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)

Effort rhabdomyolysis is a syndrome which takes the form of a pathology with different etiopathogenetic stages. General anesthesia may trigger off acute muscular cytolysis which is probably influenced by the inflammatory surgical pathology itself. The observation of two cases of post-anesthetic rhabdomyolysis following muscular stress suggest that the exhaustion of muscle energy reserves and the consequent alteration of the ATP/ADP ratio may act as a trigger mechanism for this syndrome. Its close relationship with malignant hyperthermia makes a precise anamnesis vitally important, especially in relation to recent intense physical effort in patients undergoing emergency surgery for acute pathologies.
Minerva Anestesiol 1992 Dec
PMID:[Post-anesthesia rhabdomyolysis syndrome following muscular stress. 2 case reports]. 129 23

Malignant hyperthermia may be a human stress syndrome, of which heat stroke is one manifestation. Two men in military service who had episodes of exertional heat stroke, and their immediate family members, were tested for susceptibility to malignant hyperthermia by in-vitro contracture tests on skeletal muscle samples. Muscle from both patients had a normal response to caffeine but an abnormal response to halothane. Muscle from the father of one patient had an abnormal response to halothane, and that from the father of the second patient had an abnormal response to ryanodine. The results indicate that clinical heat stroke may be associated with an underlying inherited abnormality of skeletal muscle that is similar, but not identical, to that of malignant hyperthermia.
Lancet 1991 Dec 14
PMID:Evidence for related myopathies in exertional heat stroke and malignant hyperthermia. 134 34

We have studied the effects of the calcium agonist BAY K 8644 on the in vitro halothane test in 10 malignant hyperthermia-susceptible (MHS), 12 MH "equivocal" to halothane (MHEh), 30 MH non-susceptible (MHN) and 10 control patients. BAY K 8644 potentiated the halothane-induced contracture in muscle strips from both MHS and MHEh patients. The drug produced a more obvious difference in contracture responses between the MHEh group compared with the MHN and control groups.
Br J Anaesth 1990 Dec
PMID:Use of the calcium agonist BAY K 8644 for in vitro diagnosis of susceptibility to malignant hyperthermia. 170 3

Addition of dantrolene 8.5 x 10(-5) M caused a mono-exponential decay of the depolarization contractures caused by inhibition of the sarcolemmal Na,K-ATPase with propranolol 1 mM or by depolarization of the sarcolemma and T tubular membranes with KCl 100 mM. The half-times of the inhibitory effects were 6 s for the propranolol contracture and 11 s for the KCl contracture. The inhibition of both contractures was complete. Inhibition of the caffeine (10 mM) contracture was bi-exponential with half-times of 45 s and 9.5 min. Inhibition was incomplete; 29.6 +/- 5.0% of the contracture tension could not be inhibited. The inhibition of twitch contractions was similar to that of the caffeine contracture, with half-times of 48 s and 9.1 min, and 20.6 +/- 1.2% of the initial twitch tension could not be inhibited. The contracture tensions induced by release of Ca from the mitochondria with dicumarol, and by actin-myosin binding with the sulfhydryl inhibitor, N-ethyl-maleimide, could not be inhibited by dantrolene. The present results indicate that dantrolene inhibits depolarization signals from the sarcolemma and the T tubular membranes, in addition to inhibition of the coupling between the T tubules and the sarcoplasmic reticulum, and of the release of Ca from the sarcoplasmic reticulum. All these effects of dantrolene may contribute to its therapeutic effect in malignant hyperthermia.
Eur J Pharmacol 1991 Dec 10
PMID:Separate sites for the dantrolene-induced inhibition of contracture of the rat diaphragm preparation due to depolarization or to caffeine. 172 87

A yeast artificial chromosome (YAC) library has been constructed from a somatic cell hybrid containing a t(1p;19q) chromosome and chromosome 17. After amplification, part of this library was analyzed by high-density colony filter screening with a repetitive human DNA probe (Alu). The human YACs distinguished by the screening were further analyzed by Alu fingerprinting and Alu PCR. Fluorescent in situ hybridization (FISH) was performed to localize the YACs to subchromosomal regions of chromosome 1p, 17, or 19q. We have obtained a panel of 123 individual YACs with a mean size of 160 kb, and 77 of these were regionally localized by FISH: 33 to 1p, 10 to 17p, 25 to 17q, and 9 to 19q. The YACs cover a total of 19.7 Mb or 9% of the 220 Mb of human DNA contained in the hybrid. No overlapping YACs have yet been detected. These YACs are available upon request and should be helpful in mapping studies of disease loci, e.g., Charcot-Marie-Tooth disease, Miller-Dieker syndrome, hereditary breast tumor, myotonic dystrophy, and malignant hyperthermia.
Genomics 1991 Dec
PMID:Generation and fluorescent in situ hybridization mapping of yeast artificial chromosomes of 1p, 17p, 17q, and 19q from a hybrid cell line by high-density screening of an amplified library. 178 77

