.The integration of pathophysiology with clinical Our colleagues at McGraw-Hill continue to innovate in
management is a hallmark of Harrison’s,and can be found health care publishing.This new product was champi-
throughout each of the subsequent disease-oriented oned by Jim Shanahan and impeccably produced by
chapters.The book is divided into five main sections Kim Davis.
that reflect the physiologic roots of endocrinology: (I) We hope you find this book useful in your effort to
Pituitary, Thyroid, and Adrenal Disorders; (II) Repro- achieve continuous learning on behalf of your patients.
ductive Endocrinology;(III) Diabetes Mellitus,Obesity,
Lipoprotein Metabolism;(IV) Disorders Affecting Multiple J.Larry Jameson,MD,PhD
ix