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ContinuingMedicalEducationDepartment
Course Faculty
Symposium Course Directors
Diva D. De León, M.D., M.S.C.E.
Director, Congenital Hyperinsulinism Center
Associate Professor of Pediatrics
Charles A. Stanley, M.D.
Founder, Congenital Hyperinsulinism Center
Emeritus Professor of Pediatrics
Faculty
N. Scott Adzick, M.D., M.M.M., F.A.C.S.,	
F.A.A.P.
Surgeon-in-Chief
C. Everett Koop Professor of Pediatric Surgery
Arupa Ganguly, Ph.D.
Director, Genetic Diagnostic Laboratory
Hospital of the University of Pennsylvania
Associate Professor, Department of Genetics
Jennifer Kalish, M.D., Ph.D.
Attending Physician
Division of Human Genetics and Metabolism
Instructor of Pediatrics
David Langdon, M.D., F.A.A.P.
Director, Inpatient Endocrine Service
Clinical Associate Professor
Changhong Li, M.D., Ph.D.
Division of Endocrinology and Diabetes
Research Assistant Professor of Pediatrics
Franz M. Matschinsky, M.D.
Benjamin Rush Professor of Biochemistry and
	Biophysics
Lisa J. States, M.D.
Director of Outpatient Satellite Imaging
Department of Radiology
Associate Professor of Radiology
Guest Faculty
Jean Baptiste Arnoux, M.D., M.Sc.
Senior Consultant in Inherited Metabolic
	 Disorders and Congenital Hyperinsulinism
Hôpital Universitaire Necker
Paris, France
Mark Dunne, Ph.D.
Professor
The University of Manchester
Manchester, England
Sian Ellard, Ph.D., F.R.C.Path.
Head of Molecular Genetics, Royal Devon &
	 Exeter NHS Foundation Trust
Professor of Human Molecular Genetics
Devon, England
Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., 	
M.R.C.P.C.H., M.D.
Consultant Paediatric Endocrinologist
Great Ormond Street Hospital for Children
London, England
Klaus Mohnike, M.D.
Professor
Otto-von-Guericke Universität
Magdeburg, Germany
Eva Morava-Kozicz, M.D., Ph.D.
Clinical Biochemical Geneticist

Professor of Pediatrics
Tulane University School
of Medicine
Hayward Genetics Center
New Orleans, La.
Andrew Palladino, M.D.
Clinical Director
Endocrine Therapeutics
Pfizer
Collegeville, Pa.
Show-Ling Shyng, Ph.D.
Professor
Department of Biochemistry and Molecular
	Biology
Oregon Health and Science University
Portland, Ore.
Paul Thornton, M.D.
Director, Congenital Hyperinsulinism Center
Cook Children’s Medical Center
Fort Worth, Texas
David Weinstein, M.D., M.M.Sc.
Professor

Director, Glycogen Storage Disease Program

University of Florida
Gainesville, Fla.
Joseph Wolfsdorf, M.D., B.Ch.
Associate Chief, Division of Endocrinology
Clinical Director, Division of Endocrinology
Professor of Pediatrics
Boston Children’s Hospital
Boston, Mass.
Hotel Information
The Children’s Hospital of Philadelphia has
reserved blocks of rooms at the Sheraton
University City at 3549 Chestnut St., in
Philadelphia. The Sheraton University City
(www.philadelphiasheraton.com) is located
in the heart of the University of Pennsylvania
campus, adjacent to Philadelphia’s prestigious
medical, education and corporate centers, and
just blocks away from the Colket Building.
The group overnight room rate is $179 single
and double occupancy plus a 15.5 percent
room tax. Please contact the hotel directly at
215-387-8000 to make your reservation. When
making your reservation at the hotel, please
mention the Congenital Hyperinsulinism
Symposium. For a complete listing of lodging
in Philadelphia, please visit the Philadelphia
Convention and Visitor’s Bureau website at
www.pcvb.org.
Cancellation and Refund Policy
The Children’s Hospital of Philadelphia
reserves the right to cancel or postpone
any conference due to any unforeseen
circumstances. In the event of cancellation or
postponement, Children’s Hospital will refund
any registration fees, less a $30 service charge,
but is not responsible for any related costs,
charges or expenses to participants, including
cancellation charges assessed by airlines/travel
agencies. In order to process refunds for course
withdrawals, cancellation must be received in
writing by Friday, April 1. No refunds will be
issued thereafter.
Services for People with Disabilities
If arrangements are required for an individual
with a disability to attend this meeting, please
contact the Continuing Medical Education
Department at 215-590-5263.
General
Info continued Congenital Hypoglycemia Disorders:
Hyperinsulinism and GSD
April 14 and 15, 2016
Ruth and Tristram Colket, Jr.
Translational Research
Building at
The Children’s Hospital
of Philadelphia
Sponsored by The Congenital
Hyperinsulinism Center
Division of Endocrinology and Diabetes
www.chop.edu/cme
Unless otherwise noted, faculty is from The Children’s Hospital of Philadelphia
and the Perelman School of Medicine at the University of Pennsylvania.
ADDRESSCORRECTIONREQUESTED
General InformationProgram Registration
Registeronlineatwww.chop.edu/cme
Program Overview
This two-day international symposium will be
an update on recent exciting advances in research
related to congenital hyperinsulinism and other
inherited hypoglycemia disorders, particularly
disorders of glycogenoses. The symposium is
intended to enhance the clinical management skills
and practices of endocrinologists, metabolism
specialists, neonatologists, surgeons, pathologists,
radiologists, geneticists and nurses, as well as basic
scientists interested in pancreatic islet biology and
regulation of glucose metabolism. The symposium
faculty, chosen from 10 institutions in the United
States, France, Great Britain, and Germany, will
present 27 plenary lectures on all aspects of these
disorders. Participants may register via mail or fax
by completing the Registration Form attached to the
brochure and indicating their form of payment. To
register online go to www.chop.edu/cme.
General Information
The symposium will be held on Thursday, April 14
and Friday, April 15, 2016, at the Ruth and Tristram
Colket, Jr. Translational Research Building, located
at 3614 Civic Center Blvd. on the campus of The
Children’s Hospital of Philadelphia (www.chop.edu).
Registration will begin each day at 7:30 a.m. The
registration fee includes continental breakfast, lunch,
breaks, parking and syllabus materials. Participants
are invited to attend a reception and dinner on
Thursday, April 14 for an additional fee of $75.
If you wish to attend, please check this option on
the registration form and add this fee to your total
registration fee.
Learning Objectives
Upon completion of this live activity, participants
should be able to:
•	 Provide an overview of the clinical manifestations,
genetics and natural history of congenital
hyperinsulinism and disorders of glycogenoses
•	 Review current knowledge of the normal
regulation of insulin secretion and the
pathophysiology of disorders causing
hyperinsulinism
•	 Discuss recent advances and current controversies
in the surgical and medical management of diffuse
and focal hyperinsulinism
Abstract Submissions
The program includes three sessions at which
attendees may present posters and short oral
communications of their own clinical or laboratory
research. Abstracts must be submitted electronically
using the registration form on the Congenital
Hyperinsulinism Symposium website
(www.chop.edu/cme). The deadline for abstract
submission is Friday, Jan. 8, 2016. Authors will
be notified of abstracts accepted for poster or oral
presentations by Monday, Feb. 1, 2016. A limited
number of travel grants will be available to young
investigators wishing to apply. Young investigators
must apply at the time they register for the
symposium and submit their abstract.
ACCME Accreditation Statement
The Children’s Hospital of Philadelphia is accredited
by the Accreditation Council for Continuing
Medical Education (ACCME) to provide continuing
medical education for physicians.
AMA Credit Designation Statement
The Children’s Hospital of Philadelphia designates
this live activity for a maximum of 13.0 AMA PRA
Category 1 Credit(s)™
. Physicians should claim only
the credit commensurate with their participation in
the activity.
Nursing Accreditation
This activity has been submitted to the PA State
Nurses Association for approval to award contact
hours. The PA State Nurses Association is accredited
as an approver of continuing nursing education
by the American Nurses Credentialing Center’s
Commission on Accreditation.
Congenital Hypoglycemia Disorders: Hyperinsulinism and GSD
April 14 and 15, 2016 – Registration Deadline: Friday, April 1
Name ____________________________ Credentials _____________
Hospital/Professional Affiliation _______________________________
Home Address ____________________________________________
City ____________________________ State _____ ZIP __________
Phone ________________________ Fax _______________________
Email ___________________________________________________
Conference Registration Fees
q M.D.s: $350	 q Non-M.D.s and Physicians-in-training: $250
q Reception and Dinner, Thursday, April 14: $75
Total Registration Fee: $____________
Registration will not be processed without full payment. Faxed registrations
will be accepted for credit card payments only. Please contact Micah Holliday
(hollidaydm@email.chop.edu) if you do not receive a confirmation letter.
Method of Payment
q Personal check		 q Institutional check
(Please make check payable to The Children’s Hospital of Philadelphia.)
q Discover q MasterCard q American Express q VISA
Credit Card # _____________________________________________
Exp. Date ________________________________________________
Mail/fax the completed registration form and payment to:
Ms. Micah Holliday, Continuing Medical Education Department
The Children’s Hospital of Philadelphia
34th Street and Civic Center Boulevard
Philadelphia, PA 19104
215-590-5CME • Fax 215-590-4342 • hollidaydm@email.chop.edu
The deadline for abstract submission is Friday, Jan. 8, 2016.
continued on back page
Thursday, April 14, 2016 Friday, April 15, 2016
Session 1: Congenital Hyperinsulinism (CHI)
8 – 10 a.m.
8 a.m.		 Welcome and Introductions				
Diva D. De León, M.D., M.S.C.E.
8:15 a.m.	 History of Hyperinsulinism (HI) 				
in Pediatrics and Overview of 				
Diagnostic/Therapeutic Algorithms 				
Charles A. Stanley, M.D.
8:30 a.m.	 Diazoxide-responsive Hyperinsulinism 				
a.	GDH-HI				
b.	KATP-HI				
c.	Perinatal Stress HI				
d.	HNFs-HI				
Charles A. Stanley, M.D.
9 a.m.		 Diazoxide-unresponsive Hyperinsulinism				
a.	KATP-HI: dominant vs. recessive,					
focal vs. diffuse				
b.	Atypical – LINE				
c.	GCK				
Diva D. De León, M.D., M.S.C.E.
9:30 a.m.	 Syndromic Hyperinsulinism				
BWS – Jennifer Kalish, M.D., Ph.D.				
Kabuki Syndrome – Jean Baptiste Arnoux,					
M.D., M.Sc.	
10 a.m.		 Break and Exhibits
Session 2: Other Causes of Hyperinsulinemic Hypoglycemia
10:30 – 11:20 a.m. 	
10:30 a.m.	Short Communications (10 minutes each)				
UCP2 Hyperinsulinism 				
Christine Ferrara, M.D., Ph.D.				
PGM1 Hyperinsulinism 				
Amanda Ackerman, M.D., Ph.D.				
Hyperinsulinism in Turner Syndrome 				
Christopher Gibson, M.D.	
11 a.m.		 Insulinomas and HI Mimickers 				
Andrew Palladino, M.D.
11:20 a.m.	Lunch — Exhibits and Scientific Posters
Session 3: Diagnosis and Treatment of Hyperinsulinism
12:30 – 2:20 p.m.
12:30 p.m.	Approach to the Diagnosis and 				
Management of Hyperinsulinism				
Diva D. De León, M.D., M.S.C.E.
12:50 p.m.	Genotype/Phenotype Correlation in 				
KATP Hyperinsulinism				
Arupa Ganguly, Ph.D.
1:05 p.m. 	 The Role of Genetic Testing for 				
Hyperinsulinism: New Frontiers in 				
Genetic Diagnosis				
Sian Ellard, Ph.D., F.R.C.Path.
1:25 p.m. 	 Surgical vs. Nonsurgical Decisions and 				
Options				
Paul Thornton, M.D.; Khalid Hussain, 					
M.B.Ch.B., M.R.C.P., M.Sc.,					
M.R.C.P.C.H., M.D.
2 p.m.		 Long-acting Somatostatin Analogs				
Jean Baptiste Arnoux, M.D., M.Sc.
2:20 p.m.	 Break, Exhibits and Scientific Posters
Session 4: Investigational Therapies for Hyperinsulinism
2:40 – 4:10 p.m.		
2:40 p.m.	 Investigational Therapies: Exendin-(9-39)				
Diva D. De León, M.D., M.S.C.E.
2:55 p.m.	 Investigational Therapies: Sirolimus 				
Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., 					
M.R.C.P.C.H., M.D.
3:10 p.m.	 Investigational Therapies: 				
Subcutaneous 	Glucagon				
Klaus Mohnike, M.D.
3:25 p.m. 	Investigational Therapies: Insulin 				
Receptor Antibody				
Diva D. De León, M.D., M.S.C.E.
3: 40 p.m.	 Abstract Presentations (3 – 10 min. each)
Session 5: Surgical Management Approaches to
Hyperinsulinism
4:10 – 5 p.m.
4:10 p.m.	 [18F]-DOPA PET Imaging of Focal 				
Hyperinsulinism: “GPS” for the Surgeon				
Lisa J. States, M.D.
4:30 p.m.	 Surgery for Hyperinsulinism				
N. Scott Adzick, M.D., M.M.M., F.A.C.S., 					
F.A.A.P.
5 p.m.		 Adjourn
5:30 p.m.	 Symposium Dinner				
Keynote Speaker: Franz M. Matschinsky, M.D.
Session 9: Breakout Sessions — Q&A with the Experts
3:10 – 4 p.m.					
Group A: 				
Management of the Diazoxide-responsive 					
Children				
Group B:				
Management of the Diazoxide-unresponsive 					
Children				
Group C:				
Management of Diabetes after 					
Pancreatectomy				
Group D:				
Management of Children with GSD			
4 p.m.		 Closing Remarks				
Charles A. Stanley, M.D.
4:15 p.m.	 Adjourn
Acknowledgement
The “Congenital Hypoglycemia Disorders:
Hyperinsulinism and Glycogen Storage Disease (GSD)
Symposium” is supported by an educational grant
provided by the Orphan Disease Center at the University
of Pennsylvania. The Congenital Hyperinsulinism Center
at The Children’s Hospital of Philadelphia gratefully
acknowledge this support.
Session 6: Prevention of Brain Damage
8 – 9:40 a.m.
8 a.m.		 Hypoglycemia Guidelines				
Paul Thornton, M.D.
8:20 a.m.	 Neurodevelopmental Outcomes				
Diva D. De León, M.D., M.S.C.E.
8:50 a.m.	 Management after Pancreatectomy:				
Persistent Hypoglycemia and Hyperglycemia				
David Langdon, M.D., F.A.A.P.
