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)

The purpose of the present investigation was to determine the normal perioperative variations in the serum concentration of creatine phosphokinase (CPK) and its isoenzymes MM, MB, and BB, and of lactic dehydrogenase (LDH) and its isoenzymes LDH1 to LDH5 to distinguish operation-induced changes in these enzymes from those due to acute myocardial infarction or malignant hyperthermia. In 30 patients, 52 to 75 years of age undergoing elective orthopedic operations, 10 serial blood samples were obtained in the perioperative period: two samples before skin incision and eight samples after the incision over a time span of 70 hours. The preinduction mean serum CPK level of 141 U/L increased gradually and significantly and reached a maximum mean concentration of 809 U/L 34 hours after incision (p less than 0.01). The CPK-MM percent increased after incision, whereas that of CPK-MB and CPK-BB decreased, although their absolute values in terms of U/L rose. The preinduction mean serum LDH value of 173 U/L increased gradually after incision and achieved peak levels at 34 hours (203 U/L) and 58 hours (210 U/L) after incision (p less than 0.05). The LDH1:LDH2 ratio did not change. The LDH5 percent increased and peaked 10 hours after incision (p less than 0.05). There was a significant correlation between severity of operation-induced tissue damage and the serum CPK concentration (p less than 0.001). The large increase in total CPK (primarily MM fraction) occurring after surgery may minimize the percentile effects caused by an increase in MB level due to myocardial infarction.
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PMID:Serum creatine phosphokinase, lactic dehydrogenase, and their isoenzymes in the perioperative period. 262 1

Multicore myopathy is a rare congenital myopathy. The multicores consist of numerous small areas of decreased oxidative enzyme activity. The long axis of the lesion is perpendicular or parallel to the long axis of the muscle fiber. These cores are usually smaller than central cores. For this reason they are also called minicores. Although the multicores represent a nonspecific change in that they can be observed in malignant hyperthermia, muscular dystrophy, inflammatory myopathy, etc. Muscular weakness dating from early infancy is combined large proportion of the muscle fibers. In about half of the reported cases the muscular weakness has not been progressive, while in the others a slow progression has occurred. This 9-year-old boy presented with congenital nonprogressive myopathy associated with thoracic scoliosis and bilateral equinovarus deformity. The serum creatine phosphokinase and lactic dehydrogenase levels were normal. Electromyography showed "myopathic" features. The biopsy revealed a marked size variation in myofibers, ranging from 10 microns to 100 microns. A few small angular fibers and slight endomyseal fibrosis were also noted. There was type I fiber predominance. NADH-TR reaction disclosed more well-defined cores with loss of intermyofibrillary mitochondrial activity. These cores were usually located with loss of intermyofibrillary mitochondrial activity. These cores were usually located in the peripheral portions of the myofibers and the core size measured 10-30 microns in diameter. Electron microscopic examination revealed circumscribed areas of disintegrated Z band material and disorganized sarcomeric units near the sarcolemma. A decrease in the number of mitochondria and glycogen particles was noted.
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PMID:Multicore myopathy--a case report. 819 69

