MHAUS Newsletter, February 2007
In this issue:

Genetically Developed Mouse Shows Promise for MH Research and Future of MH Patients

In the past, the pig has helped guide research into the diagnosis and treatment of malignant hyperthermia. But that animal's MH triggers have differences than those in humans, so scientists are constantly searching for ways to genetically engineer an animal into having more similar "human" MH characteristics. Progress has recently been made with the mouse by investigators from the Baylor College of Medicine, University of Rochester, and University of California, Davis.

They have introduced in mice the genetic makeup similar to that which produces MH in humans. The mutation is one of the DNA changes found in the ryanodine receptor gene.

"I think this is going to be a very useful model as they produce other mice with other knock-in mutations," says Henry Rosenberg, MD, President of the Malignant Hyperthermia Association of the United States (www.mhaus.org). "The creation of animals such as this is an important advance in our understanding of the MH syndrome and will provide us with insights in the pathophysiology and unusual presentation of this syndrome. I am sure that mice and other animals with DNA changes that are also known to produce MH will be produced in the near future. Our understanding of MH and our ability to help patients and practitioners is sure to improve."

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For Physicians and Patients: New Study to Increase Accuracy of Molecular Genetics (blood based) Test for Malignant Hyperthermia

While research with mice is opening new doors, so is research with human MH patients. Barbara Brandom, MD and Sheila Muldoon, MD (a member of the MHAUS Board of Directors) report the following:

"With support from MHAUS and approval of the Institutional Review Boards of the University of Pittsburgh and the Uniformed Services University of the Health Sciences, the North American Malignant Hyperthermia Registry staff have begun a study that could increase the sensitivity of the blood based test of Malignant Hyperthermia susceptibility. The goal of this study is to examine the entire coding region of the ryanodine receptor gene (RYR1) in people who have had strongly positive results on the caffeine-halothane contracture test. Because several studies of smaller numbers of people in 3 different parts of the world found variants of the RYR1 in 70% of subjects, it is expected that this study of people in North America will find variants in parts of the RYR1 that are not now part of the clinical genetic test of MH susceptibility. Participants in the MH Registry who meet the entry criteria of this study will receive a letter from the MH Registry office asking if they would like to participate in this study. After the potential study participant has returned their signed consent form to the Registry office, a set of instructions for sending that person's blood to the laboratory at the Uniformed Services University of the Heath Sciences is sent out. Dr. N. Sambuughin, the geneticist who worked with MH researchers in the past, is again preparing to search many specimens for variants in RYR1. The results of this study will probably be available in 2008."

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MHAUS and NMSIS Speakers Bureaus Making Strides in Educating Medical Providers

The molecular genetics test is just one of the topics included in our MHAUS Speakers Bureau. This effort by MHAUS has been scheduling speeches from Alaska to Mississippi to New York and Georgia. Our speakers have presented to anesthesiologists, several nursing disciplines, and surgeons. These speakers are top experts in MH, some are even our MH Hotline consultants (our 24/7 service, where your medical team can call at anytime to get live, real time advice on handling an MH episode) and are available throughout the year to speak on topics such as MH Diagnosis and Treatment, and an MH Hotline Case Review. We have also recently developed a speakers bureau for our sister organization, the Neuroleptic Malignant Syndrome Information Service (www.nmsis.org ). You or your facility may soon receive a flyer, e-mail, or even a telephone call letting you know about these services. It is easy to schedule a speaker. If you would like to, just contact Al Rothstein at mhausspeakers@rothsteinmedia.com, nmsspeakers@rothsteinmedia.com, or call toll free at (866) 636-3342.

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Take the Challenge - Test Yourself on the MH Hotline Case of the Month

Speaking of the MH Hotline, since February of 2005 we have been posting an "MH Case of the Month" at at www.mhaus.org. The featured case is taken from an actual MH Hotline case. It will challenge you, and help make sure your team is up to date with the latest management tools and techniques for recognizing and responding to an MH case.

See the latest case and take the challenge! The answers are posted the following month. We hope this offers you an invaluable educational experience.

MH Hotline, USA and Canada: (800) 644-9737, Outside the US: 0011 315 464 7079

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NMSIS Online Program Offers CMEs

MHAUS's sister organization, The Neuroleptic Malignant Syndrome Information Service (NMSIS), would like to invite you to gain valuable continuing education credits by participating in its new online program at www.nmsis.org. Neuroleptic Malignant Syndrome

(NMS) is a dangerous, life-threatening side effect of certain medications used mostly to treat patients suffering from psychosis. This side effect is often missed by practicing clinicians.

The program helps health professionals with diagnosis and treatment. Its objectives are:

1) The participant will recognize the clinical signs of NMS associated with typical and atypical antipsychotics to help determine a diagnosis

2) The participant will be able to discuss risk factors and the changing epidemiology of NMS.

3) The participant will identify and apply the differential diagnosis of NMS

4) The participant will employ strategies for the treatment of NMS

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The Latest Articles from The Communicator

Featured in the Latest Edition of MHAUS's newsletter, The Communicator:

MHAUS Issues Consensus Statement On MHS and OR Personnel
MHAUS has issued a consensus statement regarding MH-susceptibles working in an operating room. The statement clarifies that "there is no evidence to support restricting the professional choices of MHS individuals; however, caution should be exercised regarding the inhalation of any noxious vapor in a poorly ventilated area."

Six Honored at MHAUS Anniversary Reception
The Malignant Hyperthermia Association of the United States presented awards to six recipients during its 25th Anniversary Reception held in Chicago in October. Among the recipients, James Woods, MD, for the MHAUS Media Award; Sheila Muldoon, MD, for the Outstanding Dedication to MHAUS Award; Cristina Pamaar, MD and Denise Wedel, MD for The Hotline Partnership Award; and Dr. Richard Urman, MD, and Dr. Christopher Flood, MD., for The Daniel Massik Awards .

ASA Abstracts Available Online
Abstracts presented at the American Society of Anesthesiologists meeting are available online at www.asaabstracts.com. You can search abstracts by year or topic and can search as far back as six years.

Podcasts Available Online
There are four new podcasts from the recent American Society of Anesthesiologists meeting available on the MHAUS web site. Each podcast includes an interesting MH case from the Hotline followed by a question and answer period. The presenters are Henry Rosenberg, MD, Richard Kaplan, MD, Joseph Tobin, and Ronald Litman, MD.

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