9:10 a.m.	 Abstract Presentations (3 – 10 min. each)	
9:40 a.m.	 Break, Exhibits and Scientific Posters
Session 7: Islet Pathophysiology in Hyperinsulinism
10 – 11:30 a.m.
10 a.m.		 ß-cell KATP Channels in Health and Disease				
Show-Ling Shyng, Ph.D.
10:20 a.m. 	Insulin Secretion and Fuel Metabolism in 				
Human HI Islets				
Changhong Li, M.D., Ph.D.
10:40 a.m.	Beyond the ß-cell				
Mark Dunne, Ph.D.
11 a.m. 		 Abstract Presentations (3 – 10 min. each)
11:30 a.m. 	Lunch — Exhibits and Scientific Posters
Session 8: Hyperketotic Hypoglycemia
1 – 2:50 p.m.
1 p.m. 		 Overview of Ketotic Hypoglycemia 				
Disorders (Glycogenosis, MCT1)				
Joseph Wolfsdorf, M.D., B.Ch.
1:30 p.m.	 Natural History of GSD				
David Weinstein, M.D., M.M.Sc.
2 p.m.		 Current and Investigational Therapies 				
for GSD				
David Weinstein, M.D., M.M.Sc.
2:30 p.m.	 PGM1 in the Regulation of Glucose 				
Metabolism and Insulin Secretion				
Eva Morava-Kozicz, M.D., Ph.D.
2:50 p.m.	 Break, Exhibits and Scientific Posters	
©2015 The Children’s Hospital of Philadelphia, All Rights Reserved. 15CME0014/2.8M/09-15
Please note: This meeting will be followed by
a two-day conference for patients, parents and
families of children with HI on Saturday, April 16
and Sunday, April 17. Co-sponsored by Congenital
Hyperinsulinism International (CHI), more
information will be available on CHI’s website,
www.congenitalHI.org, and will also be posted on
our Web page, hyperinsulinism.chop.edu. Please
encourage families of any patients you follow to
consider joining us. Professional staff are also
welcome to join us for this more informal
informational exchange.
General InformationProgram Registration
Registeronlineatwww.chop.edu/cme
Program Overview
This two-day international symposium will be
an update on recent exciting advances in research
related to congenital hyperinsulinism and other
inherited hypoglycemia disorders, particularly
disorders of glycogenoses. The symposium is
intended to enhance the clinical management skills
and practices of endocrinologists, metabolism
specialists, neonatologists, surgeons, pathologists,
radiologists, geneticists and nurses, as well as basic
scientists interested in pancreatic islet biology and
regulation of glucose metabolism. The symposium
faculty, chosen from 10 institutions in the United
States, France, Great Britain, and Germany, will
present 27 plenary lectures on all aspects of these
disorders. Participants may register via mail or fax
by completing the Registration Form attached to the
brochure and indicating their form of payment. To
register online go to www.chop.edu/cme.
General Information
The symposium will be held on Thursday, April 14
and Friday, April 15, 2016, at the Ruth and Tristram
Colket, Jr. Translational Research Building, located
at 3614 Civic Center Blvd. on the campus of The
Children’s Hospital of Philadelphia (www.chop.edu).
Registration will begin each day at 7:30 a.m. The
registration fee includes continental breakfast, lunch,
breaks, parking and syllabus materials. Participants
are invited to attend a reception and dinner on
Thursday, April 14 for an additional fee of $75.
If you wish to attend, please check this option on
the registration form and add this fee to your total
registration fee.
Learning Objectives
Upon completion of this live activity, participants
should be able to:
•	 Provide an overview of the clinical manifestations,
genetics and natural history of congenital
hyperinsulinism and disorders of glycogenoses
•	 Review current knowledge of the normal
regulation of insulin secretion and the
pathophysiology of disorders causing
hyperinsulinism
•	 Discuss recent advances and current controversies
in the surgical and medical management of diffuse
and focal hyperinsulinism
Abstract Submissions
The program includes three sessions at which
attendees may present posters and short oral
communications of their own clinical or laboratory
research. Abstracts must be submitted electronically
using the registration form on the Congenital
Hyperinsulinism Symposium website
(www.chop.edu/cme). The deadline for abstract
submission is Friday, Jan. 8, 2016. Authors will
be notified of abstracts accepted for poster or oral
presentations by Monday, Feb. 1, 2016. A limited
number of travel grants will be available to young
investigators wishing to apply. Young investigators
must apply at the time they register for the
symposium and submit their abstract.
ACCME Accreditation Statement
The Children’s Hospital of Philadelphia is accredited
by the Accreditation Council for Continuing
Medical Education (ACCME) to provide continuing
medical education for physicians.
AMA Credit Designation Statement
The Children’s Hospital of Philadelphia designates
this live activity for a maximum of 13.0 AMA PRA
Category 1 Credit(s)™
. Physicians should claim only
the credit commensurate with their participation in
the activity.
Nursing Accreditation
This activity has been submitted to the PA State
Nurses Association for approval to award contact
hours. The PA State Nurses Association is accredited
as an approver of continuing nursing education
by the American Nurses Credentialing Center’s
Commission on Accreditation.
Congenital Hypoglycemia Disorders: Hyperinsulinism and GSD
April 14 and 15, 2016 – Registration Deadline: Friday, April 1
Name ____________________________ Credentials _____________
Hospital/Professional Affiliation _______________________________
Home Address ____________________________________________
City ____________________________ State _____ ZIP __________
Phone ________________________ Fax _______________________
Email ___________________________________________________
Conference Registration Fees
q M.D.s: $350	 q Non-M.D.s and Physicians-in-training: $250
q Reception and Dinner, Thursday, April 14: $75
Total Registration Fee: $____________
Registration will not be processed without full payment. Faxed registrations
will be accepted for credit card payments only. Please contact Micah Holliday
(hollidaydm@email.chop.edu) if you do not receive a confirmation letter.
Method of Payment
q Personal check		 q Institutional check
(Please make check payable to The Children’s Hospital of Philadelphia.)
q Discover q MasterCard q American Express q VISA
Credit Card # _____________________________________________
Exp. Date ________________________________________________
Mail/fax the completed registration form and payment to:
Ms. Micah Holliday, Continuing Medical Education Department
The Children’s Hospital of Philadelphia
34th Street and Civic Center Boulevard
Philadelphia, PA 19104
215-590-5CME • Fax 215-590-4342 • hollidaydm@email.chop.edu
The deadline for abstract submission is Friday, Jan. 8, 2016.
continued on back page
Thursday, April 14, 2016 Friday, April 15, 2016
Session 1: Congenital Hyperinsulinism (CHI)
8 – 10 a.m.
8 a.m.		 Welcome and Introductions				
Diva D. De León, M.D., M.S.C.E.
8:15 a.m.	 History of Hyperinsulinism (HI) 				
in Pediatrics and Overview of 				
Diagnostic/Therapeutic Algorithms 				
Charles A. Stanley, M.D.
8:30 a.m.	 Diazoxide-responsive Hyperinsulinism 				
a.	GDH-HI				
b.	KATP-HI				
c.	Perinatal Stress HI				
d.	HNFs-HI				
Charles A. Stanley, M.D.
9 a.m.		 Diazoxide-unresponsive Hyperinsulinism				
a.	KATP-HI: dominant vs. recessive,					
focal vs. diffuse				
b.	Atypical – LINE				
c.	GCK				
Diva D. De León, M.D., M.S.C.E.
9:30 a.m.	 Syndromic Hyperinsulinism				
BWS – Jennifer Kalish, M.D., Ph.D.				
Kabuki Syndrome – Jean Baptiste Arnoux,					
M.D., M.Sc.	
10 a.m.		 Break and Exhibits
Session 2: Other Causes of Hyperinsulinemic Hypoglycemia
10:30 – 11:20 a.m. 	
10:30 a.m.	Short Communications (10 minutes each)				
UCP2 Hyperinsulinism 				
Christine Ferrara, M.D., Ph.D.				
PGM1 Hyperinsulinism 				
Amanda Ackerman, M.D., Ph.D.				
Hyperinsulinism in Turner Syndrome 				
Christopher Gibson, M.D.	
11 a.m.		 Insulinomas and HI Mimickers 				
Andrew Palladino, M.D.
11:20 a.m.	Lunch — Exhibits and Scientific Posters
Session 3: Diagnosis and Treatment of Hyperinsulinism
12:30 – 2:20 p.m.
12:30 p.m.	Approach to the Diagnosis and 				
Management of Hyperinsulinism				
Diva D. De León, M.D., M.S.C.E.
12:50 p.m.	Genotype/Phenotype Correlation in 				
KATP Hyperinsulinism				
Arupa Ganguly, Ph.D.
1:05 p.m. 	 The Role of Genetic Testing for 				
Hyperinsulinism: New Frontiers in 				
Genetic Diagnosis				
Sian Ellard, Ph.D., F.R.C.Path.
1:25 p.m. 	 Surgical vs. Nonsurgical Decisions and 				
Options				
Paul Thornton, M.D.; Khalid Hussain, 					
M.B.Ch.B., M.R.C.P., M.Sc.,					
M.R.C.P.C.H., M.D.
2 p.m.		 Long-acting Somatostatin Analogs				
Jean Baptiste Arnoux, M.D., M.Sc.
2:20 p.m.	 Break, Exhibits and Scientific Posters
Session 4: Investigational Therapies for Hyperinsulinism
2:40 – 4:10 p.m.		
2:40 p.m.	 Investigational Therapies: Exendin-(9-39)				
Diva D. De León, M.D., M.S.C.E.
2:55 p.m.	 Investigational Therapies: Sirolimus 				
Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., 					
M.R.C.P.C.H., M.D.
3:10 p.m.	 Investigational Therapies: 				
Subcutaneous 	Glucagon				
Klaus Mohnike, M.D.
3:25 p.m. 	Investigational Therapies: Insulin 				
Receptor Antibody				
Diva D. De León, M.D., M.S.C.E.
3: 40 p.m.	 Abstract Presentations (3 – 10 min. each)
Session 5: Surgical Management Approaches to
Hyperinsulinism
4:10 – 5 p.m.
4:10 p.m.	 [18F]-DOPA PET Imaging of Focal 				
Hyperinsulinism: “GPS” for the Surgeon				
Lisa J. States, M.D.
4:30 p.m.	 Surgery for Hyperinsulinism				
N. Scott Adzick, M.D., M.M.M., F.A.C.S., 					
F.A.A.P.
5 p.m.		 Adjourn
5:30 p.m.	 Symposium Dinner				
Keynote Speaker: Franz M. Matschinsky, M.D.
Session 9: Breakout Sessions — Q&A with the Experts
3:10 – 4 p.m.					
Group A: 				
Management of the Diazoxide-responsive 					
Children				
Group B:				
Management of the Diazoxide-unresponsive 					
Children				
Group C:				
Management of Diabetes after 					
Pancreatectomy				
Group D:				
Management of Children with GSD			
4 p.m.		 Closing Remarks				
Charles A. Stanley, M.D.
4:15 p.m.	 Adjourn
Acknowledgement
The “Congenital Hypoglycemia Disorders:
Hyperinsulinism and Glycogen Storage Disease (GSD)
Symposium” is supported by an educational grant
provided by the Orphan Disease Center at the University
of Pennsylvania. The Congenital Hyperinsulinism Center
at The Children’s Hospital of Philadelphia gratefully
acknowledge this support.
Session 6: Prevention of Brain Damage
8 – 9:40 a.m.
8 a.m.		 Hypoglycemia Guidelines				
Paul Thornton, M.D.
8:20 a.m.	 Neurodevelopmental Outcomes				
Diva D. De León, M.D., M.S.C.E.
8:50 a.m.	 Management after Pancreatectomy:				
Persistent Hypoglycemia and Hyperglycemia				
David Langdon, M.D., F.A.A.P.
9:10 a.m.	 Abstract Presentations (3 – 10 min. each)	
9:40 a.m.	 Break, Exhibits and Scientific Posters
Session 7: Islet Pathophysiology in Hyperinsulinism
10 – 11:30 a.m.
10 a.m.		 ß-cell KATP Channels in Health and Disease				
Show-Ling Shyng, Ph.D.
10:20 a.m. 	Insulin Secretion and Fuel Metabolism in 				
Human HI Islets				
Changhong Li, M.D., Ph.D.
10:40 a.m.	Beyond the ß-cell				
Mark Dunne, Ph.D.
11 a.m. 		 Abstract Presentations (3 – 10 min. each)
11:30 a.m. 	Lunch — Exhibits and Scientific Posters
Session 8: Hyperketotic Hypoglycemia
1 – 2:50 p.m.
1 p.m. 		 Overview of Ketotic Hypoglycemia 				
Disorders (Glycogenosis, MCT1)				
Joseph Wolfsdorf, M.D., B.Ch.
1:30 p.m.	 Natural History of GSD				
David Weinstein, M.D., M.M.Sc.
2 p.m.		 Current and Investigational Therapies 				
for GSD				
David Weinstein, M.D., M.M.Sc.
2:30 p.m.	 PGM1 in the Regulation of Glucose 				
Metabolism and Insulin Secretion				
Eva Morava-Kozicz, M.D., Ph.D.
2:50 p.m.	 Break, Exhibits and Scientific Posters	
©2015 The Children’s Hospital of Philadelphia, All Rights Reserved. 15CME0014/2.8M/09-15
Please note: This meeting will be followed by
a two-day conference for patients, parents and
families of children with HI on Saturday, April 16
and Sunday, April 17. Co-sponsored by Congenital
Hyperinsulinism International (CHI), more
information will be available on CHI’s website,
www.congenitalHI.org, and will also be posted on
our Web page, hyperinsulinism.chop.edu. Please
encourage families of any patients you follow to
consider joining us. Professional staff are also
welcome to join us for this more informal
informational exchange.
General InformationProgram Registration
Registeronlineatwww.chop.edu/cme
Program Overview
This two-day international symposium will be
an update on recent exciting advances in research
related to congenital hyperinsulinism and other
inherited hypoglycemia disorders, particularly
disorders of glycogenoses. The symposium is
intended to enhance the clinical management skills
and practices of endocrinologists, metabolism
specialists, neonatologists, surgeons, pathologists,
radiologists, geneticists and nurses, as well as basic
scientists interested in pancreatic islet biology and
regulation of glucose metabolism. The symposium
faculty, chosen from 10 institutions in the United
States, France, Great Britain, and Germany, will
present 27 plenary lectures on all aspects of these
disorders. Participants may register via mail or fax
by completing the Registration Form attached to the
brochure and indicating their form of payment. To
register online go to www.chop.edu/cme.
General Information
The symposium will be held on Thursday, April 14
and Friday, April 15, 2016, at the Ruth and Tristram
Colket, Jr. Translational Research Building, located
at 3614 Civic Center Blvd. on the campus of The
Children’s Hospital of Philadelphia (www.chop.edu).
