The North American Malignant Hyperthermia Registry

Introduction to the NAMHR
If You Wish to be Registered
Why Put a Name in the Registry?
Research Using Registry Data
     Abstracts
     Publications


Introduction to the NAMHR

The North American MH Registry (NAMHR) was established in 1987 and merged with the Malignant Hyperthermia Association of the United States (MHAUS) in 1995 so that data on MH could be stored in a site that is supported by one organization to offer greater support for research initiatives. The Registry is currently directed by Dr. Barbara W. Brandom at Children's Hospital in Pittsburgh, PA.

The Registry's goal is to acquire, analyze, and disseminate case-specific clinical and laboratory information related to malignant hyperthermia susceptibility. Registry functions are consistent with HIPAA, OHRP, and IRB regulations. Registry data can be used to conduct research into the epidemology, diagnosis, clinical course, and treatment of MH. Each potential study must have IRB approval from its parent institution, and approval from our Scientific Advisory Committee.

Objectives of the Registry are to:
  • Maintain a central database of individuals with MH susceptibility and similar syndromes. (With the person's consent, specific identifiers are included in the Registry. Otherwise, only de-identified data is entered.)
  • Provide information storage and analysis services to MH diagnostic referral centers for standardization and validation of MH diagnostic testing procedures, including genetic tests.
  • Investigate the epidemology of MH in order to improve diagnosis, treatment, and prevention of MH episodes
  • Give a registered person the security of knowing that there is a central repository of information concerning their MH susceptibility status.

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If you wish to be registered, please contact us

If you wish to be registered in the NAMHR, or would like to know if you are already registered, please contact Dr. Barbara W. Brandom, Michael Young, or Kristee Adams at 1(888) 274-7899.

If you have a patient that wishes to be registered, or wants to know if they are already registered, please have them contact us directly.

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Why put a name in the Registry?

If a person knows they have MH, or if they think they might have MH, then they may want to have their name in the Registry. This could happen when there is MH in the person's family or when the individual has had an incident.

A person's participation in the North American Malignant Hyperthermia Registry (NAMHR) can help medical investigators in important ways.

The NAMHR provides researchers with information to determine how MH presents itself, how it is diagnosed, how it is treated, and how it responds to that treatment. Each case that is entered into the Registry increases the knowledge available to researchers working on MH treatment and diagnosis.

If a person agrees to participate in the NAMHR, physicians and researchers may review parts of that individual's medical information to judge if they qualify for any future research studies.

The parts of the subject's records that researchers may review will *not* include any identifying information.

The Registry can help people with appropriate cases enter family studies, which might improve the ability of that family to get a non-invasive MH test.

A person might have some information that is already in the Registry, but not connected with their name. For example, information about a subject's biopsy or other diagnostic test, including genetic tests, and history may have been transferred to the NAMHR without any specific identifiers. The individual may have had an incident report submitted, and these do not contain any specific identifiers.

People that are in the Registry may ask for a report of records we have concerning them or their case at any time. The individual may want to share this report with their health care professionals.

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Research Using Registry Data

If you are interested in conducting research with data in the NAMHR, please see the following documents for further information
Policy for Internal Researchers
Policy for External Researchers
Additionally, the NAMHR has compiled a library of works concerning MH and related topics. Researchers on approved studies will have access to this library, which contains over 600 abstracts and over 700 full text articles.

