Scientific Conference Agenda

Malignant Hyperthermia (MH)- New Insights and Connections with other Myopathies

Conference Objectives

· To discuss new advances in the pathophysiology of MH and to gain insight into the role of the skeletal muscle ryanodine receptor, type 1 (RYR1) and other genes in MH and other related disorders.

· To promote understanding of how MH susceptibility relates to other muscle disorders (e.g. Central Core Disease (CCD), muscular dystrophies, metabolic myopathies).

· To discuss advances in the genetics of MH and improvements in diagnosis for MH susceptibility.

· To improve the ability of anesthesiologists who volunteer as consultants (the HLCs) to provide up-to-date support to medical practitioners on the identification and management of MH and other muscle disorders.

· To introduce young anesthesiologists and other interested investigators to this area of medicine.

Time	Topic	Speaker(s)
8:00-8:30	Welcome and Introductions	Henry Rosenberg, MD, President, MHAUS Andrew Herlich, MD, UPMC
Session 1	Pathophysiology of MH – Overview and Recent Advances in Understanding	Session Chairperson: Jerome Parness, MD, PhD, UPMC
8:30-9:00	Introduction to MH Pathophysiology; fresh approaches to understanding MH at cellular level; dantrolene & mechanism of action	Jerome Parness, MD, PhD, UPMC
9:00-9:45	Lessons from a Mouse Model of Malignant Hyperthermia: Mechanisms and New Interventions 9:45-10:00: Questions/Discussion	Susan Hamilton, PhD, Baylor College of Medicine Keynote Speaker
10:00-10:30	Mechanistic Models for the Cause of MH and for Core Formation in CCD 10:30-10:45 Questions/Discussion	David MacLennan, PhD University of Toronto
10:45-11:15	COFFEE BREAK
11:15-11:45	New Frontiers in Personalized Medicine: RyR1 Allele-Specific Gene Silencing in Mouse Models of MH and CCD 11:45-12:00: Questions/Discussion	Robert T. Dirksen, PhD University of Rochester
12:00-12:30	Changes in DHPR kinetics induced by MH related conformational changes in RyR1 12:30-12:45: Questions/Discussion	Paul Allen, MD, PhD Brigham and Women’s
12:45-1:45	LUNCH
Session 2	Genetics of MH: Results and Future Directions	Chairperson: Sheila Muldoon, MD, USUHS
1:45-2:15	Genetics testing for MH susceptibility in Europe; role of new diagnostic testing strategies in evaluating MH risk	Henrick Rueffert, MD University of Leipzig, Germany
2:15-2:45	Reassessment of the operating characteristics of IVCT given genetic RYR1 results; update on the EMHG	Thierry Girard, MD University Hospital of Basel, Switzerland
2:45-3:15	Results of genetic testing (RYR1 gene findings) in basic research laboratories	Khishge Sambuughin, PhD, USUHS
3:15-3:30	COFFEE BREAK
3:30-4:00	Assessment of RYR1 gene variants for Pathogenicity: Theory and Examples	Jeffrey Kant, MD, PhD UPMC
4:00-4:30	Results of genetic testing (RYR1 gene findings) in CLIA-approved clinical laboratories	Jim Weber, PhD PreventionGenetics
4:30-5:15	Discussion of related questions	Sheila Muldoon, MD USUHS
5:15-6:30	POSTER SESSION

Time	Topic	Speaker(s)
Session 3	Relationship between MH-susceptibility and other muscle conditions/disorders	Chairperson: Barbara Brandom, MD, UPMC
8:30-8:45	Review of 2008 SPA Meeting Recommendations (anesthetic considerations in MH-associated diseases)	Barbara Brandom, MD UPMC
8:45-9:15	Creatine Kinase and Heat Tolerance in Mice and Men 9:15-9:30: Questions/Discussion	Patricia Deuster, PhD, MPH USUHS
9:30-10:00	COFFEE BREAK
10:00-10:30	Confounding Cases: Is this MH and How Would You Provide Anesthesia? 10:30-10:45: Questions/Discussion	John Capacchione, MD USUHS
10:45-11:15	Genetic susceptibility to statin-induced myopathy 11:15-11:30: Questions/Discussion	Georgirene Vladutiu, PhD SUNY at Buffalo
11:30-1:00	LUNCH & ADDITIONAL TIME TO VISIT POSTERS
Session 4	MH Epidemiology	Chairperson: Henry Rosenberg, President, MHAUS
1:00-1:15	Introduction to Epidemiology of MH; role of MH Hotline and North American MH Registry (NAMHR) databases as research and educational tools; implications and limitations of epidemiologic studies	Henry Rosenberg President, MHAUS
1:15-1:45	MH Comorbidities 1:45-2:00: Questions/Discussion	Guohua Li, MD, DrPH Columbia University
2:00-2:45	What the Registry has Taught Us: MH Presentation, Treatment, and Outcome; Is it Time for Revisions to MHAUS Clinical Care Recommendations? 2:45-3:00: Questions/Discussion	Marilyn Larach, MD Penn State College of Medicine
3:00-3:15	COFFEE BREAK
Session 5	Future Directions/Challenges	Chairperson: Henry Rosenberg, MD, President, MHAUS
3:15-3:30	Changing Recommendations for Anesthesia Machine Preparation 3:30-3:45: Questions/Discussion	David Rymer, MD UPMC
3:45-4:00	Need for building the NAMHR database as source for future clinical trials; Recent reporting trends; Current contributions from the MH Diagnostic Centers	Barbara Brandom, MD UPMC
4:00-4:30	Patient Safety First	Dale Micalizzi Board of Directors, MHAUS
4:30-5:00	Closing Remarks: Future directions for MH – research and non-invasive diagnostic testing; Future Directions for MHAUS –patient safety initiatives (ambulatory surgery issues, educational initiatives [simulation]); advice for MHS individuals.	Henry Rosenberg, MD MHAUS