Registration will begin each day at 7:30 a.m. The
registration fee includes continental breakfast, lunch,
breaks, parking and syllabus materials. Participants
are invited to attend a reception and dinner on
Thursday, April 14 for an additional fee of $75.
If you wish to attend, please check this option on
the registration form and add this fee to your total
registration fee.
Learning Objectives
Upon completion of this live activity, participants
should be able to:
•	 Provide an overview of the clinical manifestations,
genetics and natural history of congenital
hyperinsulinism and disorders of glycogenoses
•	 Review current knowledge of the normal
regulation of insulin secretion and the
pathophysiology of disorders causing
hyperinsulinism
•	 Discuss recent advances and current controversies
in the surgical and medical management of diffuse
and focal hyperinsulinism
Abstract Submissions
The program includes three sessions at which
attendees may present posters and short oral
communications of their own clinical or laboratory
research. Abstracts must be submitted electronically
using the registration form on the Congenital
Hyperinsulinism Symposium website
(www.chop.edu/cme). The deadline for abstract
submission is Friday, Jan. 8, 2016. Authors will
be notified of abstracts accepted for poster or oral
presentations by Monday, Feb. 1, 2016. A limited
number of travel grants will be available to young
investigators wishing to apply. Young investigators
must apply at the time they register for the
symposium and submit their abstract.
ACCME Accreditation Statement
The Children’s Hospital of Philadelphia is accredited
by the Accreditation Council for Continuing
Medical Education (ACCME) to provide continuing
medical education for physicians.
AMA Credit Designation Statement
The Children’s Hospital of Philadelphia designates
this live activity for a maximum of 13.0 AMA PRA
Category 1 Credit(s)™
. Physicians should claim only
the credit commensurate with their participation in
the activity.
Nursing Accreditation
This activity has been submitted to the PA State
Nurses Association for approval to award contact
hours. The PA State Nurses Association is accredited
as an approver of continuing nursing education
by the American Nurses Credentialing Center’s
Commission on Accreditation.
Congenital Hypoglycemia Disorders: Hyperinsulinism and GSD
April 14 and 15, 2016 – Registration Deadline: Friday, April 1
Name ____________________________ Credentials _____________
Hospital/Professional Affiliation _______________________________
Home Address ____________________________________________
City ____________________________ State _____ ZIP __________
Phone ________________________ Fax _______________________
Email ___________________________________________________
Conference Registration Fees
q M.D.s: $350	 q Non-M.D.s and Physicians-in-training: $250
q Reception and Dinner, Thursday, April 14: $75
Total Registration Fee: $____________
Registration will not be processed without full payment. Faxed registrations
will be accepted for credit card payments only. Please contact Micah Holliday
(hollidaydm@email.chop.edu) if you do not receive a confirmation letter.
Method of Payment
q Personal check		 q Institutional check
(Please make check payable to The Children’s Hospital of Philadelphia.)
q Discover q MasterCard q American Express q VISA
Credit Card # _____________________________________________
Exp. Date ________________________________________________
Mail/fax the completed registration form and payment to:
Ms. Micah Holliday, Continuing Medical Education Department
The Children’s Hospital of Philadelphia
34th Street and Civic Center Boulevard
Philadelphia, PA 19104
215-590-5CME • Fax 215-590-4342 • hollidaydm@email.chop.edu
The deadline for abstract submission is Friday, Jan. 8, 2016.
continued on back page
Thursday, April 14, 2016 Friday, April 15, 2016
Session 1: Congenital Hyperinsulinism (CHI)
8 – 10 a.m.
8 a.m.		 Welcome and Introductions				
Diva D. De León, M.D., M.S.C.E.
8:15 a.m.	 History of Hyperinsulinism (HI) 				
in Pediatrics and Overview of 				
Diagnostic/Therapeutic Algorithms 				
Charles A. Stanley, M.D.
8:30 a.m.	 Diazoxide-responsive Hyperinsulinism 				
a.	GDH-HI				
b.	KATP-HI				
c.	Perinatal Stress HI				
d.	HNFs-HI				
Charles A. Stanley, M.D.
9 a.m.		 Diazoxide-unresponsive Hyperinsulinism				
a.	KATP-HI: dominant vs. recessive,					
focal vs. diffuse				
b.	Atypical – LINE				
c.	GCK				
Diva D. De León, M.D., M.S.C.E.
9:30 a.m.	 Syndromic Hyperinsulinism				
BWS – Jennifer Kalish, M.D., Ph.D.				
Kabuki Syndrome – Jean Baptiste Arnoux,					
M.D., M.Sc.	
10 a.m.		 Break and Exhibits
Session 2: Other Causes of Hyperinsulinemic Hypoglycemia
10:30 – 11:20 a.m. 	
10:30 a.m.	Short Communications (10 minutes each)				
UCP2 Hyperinsulinism 				
Christine Ferrara, M.D., Ph.D.				
PGM1 Hyperinsulinism 				
Amanda Ackerman, M.D., Ph.D.				
Hyperinsulinism in Turner Syndrome 				
Christopher Gibson, M.D.	
11 a.m.		 Insulinomas and HI Mimickers 				
Andrew Palladino, M.D.
11:20 a.m.	Lunch — Exhibits and Scientific Posters
Session 3: Diagnosis and Treatment of Hyperinsulinism
12:30 – 2:20 p.m.
12:30 p.m.	Approach to the Diagnosis and 				
Management of Hyperinsulinism				
Diva D. De León, M.D., M.S.C.E.
12:50 p.m.	Genotype/Phenotype Correlation in 				
KATP Hyperinsulinism				
Arupa Ganguly, Ph.D.
1:05 p.m. 	 The Role of Genetic Testing for 				
Hyperinsulinism: New Frontiers in 				
Genetic Diagnosis				
Sian Ellard, Ph.D., F.R.C.Path.
1:25 p.m. 	 Surgical vs. Nonsurgical Decisions and 				
Options				
Paul Thornton, M.D.; Khalid Hussain, 					
M.B.Ch.B., M.R.C.P., M.Sc.,					
M.R.C.P.C.H., M.D.
2 p.m.		 Long-acting Somatostatin Analogs				
Jean Baptiste Arnoux, M.D., M.Sc.
2:20 p.m.	 Break, Exhibits and Scientific Posters
Session 4: Investigational Therapies for Hyperinsulinism
2:40 – 4:10 p.m.		
2:40 p.m.	 Investigational Therapies: Exendin-(9-39)				
Diva D. De León, M.D., M.S.C.E.
2:55 p.m.	 Investigational Therapies: Sirolimus 				
Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., 					
M.R.C.P.C.H., M.D.
3:10 p.m.	 Investigational Therapies: 				
Subcutaneous 	Glucagon				
Klaus Mohnike, M.D.
3:25 p.m. 	Investigational Therapies: Insulin 				
Receptor Antibody				
Diva D. De León, M.D., M.S.C.E.
3: 40 p.m.	 Abstract Presentations (3 – 10 min. each)
Session 5: Surgical Management Approaches to
Hyperinsulinism
4:10 – 5 p.m.
4:10 p.m.	 [18F]-DOPA PET Imaging of Focal 				
Hyperinsulinism: “GPS” for the Surgeon				
Lisa J. States, M.D.
4:30 p.m.	 Surgery for Hyperinsulinism				
N. Scott Adzick, M.D., M.M.M., F.A.C.S., 					
F.A.A.P.
5 p.m.		 Adjourn
5:30 p.m.	 Symposium Dinner				
Keynote Speaker: Franz M. Matschinsky, M.D.
Session 9: Breakout Sessions — Q&A with the Experts
3:10 – 4 p.m.					
Group A: 				
Management of the Diazoxide-responsive 					
Children				
Group B:				
Management of the Diazoxide-unresponsive 					
Children				
Group C:				
Management of Diabetes after 					
Pancreatectomy				
Group D:				
Management of Children with GSD			
4 p.m.		 Closing Remarks				
Charles A. Stanley, M.D.
4:15 p.m.	 Adjourn
Acknowledgement
The “Congenital Hypoglycemia Disorders:
Hyperinsulinism and Glycogen Storage Disease (GSD)
Symposium” is supported by an educational grant
provided by the Orphan Disease Center at the University
of Pennsylvania. The Congenital Hyperinsulinism Center
at The Children’s Hospital of Philadelphia gratefully
acknowledge this support.
Session 6: Prevention of Brain Damage
8 – 9:40 a.m.
8 a.m.		 Hypoglycemia Guidelines				
Paul Thornton, M.D.
8:20 a.m.	 Neurodevelopmental Outcomes				
Diva D. De León, M.D., M.S.C.E.
8:50 a.m.	 Management after Pancreatectomy:				
Persistent Hypoglycemia and Hyperglycemia				
David Langdon, M.D., F.A.A.P.
9:10 a.m.	 Abstract Presentations (3 – 10 min. each)	
9:40 a.m.	 Break, Exhibits and Scientific Posters
Session 7: Islet Pathophysiology in Hyperinsulinism
10 – 11:30 a.m.
10 a.m.		 ß-cell KATP Channels in Health and Disease				
Show-Ling Shyng, Ph.D.
10:20 a.m. 	Insulin Secretion and Fuel Metabolism in 				
Human HI Islets				
Changhong Li, M.D., Ph.D.
10:40 a.m.	Beyond the ß-cell				
Mark Dunne, Ph.D.
11 a.m. 		 Abstract Presentations (3 – 10 min. each)
11:30 a.m. 	Lunch — Exhibits and Scientific Posters
Session 8: Hyperketotic Hypoglycemia
1 – 2:50 p.m.
1 p.m. 		 Overview of Ketotic Hypoglycemia 				
Disorders (Glycogenosis, MCT1)				
Joseph Wolfsdorf, M.D., B.Ch.
1:30 p.m.	 Natural History of GSD				
David Weinstein, M.D., M.M.Sc.
2 p.m.		 Current and Investigational Therapies 				
for GSD				
David Weinstein, M.D., M.M.Sc.
2:30 p.m.	 PGM1 in the Regulation of Glucose 				
Metabolism and Insulin Secretion				
Eva Morava-Kozicz, M.D., Ph.D.
2:50 p.m.	 Break, Exhibits and Scientific Posters	
©2015 The Children’s Hospital of Philadelphia, All Rights Reserved. 15CME0014/2.8M/09-15
Please note: This meeting will be followed by
a two-day conference for patients, parents and
families of children with HI on Saturday, April 16
and Sunday, April 17. Co-sponsored by Congenital
Hyperinsulinism International (CHI), more
information will be available on CHI’s website,
www.congenitalHI.org, and will also be posted on
our Web page, hyperinsulinism.chop.edu. Please
encourage families of any patients you follow to
consider joining us. Professional staff are also
welcome to join us for this more informal
informational exchange.
General InformationProgram Registration
Registeronlineatwww.chop.edu/cme
Program Overview
This two-day international symposium will be
an update on recent exciting advances in research
related to congenital hyperinsulinism and other
inherited hypoglycemia disorders, particularly
disorders of glycogenoses. The symposium is
intended to enhance the clinical management skills
and practices of endocrinologists, metabolism
specialists, neonatologists, surgeons, pathologists,
radiologists, geneticists and nurses, as well as basic
scientists interested in pancreatic islet biology and
regulation of glucose metabolism. The symposium
faculty, chosen from 10 institutions in the United
States, France, Great Britain, and Germany, will
present 27 plenary lectures on all aspects of these
disorders. Participants may register via mail or fax
by completing the Registration Form attached to the
brochure and indicating their form of payment. To
register online go to www.chop.edu/cme.
General Information
The symposium will be held on Thursday, April 14
and Friday, April 15, 2016, at the Ruth and Tristram
Colket, Jr. Translational Research Building, located
at 3614 Civic Center Blvd. on the campus of The
Children’s Hospital of Philadelphia (www.chop.edu).
Registration will begin each day at 7:30 a.m. The
registration fee includes continental breakfast, lunch,
breaks, parking and syllabus materials. Participants
are invited to attend a reception and dinner on
Thursday, April 14 for an additional fee of $75.
If you wish to attend, please check this option on
the registration form and add this fee to your total
registration fee.
Learning Objectives
Upon completion of this live activity, participants
should be able to:
•	 Provide an overview of the clinical manifestations,
genetics and natural history of congenital
hyperinsulinism and disorders of glycogenoses
•	 Review current knowledge of the normal
regulation of insulin secretion and the
pathophysiology of disorders causing
hyperinsulinism
•	 Discuss recent advances and current controversies
in the surgical and medical management of diffuse
and focal hyperinsulinism
Abstract Submissions
The program includes three sessions at which
attendees may present posters and short oral
communications of their own clinical or laboratory
research. Abstracts must be submitted electronically
using the registration form on the Congenital
Hyperinsulinism Symposium website
(www.chop.edu/cme). The deadline for abstract
submission is Friday, Jan. 8, 2016. Authors will
be notified of abstracts accepted for poster or oral
presentations by Monday, Feb. 1, 2016. A limited
number of travel grants will be available to young
investigators wishing to apply. Young investigators
must apply at the time they register for the
symposium and submit their abstract.
ACCME Accreditation Statement
The Children’s Hospital of Philadelphia is accredited
by the Accreditation Council for Continuing
Medical Education (ACCME) to provide continuing
medical education for physicians.
AMA Credit Designation Statement
The Children’s Hospital of Philadelphia designates
this live activity for a maximum of 13.0 AMA PRA
Category 1 Credit(s)™
. Physicians should claim only
the credit commensurate with their participation in
the activity.
Nursing Accreditation
This activity has been submitted to the PA State
Nurses Association for approval to award contact
hours. The PA State Nurses Association is accredited
as an approver of continuing nursing education
by the American Nurses Credentialing Center’s
Commission on Accreditation.
Congenital Hypoglycemia Disorders: Hyperinsulinism and GSD
April 14 and 15, 2016 – Registration Deadline: Friday, April 1
Name ____________________________ Credentials _____________
Hospital/Professional Affiliation _______________________________
Home Address ____________________________________________
City ____________________________ State _____ ZIP __________
Phone ________________________ Fax _______________________
Email ___________________________________________________
Conference Registration Fees
q M.D.s: $350	 q Non-M.D.s and Physicians-in-training: $250
q Reception and Dinner, Thursday, April 14: $75
Total Registration Fee: $____________
Registration will not be processed without full payment. Faxed registrations
will be accepted for credit card payments only. Please contact Micah Holliday
(hollidaydm@email.chop.edu) if you do not receive a confirmation letter.
