Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024591 (malignant hyperthermia)
 2,353 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We encountered a case of malignant hyperthermia caused by intravenous lidocaine which had been administered as treatment for a ventricular arrhythmia. The patient, a 72-year-old male, was admitted with chronic renal failure and aortic valvular stenosis. His chronic renal failure progressed, and congestive heart failure developed, and ventricular arrhythmias occurred frequently. For the treatment of these arrhythmias, lidocaine was injected and continuous infusion was started. Despite initial improvement in symptoms and laboratory data following hemofiltration, refractory ventricular tachycardia occurred. The patient was treated with large doses of lidocaine. His body temperature rose to a maximum of 41.7 degrees C, and generalized muscular twitching was observed before he lost consciousness. Serum and urinary myoglobin levels became elevated. This abnormally high fever was relieved only by dantrolene sodium. After we made a diagnosis of malignant hyperthermia and stopped the lidocaine infusion, the high fever resolved quickly. It is important to note that malignant hyperthermia can be caused by lidocaine and amide-linked local anesthetics.
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PMID:Malignant hyperthermia caused by intravenous lidocaine for ventricular arrhythmia. 147 68

We report a series of 22 children with complete atrioventricular canal (CAVC) operated upon before the age of one year. The youngest patient was 1 month old and weighed 3 kg. The patients' mean age was 7 months and their mean weight was 5.4 kg. 15 patients had trisomy 21, and in one patient the CAVC was associated with tetralogy of Fallot. The remaining 21 patients had congestive heart failure resistant to medical treatment, with clinical evidence of pulmonary arterial hypertension (PAHT). At the time of surgery, 2 patients had been under artificial respiration for one month. All patients were explored by echocardiography and cardiac catheterization. The mean pulmonary pressure/aortic pressure ratio was 0.92; the mean pulmonary flow rate/systemic flow rate ratio (Qp/Qs) was 2.9/1 and the mean pulmonary resistance/systemic resistance ratio (Rp/Rs) was 0.22. All children were operated upon under deep hypothermia with circulatory arrest (mean 54 min); the patient with tetralogy of Fallot had an additional period of extracorporeal circulation. Fourteen patients had Rastelli's type A CAVC and 8 had type C CAVC. All were operated upon by the classical Rastelli technique, using a single autologous pericardial patch; in none of the patients was the septal "slit" or "commissure" entirely closed. Three patients died within 48 hours of the operation: the first one died of sudden low cardiac output 18 hours after surgery, the second one of persistent PAHT and the third one of malignant hyperthermia. The patient under artificial respiration before surgery could not be disconnected and died on the 30th post-operative day.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Repair of complete atrioventricular canal before one year of age]. 250 95

P31 nuclear magnetic resonance spectroscopy (NMRS) is a new noninvasive method for the study of normal and diseased muscle. Significant differences between the spectra of normal and pathological human muscles were reported in some known myopathies and in patients with congestive heart failure. The possibility that metabolic disturbances are linked to contraction abnormalities led to the investigation of malignant hyperthermia susceptible (MHs) muscle with NMRS. At rest, no differences were found between patients and normal subjects. During exercise, and recovery, MHs exercise hyperthermic patients developed an abnormal P31 spectral configuration, indicating a defective energy metabolism. This technique is of great interest, and could be used in patients with muscle diseases of unknown origin. NMRS with P31 and, simultaneously, other nuclei (C13, H1) could refine the results already obtained.
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PMID:[New research on muscular function: NMR spectroscopy. Application to malignant hyperthermia]. 262 42

Ryanodine receptors (RyR) are the Ca2+ release channels of sarcoplasmic reticulum that provide the majority of the [Ca2+] necessary to induce contraction of cardiac and skeletal muscle cells. In their cellular environment, RyRs are exquisitely regulated by a variety of cytosolic factors and accessory proteins so that their output signal (Ca2+) induces cell contraction without igniting signaling pathways that eventually lead to contractile dysfunction or pathological cellular remodeling. Here we review how dysfunction of RyRs, most commonly expressed as enhanced Ca2+ release at rest (skeletal muscle) or during diastole (cardiac muscle), appears to be the fundamental mechanism underlying several genetic or acquired syndromes. In skeletal muscle, malignant hyperthermia and central core disease result from point mutations in RYR1, the skeletal isoform of RyRs. In cardiac muscle, RYR2 mutations lead to catecholaminergic polymorphic ventricular tachycardia and other cardiac arrhythmias. Lastly, an altered phosphorylation of the RyR2 protein may be involved in some forms of congestive heart failure.
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PMID:Ryanodine receptor channelopathies. 1533 75