2 0 1 7 M E E T - T 2017 Meet-the-Professor Endocrine Case Management is your source for the H latest updates in the diagnosis and management of a wide range of endocrine E F R O M T H E E X P E RT S I N E N D O C R I N O L O G Y - disorders. This valuable resource allows you to evaluate your endocrine P knowledge and gain insight into the strategies used by clinical experts. R 7MEET-THE-PROFESSOR O F Features of Meet-the-Professor Endocrine Case Management 2017 include: E 1ENDOCRINE S S (cid:127) Signifi cance of the Clinical Problem O 0 (cid:127) Key Learning Objectives R (cid:127) Strategies for Diagnosis and Management E (cid:127) Clinical Pearls and Pertinent References N 2CASE MANAGEMENT (cid:127) Cases and Questions D O C R I N E C A S E M A N A G E M E N T 2055 L Street NW, Suite 600 Washington, DC 20036 ENDOCRINE ~, endocrine.org SOCIETY..,1 education.endocrine.org CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS WHEN YOU NEED THE BEST, CESS ■ OPEN ACCESISN ■ OFPELN AUCCEESNS ■ COPEEN ATCCHESSE ■ O WPEN AOCCRESSL ■ DOPEN ACCESS ■ OPEN OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS CESS ■ OPEN AWCCESSI ■T OPHEN A CYCESOS ■ OUPENR AC CRESSE ■ OSPENE ACACESRS ■ COPEHN ACCESS ■ OPEN ESAP DELIVERS ™ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS Deliver outstanding patient CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN care with our premier OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN self-assessment program. OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN (cid:127) Completely updated OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS content with 120 new cases CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS (cid:127) Online module, hard CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN copy reference book, OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN conventional and SI Units OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS (cid:127) Eligible for up to 40 AMA CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS PRA Category 1 Credits™ CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN and 40 ABIM MOC points OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS CESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN ACCESS ■ OPEN Streamlined peer review and continuous online publication An Open Access format guaranteeing global readership Distinguished and international editorial board of more than 120 experts “I use ESAP to refresh my memory on endocrine topics, keep up-to-date with changes in endocrinology, and Welcoming a wide range of article types from basic research accumulate CME credits in order to maintain my medical to clinical applications license. ESAP serves this purpose well, and I plan on continuing to purchase it regularly.” — Roger Rittmaster, MD SUBMIT TODAY Update your clinical practice with ESAP. Order online at endocrine.org/store Visit academic.oup.com/jes •1 ENDOCRINE SOCIETY.,J © 2017 ENDOCRINE SOCIETY © 2017 ENDOCRINE SOCIETY ESAP_2017_Letter_Size.indd 1 2/10/17 3:23 PM JES_Open_Acess_Letter_Size.indd 1 2/13/17 12:37 PM FROM THE EXPERTS IN ENDOCRINOLOGY ENDO 2017 MEET-THE-PROFESSOR ENDOCRINE CASE MANAGEMENT END8 2017 ENDOCRINE._, SOCIETY_,1 •1 ENDOCRINE SOCIETY_,.1 2055 L Street, NW, Suite 600 Washington, DC 20036 www.endocrine.org Other Publications: press.endocrine.org