Method of Payment
q Personal check		 q Institutional check
(Please make check payable to The Children’s Hospital of Philadelphia.)
q Discover q MasterCard q American Express q VISA
Credit Card # _____________________________________________
Exp. Date ________________________________________________
Mail/fax the completed registration form and payment to:
Ms. Micah Holliday, Continuing Medical Education Department
The Children’s Hospital of Philadelphia
34th Street and Civic Center Boulevard
Philadelphia, PA 19104
215-590-5CME • Fax 215-590-4342 • hollidaydm@email.chop.edu
The deadline for abstract submission is Friday, Jan. 8, 2016.
continued on back page
Thursday, April 14, 2016 Friday, April 15, 2016
Session 1: Congenital Hyperinsulinism (CHI)
8 – 10 a.m.
8 a.m.		 Welcome and Introductions				
Diva D. De León, M.D., M.S.C.E.
8:15 a.m.	 History of Hyperinsulinism (HI) 				
in Pediatrics and Overview of 				
Diagnostic/Therapeutic Algorithms 				
Charles A. Stanley, M.D.
8:30 a.m.	 Diazoxide-responsive Hyperinsulinism 				
a.	GDH-HI				
b.	KATP-HI				
c.	Perinatal Stress HI				
d.	HNFs-HI				
Charles A. Stanley, M.D.
9 a.m.		 Diazoxide-unresponsive Hyperinsulinism				
a.	KATP-HI: dominant vs. recessive,					
focal vs. diffuse				
b.	Atypical – LINE				
c.	GCK				
Diva D. De León, M.D., M.S.C.E.
9:30 a.m.	 Syndromic Hyperinsulinism				
BWS – Jennifer Kalish, M.D., Ph.D.				
Kabuki Syndrome – Jean Baptiste Arnoux,					
M.D., M.Sc.	
10 a.m.		 Break and Exhibits
Session 2: Other Causes of Hyperinsulinemic Hypoglycemia
10:30 – 11:20 a.m. 	
10:30 a.m.	Short Communications (10 minutes each)				
UCP2 Hyperinsulinism 				
Christine Ferrara, M.D., Ph.D.				
PGM1 Hyperinsulinism 				
Amanda Ackerman, M.D., Ph.D.				
Hyperinsulinism in Turner Syndrome 				
Christopher Gibson, M.D.	
11 a.m.		 Insulinomas and HI Mimickers 				
Andrew Palladino, M.D.
11:20 a.m.	Lunch — Exhibits and Scientific Posters
Session 3: Diagnosis and Treatment of Hyperinsulinism
12:30 – 2:20 p.m.
12:30 p.m.	Approach to the Diagnosis and 				
Management of Hyperinsulinism				
Diva D. De León, M.D., M.S.C.E.
12:50 p.m.	Genotype/Phenotype Correlation in 				
KATP Hyperinsulinism				
Arupa Ganguly, Ph.D.
1:05 p.m. 	 The Role of Genetic Testing for 				
Hyperinsulinism: New Frontiers in 				
Genetic Diagnosis				
Sian Ellard, Ph.D., F.R.C.Path.
1:25 p.m. 	 Surgical vs. Nonsurgical Decisions and 				
Options				
Paul Thornton, M.D.; Khalid Hussain, 					
M.B.Ch.B., M.R.C.P., M.Sc.,					
M.R.C.P.C.H., M.D.
2 p.m.		 Long-acting Somatostatin Analogs				
Jean Baptiste Arnoux, M.D., M.Sc.
2:20 p.m.	 Break, Exhibits and Scientific Posters
Session 4: Investigational Therapies for Hyperinsulinism
2:40 – 4:10 p.m.		
2:40 p.m.	 Investigational Therapies: Exendin-(9-39)				
Diva D. De León, M.D., M.S.C.E.
2:55 p.m.	 Investigational Therapies: Sirolimus 				
Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., 					
M.R.C.P.C.H., M.D.
3:10 p.m.	 Investigational Therapies: 				
Subcutaneous 	Glucagon				
Klaus Mohnike, M.D.
3:25 p.m. 	Investigational Therapies: Insulin 				
Receptor Antibody				
Diva D. De León, M.D., M.S.C.E.
3: 40 p.m.	 Abstract Presentations (3 – 10 min. each)
Session 5: Surgical Management Approaches to
Hyperinsulinism
4:10 – 5 p.m.
4:10 p.m.	 [18F]-DOPA PET Imaging of Focal 				
Hyperinsulinism: “GPS” for the Surgeon				
Lisa J. States, M.D.
4:30 p.m.	 Surgery for Hyperinsulinism				
N. Scott Adzick, M.D., M.M.M., F.A.C.S., 					
F.A.A.P.
5 p.m.		 Adjourn
5:30 p.m.	 Symposium Dinner				
Keynote Speaker: Franz M. Matschinsky, M.D.
Session 9: Breakout Sessions — Q&A with the Experts
3:10 – 4 p.m.					
Group A: 				
Management of the Diazoxide-responsive 					
Children				
Group B:				
Management of the Diazoxide-unresponsive 					
Children				
Group C:				
Management of Diabetes after 					
Pancreatectomy				
Group D:				
Management of Children with GSD			
4 p.m.		 Closing Remarks				
Charles A. Stanley, M.D.
4:15 p.m.	 Adjourn
Acknowledgement
The “Congenital Hypoglycemia Disorders:
Hyperinsulinism and Glycogen Storage Disease (GSD)
Symposium” is supported by an educational grant
provided by the Orphan Disease Center at the University
of Pennsylvania. The Congenital Hyperinsulinism Center
at The Children’s Hospital of Philadelphia gratefully
acknowledge this support.
Session 6: Prevention of Brain Damage
8 – 9:40 a.m.
8 a.m.		 Hypoglycemia Guidelines				
Paul Thornton, M.D.
8:20 a.m.	 Neurodevelopmental Outcomes				
Diva D. De León, M.D., M.S.C.E.
8:50 a.m.	 Management after Pancreatectomy:				
Persistent Hypoglycemia and Hyperglycemia				
David Langdon, M.D., F.A.A.P.
9:10 a.m.	 Abstract Presentations (3 – 10 min. each)	
9:40 a.m.	 Break, Exhibits and Scientific Posters
Session 7: Islet Pathophysiology in Hyperinsulinism
10 – 11:30 a.m.
10 a.m.		 ß-cell KATP Channels in Health and Disease				
Show-Ling Shyng, Ph.D.
10:20 a.m. 	Insulin Secretion and Fuel Metabolism in 				
Human HI Islets				
Changhong Li, M.D., Ph.D.
10:40 a.m.	Beyond the ß-cell				
Mark Dunne, Ph.D.
11 a.m. 		 Abstract Presentations (3 – 10 min. each)
11:30 a.m. 	Lunch — Exhibits and Scientific Posters
Session 8: Hyperketotic Hypoglycemia
1 – 2:50 p.m.
1 p.m. 		 Overview of Ketotic Hypoglycemia 				
Disorders (Glycogenosis, MCT1)				
Joseph Wolfsdorf, M.D., B.Ch.
1:30 p.m.	 Natural History of GSD				
David Weinstein, M.D., M.M.Sc.
2 p.m.		 Current and Investigational Therapies 				
for GSD				
David Weinstein, M.D., M.M.Sc.
2:30 p.m.	 PGM1 in the Regulation of Glucose 				
Metabolism and Insulin Secretion				
Eva Morava-Kozicz, M.D., Ph.D.
2:50 p.m.	 Break, Exhibits and Scientific Posters	
©2015 The Children’s Hospital of Philadelphia, All Rights Reserved. 15CME0014/2.8M/09-15
Please note: This meeting will be followed by
a two-day conference for patients, parents and
families of children with HI on Saturday, April 16
and Sunday, April 17. Co-sponsored by Congenital
Hyperinsulinism International (CHI), more
information will be available on CHI’s website,
www.congenitalHI.org, and will also be posted on
our Web page, hyperinsulinism.chop.edu. Please
encourage families of any patients you follow to
consider joining us. Professional staff are also
welcome to join us for this more informal
informational exchange.
ContinuingMedicalEducationDepartment
Course Faculty
Symposium Course Directors
Diva D. De León, M.D., M.S.C.E.
Director, Congenital Hyperinsulinism Center
Associate Professor of Pediatrics
Charles A. Stanley, M.D.
Founder, Congenital Hyperinsulinism Center
Emeritus Professor of Pediatrics
Faculty
N. Scott Adzick, M.D., M.M.M., F.A.C.S.,	
F.A.A.P.
Surgeon-in-Chief
C. Everett Koop Professor of Pediatric Surgery
Arupa Ganguly, Ph.D.
Director, Genetic Diagnostic Laboratory
Hospital of the University of Pennsylvania
Associate Professor, Department of Genetics
Jennifer Kalish, M.D., Ph.D.
Attending Physician
Division of Human Genetics and Metabolism
Instructor of Pediatrics
David Langdon, M.D., F.A.A.P.
Director, Inpatient Endocrine Service
Clinical Associate Professor
Changhong Li, M.D., Ph.D.
Division of Endocrinology and Diabetes
Research Assistant Professor of Pediatrics
Franz M. Matschinsky, M.D.
Benjamin Rush Professor of Biochemistry and
	Biophysics
Lisa J. States, M.D.
Director of Outpatient Satellite Imaging
Department of Radiology
Associate Professor of Radiology
Guest Faculty
Jean Baptiste Arnoux, M.D., M.Sc.
Senior Consultant in Inherited Metabolic
	 Disorders and Congenital Hyperinsulinism
Hôpital Universitaire Necker
Paris, France
Mark Dunne, Ph.D.
Professor
The University of Manchester
Manchester, England
Sian Ellard, Ph.D., F.R.C.Path.
Head of Molecular Genetics, Royal Devon &
	 Exeter NHS Foundation Trust
Professor of Human Molecular Genetics
Devon, England
Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., 	
M.R.C.P.C.H., M.D.
Consultant Paediatric Endocrinologist
Great Ormond Street Hospital for Children
London, England
Klaus Mohnike, M.D.
Professor
Otto-von-Guericke Universität
Magdeburg, Germany
Eva Morava-Kozicz, M.D., Ph.D.
Clinical Biochemical Geneticist

Professor of Pediatrics
Tulane University School
of Medicine
Hayward Genetics Center
New Orleans, La.
Andrew Palladino, M.D.
Clinical Director
Endocrine Therapeutics
Pfizer
Collegeville, Pa.
Show-Ling Shyng, Ph.D.
Professor
Department of Biochemistry and Molecular
	Biology
Oregon Health and Science University
Portland, Ore.
Paul Thornton, M.D.
Director, Congenital Hyperinsulinism Center
Cook Children’s Medical Center
Fort Worth, Texas
David Weinstein, M.D., M.M.Sc.
Professor

Director, Glycogen Storage Disease Program

University of Florida
Gainesville, Fla.
Joseph Wolfsdorf, M.D., B.Ch.
Associate Chief, Division of Endocrinology
Clinical Director, Division of Endocrinology
Professor of Pediatrics
Boston Children’s Hospital
Boston, Mass.
Hotel Information
The Children’s Hospital of Philadelphia has
reserved blocks of rooms at the Sheraton
University City at 3549 Chestnut St., in
Philadelphia. The Sheraton University City
(www.philadelphiasheraton.com) is located
in the heart of the University of Pennsylvania
campus, adjacent to Philadelphia’s prestigious
medical, education and corporate centers, and
just blocks away from the Colket Building.
The group overnight room rate is $179 single
and double occupancy plus a 15.5 percent
room tax. Please contact the hotel directly at
215-387-8000 to make your reservation. When
making your reservation at the hotel, please
mention the Congenital Hyperinsulinism
Symposium. For a complete listing of lodging
in Philadelphia, please visit the Philadelphia
Convention and Visitor’s Bureau website at
www.pcvb.org.
Cancellation and Refund Policy
The Children’s Hospital of Philadelphia
reserves the right to cancel or postpone
any conference due to any unforeseen
circumstances. In the event of cancellation or
postponement, Children’s Hospital will refund
any registration fees, less a $30 service charge,
but is not responsible for any related costs,
charges or expenses to participants, including
cancellation charges assessed by airlines/travel
agencies. In order to process refunds for course
withdrawals, cancellation must be received in
writing by Friday, April 1. No refunds will be
issued thereafter.
Services for People with Disabilities
If arrangements are required for an individual
with a disability to attend this meeting, please
contact the Continuing Medical Education
Department at 215-590-5263.
General
Info continued Congenital Hypoglycemia Disorders:
Hyperinsulinism and GSD
April 14 and 15, 2016
Ruth and Tristram Colket, Jr.
Translational Research
Building at
The Children’s Hospital
of Philadelphia
Sponsored by The Congenital
Hyperinsulinism Center
Division of Endocrinology and Diabetes
www.chop.edu/cme
Unless otherwise noted, faculty is from The Children’s Hospital of Philadelphia
and the Perelman School of Medicine at the University of Pennsylvania.
ADDRESSCORRECTIONREQUESTED
ContinuingMedicalEducationDepartment
Course Faculty
Symposium Course Directors
Diva D. De León, M.D., M.S.C.E.
Director, Congenital Hyperinsulinism Center
Associate Professor of Pediatrics
Charles A. Stanley, M.D.
Founder, Congenital Hyperinsulinism Center
Emeritus Professor of Pediatrics
Faculty
N. Scott Adzick, M.D., M.M.M., F.A.C.S.,	
F.A.A.P.
Surgeon-in-Chief
C. Everett Koop Professor of Pediatric Surgery
Arupa Ganguly, Ph.D.
Director, Genetic Diagnostic Laboratory
Hospital of the University of Pennsylvania
Associate Professor, Department of Genetics
Jennifer Kalish, M.D., Ph.D.
Attending Physician
Division of Human Genetics and Metabolism
Instructor of Pediatrics
David Langdon, M.D., F.A.A.P.
Director, Inpatient Endocrine Service
Clinical Associate Professor
Changhong Li, M.D., Ph.D.
Division of Endocrinology and Diabetes
Research Assistant Professor of Pediatrics
Franz M. Matschinsky, M.D.
Benjamin Rush Professor of Biochemistry and
	Biophysics
Lisa J. States, M.D.
Director of Outpatient Satellite Imaging
Department of Radiology
Associate Professor of Radiology
Guest Faculty
Jean Baptiste Arnoux, M.D., M.Sc.
Senior Consultant in Inherited Metabolic
	 Disorders and Congenital Hyperinsulinism
Hôpital Universitaire Necker
Paris, France
Mark Dunne, Ph.D.
Professor
The University of Manchester
Manchester, England
Sian Ellard, Ph.D., F.R.C.Path.
Head of Molecular Genetics, Royal Devon &
	 Exeter NHS Foundation Trust
Professor of Human Molecular Genetics
Devon, England
Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., 	
M.R.C.P.C.H., M.D.
Consultant Paediatric Endocrinologist
Great Ormond Street Hospital for Children
London, England
Klaus Mohnike, M.D.
Professor
Otto-von-Guericke Universität
Magdeburg, Germany
Eva Morava-Kozicz, M.D., Ph.D.
