malignant hyperthermia
Noun | 1. | malignant hyperthermia - hereditary condition in which certain anesthetics (e.g., halothane) cause high body temperatures and muscle rigidity |
单词 | malignant hyperthermia | |||
释义 | malignant hyperthermia
malignant hyperthermiahyperthermia[hi″per-ther´me-ah]ma·lig·nant hy·per·ther·mi·amalignant hyperthermia, susceptibility to, 1An autosomal dominant (OMIM:145600) condition of variable penetration in which a patient, when subjected to certain anaesthetics (halothane, diethyl-ether, cyclopropane, enflurane and psychotropics), develops a potentially fatal reaction (up to 70% mortality in acute episodes), which occurs in 1/15,000 administrations of anaesthesics in children and 1/50–100,000 in adults; 1/2 of cases had not been previously sensitive to anaesthesia. Anaesthetised patients may develop high fever and muscle rigidity with rhabdomyolysis, release of myoglobin, renal damage and acute renal failure.Clinical findings Tachycardia, tachypnoea, cyanosis, labile blood pressure, muscle rigidity, rapid and marked hyperpyrexia, acidotic, hyperkalemic, possibly DIC, renal failure; similar reactions may be evoked in these patients by warm weather, exercise, emotional stress or without known environmental cues, and are initiated by muscular hypermetabolism due to an idiopathic increase in sarcoplasmic calcium occurring under general anaesthesia. Diagnosis Muscle contraction test with halothane or caffeine challenge. Management Hypothermia, hydration, sodium bicarbonate infusion, mechanical hyperventilation, diuretics to increased urine flow, dantrolene (an agent that blocks excitation-contraction coupling between T tubules and sarcoplasmic reticulum). Molecular pathology Caused by defects in RYR1, which encodes ryanodine receptor 1, a calcium channel that mediates the release of Ca2+ from the sarcoplasmic reticulum and plays a key role in triggering muscle contraction. ma·lig·nant hy·per·ther·mi·a(mă-lig'nănt hī'pĕr-thĕr'mē-ă)malignant hyperthermiaA rare inherited muscle disorder that leads to dangerously high fever from intense muscle contraction when the affected person is given a general anaesthetic drug such as halothane, cyclopropane or ethyl ether, or a muscle relaxant drug such as succinylcholine. Emergency treatment to cool the patient, to neutralize the rapid rise in blood lactic acid from the muscles, and to reverse the abnormal muscle response, is needed to save life.Malignant hyperthermiama·lig·nant hy·per·ther·mi·a(mă-lig'nănt hī'pĕr-thĕr'mē-ă)malignant hyperthermia
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