TheEndocrineSocietyistheworld’slargest,oldest,andmostactiveorganizationworkingtoadvancetheclinicalpracticeof endocrinologyandhormoneresearch.Foundedin1916,theSocietynowhasmorethan18,000globalmembersacrossarange of disciplines. The Society has earned an international reputation for excellence in the quality of its peer-reviewed journals, educational resources, meetings, and programs that improve public health through the practice and science of endocrinology. Clinical Science Chair, ENDO 2017 John (Jack) Leahy, MD Physician-In-Practice Chair, ENDO 2017 Ann Danoff, MD Thestatementsandopinionsexpressedinthispublicationarethoseoftheindividualauthorsanddonotnecessarilyreflectthe views of the Endocrine Society. The Endocrine Society is not responsible or liable in any way for the currency of the information,foranyerrors,omissionsorinaccuracies,orforanyconsequencesarisingtherefrom.Withrespecttoanydrugs mentioned,thereaderisadvisedtorefertotheappropriatemedicalliteratureandtheproductinformationcurrentlyprovidedby themanufacturertoverifyappropriatedosage,methodanddurationofadministration,andotherrelevantinformation.Inall instances,itistheresponsibilityofthetreatingphysicianorotherhealthcareprofessional,relyingonindependentexperience and expertise, as well as knowledge of the patient, to determine the best treatment for the patient. Copyright©2017bytheEndocrineSociety,2055LStreet,NW,Suite600,Washington,DC20036.Allrightsreserved.No partofthispublicationmaybereproduced,storedinaretrievalsystem,postedontheinternet,ortransmittedinanyform,by any means, electronic, mechanical, photocopying, recording, or otherwise, without written permission of the publisher. 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ISBN: 978-1-879225-32-9 eISBN: 978-1-879225-34-3 Library of Congress Control Number: 2017932571 ENDO2017 · CONTENTS iii ENDO 2017 CONTENTS DISCLOSURE INDEX ........................................................................... iv FOREWORD ...................................................................................... v TOPIC INDEX ................................................................................... vi SPEAKER HANDOUT INDEX .................................................................. xi ADRENAL/HPA AXIS .......................................................................... 1 BONE, CALCIOTROPIC HORMONES, AND VITAMIN D .................................. 33 DIABETES AND GLUCOSE METABOLISM ................................................. 59 GENERAL ENDOCRINOLOGY .............................................................. 111 NEUROENDOCRINOLOGY AND PITUITARY ............................................. 141 OBESITY AND LIPIDS ...................................................................... 177 PEDIATRIC ENDOCRINOLOGY ............................................................ 203 REPRODUCTIVE ENDOCRINOLOGY ...................................................... 233 THYROID/HPT AXIS ........................................................................ 