Related Experiment Videos
Malignant hyperthermia: pathophysiology, clinical presentation, and treatment
1University of Maryland, School of Nursing, Baltimore, USA. mcca003@son.umaryland.edu
AACN Clinical Issues
|October 6, 2004
Summary
Malignant hyperthermia (MH) is a rare inherited disorder triggered by anesthesia. Early identification of susceptible individuals and preparedness can prevent hypermetabolic crises and reduce mortality.
Area of Science:
- Anesthesiology
- Genetics
- Pharmacology
Background:
- Malignant hyperthermia (MH) is a life-threatening inherited disorder first described in 1960.
- Significant advancements have been made in clinical management, diagnosis, and understanding MH pathophysiology.
- Current MH mortality is less than 10%, with potential for further reduction through enhanced preparedness.
Purpose of the Study:
- To review the current understanding of malignant hyperthermia.
- To cover pathophysiology, diagnosis, clinical presentation, and treatment of MH.
Main Methods:
- Review of current literature on malignant hyperthermia.
- Analysis of molecular genetic studies related to MH.
- Synthesis of information on clinical management and patient identification.
Main Results:
- MH is triggered by specific anesthetic agents, causing uncontrolled calcium release from skeletal muscle sarcoplasmic reticulum.
- This leads to a hypermetabolic state.
- Abnormalities in the ryanodine receptor are implicated in MH pathophysiology.
Conclusions:
- MH episodes can be avoided in known susceptible individuals by using non-triggering anesthetic agents.
- Improved MH preparedness is crucial for further reducing mortality.
- Understanding the genetic basis and pathophysiology aids in diagnosis and management.