Clinical Biochemical Geneticist

Professor of Pediatrics
Tulane University School
of Medicine
Hayward Genetics Center
New Orleans, La.
Andrew Palladino, M.D.
Clinical Director
Endocrine Therapeutics
Pfizer
Collegeville, Pa.
Show-Ling Shyng, Ph.D.
Professor
Department of Biochemistry and Molecular
	Biology
Oregon Health and Science University
Portland, Ore.
Paul Thornton, M.D.
Director, Congenital Hyperinsulinism Center
Cook Children’s Medical Center
Fort Worth, Texas
David Weinstein, M.D., M.M.Sc.
Professor

Director, Glycogen Storage Disease Program

University of Florida
Gainesville, Fla.
Joseph Wolfsdorf, M.D., B.Ch.
Associate Chief, Division of Endocrinology
Clinical Director, Division of Endocrinology
Professor of Pediatrics
Boston Children’s Hospital
Boston, Mass.
Hotel Information
The Children’s Hospital of Philadelphia has
reserved blocks of rooms at the Sheraton
University City at 3549 Chestnut St., in
Philadelphia. The Sheraton University City
(www.philadelphiasheraton.com) is located
in the heart of the University of Pennsylvania
campus, adjacent to Philadelphia’s prestigious
medical, education and corporate centers, and
just blocks away from the Colket Building.
The group overnight room rate is $179 single
and double occupancy plus a 15.5 percent
room tax. Please contact the hotel directly at
215-387-8000 to make your reservation. When
making your reservation at the hotel, please
mention the Congenital Hyperinsulinism
Symposium. For a complete listing of lodging
in Philadelphia, please visit the Philadelphia
Convention and Visitor’s Bureau website at
www.pcvb.org.
Cancellation and Refund Policy
The Children’s Hospital of Philadelphia
reserves the right to cancel or postpone
any conference due to any unforeseen
circumstances. In the event of cancellation or
postponement, Children’s Hospital will refund
any registration fees, less a $30 service charge,
but is not responsible for any related costs,
charges or expenses to participants, including
cancellation charges assessed by airlines/travel
agencies. In order to process refunds for course
withdrawals, cancellation must be received in
writing by Friday, April 1. No refunds will be
issued thereafter.
Services for People with Disabilities
If arrangements are required for an individual
with a disability to attend this meeting, please
contact the Continuing Medical Education
Department at 215-590-5263.
General
Info continued Congenital Hypoglycemia Disorders:
Hyperinsulinism and GSD
April 14 and 15, 2016
Ruth and Tristram Colket, Jr.
Translational Research
Building at
The Children’s Hospital
of Philadelphia
Sponsored by The Congenital
Hyperinsulinism Center
Division of Endocrinology and Diabetes
www.chop.edu/cme
Unless otherwise noted, faculty is from The Children’s Hospital of Philadelphia
and the Perelman School of Medicine at the University of Pennsylvania.
ADDRESSCORRECTIONREQUESTED

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Simposio de Desórdenes de Hipoglucemia Congénita: Hiperinsulinismo y Desórdenes del Almacenamiento del Glucógeno

  • 1. ContinuingMedicalEducationDepartment Course Faculty Symposium Course Directors Diva D. De León, M.D., M.S.C.E. Director, Congenital Hyperinsulinism Center Associate Professor of Pediatrics Charles A. Stanley, M.D. Founder, Congenital Hyperinsulinism Center Emeritus Professor of Pediatrics Faculty N. Scott Adzick, M.D., M.M.M., F.A.C.S., F.A.A.P. Surgeon-in-Chief C. Everett Koop Professor of Pediatric Surgery Arupa Ganguly, Ph.D. Director, Genetic Diagnostic Laboratory Hospital of the University of Pennsylvania Associate Professor, Department of Genetics Jennifer Kalish, M.D., Ph.D. Attending Physician Division of Human Genetics and Metabolism Instructor of Pediatrics David Langdon, M.D., F.A.A.P. Director, Inpatient Endocrine Service Clinical Associate Professor Changhong Li, M.D., Ph.D. Division of Endocrinology and Diabetes Research Assistant Professor of Pediatrics Franz M. Matschinsky, M.D. Benjamin Rush Professor of Biochemistry and Biophysics Lisa J. States, M.D. Director of Outpatient Satellite Imaging Department of Radiology Associate Professor of Radiology Guest Faculty Jean Baptiste Arnoux, M.D., M.Sc. Senior Consultant in Inherited Metabolic Disorders and Congenital Hyperinsulinism Hôpital Universitaire Necker Paris, France Mark Dunne, Ph.D. Professor The University of Manchester Manchester, England Sian Ellard, Ph.D., F.R.C.Path. Head of Molecular Genetics, Royal Devon & Exeter NHS Foundation Trust Professor of Human Molecular Genetics Devon, England Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., M.R.C.P.C.H., M.D. Consultant Paediatric Endocrinologist Great Ormond Street Hospital for Children London, England Klaus Mohnike, M.D. Professor Otto-von-Guericke Universität Magdeburg, Germany Eva Morava-Kozicz, M.D., Ph.D. Clinical Biochemical Geneticist
 Professor of Pediatrics Tulane University School
of Medicine Hayward Genetics Center New Orleans, La. Andrew Palladino, M.D. Clinical Director Endocrine Therapeutics Pfizer Collegeville, Pa. Show-Ling Shyng, Ph.D. Professor Department of Biochemistry and Molecular Biology Oregon Health and Science University Portland, Ore. Paul Thornton, M.D. Director, Congenital Hyperinsulinism Center Cook Children’s Medical Center Fort Worth, Texas David Weinstein, M.D., M.M.Sc. Professor
 Director, Glycogen Storage Disease Program
 University of Florida Gainesville, Fla. Joseph Wolfsdorf, M.D., B.Ch. Associate Chief, Division of Endocrinology Clinical Director, Division of Endocrinology Professor of Pediatrics Boston Children’s Hospital Boston, Mass. Hotel Information The Children’s Hospital of Philadelphia has reserved blocks of rooms at the Sheraton University City at 3549 Chestnut St., in Philadelphia. The Sheraton University City (www.philadelphiasheraton.com) is located in the heart of the University of Pennsylvania campus, adjacent to Philadelphia’s prestigious medical, education and corporate centers, and just blocks away from the Colket Building. The group overnight room rate is $179 single and double occupancy plus a 15.5 percent room tax. Please contact the hotel directly at 215-387-8000 to make your reservation. When making your reservation at the hotel, please mention the Congenital Hyperinsulinism Symposium. For a complete listing of lodging in Philadelphia, please visit the Philadelphia Convention and Visitor’s Bureau website at www.pcvb.org. Cancellation and Refund Policy The Children’s Hospital of Philadelphia reserves the right to cancel or postpone any conference due to any unforeseen circumstances. In the event of cancellation or postponement, Children’s Hospital will refund any registration fees, less a $30 service charge, but is not responsible for any related costs, charges or expenses to participants, including cancellation charges assessed by airlines/travel agencies. In order to process refunds for course withdrawals, cancellation must be received in writing by Friday, April 1. No refunds will be issued thereafter. Services for People with Disabilities If arrangements are required for an individual with a disability to attend this meeting, please contact the Continuing Medical Education Department at 215-590-5263. General Info continued Congenital Hypoglycemia Disorders: Hyperinsulinism and GSD April 14 and 15, 2016 Ruth and Tristram Colket, Jr. Translational Research Building at The Children’s Hospital of Philadelphia Sponsored by The Congenital Hyperinsulinism Center Division of Endocrinology and Diabetes www.chop.edu/cme Unless otherwise noted, faculty is from The Children’s Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania. ADDRESSCORRECTIONREQUESTED
  • 2. General InformationProgram Registration Registeronlineatwww.chop.edu/cme Program Overview This two-day international symposium will be an update on recent exciting advances in research related to congenital hyperinsulinism and other inherited hypoglycemia disorders, particularly disorders of glycogenoses. The symposium is intended to enhance the clinical management skills and practices of endocrinologists, metabolism specialists, neonatologists, surgeons, pathologists, radiologists, geneticists and nurses, as well as basic scientists interested in pancreatic islet biology and regulation of glucose metabolism. The symposium faculty, chosen from 10 institutions in the United States, France, Great Britain, and Germany, will present 27 plenary lectures on all aspects of these disorders. Participants may register via mail or fax by completing the Registration Form attached to the brochure and indicating their form of payment. To register online go to www.chop.edu/cme. General Information The symposium will be held on Thursday, April 14 and Friday, April 15, 2016, at the Ruth and Tristram Colket, Jr. Translational Research Building, located at 3614 Civic Center Blvd. on the campus of The Children’s Hospital of Philadelphia (www.chop.edu). Registration will begin each day at 7:30 a.m. The registration fee includes continental breakfast, lunch, breaks, parking and syllabus materials. Participants are invited to attend a reception and dinner on Thursday, April 14 for an additional fee of $75. If you wish to attend, please check this option on the registration form and add this fee to your total registration fee. Learning Objectives Upon completion of this live activity, participants should be able to: • Provide an overview of the clinical manifestations, genetics and natural history of congenital hyperinsulinism and disorders of glycogenoses • Review current knowledge of the normal regulation of insulin secretion and the pathophysiology of disorders causing hyperinsulinism • Discuss recent advances and current controversies in the surgical and medical management of diffuse and focal hyperinsulinism Abstract Submissions The program includes three sessions at which attendees may present posters and short oral communications of their own clinical or laboratory research. Abstracts must be submitted electronically using the registration form on the Congenital Hyperinsulinism Symposium website (www.chop.edu/cme). The deadline for abstract submission is Friday, Jan. 8, 2016. Authors will be notified of abstracts accepted for poster or oral presentations by Monday, Feb. 1, 2016. A limited number of travel grants will be available to young investigators wishing to apply. Young investigators must apply at the time they register for the symposium and submit their abstract. ACCME Accreditation Statement The Children’s Hospital of Philadelphia is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians. AMA Credit Designation Statement The Children’s Hospital of Philadelphia designates this live activity for a maximum of 13.0 AMA PRA Category 1 Credit(s)™ . Physicians should claim only the credit commensurate with their participation in the activity. Nursing Accreditation This activity has been submitted to the PA State Nurses Association for approval to award contact hours. The PA State Nurses Association is accredited as an approver of continuing nursing education by the American Nurses Credentialing Center’s Commission on Accreditation. Congenital Hypoglycemia Disorders: Hyperinsulinism and GSD April 14 and 15, 2016 – Registration Deadline: Friday, April 1 Name ____________________________ Credentials _____________ Hospital/Professional Affiliation _______________________________ Home Address ____________________________________________ City ____________________________ State _____ ZIP __________ Phone ________________________ Fax _______________________ Email ___________________________________________________ Conference Registration Fees q M.D.s: $350 q Non-M.D.s and Physicians-in-training: $250 q Reception and Dinner, Thursday, April 14: $75 Total Registration Fee: $____________ Registration will not be processed without full payment. Faxed registrations will be accepted for credit card payments only. Please contact Micah Holliday (hollidaydm@email.chop.edu) if you do not receive a confirmation letter. Method of Payment q Personal check q Institutional check (Please make check payable to The Children’s Hospital of Philadelphia.) q Discover q MasterCard q American Express q VISA Credit Card # _____________________________________________ Exp. Date ________________________________________________ Mail/fax the completed registration form and payment to: Ms. Micah Holliday, Continuing Medical Education Department The Children’s Hospital of Philadelphia 34th Street and Civic Center Boulevard Philadelphia, PA 19104 215-590-5CME • Fax 215-590-4342 • hollidaydm@email.chop.edu The deadline for abstract submission is Friday, Jan. 8, 2016. continued on back page Thursday, April 14, 2016 Friday, April 15, 2016 Session 1: Congenital Hyperinsulinism (CHI) 8 – 10 a.m. 8 a.m. Welcome and Introductions Diva D. De León, M.D., M.S.C.E. 8:15 a.m. History of Hyperinsulinism (HI) in Pediatrics and Overview of Diagnostic/Therapeutic Algorithms Charles A. Stanley, M.D. 8:30 a.m. Diazoxide-responsive Hyperinsulinism a. GDH-HI b. KATP-HI c. Perinatal Stress HI d. HNFs-HI Charles A. Stanley, M.D. 9 a.m. Diazoxide-unresponsive Hyperinsulinism a. KATP-HI: dominant vs. recessive, focal vs. diffuse b. Atypical – LINE c. GCK Diva D. De León, M.D., M.S.C.E. 9:30 a.m. Syndromic Hyperinsulinism BWS – Jennifer Kalish, M.D., Ph.D. Kabuki Syndrome – Jean Baptiste Arnoux, M.D., M.Sc. 10 a.m. Break and Exhibits Session 2: Other Causes of Hyperinsulinemic Hypoglycemia 10:30 – 11:20 a.m. 10:30 a.m. Short Communications (10 minutes each) UCP2 Hyperinsulinism Christine Ferrara, M.D., Ph.D. PGM1 Hyperinsulinism Amanda Ackerman, M.D., Ph.D. Hyperinsulinism in Turner Syndrome Christopher Gibson, M.D. 11 a.m. Insulinomas and HI Mimickers Andrew Palladino, M.D. 11:20 a.m. Lunch — Exhibits and Scientific Posters Session 3: Diagnosis and Treatment of Hyperinsulinism 12:30 – 2:20 p.m. 12:30 p.m. Approach to the Diagnosis and Management of Hyperinsulinism Diva D. De León, M.D., M.S.C.E. 12:50 p.m. Genotype/Phenotype Correlation in KATP Hyperinsulinism Arupa Ganguly, Ph.D. 1:05 p.m. The Role of Genetic Testing for Hyperinsulinism: New Frontiers in Genetic Diagnosis Sian Ellard, Ph.D., F.R.C.Path. 1:25 p.m. Surgical vs. Nonsurgical Decisions and Options Paul Thornton, M.D.; Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., M.R.C.P.C.H., M.D. 2 p.m. Long-acting Somatostatin Analogs Jean Baptiste Arnoux, M.D., M.Sc. 2:20 p.m. Break, Exhibits and Scientific Posters Session 4: Investigational Therapies for Hyperinsulinism 2:40 – 4:10 p.m. 2:40 p.m. Investigational Therapies: Exendin-(9-39) Diva D. De León, M.D., M.S.C.E. 2:55 p.m. Investigational Therapies: Sirolimus Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., M.R.C.P.C.H., M.D. 3:10 p.m. Investigational Therapies: Subcutaneous Glucagon Klaus Mohnike, M.D. 3:25 p.m. Investigational Therapies: Insulin Receptor Antibody Diva D. De León, M.D., M.S.C.E. 3: 40 p.m. Abstract Presentations (3 – 10 min. each) Session 5: Surgical Management Approaches to Hyperinsulinism 4:10 – 5 p.m. 4:10 p.m. [18F]-DOPA PET Imaging of Focal Hyperinsulinism: “GPS” for the Surgeon Lisa J. States, M.D. 4:30 p.m. Surgery for Hyperinsulinism N. Scott Adzick, M.D., M.M.M., F.A.C.S., F.A.A.P. 5 p.m. Adjourn 5:30 p.m. Symposium Dinner Keynote Speaker: Franz M. Matschinsky, M.D. Session 9: Breakout Sessions — Q&A with the Experts 3:10 – 4 p.m. Group A: Management of the Diazoxide-responsive Children Group B: Management of the Diazoxide-unresponsive Children Group C: Management of Diabetes after Pancreatectomy Group D: Management of Children with GSD 4 p.m. Closing Remarks Charles A. Stanley, M.D. 4:15 p.m. Adjourn Acknowledgement The “Congenital Hypoglycemia Disorders: Hyperinsulinism and Glycogen Storage Disease (GSD) Symposium” is supported by an educational grant provided by the Orphan Disease Center at the University of Pennsylvania. The Congenital Hyperinsulinism Center at The Children’s Hospital of Philadelphia gratefully acknowledge this support. Session 6: Prevention of Brain Damage 8 – 9:40 a.m. 8 a.m. Hypoglycemia Guidelines Paul Thornton, M.D. 8:20 a.m. Neurodevelopmental Outcomes Diva D. De León, M.D., M.S.C.E. 8:50 a.m. Management after Pancreatectomy: Persistent Hypoglycemia and Hyperglycemia David Langdon, M.D., F.A.A.P. 9:10 a.m. Abstract Presentations (3 – 10 min. each) 9:40 a.m. Break, Exhibits and Scientific Posters Session 7: Islet Pathophysiology in Hyperinsulinism 10 – 11:30 a.m. 10 a.m. ß-cell KATP Channels in Health and Disease Show-Ling Shyng, Ph.D. 10:20 a.m. Insulin Secretion and Fuel Metabolism in Human HI Islets Changhong Li, M.D., Ph.D. 10:40 a.m. Beyond the ß-cell Mark Dunne, Ph.D. 11 a.m. Abstract Presentations (3 – 10 min. each) 11:30 a.m. Lunch — Exhibits and Scientific Posters Session 8: Hyperketotic Hypoglycemia 1 – 2:50 p.m. 1 p.m. Overview of Ketotic Hypoglycemia Disorders (Glycogenosis, MCT1) Joseph Wolfsdorf, M.D., B.Ch. 1:30 p.m. Natural History of GSD David Weinstein, M.D., M.M.Sc. 2 p.m. Current and Investigational Therapies for GSD David Weinstein, M.D., M.M.Sc. 2:30 p.m. PGM1 in the Regulation of Glucose Metabolism and Insulin Secretion Eva Morava-Kozicz, M.D., Ph.D. 2:50 p.m. Break, Exhibits and Scientific Posters ©2015 The Children’s Hospital of Philadelphia, All Rights Reserved. 15CME0014/2.8M/09-15 Please note: This meeting will be followed by a two-day conference for patients, parents and families of children with HI on Saturday, April 16 and Sunday, April 17. Co-sponsored by Congenital Hyperinsulinism International (CHI), more information will be available on CHI’s website, www.congenitalHI.org, and will also be posted on our Web page, hyperinsulinism.chop.edu. Please encourage families of any patients you follow to consider joining us. Professional staff are also welcome to join us for this more informal informational exchange.