279 iv ENDO2017 · DISCLOSUREINDEX ENDO 2017 DISCLOSURE INDEX Thefaculty,committeemembers,andstaffwhoareinpositiontocontrol Consultant,ONO-Pharma.Leffert,J:StudyInvestigator,AbbottLaboratories, thecontentofthisactivityarerequiredtodisclosetotheEndocrineSociety Bristol-MyersSquibb,Merck&Co.,AstraZeneca,RocheDiagnostics,Novo and to learners any relevant financial relationship(s) of the individual or Nordisk,Sanofi.Mannstadt,M:AdvisoryGroupMember,Shire.Mauras,N: spouse/partner that have occurred within the last 12 months with any PrincipalInvestigator,Pfizer,Inc.,Orphan;AdvisoryGroupMember,OPKO. commercialinterest(s)whoseproductsorservicesarerelatedtotheCME McGill,J:AdvisoryGroupMember,JansenPharmaceuticals,Merck&Co., content.Financialrelationshipsaredefinedbyremunerationinanyamount Novo Nordisk, Calibra,Dynavax,Intarcia; Principal Investigator, Novartis from the commercial interest(s) in the form of grants; research support; Pharmaceuticals, Lexicon Pharmaceuticals, Inc.; Speaker, Jansen Pharma- consulting fees; salary; ownership interest (e.g., stocks, stock options, or ceuticals; Committee Member, Boehringer-Ingelheim; Ad Hoc Consultant, ownershipinterestexcludingdiversifiedmutualfunds);honorariaorother Dexcom.Merke,D:PrincipalInvestigator,Diurnal,MillendoTherapeutics. paymentsforparticipationinspeakers’bureaus,advisoryboards,orboards Molitch, M: Ad Hoc Consultant, Abbott Laboratories; Owner, Amgen; ofdirectors;orotherfinancialbenefits.Theintentofthisdisclosureisnotto Investigator, Bayer, Inc., Novartis Pharmaceuticals, Novo Nordisk, Ipsen, preventCMEplannerswithrelevantfinancialrelationshipsfromplanningor Johnson & Johnson; Advisory Group Member, Jansen Pharmaceuticals; delivery of content, but rather to provide learners with information that MemberofAdvisoryCommitteesorReviewPanels,Merck&Co.,Pfizer,Inc. allows them to make their own judgments of whether these financial re- Peters,A:AdHocConsultant,AbbottLaboratories,EliLilly&Company, lationships may have influenced the educational activity with regards to Merck&Co.,NovoNordisk,Sanofi,MedtronicMinimed;SpeakerBureau expositionorconclusion. Member,EliLilly&Company,NovoNordisk;Speaker,RocheDiagnostics; Investigator,Dexcom.Rosenthal,S:Consultant,ABBVIE,INC.Salvatori,R: TheEndocrineSocietyhasreviewedalldisclosuresandresolvedormanaged AdvisoryGroupMember,NovoNordisk,ionis;AdHocConsultant,Pfizer, allidentifiedconflictsofinterest,asapplicable. Inc. Santoro, N: Principal Investigator, Bayer, Inc.; Ad Hoc Consultant, Menogenix. Schwartz, A: Advisory Group Member, Amgen. Siraj, E: Thefollowingfacultyreportedrelevantfinancialrelationship,asidentified Consultant, GlaxoSmithKline. Terzolo M: HRA Pharma; Advisory Group below:Azziz,R:AdHocConsultant,Bayer,Inc.,JDSTherapeutics;Advisory Member,Atterocor.Verbalis,J:AdHocConsultant,FerringPharmaceuticals, GroupMember,GlobalPETImaging.Bessesen,D:DataSafetyMonitoring Otsuka.WeltC:Speaker,NovartisPharmaceuticals.Wysham,C:Speaker, BoardMember,EnteromedicsInc.Brown,T:Consultant,Gilead,Merck& AstraZeneca,EliLilly&Company,JansenPharmaceuticals,NovoNordisk, Co.,Bristol-MyersSquibb,EMDSerono,Theratechnologies.Cabanillas,M: Sanofi, Boehringer-Ingelheim; Advisory Group Member, Sanofi, Novo Consultant, Eisai. Advisory Group Member, LOXO. Dad-u,-R: Advisory Nordisk; Consultant, Jansen Pharmaceuticals, Novo Nordisk, Sanofi; Sci- Group Member, Bristol-Myers Squibb. Econs, M: Inventor, Kyowa Hakki entificBoardMember,JansenPharmaceuticals.Young,W:Consultant,Nihon Kirin Pharma. Eugster, E: Principal Investigator, Endo Pharmaceuticals; Medi-Physics.Zeitler,P:Consultant,DaiichiSankyo,Merck&Co.,Takeda, Study Investigator, Astra Zeneca, Eli Lilly & Company, Tolmar Orphan JansenPharmaceuticals,Boehringer-Ingelheim. Reach. Fleseriu, M: Principal Investigator, Ad Hoc Consultant, Novartis