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Abstracts

  • Presented to the European Malignant Hyperthermia Group 2010:
    Brandom BW, Muldoon, SM, Sambuughin N. More than Anesthetic Induced Malignant Hyperthermia (MH)
  • Presented to the European Malignant Hyperthermia Group 2009:
    Brandom BW, Muldoon SM, Wong C, Rosenberg H, Tautz T, Sambuughin N. An Update on the Ryanodin Receptor Gene Variants found in the USA.
  • Presented to the American Society of Anesthesiologists (ASA) 2008:
    Larach MG, Allen GC, Brandom BW, Gronert GA, Lehman EB. Temperature changes are not late signs of malignant hyperthermia: A NAMH Registry of MHAUS Study
  • Presented to the American Society of Anesthesiologists (ASA) 2008:
    Scientific Papers, in review. Larach MG, Allen GC, Brandom BW, Gronert GA, Lehman EB. Serious complications associated with malignant hyperthermia events: A NAMH Registry of MHAUS Study
  • Presented to the American Society of Anesthesiologists (ASA) 2007:
    Sambuughin N, Brandom B, Capacchione J, Rosenberg H, Muldoon S. Toward updating the North American Malignant Hyperthermia Mutation Panel
  • Presented to the American Society of Anesthesiologists (ASA) 2006:
    Larach MG, Brandom BW, Allen GC, Gronert GA, Lehman EB. Deaths associated with malignant hyperthermia (1987-2006) ? A North American MH Registry of MHAUS Study
  • Presented to the American Society of Anesthesiologists (ASA) 2005:
    Torp KD, Brandom BW, Capacchione JF, Voelkel ML, Muldoon SM. Caffeine halothane contracture test and ryanodine receptor type 1 analysis in patients who experienced MH episodes
  • Presented to the American Society of Anesthesiologists (ASA) in 2005:
    Burkman JM, Posner KL, Domino KB: Analysis of the clinical variables associated with recrudescence after malignant hyperthermia reactions
  • Presented to the American Society of Anesthesiologists (ASA) in 2004:
    Sambuughin N, Holley H, Brandom B, Nelson T, Muldoon S: Comprehensive screening of the RYR1 gene for malignant hyperthermia susceptibility
  • Presented to the European Congress of Nursing in 2004,
    previously presented to the American Association of Nurse Anesthethists, 2003:

    Ciceron MC, Lauriello R, McCarthy EJ, McDonough JP: Differences in clinical manifestations in malignant hyperthermia episodes with succinylcholine or volatile anesthetics
  • Presented to the Society for Pediatric Anesthesia, 2003:
    Brandom BW, Larach MG, Gurgis F: Comparison of pediatric and adult patients with regard to the safety and efficacy of dantrolene
  • Presented to the American Society of Anesthesiologists, 2002:
    Kozack JK: A survey of chronic muscle pain and other symptoms in malignant hyperthermia susceptible individuals
  • Presented to the American Society of Anesthesiologists, 2002:
    Brandom BW, Larach MG: Reassessment of the safety and efficacy of dantrolene