  • 3. General InformationProgram Registration Registeronlineatwww.chop.edu/cme Program Overview This two-day international symposium will be an update on recent exciting advances in research related to congenital hyperinsulinism and other inherited hypoglycemia disorders, particularly disorders of glycogenoses. The symposium is intended to enhance the clinical management skills and practices of endocrinologists, metabolism specialists, neonatologists, surgeons, pathologists, radiologists, geneticists and nurses, as well as basic scientists interested in pancreatic islet biology and regulation of glucose metabolism. The symposium faculty, chosen from 10 institutions in the United States, France, Great Britain, and Germany, will present 27 plenary lectures on all aspects of these disorders. Participants may register via mail or fax by completing the Registration Form attached to the brochure and indicating their form of payment. To register online go to www.chop.edu/cme. General Information The symposium will be held on Thursday, April 14 and Friday, April 15, 2016, at the Ruth and Tristram Colket, Jr. Translational Research Building, located at 3614 Civic Center Blvd. on the campus of The Children’s Hospital of Philadelphia (www.chop.edu). Registration will begin each day at 7:30 a.m. The registration fee includes continental breakfast, lunch, breaks, parking and syllabus materials. Participants are invited to attend a reception and dinner on Thursday, April 14 for an additional fee of $75. If you wish to attend, please check this option on the registration form and add this fee to your total registration fee. Learning Objectives Upon completion of this live activity, participants should be able to: • Provide an overview of the clinical manifestations, genetics and natural history of congenital hyperinsulinism and disorders of glycogenoses • Review current knowledge of the normal regulation of insulin secretion and the pathophysiology of disorders causing hyperinsulinism • Discuss recent advances and current controversies in the surgical and medical management of diffuse and focal hyperinsulinism Abstract Submissions The program includes three sessions at which attendees may present posters and short oral communications of their own clinical or laboratory research. Abstracts must be submitted electronically using the registration form on the Congenital Hyperinsulinism Symposium website (www.chop.edu/cme). The deadline for abstract submission is Friday, Jan. 8, 2016. Authors will be notified of abstracts accepted for poster or oral presentations by Monday, Feb. 1, 2016. A limited number of travel grants will be available to young investigators wishing to apply. Young investigators must apply at the time they register for the symposium and submit their abstract. ACCME Accreditation Statement The Children’s Hospital of Philadelphia is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians. AMA Credit Designation Statement The Children’s Hospital of Philadelphia designates this live activity for a maximum of 13.0 AMA PRA Category 1 Credit(s)™ . Physicians should claim only the credit commensurate with their participation in the activity. Nursing Accreditation This activity has been submitted to the PA State Nurses Association for approval to award contact hours. The PA State Nurses Association is accredited as an approver of continuing nursing education by the American Nurses Credentialing Center’s Commission on Accreditation. Congenital Hypoglycemia Disorders: Hyperinsulinism and GSD April 14 and 15, 2016 – Registration Deadline: Friday, April 1 Name ____________________________ Credentials _____________ Hospital/Professional Affiliation _______________________________ Home Address ____________________________________________ City ____________________________ State _____ ZIP __________ Phone ________________________ Fax _______________________ Email ___________________________________________________ Conference Registration Fees q M.D.s: $350 q Non-M.D.s and Physicians-in-training: $250 q Reception and Dinner, Thursday, April 14: $75 Total Registration Fee: $____________ Registration will not be processed without full payment. Faxed registrations will be accepted for credit card payments only. Please contact Micah Holliday (hollidaydm@email.chop.edu) if you do not receive a confirmation letter. Method of Payment q Personal check q Institutional check (Please make check payable to The Children’s Hospital of Philadelphia.) q Discover q MasterCard q American Express q VISA Credit Card # _____________________________________________ Exp. Date ________________________________________________ Mail/fax the completed registration form and payment to: Ms. Micah Holliday, Continuing Medical Education Department The Children’s Hospital of Philadelphia 34th Street and Civic Center Boulevard Philadelphia, PA 19104 215-590-5CME • Fax 215-590-4342 • hollidaydm@email.chop.edu The deadline for abstract submission is Friday, Jan. 8, 2016. continued on back page Thursday, April 14, 2016 Friday, April 15, 2016 Session 1: Congenital Hyperinsulinism (CHI) 8 – 10 a.m. 8 a.m. Welcome and Introductions Diva D. De León, M.D., M.S.C.E. 8:15 a.m. History of Hyperinsulinism (HI) in Pediatrics and Overview of Diagnostic/Therapeutic Algorithms Charles A. Stanley, M.D. 8:30 a.m. Diazoxide-responsive Hyperinsulinism a. GDH-HI b. KATP-HI c. Perinatal Stress HI d. HNFs-HI Charles A. Stanley, M.D. 9 a.m. Diazoxide-unresponsive Hyperinsulinism a. KATP-HI: dominant vs. recessive, focal vs. diffuse b. Atypical – LINE c. GCK Diva D. De León, M.D., M.S.C.E. 9:30 a.m. Syndromic Hyperinsulinism BWS – Jennifer Kalish, M.D., Ph.D. Kabuki Syndrome – Jean Baptiste Arnoux, M.D., M.Sc. 10 a.m. Break and Exhibits Session 2: Other Causes of Hyperinsulinemic Hypoglycemia 10:30 – 11:20 a.m. 10:30 a.m. Short Communications (10 minutes each) UCP2 Hyperinsulinism Christine Ferrara, M.D., Ph.D. PGM1 Hyperinsulinism Amanda Ackerman, M.D., Ph.D. Hyperinsulinism in Turner Syndrome Christopher Gibson, M.D. 11 a.m. Insulinomas and HI Mimickers Andrew Palladino, M.D. 11:20 a.m. Lunch — Exhibits and Scientific Posters Session 3: Diagnosis and Treatment of Hyperinsulinism 12:30 – 2:20 p.m. 12:30 p.m. Approach to the Diagnosis and Management of Hyperinsulinism Diva D. De León, M.D., M.S.C.E. 12:50 p.m. Genotype/Phenotype Correlation in KATP Hyperinsulinism Arupa Ganguly, Ph.D. 1:05 p.m. The Role of Genetic Testing for Hyperinsulinism: New Frontiers in Genetic Diagnosis Sian Ellard, Ph.D., F.R.C.Path. 1:25 p.m. Surgical vs. Nonsurgical Decisions and Options Paul Thornton, M.D.; Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., M.R.C.P.C.H., M.D. 2 p.m. Long-acting Somatostatin Analogs Jean Baptiste Arnoux, M.D., M.Sc. 2:20 p.m. Break, Exhibits and Scientific Posters Session 4: Investigational Therapies for Hyperinsulinism 2:40 – 4:10 p.m. 2:40 p.m. Investigational Therapies: Exendin-(9-39) Diva D. De León, M.D., M.S.C.E. 2:55 p.m. Investigational Therapies: Sirolimus Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., M.R.C.P.C.H., M.D. 3:10 p.m. Investigational Therapies: Subcutaneous Glucagon Klaus Mohnike, M.D. 3:25 p.m. Investigational Therapies: Insulin Receptor Antibody Diva D. De León, M.D., M.S.C.E. 3: 40 p.m. Abstract Presentations (3 – 10 min. each) Session 5: Surgical Management Approaches to Hyperinsulinism 4:10 – 5 p.m. 4:10 p.m. [18F]-DOPA PET Imaging of Focal Hyperinsulinism: “GPS” for the Surgeon Lisa J. States, M.D. 4:30 p.m. Surgery for Hyperinsulinism N. Scott Adzick, M.D., M.M.M., F.A.C.S., F.A.A.P. 5 p.m. Adjourn 5:30 p.m. Symposium Dinner Keynote Speaker: Franz M. Matschinsky, M.D. Session 9: Breakout Sessions — Q&A with the Experts 3:10 – 4 p.m. Group A: Management of the Diazoxide-responsive Children Group B: Management of the Diazoxide-unresponsive Children Group C: Management of Diabetes after Pancreatectomy Group D: Management of Children with GSD 4 p.m. Closing Remarks Charles A. Stanley, M.D. 4:15 p.m. Adjourn Acknowledgement The “Congenital Hypoglycemia Disorders: Hyperinsulinism and Glycogen Storage Disease (GSD) Symposium” is supported by an educational grant provided by the Orphan Disease Center at the University of Pennsylvania. The Congenital Hyperinsulinism Center at The Children’s Hospital of Philadelphia gratefully acknowledge this support. Session 6: Prevention of Brain Damage 8 – 9:40 a.m. 8 a.m. Hypoglycemia Guidelines Paul Thornton, M.D. 8:20 a.m. Neurodevelopmental Outcomes Diva D. De León, M.D., M.S.C.E. 8:50 a.m. Management after Pancreatectomy: Persistent Hypoglycemia and Hyperglycemia David Langdon, M.D., F.A.A.P. 9:10 a.m. Abstract Presentations (3 – 10 min. each) 9:40 a.m. Break, Exhibits and Scientific Posters Session 7: Islet Pathophysiology in Hyperinsulinism 10 – 11:30 a.m. 10 a.m. ß-cell KATP Channels in Health and Disease Show-Ling Shyng, Ph.D. 10:20 a.m. Insulin Secretion and Fuel Metabolism in Human HI Islets Changhong Li, M.D., Ph.D. 10:40 a.m. Beyond the ß-cell Mark Dunne, Ph.D. 11 a.m. Abstract Presentations (3 – 10 min. each) 11:30 a.m. Lunch — Exhibits and Scientific Posters Session 8: Hyperketotic Hypoglycemia 1 – 2:50 p.m. 1 p.m. Overview of Ketotic Hypoglycemia Disorders (Glycogenosis, MCT1) Joseph Wolfsdorf, M.D., B.Ch. 1:30 p.m. Natural History of GSD David Weinstein, M.D., M.M.Sc. 2 p.m. Current and Investigational Therapies for GSD David Weinstein, M.D., M.M.Sc. 2:30 p.m. PGM1 in the Regulation of Glucose Metabolism and Insulin Secretion Eva Morava-Kozicz, M.D., Ph.D. 2:50 p.m. Break, Exhibits and Scientific Posters ©2015 The Children’s Hospital of Philadelphia, All Rights Reserved. 15CME0014/2.8M/09-15 Please note: This meeting will be followed by a two-day conference for patients, parents and families of children with HI on Saturday, April 16 and Sunday, April 17. Co-sponsored by Congenital Hyperinsulinism International (CHI), more information will be available on CHI’s website, www.congenitalHI.org, and will also be posted on our Web page, hyperinsulinism.chop.edu. Please encourage families of any patients you follow to consider joining us. Professional staff are also welcome to join us for this more informal informational exchange.