AGENDA Day 1- Friday, April 23, 2010

POSTERS SHOULD BE SET UP 7:00-8:00 AM

Time

Topic

Speaker(s)

8:00-8:30

Welcome and Introductions

Henry Rosenberg, MD,

President, MHAUS

Andrew Herlich, MD, UPMC

Session 1

Pathophysiology of MH – Overview and Recent Advances in Understanding

Session Chairperson: Jerome Parness, MD, PhD, UPMC

8:30-9:00

Introduction to MH Pathophysiology; fresh approaches to understanding MH at cellular level; dantrolene & mechanism of action

Jerome Parness, MD, PhD, UPMC

9:00-9:45

Lessons from a Mouse Model of Malignant Hyperthermia: Mechanisms and New Interventions

9:45-10:00: Questions/Discussion

Susan Hamilton, PhD,

Baylor College of Medicine

Keynote Speaker

10:00-10:30

Mechanistic Models for the Cause of MH and for Core Formation in CCD

10:30-10:45 Questions/Discussion

David MacLennan, PhD

University of Toronto

10:45-11:15

COFFEE BREAK

11:15-11:45

New Frontiers in Personalized Medicine: RyR1 Allele-Specific Gene Silencing in Mouse Models of MH and CCD

11:45-12:00: Questions/Discussion

Robert T. Dirksen, PhD

University of Rochester

12:00-12:30

Changes in DHPR kinetics induced by MH related conformational changes in RyR1

12:30-12:45: Questions/Discussion

Paul Allen, MD, PhD

Brigham and Women’s

12:45-1:45

LUNCH

Session 2

Genetics of MH: Results and Future Directions

Chairperson: Sheila Muldoon, MD, USUHS

1:45-2:15

Genetics testing for MH susceptibility in Europe; role of new diagnostic testing strategies in evaluating MH risk

Henrick Rueffert, MD

University of Leipzig, Germany

2:15-2:45

Reassessment of the operating characteristics of IVCT given genetic RYR1 results; update on the EMHG

Thierry Girard, MD

University Hospital of Basel, Switzerland

2:45-3:15

Results of genetic testing (RYR1 gene findings) in basic research laboratories

Khishge Sambuughin, PhD,

USUHS

3:15-3:30

COFFEE BREAK

3:30-4:00

Assessment of RYR1 gene variants for Pathogenicity: Theory and Examples

Jeffrey Kant, MD, PhD

UPMC

4:00-4:30

Results of genetic testing (RYR1 gene findings) in CLIA-approved clinical laboratories

Jim Weber, PhD

PreventionGenetics

4:30-5:15

Discussion of related questions

Sheila Muldoon, MD

USUHS

5:15-6:30

POSTER SESSION

AGENDA Day 2- Saturday, April 24, 2010

Time

Topic

Speaker(s)

8:00-8:30

Welcome, Comment on Day 1

Jerome Parness, MD, PhD, UPMC

Sheila Muldoon, MD, USUHS

Session 3

Relationship between MH-susceptibility and other muscle conditions/disorders

Chairperson: Barbara Brandom, MD, UPMC

8:30-8:45

Review of 2008 SPA Meeting Recommendations (anesthetic considerations in MH-associated diseases)

Barbara Brandom, MD

UPMC

8:45-9:15

Creatine Kinase and Heat Tolerance in Mice and Men

9:15-9:30: Questions/Discussion

Patricia Deuster, PhD, MPH

USUHS

9:30-10:00

COFFEE BREAK

10:00-10:30

Confounding Cases: Is this MH and How Would You Provide Anesthesia?

10:30-10:45: Questions/Discussion

John Capacchione, MD

USUHS

10:45-11:15

Genetic susceptibility to statin-induced myopathy

11:15-11:30: Questions/Discussion

Georgirene Vladutiu, PhD

SUNY at Buffalo

11:30-1:00

LUNCH & ADDITIONAL TIME TO VISIT POSTERS

Session 4

MH Epidemiology

Chairperson: Henry Rosenberg, President, MHAUS

1:00-1:15

Introduction to Epidemiology of MH; role of MH Hotline and North American MH Registry (NAMHR) databases as research and educational tools; implications and limitations of epidemiologic studies

Henry Rosenberg

President, MHAUS

1:15-1:45

MH Comorbidities

1:45-2:00: Questions/Discussion

Guohua Li, MD, DrPH

Columbia University

2:00-2:45

What the Registry has Taught Us: MH Presentation, Treatment, and Outcome; Is it Time for Revisions to MHAUS Clinical Care Recommendations?

2:45-3:00: Questions/Discussion

Marilyn Larach, MD

Penn State College of Medicine

3:00-3:15

COFFEE BREAK

Session 5

Future Directions/Challenges

Chairperson: Henry Rosenberg, MD, President, MHAUS

3:15-3:30

Changing Recommendations for Anesthesia Machine Preparation

3:30-3:45: Questions/Discussion

David Rymer, MD

UPMC

3:45-4:00

Need for building the NAMHR database as source for future clinical trials; Recent reporting trends; Current contributions from the MH Diagnostic Centers

Barbara Brandom, MD

UPMC

4:00-4:30

Patient Safety First

Dale Micalizzi

Board of Directors, MHAUS

4:30-5:00

Closing Remarks: Future directions for MH – research and non-invasive diagnostic testing; Future Directions for MHAUS –patient safety initiatives (ambulatory surgery issues, educational initiatives [simulation]); advice for MHS individuals.

Henry Rosenberg, MD

MHAUS