Pharmaceuticals, Chiasma, Pfizer Global R&D. Garg, A: Consultant, The following faculty reported no relevant financial relationships: Aegerion Pharmaceuticals, Inc.; Study Investigator, Aegerion, Principal Anawalt,BD;Balasubramanian,R;Baron,J;Becker,C;Brent,G;Cappola,A; Investigator, Ionis Pharmaceuticals, Inc., Intercept Pharmaceuticals, Inc., Carlson, HE; Chen, H; Christ-Crain. M; Colao, A; Collazo-Clavell, ML; Kadmon Pharmaceuticals, Inc.; Consultant, Ionis Pharmaceuticals, Inc. DiMeglio,LA;Doherty,G;El-HajjFuleihan,G;Else,T;Grossman,A;Han,J; Akcea Pharmaceuticals, Inc. Goldberg, A: Investigator, Amarin, Genzyme Jonklaas,J;Katznelson,L;Kellogg,TA;Kirk,SE;Klonoff,D;Lipska,K; Corporation, ISIS/IONIS, Amgen, Madrigal, Pfizer, Inc. Genentech, Inc., McCall, AL; McCartney, C; Newman, C; Nickolas, T; Rosen, CJ; Safer, J; GlaxoSmithKline,Merck&Co.,Sanofi,Regeneron;Editor,MerckManual; Speiser,PW;Tannock,LR;Timmers,JLM;Torpy,DJ;Tuttle,RM;Weber,TJ. Advisory Group Member, Regeneron, UniQure, Consultant, OptumRX. Korbonits, M: Principal Investigator, Teacher, Advisory Group Member; EndocrineSocietystaffreportnorelevantfinancialrelationships. ENDO2017 · FOREWORD v ENDO 2017 FOREWORD Intoday’sdemandingandrapidlychanginghealthcareenvironment,finding thetimetokeepabreastofthelatestendocrinecasemanagementstrategies and state-of-the-art treatments has become increasingly difficult. The Meet-The-Professor Endocrine Case Management book is designed to provide physicians with a concise and high-quality review of over 60 commonandrareendocrinedisorderstohelpyoukeepyourpracticecurrent. Thecontributorsofthisbookareexpertsintheirrespectivefieldsandhave been selected for their deep understanding of the topic, their clinical ex- perience, and their ability to clearly communicate complex information. Thebookutilizesacase-basedformattofacilitateanenjoyablelearning experienceandhelpsolidifyretentionoflessonslearned.Eachcontributor hasprovidedlearningobjectives,aconciseup-to-datereviewoftheirtopic, aswellasasummaryofcasesfollowedbyabriefdiscussionofthecasesfor your edification. This format promotes active learning by enhancing your knowledge of clinical endocrinology, and challenging you to apply that knowledge to the cases included in the following pages, which mirror the patients we see daily in the office and the hospital. We thank the many experts who have shared their vast knowledge and insightsinthelivesessionsandthisaccompanyingbook,whichalsoserves as a wonderful resource for sessions that you may have been unable to attend. We are also grateful for the Endocrine Society staff whose efforts were instrumental to the successful publication of this book. Ann Danoff, MD Physician-In-Practice Chair, ENDO 2017 Philadelphia Veterans Affairs Hospital Philadelphia, Pennsylvania John (Jack) Leahy, MD Clinical Science Chair, ENDO 2017 University of Vermont, College of Medicine Colchester, Vermont vi ENDO2017 · TOPICINDEX ENDO 2017 TOPIC INDEX ADRENAL/HPA AXIS M01 – Adrenal Incidentalomas Page 2 Massimo Terzolo, MD M02 – Attenuated Congenital Adrenal Hyperplasia Page 8 Deborah P. Merke, MS, MD M03 – Diagnosis and Management of Pheochromocytoma/Paraganglioma Page 11 Henri J.L.M. Timmers, MD, PhD M04 – Diagnosis and Treatment of Adrenal Insufficiency Page 19 David J. Torpy, FRACP M05 – Primary Aldosteronism: What Is New and Clinically Relevant? Page 29 William