  • 4. General InformationProgram Registration Registeronlineatwww.chop.edu/cme Program Overview This two-day international symposium will be an update on recent exciting advances in research related to congenital hyperinsulinism and other inherited hypoglycemia disorders, particularly disorders of glycogenoses. The symposium is intended to enhance the clinical management skills and practices of endocrinologists, metabolism specialists, neonatologists, surgeons, pathologists, radiologists, geneticists and nurses, as well as basic scientists interested in pancreatic islet biology and regulation of glucose metabolism. The symposium faculty, chosen from 10 institutions in the United States, France, Great Britain, and Germany, will present 27 plenary lectures on all aspects of these disorders. Participants may register via mail or fax by completing the Registration Form attached to the brochure and indicating their form of payment. To register online go to www.chop.edu/cme. General Information The symposium will be held on Thursday, April 14 and Friday, April 15, 2016, at the Ruth and Tristram Colket, Jr. Translational Research Building, located at 3614 Civic Center Blvd. on the campus of The Children’s Hospital of Philadelphia (www.chop.edu). Registration will begin each day at 7:30 a.m. The registration fee includes continental breakfast, lunch, breaks, parking and syllabus materials. Participants are invited to attend a reception and dinner on Thursday, April 14 for an additional fee of $75. If you wish to attend, please check this option on the registration form and add this fee to your total registration fee. Learning Objectives Upon completion of this live activity, participants should be able to: • Provide an overview of the clinical manifestations, genetics and natural history of congenital hyperinsulinism and disorders of glycogenoses • Review current knowledge of the normal regulation of insulin secretion and the pathophysiology of disorders causing hyperinsulinism • Discuss recent advances and current controversies in the surgical and medical management of diffuse and focal hyperinsulinism Abstract Submissions The program includes three sessions at which attendees may present posters and short oral communications of their own clinical or laboratory research. Abstracts must be submitted electronically using the registration form on the Congenital Hyperinsulinism Symposium website (www.chop.edu/cme). The deadline for abstract submission is Friday, Jan. 8, 2016. Authors will be notified of abstracts accepted for poster or oral presentations by Monday, Feb. 1, 2016. A limited number of travel grants will be available to young investigators wishing to apply. Young investigators must apply at the time they register for the symposium and submit their abstract. ACCME Accreditation Statement The Children’s Hospital of Philadelphia is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians. AMA Credit Designation Statement The Children’s Hospital of Philadelphia designates this live activity for a maximum of 13.0 AMA PRA Category 1 Credit(s)™ . Physicians should claim only the credit commensurate with their participation in the activity. Nursing Accreditation This activity has been submitted to the PA State Nurses Association for approval to award contact hours. The PA State Nurses Association is accredited as an approver of continuing nursing education by the American Nurses Credentialing Center’s Commission on Accreditation. Congenital Hypoglycemia Disorders: Hyperinsulinism and GSD April 14 and 15, 2016 – Registration Deadline: Friday, April 1 Name ____________________________ Credentials _____________ Hospital/Professional Affiliation _______________________________ Home Address ____________________________________________ City ____________________________ State _____ ZIP __________ Phone ________________________ Fax _______________________ Email ___________________________________________________ Conference Registration Fees q M.D.s: $350 q Non-M.D.s and Physicians-in-training: $250 q Reception and Dinner, Thursday, April 14: $75 Total Registration Fee: $____________ Registration will not be processed without full payment. Faxed registrations will be accepted for credit card payments only. Please contact Micah Holliday (hollidaydm@email.chop.edu) if you do not receive a confirmation letter. Method of Payment q Personal check q Institutional check (Please make check payable to The Children’s Hospital of Philadelphia.) q Discover q MasterCard q American Express q VISA Credit Card # _____________________________________________ Exp. Date ________________________________________________ Mail/fax the completed registration form and payment to: Ms. Micah Holliday, Continuing Medical Education Department The Children’s Hospital of Philadelphia 34th Street and Civic Center Boulevard Philadelphia, PA 19104 215-590-5CME • Fax 215-590-4342 • hollidaydm@email.chop.edu The deadline for abstract submission is Friday, Jan. 8, 2016. continued on back page Thursday, April 14, 2016 Friday, April 15, 2016 Session 1: Congenital Hyperinsulinism (CHI) 8 – 10 a.m. 8 a.m. Welcome and Introductions Diva D. De León, M.D., M.S.C.E. 8:15 a.m. History of Hyperinsulinism (HI) in Pediatrics and Overview of Diagnostic/Therapeutic Algorithms Charles A. Stanley, M.D. 8:30 a.m. Diazoxide-responsive Hyperinsulinism a. GDH-HI b. KATP-HI c. Perinatal Stress HI d. HNFs-HI Charles A. Stanley, M.D. 9 a.m. Diazoxide-unresponsive Hyperinsulinism a. KATP-HI: dominant vs. recessive, focal vs. diffuse b. Atypical – LINE c. GCK Diva D. De León, M.D., M.S.C.E. 9:30 a.m. Syndromic Hyperinsulinism BWS – Jennifer Kalish, M.D., Ph.D. Kabuki Syndrome – Jean Baptiste Arnoux, M.D., M.Sc. 10 a.m. Break and Exhibits Session 2: Other Causes of Hyperinsulinemic Hypoglycemia 10:30 – 11:20 a.m. 10:30 a.m. Short Communications (10 minutes each) UCP2 Hyperinsulinism Christine Ferrara, M.D., Ph.D. PGM1 Hyperinsulinism Amanda Ackerman, M.D., Ph.D. Hyperinsulinism in Turner Syndrome Christopher Gibson, M.D. 11 a.m. Insulinomas and HI Mimickers Andrew Palladino, M.D. 11:20 a.m. Lunch — Exhibits and Scientific Posters Session 3: Diagnosis and Treatment of Hyperinsulinism 12:30 – 2:20 p.m. 12:30 p.m. Approach to the Diagnosis and Management of Hyperinsulinism Diva D. De León, M.D., M.S.C.E. 12:50 p.m. Genotype/Phenotype Correlation in KATP Hyperinsulinism Arupa Ganguly, Ph.D. 1:05 p.m. The Role of Genetic Testing for Hyperinsulinism: New Frontiers in Genetic Diagnosis Sian Ellard, Ph.D., F.R.C.Path. 1:25 p.m. Surgical vs. Nonsurgical Decisions and Options Paul Thornton, M.D.; Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., M.R.C.P.C.H., M.D. 2 p.m. Long-acting Somatostatin Analogs Jean Baptiste Arnoux, M.D., M.Sc. 2:20 p.m. Break, Exhibits and Scientific Posters Session 4: Investigational Therapies for Hyperinsulinism 2:40 – 4:10 p.m. 2:40 p.m. Investigational Therapies: Exendin-(9-39) Diva D. De León, M.D., M.S.C.E. 2:55 p.m. Investigational Therapies: Sirolimus Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., M.R.C.P.C.H., M.D. 3:10 p.m. Investigational Therapies: Subcutaneous Glucagon Klaus Mohnike, M.D. 3:25 p.m. Investigational Therapies: Insulin Receptor Antibody Diva D. De León, M.D., M.S.C.E. 3: 40 p.m. Abstract Presentations (3 – 10 min. each) Session 5: Surgical Management Approaches to Hyperinsulinism 4:10 – 5 p.m. 4:10 p.m. [18F]-DOPA PET Imaging of Focal Hyperinsulinism: “GPS” for the Surgeon Lisa J. States, M.D. 4:30 p.m. Surgery for Hyperinsulinism N. Scott Adzick, M.D., M.M.M., F.A.C.S., F.A.A.P. 5 p.m. Adjourn 5:30 p.m. Symposium Dinner Keynote Speaker: Franz M. Matschinsky, M.D. Session 9: Breakout Sessions — Q&A with the Experts 3:10 – 4 p.m. Group A: Management of the Diazoxide-responsive Children Group B: Management of the Diazoxide-unresponsive Children Group C: Management of Diabetes after Pancreatectomy Group D: Management of Children with GSD 4 p.m. Closing Remarks Charles A. Stanley, M.D. 4:15 p.m. Adjourn Acknowledgement The “Congenital Hypoglycemia Disorders: Hyperinsulinism and Glycogen Storage Disease (GSD) Symposium” is supported by an educational grant provided by the Orphan Disease Center at the University of Pennsylvania. The Congenital Hyperinsulinism Center at The Children’s Hospital of Philadelphia gratefully acknowledge this support. Session 6: Prevention of Brain Damage 8 – 9:40 a.m. 8 a.m. Hypoglycemia Guidelines Paul Thornton, M.D. 8:20 a.m. Neurodevelopmental Outcomes Diva D. De León, M.D., M.S.C.E. 8:50 a.m. Management after Pancreatectomy: Persistent Hypoglycemia and Hyperglycemia David Langdon, M.D., F.A.A.P. 9:10 a.m. Abstract Presentations (3 – 10 min. each) 9:40 a.m. Break, Exhibits and Scientific Posters Session 7: Islet Pathophysiology in Hyperinsulinism 10 – 11:30 a.m. 10 a.m. ß-cell KATP Channels in Health and Disease Show-Ling Shyng, Ph.D. 10:20 a.m. Insulin Secretion and Fuel Metabolism in Human HI Islets Changhong Li, M.D., Ph.D. 10:40 a.m. Beyond the ß-cell Mark Dunne, Ph.D. 11 a.m. Abstract Presentations (3 – 10 min. each) 11:30 a.m. Lunch — Exhibits and Scientific Posters Session 8: Hyperketotic Hypoglycemia 1 – 2:50 p.m. 1 p.m. Overview of Ketotic Hypoglycemia Disorders (Glycogenosis, MCT1) Joseph Wolfsdorf, M.D., B.Ch. 1:30 p.m. Natural History of GSD David Weinstein, M.D., M.M.Sc. 2 p.m. Current and Investigational Therapies for GSD David Weinstein, M.D., M.M.Sc. 2:30 p.m. PGM1 in the Regulation of Glucose Metabolism and Insulin Secretion Eva Morava-Kozicz, M.D., Ph.D. 2:50 p.m. Break, Exhibits and Scientific Posters ©2015 The Children’s Hospital of Philadelphia, All Rights Reserved. 15CME0014/2.8M/09-15 Please note: This meeting will be followed by a two-day conference for patients, parents and families of children with HI on Saturday, April 16 and Sunday, April 17. Co-sponsored by Congenital Hyperinsulinism International (CHI), more information will be available on CHI’s website, www.congenitalHI.org, and will also be posted on our Web page, hyperinsulinism.chop.edu. Please encourage families of any patients you follow to consider joining us. Professional staff are also welcome to join us for this more informal informational exchange.
  • 5. General InformationProgram Registration Registeronlineatwww.chop.edu/cme Program Overview This two-day international symposium will be an update on recent exciting advances in research related to congenital hyperinsulinism and other inherited hypoglycemia disorders, particularly disorders of glycogenoses. The symposium is intended to enhance the clinical management skills and practices of endocrinologists, metabolism specialists, neonatologists, surgeons, pathologists, radiologists, geneticists and nurses, as well as basic scientists interested in pancreatic islet biology and regulation of glucose metabolism. The symposium faculty, chosen from 10 institutions in the United States, France, Great Britain, and Germany, will present 27 plenary lectures on all aspects of these disorders. Participants may register via mail or fax by completing the Registration Form attached to the brochure and indicating their form of payment. To register online go to www.chop.edu/cme. General Information The symposium will be held on Thursday, April 14 and Friday, April 15, 2016, at the Ruth and Tristram Colket, Jr. Translational Research Building, located at 3614 Civic Center Blvd. on the campus of The Children’s Hospital of Philadelphia (www.chop.edu). Registration will begin each day at 7:30 a.m. The registration fee includes continental breakfast, lunch, breaks, parking and syllabus materials. Participants are invited to attend a reception and dinner on Thursday, April 14 for an additional fee of $75. If you wish to attend, please check this option on the registration form and add this fee to your total registration fee. Learning Objectives Upon completion of this live activity, participants should be able to: • Provide an overview of the clinical manifestations, genetics and natural history of congenital hyperinsulinism and disorders of glycogenoses • Review current knowledge of the normal regulation of insulin secretion and the pathophysiology of disorders causing hyperinsulinism • Discuss recent advances and current controversies in the surgical and medical management of diffuse and focal hyperinsulinism Abstract Submissions The program includes three sessions at which attendees may present posters and short oral communications of their own clinical or laboratory research. Abstracts must be submitted electronically using the registration form on the Congenital Hyperinsulinism Symposium website (www.chop.edu/cme). The deadline for abstract submission is Friday, Jan. 8, 2016. Authors will be notified of abstracts accepted for poster or oral presentations by Monday, Feb. 1, 2016. A limited number of travel grants will be available to young investigators wishing to apply. Young investigators must apply at the time they register for the symposium and submit their abstract. ACCME Accreditation Statement The Children’s Hospital of Philadelphia is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians. AMA Credit Designation Statement The Children’s Hospital of Philadelphia designates this live activity for a maximum of 13.0 AMA PRA Category 1 Credit(s)™ . Physicians should claim only the credit commensurate with their participation in the activity. Nursing Accreditation This activity has been submitted to the PA State Nurses Association for approval to award contact hours. The PA State Nurses Association is accredited as an approver of continuing nursing education by the American Nurses Credentialing Center’s Commission on Accreditation. Congenital Hypoglycemia Disorders: Hyperinsulinism and GSD April 14 and 15, 2016 – Registration Deadline: Friday, April 1 Name ____________________________ Credentials _____________ Hospital/Professional Affiliation _______________________________ Home Address ____________________________________________ City ____________________________ State _____ ZIP __________ Phone ________________________ Fax _______________________ Email ___________________________________________________ Conference Registration Fees q M.D.s: $350 q Non-M.D.s and Physicians-in-training: $250 q Reception and Dinner, Thursday, April 14: $75 Total Registration Fee: $____________ Registration will not be processed without full payment. Faxed registrations will be accepted for credit card payments only. Please contact Micah Holliday (hollidaydm@email.chop.edu) if you do not receive a confirmation letter. Method of Payment q Personal check q Institutional check (Please make check payable to The Children’s Hospital of Philadelphia.) q Discover q MasterCard q American Express q VISA Credit Card # _____________________________________________ Exp. Date ________________________________________________ Mail/fax the completed registration form and payment to: Ms. Micah Holliday, Continuing Medical Education Department The Children’s Hospital of Philadelphia 34th Street and Civic Center Boulevard Philadelphia, PA 19104 215-590-5CME • Fax 215-590-4342 • hollidaydm@email.chop.edu The deadline for abstract submission is Friday, Jan. 8, 2016. continued on back page Thursday, April 14, 2016 Friday, April 15, 2016 Session 1: Congenital Hyperinsulinism (CHI) 8 – 10 a.m. 8 a.m. Welcome and Introductions Diva D. De León, M.D., M.S.C.E. 8:15 a.m. History of Hyperinsulinism (HI) in Pediatrics and Overview of Diagnostic/Therapeutic Algorithms Charles A. Stanley, M.D. 8:30 a.m. Diazoxide-responsive Hyperinsulinism a. GDH-HI b. KATP-HI c. Perinatal Stress HI d. HNFs-HI Charles A. Stanley, M.D. 9 a.m. Diazoxide-unresponsive Hyperinsulinism a. KATP-HI: dominant vs. recessive, focal vs. diffuse b. Atypical – LINE c. GCK Diva D. De León, M.D., M.S.C.E. 9:30 a.m. Syndromic Hyperinsulinism BWS – Jennifer Kalish, M.D., Ph.D. Kabuki Syndrome – Jean Baptiste Arnoux, M.D., M.Sc. 10 a.m. Break and Exhibits Session 2: Other Causes of Hyperinsulinemic Hypoglycemia 10:30 – 11:20 a.m. 10:30 a.m. Short Communications (10 minutes each) UCP2 Hyperinsulinism Christine Ferrara, M.D., Ph.D. PGM1 Hyperinsulinism Amanda Ackerman, M.D., Ph.D. Hyperinsulinism in Turner Syndrome Christopher Gibson, M.D. 11 a.m. Insulinomas and HI Mimickers Andrew Palladino, M.D. 11:20 a.m. Lunch — Exhibits and Scientific Posters Session 3: Diagnosis and Treatment of Hyperinsulinism 12:30 – 2:20 p.m. 12:30 p.m. Approach to the Diagnosis and Management of Hyperinsulinism Diva D. De León, M.D., M.S.C.E. 12:50 p.m. Genotype/Phenotype Correlation in KATP Hyperinsulinism Arupa Ganguly, Ph.D. 1:05 p.m. The Role of Genetic Testing for Hyperinsulinism: New Frontiers in Genetic Diagnosis Sian Ellard, Ph.D., F.R.C.Path. 1:25 p.m. Surgical vs. Nonsurgical Decisions and Options Paul Thornton, M.D.; Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., M.R.C.P.C.H., M.D. 2 p.m. Long-acting Somatostatin Analogs Jean Baptiste Arnoux, M.D., M.Sc. 2:20 p.m. Break, Exhibits and Scientific Posters Session 4: Investigational Therapies for Hyperinsulinism 2:40 – 4:10 p.m. 2:40 p.m. Investigational Therapies: Exendin-(9-39) Diva D. De León, M.D., M.S.C.E. 2:55 p.m. Investigational Therapies: Sirolimus Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., M.R.C.P.C.H., M.D. 3:10 p.m. Investigational Therapies: Subcutaneous Glucagon Klaus Mohnike, M.D. 3:25 p.m. Investigational Therapies: Insulin Receptor Antibody Diva D. De León, M.D., M.S.C.E. 3: 40 p.m. Abstract Presentations (3 – 10 min. each) Session 5: Surgical Management Approaches to Hyperinsulinism 4:10 – 5 p.m. 4:10 p.m. [18F]-DOPA PET Imaging of Focal Hyperinsulinism: “GPS” for the Surgeon Lisa J. States, M.D. 4:30 p.m. Surgery for Hyperinsulinism N. Scott Adzick, M.D., M.M.M., F.A.C.S., F.A.A.P. 5 p.m. Adjourn 5:30 p.m. Symposium Dinner Keynote Speaker: Franz M. Matschinsky, M.D. Session 9: Breakout Sessions — Q&A with the Experts 3:10 – 4 p.m. Group A: Management of the Diazoxide-responsive Children Group B: Management of the Diazoxide-unresponsive Children Group C: Management of Diabetes after Pancreatectomy Group D: Management of Children with GSD 4 p.m. Closing Remarks Charles A. Stanley, M.D. 4:15 p.m. Adjourn Acknowledgement The “Congenital Hypoglycemia Disorders: Hyperinsulinism and Glycogen Storage Disease (GSD) Symposium” is supported by an educational grant provided by the Orphan Disease Center at the University of Pennsylvania. The Congenital Hyperinsulinism Center at The Children’s Hospital of Philadelphia gratefully acknowledge this support. Session 6: Prevention of Brain Damage 8 – 9:40 a.m. 8 a.m. Hypoglycemia Guidelines Paul Thornton, M.D. 8:20 a.m. Neurodevelopmental Outcomes Diva D. De León, M.D., M.S.C.E. 8:50 a.m. Management after Pancreatectomy: Persistent Hypoglycemia and Hyperglycemia David Langdon, M.D., F.A.A.P. 9:10 a.m. Abstract Presentations (3 – 10 min. each) 9:40 a.m. Break, Exhibits and Scientific Posters Session 7: Islet Pathophysiology in Hyperinsulinism 10 – 11:30 a.m. 10 a.m. ß-cell KATP Channels in Health and Disease Show-Ling Shyng, Ph.D. 10:20 a.m. Insulin Secretion and Fuel Metabolism in Human HI Islets Changhong Li, M.D., Ph.D. 10:40 a.m. Beyond the ß-cell Mark Dunne, Ph.D. 11 a.m. Abstract Presentations (3 – 10 min. each) 11:30 a.m. Lunch — Exhibits and Scientific Posters Session 8: Hyperketotic Hypoglycemia 1 – 2:50 p.m. 1 p.m. Overview of Ketotic Hypoglycemia Disorders (Glycogenosis, MCT1) Joseph Wolfsdorf, M.D., B.Ch. 1:30 p.m. Natural History of GSD David Weinstein, M.D., M.M.Sc. 2 p.m. Current and Investigational Therapies for GSD David Weinstein, M.D., M.M.Sc. 2:30 p.m. PGM1 in the Regulation of Glucose Metabolism and Insulin Secretion Eva Morava-Kozicz, M.D., Ph.D. 2:50 p.m. Break, Exhibits and Scientific Posters ©2015 The Children’s Hospital of Philadelphia, All Rights Reserved. 15CME0014/2.8M/09-15 Please note: This meeting will be followed by a two-day conference for patients, parents and families of children with HI on Saturday, April 16 and Sunday, April 17. Co-sponsored by Congenital Hyperinsulinism International (CHI), more information will be available on CHI’s website, www.congenitalHI.org, and will also be posted on our Web page, hyperinsulinism.chop.edu. Please encourage families of any patients you follow to consider joining us. Professional staff are also welcome to join us for this more informal informational exchange.
  • 6. ContinuingMedicalEducationDepartment Course Faculty Symposium Course Directors Diva D. De León, M.D., M.S.C.E. Director, Congenital Hyperinsulinism Center Associate Professor of Pediatrics Charles A. Stanley, M.D. Founder, Congenital Hyperinsulinism Center Emeritus Professor of Pediatrics Faculty N. Scott Adzick, M.D., M.M.M., F.A.C.S., F.A.A.P. Surgeon-in-Chief C. Everett Koop Professor of Pediatric Surgery Arupa Ganguly, Ph.D. Director, Genetic Diagnostic Laboratory Hospital of the University of Pennsylvania Associate Professor, Department of Genetics Jennifer Kalish, M.D., Ph.D. Attending Physician Division of Human Genetics and Metabolism Instructor of Pediatrics David Langdon, M.D., F.A.A.P. Director, Inpatient Endocrine Service Clinical Associate Professor Changhong Li, M.D., Ph.D. Division of Endocrinology and Diabetes Research Assistant Professor of Pediatrics Franz M. Matschinsky, M.D. Benjamin Rush Professor of Biochemistry and Biophysics Lisa J. States, M.D. Director of Outpatient Satellite Imaging Department of Radiology Associate Professor of Radiology Guest Faculty Jean Baptiste Arnoux, M.D., M.Sc. Senior Consultant in Inherited Metabolic Disorders and Congenital Hyperinsulinism Hôpital Universitaire Necker Paris, France Mark Dunne, Ph.D. Professor The University of Manchester Manchester, England Sian Ellard, Ph.D., F.R.C.Path. Head of Molecular Genetics, Royal Devon & Exeter NHS Foundation Trust Professor of Human Molecular Genetics Devon, England Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., M.R.C.P.C.H., M.D. Consultant Paediatric Endocrinologist Great Ormond Street Hospital for Children London, England Klaus Mohnike, M.D. Professor Otto-von-Guericke Universität Magdeburg, Germany Eva Morava-Kozicz, M.D., Ph.D. Clinical Biochemical Geneticist
 Professor of Pediatrics Tulane University School
of Medicine Hayward Genetics Center New Orleans, La. Andrew Palladino, M.D. Clinical Director Endocrine Therapeutics Pfizer Collegeville, Pa. Show-Ling Shyng, Ph.D. Professor Department of Biochemistry and Molecular Biology Oregon Health and Science University Portland, Ore. Paul Thornton, M.D. Director, Congenital Hyperinsulinism Center Cook Children’s Medical Center Fort Worth, Texas David Weinstein, M.D., M.M.Sc. Professor
 Director, Glycogen Storage Disease Program
 University of Florida Gainesville, Fla. Joseph Wolfsdorf, M.D., B.Ch. Associate Chief, Division of Endocrinology Clinical Director, Division of Endocrinology Professor of Pediatrics Boston Children’s Hospital Boston, Mass. Hotel Information The Children’s Hospital of Philadelphia has reserved blocks of rooms at the Sheraton University City at 3549 Chestnut St., in Philadelphia. The Sheraton University City (www.philadelphiasheraton.com) is located in the heart of the University of Pennsylvania campus, adjacent to Philadelphia’s prestigious medical, education and corporate centers, and just blocks away from the Colket Building. The group overnight room rate is $179 single and double occupancy plus a 15.5 percent room tax. Please contact the hotel directly at 215-387-8000 to make your reservation. When making your reservation at the hotel, please mention the Congenital Hyperinsulinism Symposium. For a complete listing of lodging in Philadelphia, please visit the Philadelphia Convention and Visitor’s Bureau website at www.pcvb.org. Cancellation and Refund Policy The Children’s Hospital of Philadelphia reserves the right to cancel or postpone any conference due to any unforeseen circumstances. In the event of cancellation or postponement, Children’s Hospital will refund any registration fees, less a $30 service charge, but is not responsible for any related costs, charges or expenses to participants, including cancellation charges assessed by airlines/travel agencies. In order to process refunds for course withdrawals, cancellation must be received in writing by Friday, April 1. No refunds will be issued thereafter. Services for People with Disabilities If arrangements are required for an individual with a disability to attend this meeting, please contact the Continuing Medical Education Department at 215-590-5263. General Info continued Congenital Hypoglycemia Disorders: Hyperinsulinism and GSD April 14 and 15, 2016 Ruth and Tristram Colket, Jr. Translational Research Building at The Children’s Hospital of Philadelphia Sponsored by The Congenital Hyperinsulinism Center Division of Endocrinology and Diabetes www.chop.edu/cme Unless otherwise noted, faculty is from The Children’s Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania. ADDRESSCORRECTIONREQUESTED
  • 7. ContinuingMedicalEducationDepartment Course Faculty Symposium Course Directors Diva D. De León, M.D., M.S.C.E. Director, Congenital Hyperinsulinism Center Associate Professor of Pediatrics Charles A. Stanley, M.D. Founder, Congenital Hyperinsulinism Center Emeritus Professor of Pediatrics Faculty N. Scott Adzick, M.D., M.M.M., F.A.C.S., F.A.A.P. Surgeon-in-Chief C. Everett Koop Professor of Pediatric Surgery Arupa Ganguly, Ph.D. Director, Genetic Diagnostic Laboratory Hospital of the University of Pennsylvania Associate Professor, Department of Genetics Jennifer Kalish, M.D., Ph.D. Attending Physician Division of Human Genetics and Metabolism Instructor of Pediatrics David Langdon, M.D., F.A.A.P. Director, Inpatient Endocrine Service Clinical Associate Professor Changhong Li, M.D., Ph.D. Division of Endocrinology and Diabetes Research Assistant Professor of Pediatrics Franz M. Matschinsky, M.D. Benjamin Rush Professor of Biochemistry and Biophysics Lisa J. States, M.D. Director of Outpatient Satellite Imaging Department of Radiology Associate Professor of Radiology Guest Faculty Jean Baptiste Arnoux, M.D., M.Sc. Senior Consultant in Inherited Metabolic Disorders and Congenital Hyperinsulinism Hôpital Universitaire Necker Paris, France Mark Dunne, Ph.D. Professor The University of Manchester Manchester, England Sian Ellard, Ph.D., F.R.C.Path. Head of Molecular Genetics, Royal Devon & Exeter NHS Foundation Trust Professor of Human Molecular Genetics Devon, England Khalid Hussain, M.B.Ch.B., M.R.C.P., M.Sc., M.R.C.P.C.H., M.D. Consultant Paediatric Endocrinologist Great Ormond Street Hospital for Children London, England Klaus Mohnike, M.D. Professor Otto-von-Guericke Universität Magdeburg, Germany Eva Morava-Kozicz, M.D., Ph.D. Clinical Biochemical Geneticist
 Professor of Pediatrics Tulane University School
of Medicine Hayward Genetics Center New Orleans, La. Andrew Palladino, M.D. Clinical Director Endocrine Therapeutics Pfizer Collegeville, Pa. Show-Ling Shyng, Ph.D. Professor Department of Biochemistry and Molecular Biology Oregon Health and Science University Portland, Ore. Paul Thornton, M.D. Director, Congenital Hyperinsulinism Center Cook Children’s Medical Center Fort Worth, Texas David Weinstein, M.D., M.M.Sc. Professor
 Director, Glycogen Storage Disease Program
 University of Florida Gainesville, Fla. Joseph Wolfsdorf, M.D., B.Ch. Associate Chief, Division of Endocrinology Clinical Director, Division of Endocrinology Professor of Pediatrics Boston Children’s Hospital Boston, Mass. Hotel Information The Children’s Hospital of Philadelphia has reserved blocks of rooms at the Sheraton University City at 3549 Chestnut St., in Philadelphia. The Sheraton University City (www.philadelphiasheraton.com) is located in the heart of the University of Pennsylvania campus, adjacent to Philadelphia’s prestigious medical, education and corporate centers, and just blocks away from the Colket Building. The group overnight room rate is $179 single and double occupancy plus a 15.5 percent room tax. Please contact the hotel directly at 215-387-8000 to make your reservation. When making your reservation at the hotel, please mention the Congenital Hyperinsulinism Symposium. For a complete listing of lodging in Philadelphia, please visit the Philadelphia Convention and Visitor’s Bureau website at www.pcvb.org. Cancellation and Refund Policy The Children’s Hospital of Philadelphia reserves the right to cancel or postpone any conference due to any unforeseen circumstances. In the event of cancellation or postponement, Children’s Hospital will refund any registration fees, less a $30 service charge, but is not responsible for any related costs, charges or expenses to participants, including cancellation charges assessed by airlines/travel agencies. In order to process refunds for course withdrawals, cancellation must be received in writing by Friday, April 1. No refunds will be issued thereafter. Services for People with Disabilities If arrangements are required for an individual with a disability to attend this meeting, please contact the Continuing Medical Education Department at 215-590-5263. General Info continued Congenital Hypoglycemia Disorders: Hyperinsulinism and GSD April 14 and 15, 2016 Ruth and Tristram Colket, Jr. Translational Research Building at The Children’s Hospital of Philadelphia Sponsored by The Congenital Hyperinsulinism Center Division of Endocrinology and Diabetes www.chop.edu/cme Unless otherwise noted, faculty is from The Children’s Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania. ADDRESSCORRECTIONREQUESTED