Monolayers of the porcine-derived renal epithelial cell line, LLC-PK1, were used to characterize the effects of heat stress on Na+-glucose cotransport. Transepithelial current dependent on 5 mM glucose (I(Glc)), phloridzin-sensitive current (I(phz)), and total transepithelial current (I(total)) were measured as indicators of Na+-glucose cotransport. Severe heat shock (SHS; 45 degrees C for 1 h, then 37 degrees C for measurements) decreased transepithelial electrical resistance (TER), I(Glc), I(phz), and I(total) 50-70%. Mild heat shock (MHS; 42 degrees C for 3 h, then 37 degrees C for 12 h) induced accumulation of 72-kDa heat shock protein (HSP-72), decreased damage to TER from SHS, and prevented damage to I(Glc), I(phz), and I(total). Kinetic analysis showed that SHS damaged and MHS protected total Na+-glucose transport capacity (Vmax of I(Glc)). MHS alone increased TER (50%), I(Glc) (20%), I(total) (20%), and Vmax of I(Glc) (25%). On enhancement of the Na+ gradient by depletion of intracellular Na+, MHS increased I(Glc) 50% and had no effect on transepithelial Na+-dependent sulfate reabsorptive flux measured concurrently or in Na+-replete tissues. These effects of MHS were not reflected in effects on cell survival or luminal membrane surface area as indicated by lactate dehydrogenase or alkaline phosphatase release. In conclusion, HSP-72-inducing heat treatment both protected and enhanced Na+-glucose cotransport independently of the luminal membrane Na+ gradient and selectively with respect to effects on TER, reabsorptive sulfate transport, cell survival, and luminal membrane surface area.
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PMID:Heat shock-induced protection and enhancement of Na+-glucose cotransport by LLC-PK1 monolayers. 936 30

The authors submit the case-history of a 29-year-old man, followed up on account of liver steatosis with a toxic-nutritional etiology who developed, after previous increased physical exertion and alcohol abuse, fever associated with major muscular weakness. Gradually he developed an amental delirious state which was evaluated as suspect delirium tremens. Fever of 40-41 degrees C continued, the patient developed muscular rigidity, tremor and hypotension. After intubation during which succinylcholine was administered, the patient's condition deteriorated further with a rise of temperature and muscular rigidity. The patient developed acute renal failure with anuria and the necessity of repeated haemodialyses and severe acidosis of the mixed type on account of which he was intubated and switched to artificial ventilation. According to the case-history clinical and laboratory picture of the disease (extremely high creatine kinase activity, hyperkalaemia, acidosis, hepatorenal failure) malignant hyperthermia was suspected. After a single intravenous injection of sodium dantrolene, 2.5 mg/kg, the temperature dropped and within 24 hours the patient was afebrile. Gradually the acidosis improved, the blood pressure became stabilized and artificial ventilation was no longer used. The patient was discharged after 34 days in hospital in a state of cardiopulmonary compensation with mild polyuria but without signs of retention of nitrogenous substances with sideropenic anaemia and marginal creatine kinase and lactate dehydrogenase values. Within one month after discharge the laboratory values reached normal levels and only slight muscular weakness and greater fatiguability persisted.
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PMID:[An attack of malignant hyperthermia caused by a combination of the effects of succinylcholine, increased physical exertion and alcohol abuse]. 1095 47

A case of rhabdomyolysis from malignant hyperthermia occurred during renal transplantation surgery is presented. After the completion of vascular and uretherovesical anostomosis, the patient's heart rate began to rise, sweatiness was observed and body temperature increased to 41 degrees C. Additionally, metabolic and respiratory acidosis and hyperkalemia were detected. Serum creatine kinase and lactic dehydrogenase levels were increased significantly. After external cooling and the administration of dantrolene sodium, body temperature and heart rate were decreased. During this period; furosemide, mannitol and sodium bicarbonate were given. Three hours after the completion of surgery, urine output was begun and urine myoglobin was found to be positive. Renal function improved gradually and serum creatinine level decreased to 1.6 mg/dl on the 14th postoperative day. Malignant hyperthermia can lead to severe rhabdomyolysis and delayed graft function in renal transplant recipients. Early diagnosis and intervention is crucial for protecting renal function.
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PMID:Severe rhabdomyolysis due to malignant hyperthermia during renal transplantation procedure can cause delayed graft function. 1191 7

