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Excess comorbidities associated with malignant hyperthermia diagnosis in pediatric hospital discharge records

Guohua Li1, Joanne E Brady, Henry Rosenberg

  • 1Department of Anesthesiology, Columbia University College of Physicians and Surgeons, 622 West 168th Street, New York, NY 10032, USA. GL2240@columbia.edu

Paediatric Anaesthesia
|July 5, 2011
PubMed

Insights

Malignant hyperthermia (MH) in children is significantly linked to musculoskeletal and circulatory system diseases, and congenital anomalies. Muscular dystrophies show the strongest association, warranting further research into MH susceptibility.

Area of Science:

  • Pediatric Anesthesiology
  • Medical Genetics
  • Health Informatics

Background:

  • Malignant hyperthermia (MH) is a rare, life-threatening pharmacogenetic disorder of skeletal muscle.
  • Previous case reports suggest associations between MH and various genetic conditions.
  • Quantitative data on comorbidities in pediatric MH cases are limited.

Purpose of the Study:

  • To quantitatively assess the excess comorbidities associated with a diagnosis of malignant hyperthermia (MH) in pediatric hospital discharge records.
  • To identify specific disease groups and conditions that are disproportionately represented in children with MH.

Main Methods:

  • Utilized data from the Kids' Inpatient Database (KID) for 2000, 2003, and 2006, encompassing a large sample of US pediatric hospital discharges.
  • Calculated standardized morbidity ratios (SMRs) and 95% confidence intervals (CIs) comparing children with MH diagnosis to the general pediatric inpatient population.
  • Analyzed comorbidities across major disease groups and specific medical conditions.

Main Results:

  • Out of nearly 6 million pediatric discharges, 175 had an MH diagnosis.
  • Children with MH showed significantly higher rates of musculoskeletal/connective tissue diseases (SMR 5.7), circulatory system diseases (SMR 3.3), and congenital anomalies (SMR 3.2).
  • Muscular dystrophies were the most strongly associated specific diagnosis (SMR 31.3).

Conclusions:

  • Significant associations exist between malignant hyperthermia (MH) and diseases of the musculoskeletal system, connective tissue, and circulatory system in children.
  • Congenital anomalies are also more prevalent in pediatric MH cases.
  • Further investigation is needed to explore the clinical utility of these comorbidities in assessing MH susceptibility.
Abstract

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