F. Young, Jr., MD, MSc BONE, CALCIOTROPIC HORMONES, AND VITAMIN D M06 – Bone Health in Patients With Cancer Page 34 Carolyn Becker, MD M07 – Challenges in DXA Interpretation: Do Expect Anomalies Page 38 Thomas J. Weber, MD M08 – Hypocalcemia Page 43 Clifford J. Rosen, MD M09 – Hypoparathyroidism Page 47 Michael Mannstadt, MD M10 – Hypophosphatemic Disorders Page 50 Michael J. Econs, MD M11 – Osteoporosis Treatment Failure, Change of Therapy, and Drug Holidays Page 54 Ghada El-Hajj Fuleihan, MD, MPH DIABETES AND GLUCOSE METABOLISM M13 – Comorbidities in Diabetes and Cardiovascular Disease Page 60 Janet B. McGill, MD M14 – Diabetes and Metabolic Bone Health Page 63 Ann Schwartz, PhD ENDO2017 · TOPICINDEX vii M15 – Continuous Glucose Monitoring Page 66 David C. Klonoff, MD, FACP, FRCP (Edin), Fellow AIMBE M16 – Diagnosis and Management of Diabetes in Pregnancy Page 71 Susan E. Kirk, MD M17 – Diagnosis and Management of Diabetic Neuropathies Page 76 Aaron I. Vinik, MD, PhD, FCP, MACP, FACE M18 – New Insulins and New Combinations Page 86 Carol H. Wysham, MD M19 – Goals of Diabetes Care in the Oldest of the Old Page 91 Kasia J. Lipska, MHS M20 – How To Be a Change Agent Page 95 Ann Peters, MD M21 – Hypoglycemia Page 98 Anthony L. McCall, MD, PhD M22 – Posttransplantation Diabetes Mellitus Page 106 Elias S. Siraj, MD, FACP, FACE GENERAL ENDOCRINOLOGY CMF1 – Endocrine Tumor Genetics: Challenging Issues Page 112 Tobias Else, MD and Ma´rta Korbonits, MD, PhD M23 – Endocrine Immune-Related Adverse Events Page 123 Ramona Dadu, MD M24 – Challenging Patients: Hey Doc, Something Is Wrong With My Glands Page 127 Jonathan D. Leffert, MD, FACP, FACE, ECNU M25 – Growth Hormone Therapy in Adults: Getting It Right! Page 129 Annamaria Colao, MD, PhD M27 – HIV and Endocrine Disorders Page 135 Todd T. Brown, MD, PhD NEUROENDOCRINOLOGY AND PITUITARY M28 – Diagnosis and Management of Diabetes Insipidus Page 142 Mirjam Christ-Crain, MD, PhD M29 – Diagnosis and Management of Hyponatremia Page 147 Joseph G. Verbalis, MD M30 – Diagnosis and Management of Prolactinoma Page 156 Mark E. Molitch, MD viii ENDO2017 · TOPICINDEX M31 – Medical Management of Acromegaly: An Update Page 160 Maria Fleseriu, FACE M32 – Metastatic Neuroendocrine Tumors Page 167 Ashley B. Grossman, MD M33 – Perioperative Management of Pituitary Tumors Page 171 Roberto Salvatori, MD OBESITY AND LIPIDS CMF5 – Care of the Post–Bariatric Surgery Patient: The Good, the Bad, and the Ugly Page 178 Maria L. Collazo-Clavell, MD M34 – Appropriate Use of PCSK9 Monoclonal Antibodies Page 181 Anne Carol Goldberg, MD M36 – Lipid Disorders in Women Page 184 Lisa Tannock, MD M37 – Lipodystrophy Syndromes Page 188 Abhimanyu Garg, MD M38 – Statin Intolerance Page 193 Connie B. Newman, MD PEDIATRIC ENDOCRINOLOGY M41 – CAH in the Pediatric Patient Page 204 Phyllis W. Speiser, MD M42 – Management of Precocious Puberty: Questions and Considerations Page 208 Erica A. Eugster, MD M43 – Short Stature Page 213 Jeffrey Baron, MD M44 – Treating Toddlers: Care of Preschool Children With Type 1 Diabetes Page 217 Linda A. DiMeglio, MD, MPH M45 – Turner Syndrome: A Focus on Growth, Puberty, and Fertility Page 221 Nelly Mauras, MD M46 – Youth-Onset Type 2 Diabetes Page 228 Philip Zeitler, MD, PhD REPRODUCTIVE ENDOCRINOLOGY CMF3 – Case Management Forum: Polycystic Ovary Syndrome Page 234 Christopher R. McCartney, MD and Corrine K. Welt, MD