The perioperative changes in the serum concentration of creatine phosphokinase (CPK) and its isoenzymes MM, MB, and BB and of lactic dehydrogenase (LDH) and its isoenzymes LDH1 to LDH5 were determined during craniotomy in order to distinguish operation-induced changes in these enzymes from those due to acute myocardial infarction and malignant hyperthermia. Twenty-eight male patients, 29 to 76 years of age (mean +/- SD = 58 +/- 13.2 years), undergoing craniotomy for tumor reseaction (n = 26) or cerebral artery aneurysm clipping (n = 2) were included in this study. Ten serial blood samples were obtained from each patient: one sample before and another after induction of anesthesia, and eight samples after the incision, over a period of 70 h. The preinduction serum CPK level of 97 +/- 32 U/L (mean +/- SD) increased gradually and significantly and reached the peak level of 542 +/- 116 U/L 34 h after incision (p <0.05). Whereas all of the CPK isoenzymes increased in terms of U/L after incision, only the MM fraction (expressed as percent of total CPK) increased, and the MB and BB fractions (expressed as percent of total CPK) decreased. The preinduction serum LDH level of 150 +/- 42 U/L (mean +/- SD) increased gradually after incision and reached the peak level of 210 +/- 32 U/L 58 h after incision (p <0.05). LDH2 as a percent of total LDH decreased significantly, but the LDH1/LDH2 ratio did not change. LDH4 and LDH5, as percents of total LDH, increased significantly. The large increases in total serum CPK and the concomitant decrease in MB percent after craniotomy may minimize and/or mask the percentage increase in the MB level following acute myocardial infarction. The perioperative serum CPK level as a marker in the diagnosis of malignant hyperthermia should be interpreted in light of the present results and in conjunction with clinical symptomatology.
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PMID:Changes in serum CPK, LDH, and their isoenzymes in the perioperative period in patients undergoing craniotomy. 1581 62

Longissimus muscle samples from the pig genotypes Duroc (Du), Pietrain (MHS homozygote negative (PiNN), positive (PiPP)) and a Duroc-Pietrain crossbreed (DuPi) were analyzed. The PiPP samples showed a faster pH drop and higher electrical conductivity, drip loss and lightness values. Before slaughter the concentrations of the adenine nucleotides were comparable between the genotypes, but 40 min after slaughter (p.m.) the ATP concentrations decreased and IMP increased, to a higher extent in the PiPP pigs. The nucleotide values of the 12 h p.m. samples were again comparable. Activities of glycogen phosporylase (GP), phosphofructokinase (PFK) and lactate dehydrogenase (LDH) were nearly similar before slaughter. Forty minutes after slaughter the LDH activities increased in all pigs and the PFK activities in all genotypes but not in the PiPP. GP results were rather inconsistent indicating an earlier activation of this enzyme. The study showed that the reduced meat quality in the PiPP pigs is accompanied with rapid ATP degradation and accelerated enzyme activation.
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PMID:Adenine nucleotide concentrations and glycolytic enzyme activities in longissimus muscle samples of different pig genotypes collected before and after slaughter. 2159 77

We present 2 cases of fulminant malignant hyperthermia (MH), complicated with massive rhabdomyolysis. The patients were successfully treated in the intensive care unit of our university teaching hospital, despite the lack of availability of dantrolene in our country, by early application of continuous veno-venous hemofiltration (CVVH). Both male patients developed fulminant malignant hyperthermia during anesthesia for oromaxillofacial surgery. CVVH was employed when the values of creatine phosphokinase (CPK), myoglobin (Mb), and lactate dehydrogenase (LDH) increased significantly. After emergency treatment and CVVH therapy, the values of CPK, Mb, and LDH in the blood plasma of the patients decreased significantly. The complications, including acute renal failure, disseminated intravascular coagulation, and acute respiratory distress syndrome were also treated without any obvious organ damage. Early detection and management are the keys to treat MH successfully. CVVH is a valuable therapeutic application in the initial/critical management of severe rhabdomyolysis. If these complications occur even with initial treatment with dantrolene, our experiences may be useful adjunctive treatments to consider.
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PMID:Continuous veno-venous hemofiltration for massive rhabdomyolysis after malignant hyperthermia: report of 2 cases. 2350 80