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Publications

  • Brandom BW, Bina S, Wong CA, Wallace T, Visoiu M, Isackson PJ, Vladutiu GD, Sambuughin N, Muldoon SM. Ryanodine Receptor Type 1 Gene Variants in the Malignant Hyperthermia-Susceptible Population of the United States. Anesth Analg 2013; 116:1078-86
  • Lavezzi WA, Capacchione JF, Muldoon SM, Sambuughin N, Bina S, Steele D, Brandom BW. Death in the Emergency Department: An Unrecognized Awake Malignant Hyperthermia-Like Reaction in a Six-Year-Old. Anesth Analg 2013; 116:420-3
  • Brandom BW, Larach MG, Chen MA, Young MC. Complications Associated with the Administration of Dantrolene 1987 to 2006: A Report from the North American Malignant Hyperthermia Association of the United States. Anesth Analg 2011; 112:1115-23
  • Larach MG, Gronert GA, Allen GC, Brandom BW, Lehman EB. Clinical Presentation, Treatment, and Complications of Malignant Hyperthermia in North America from 1987 to 2006. Anesth Analg 2010; 110:498-507
  • RS Litman, CD Flood, RF Kaplan, YL Kim, JR Tobin. Postoperative Malignant Hyperthermia; An Analysis of Cases from the North American Malignant Hyperthermia Registry. Anesthesiology 2008; 109:825-9
  • Larach MG, Brandom BW, Allen GC, Gronert GA, Lehman EB. Cardiac arrests and deaths associated with malignant hyperthermia in North America from 1987 to 2006. Anesthesiology 2008; 108:603-11
  • Newmark JL, Voelkel M, Brandom BW, Wu J. Delayed onset of malignant hyperthermia without creatine kinase elevation in a geriatric, ryanodine receptor type 1 gene compound heterozygous patient. Anesthesiology 2007; 107(2):350-3
  • JM Burkman, KL Posner, KB Domino. Analysis of the Clinical Variables Associated with Recrudescence after Malignant Hyperthermia Reactions. Anesthesiology 2007; 106:901-6
  • Fink EL, Brandom BW, Torp KD. Heat stroke in the super-sized athlete. Pediatric Emergency Care 2006; 22: 510-3
  • Sambuughin N. Holley H, Muldoon S, Brandom BW, de Bantel AM, Tobin JR, Nelson TE, Goldfarb LG. Screening of the entire ryanodine receptor type 1 coding region for sequence variants associated with malignant hyperthermia susceptibility in the North American Population. Anesthesiology 2005; 102:515-521
  • Sei Y, Sambuughin NN, Davis EJ, Sachs D, Cuenca B, Brandom BW, Tautz T, Rosenberg H, Nelson TE, Muldoon SM. Malignant hyperthermia in North America; Genetic screening of the three hot spots in the type I ryanodine receptor gene. Anesthesiology 2004; 101:824-830
  • Muldoon SM, Deuster P, Brandom BW, Bunger R: Is There a Link between Malignant Hyperthermia and Exertional Heat Illness. Exercise and Sport Sciences Reviews 2004; 32:174-179
  • Sei Y, Sambuughin NN, Davis EJ, Sachs D, Cuenca PB, Brandom BW, Tautz T, Rosenberg H, Nelson TE, Muldoon SM: Malignant Hyperthermia in North America: Genetic Screening of the Three Hot Spots in the Type I Ryanodine Receptor Gene. Anesthesiology 2004; 101:824-830
  • Sei Y, Brandom BW, Bina S, Hosio E, Gallagher KL, Wyre HW, Pudimat PA, Holman SJ, Venzon DJ, Daly JW, Muldoon SM: Patients with malignant hyperthermia demonstrate an altered calcium control mechanism in B lymphocytes. Anesthesiology 2002; 97:1052-1058
  • Allen GC, Larach MG, Kunselman AR, The North American MH Registry: The sensitivity and specificity of the caffeine halothane contracture test. Anesthesiology 1998; 88:579-588
  • Albrecht A, Wedel DJ, Gronert GA: Masseter muscle rigidity and non depolarizing neuromuscular blocking agents. Mayo Clin Proc 1997; 72:329-332
  • Larach MG, Rosenberg H, Gronert GA, Allen GC: Hyperkalemic cardiac arrest during anesthesia in infants and children with occult myopathies. Clin Pediatr (Phila) 1997; 36:9-16
  • Antognini JF: Creatine kinase alterations after acute malignant hyperthermia episodes and common surgical procedures. Anesth Analg 1995; 81:1039-1042
  • Larach MG, Localio AR, Allen GC, Denborough MA, Ellis FR, Gronert GA, Kaplan RF, Muldoon SM, Nelson TE, Ording H, et al: A clinical grading scale to predict malignant hyperthermia susceptibility. Anesthesiology 1994; 80:771-779
  • Larach MG, Landis JR, Bunn JS, Diaz M, The North American MH Registry: Prediction of malignant hyperthermia susceptibility in low-risk subjects. Anesthesiology 1992; 76:16-27
  • Larach MG: Standardization of the caffeine halothane muscle contracture test, North American Malignant Hyperthermia Group. Anesth Analg 1989; 69:511-515

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