Besides offering a concise recapitulation of known facts, experiences and related viewpoints concerning malignant hyperthermia (MH), the present article offers a review of the most relevant new aspects in this field. Special emphasis is on genetics and pathogenesis of MH. The contents of the review are as follows: History; Definition of MH; Epidemiological aspects; Inheritance; Molecular genetics; Pathogenesis; Triggering agents; Awake Triggering; Sympathetic nervous system; Serotoninergic system; Involvement of other organs and cell systems; Clinical symptoms and diagnosis; MH and myopathies; Associated disorders; Treatment; Prophylaxis; Identification of susceptibility; MH testing centres in the FRG; Hot-line for MH emergencies
Anasthesiol Intensivmed Notfallmed Schmerzther 1991 Dec
PMID:[Malignant hyperthermia today]. 178 3

There are recent reports that inositol phosphate metabolism is involved in the development of malignant hyperthermia (MH). Consequently, we investigated the basal concentration of inositol phosphate products in skeletal and heart muscles of malignant hyperthermia-susceptible (MHS) and healthy control (MHN) swine. Different inositol phosphates were measured by high pressure liquid chromatography, including inositol trisphosphate, tetrakisphosphate, pentakisphosphate and hexakisphosphate. All inositol phosphate products measured had a higher concentration in MHS than MHN in skeletal (304-1330%) as well as heart muscles (134-440%). An activation of the inositol phosphate metabolism has been shown to mobilise intracellular calcium from the sarcoplasmic reticulum. It is therefore concluded that, firstly, besides involvement of the skeletal muscles a primary myocardial abnormality in MHS is possible; and secondly, the idea that the inositol phosphate metabolism could be involved in the development of MH is additionally supported.
Anasthesiol Intensivmed Notfallmed Schmerzther 1991 Dec
PMID:[Malignant hyperthermia and inositol phosphate metabolism in the heart and skeletal musculature]. 178 4

In accordance with the protocol of the European Malignant Hyperpyrexia Group, an account is given of the experience regarding establishment of the "in vitro contracture test" for diagnosis and exclusion of malignant hyperpyrexia disposition at the University Clinic of Leipzig. Since its commencement in 1986 these diagnostic possibilities are being increasingly utilized, and--basing on 76 MH manifestations and suspected cases--324 persons have been examined until now. Besides the representation of the methodical features (e.g. performance of biopsy in tranquanalgesia in children), emphasis was on the safety of executing this test (attaining higher specificity, total absence of MH manifestations). The epidemiological study of the results shows the varying knowledge of physicians and the interference by insufficient investigation alternatives in individual areas. On the basis of the results, the use of the in vitro contracture test is recommended in all MH suspected cases by the authorized and qualified regional centres.
Anasthesiol Intensivmed Notfallmed Schmerzther 1991 Dec
PMID:[A new emphasis on the diagnosis of malignant hyperthermia using the in vitro contracture test?]. 178 5

The halothane-caffeine contracture test is presently the most well-established method for identification of malignant hyperthermia susceptibility (MHS) or non-susceptibility (MHN). However, 10-20% of the patients tested are classified as equivocal (MHE), i.e. their susceptibility remains uncertain. A genetic disorder of the calcium releasing ryanodine receptor has been postulated recently. Therefore, 12 patients were tested in addition to the protocol of the European Malignant Hyperthermia Group (EMHG) for dose- and time-dependent contracture after ryanodine application. In this study, contracture of 0.2g appeared significantly earlier in MHS patients (17.5 +/- 1.7 min; n = 5) during cumulative ryanodine exposition (0.4-0.8-1.6-10.0 mumol/l) than in MHN (38.2 +/- 5.4 min; n = 5). A significant difference between MHS (10.0 +/- 1.7 min; n = 6) and MHN (19.8 +/- 0.6 min; n = 3) was also seen after bolus application of ryanodine (10.0 mumol/l). One patient classified as MHE according to the EMHG protocol, manifested as MHN after the ryanodine contracture test. This study supports previous work suggesting the ryanodine contracture test as an improvement in the in-vitro diagnosis of MH susceptibility.
Anasthesiol Intensivmed Notfallmed Schmerzther 1991 Dec
PMID:[Ryanodine-induced contractures for the diagnosis of malignant hyperthermia susceptibility]. 178 6

Two of the persisting controversies concerning malignant hyperthermia (MH) are discussed: storing and dosage of dantrolene and preoperative tactics to be followed with patients who are MH-susceptible. Reasons are presented for the obligation to store sufficient amounts of dantrolene in every operating suite. The second part discusses the question of pretreatment of MH-susceptible patients with dantrolene.
Anasthesiol Intensivmed Notfallmed Schmerzther 1991 Dec
PMID:[An attempt to reconcile differences of opinion in the field of malignant hyperthermia]. 178 7


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