Skip to main content
SpringerLink
Log in

Maligne Hyperthermie

Allgemeine, klinische und experimentelle Aspekte

  • Chapter
Weiterbildung für Anästhesisten 2002

  • 84 Accesses

Zusammenfassung

Bei der malignen Hyperthermie (MH) handelt es sich um eine genetisch determinierte, latente Anomalie, die nach Exposition mit MH-Triggersubstanzen (volatile Inhalations-anästhetika und depolarisierende Muskelrelaxanzien vom Typ des Succinylcholins) auf zellulärer Ebene durch eine Dysregulation der myoplasmatischen Kalziumhomöostase charakterisiert ist. Der „Dihydropyridin-Ryanodin-Rezeptorkomplex“ der Skelettmuskulatur, der einen wesentlichen Einfluss auf die myoplasmatische Kalziumregulation ausübt, wird als hauptverantwortlicher Strukturkomplex für das MH-Syndrom angesehen. Die hypermetabole Stoffwechselentgleisung als Konsequenz der myoplasmatischen Kalziumüberladung im Rahmen einer MH-Krise kann sich bei allen Menschenrassen und verschiedenen Tierspezies manifestieren. Das klinische Bild einer MH-Episode kann sehr vielfältig sein, wobei Hyperkapnie und Herzrhythmusstörungen zu den Frühsymptomen gehören und der Temperaturanstieg eher ein Spätsymptom ist. Mit der Einführung des Hydantoinderivats Dantrolen konnte die Letalitätsrate fulminanter MH-Verläufe von 70–80% auf unter 10% reduziert werden.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 54.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 69.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Literatur

  1. Ball SP, Johnson KJ (1993) The genetics of malignant hyperthermia. J Med Genet 30:89–93

    Article  PubMed  CAS  Google Scholar 

  2. Berridge MJ (1993) Inositol trisphosphate and calcium signalling. Nature 361:315–325

    Article  PubMed  CAS  Google Scholar 

  3. Brandt A, Schleithoff L, Jurkat-Rott K, Klingler W, Baur C, Lehmann-Horn F (1999) Screening of the ryanodine receptor gene in 105 malignant hyperthermia families: novel mutations and concordance with the in vitro contracture test. Hum Mol Genet 8:2055–2062

    Article  PubMed  CAS  Google Scholar 

  4. Censier K, Urwyler A, Zorzato F, Treves S (1998) Intracellular calcium homeostasis in human primary muscle cells from malignant hyperthermia-susceptible and normal individuals. J Clin Invest 101:1233–1242

    Article  PubMed  CAS  Google Scholar 

  5. Deufel T, Sudbrak R, Feist Y et al. (1995) Discordance, in a malignant hyperthermia pedigree, between in vitro contracture-test phenotype and haplotypes for the MHSI region on chromosome 19q12–13.2,comprising the C1840 T transition in the RYRI gene. Am J Hum Genet 56:1334–1342

    PubMed  CAS  Google Scholar 

  6. Ducart A, Adnet P, Renaud B, Riou B, Krivosic-Horber R (1995) Malignant hyperthermia during sevoflurane administration. Anesth Analg 80:609–611

    PubMed  CAS  Google Scholar 

  7. Ellis FR, Harriman DGF, Keaney NP, Kyei-Mensah K, Tyrrell JH (1971) Halothane-induced muscle contracture as a cause of hyperpyrexia. Br J Anaesth 43:721–722

    PubMed  CAS  Google Scholar 

  8. European Malignant Hyperthermia Group (1984) A protocol for the investigation of malignant hyperpyrexia (MH) susceptibility. Br J Anaesth 56:1267–1269

    Article  Google Scholar 

  9. Fill M, Coronado R, Mickelson JR, Vilven J, Ma JJ, Jacobson BA, Louis CF (1990) Abnormal ryanodine receptor channels in malignant hyperthermia. Biophys J 57:471–475

    Article  PubMed  CAS  Google Scholar 

  10. Fujii J, Otsu K, Zorzato F et al. (1991) Identification of a mutation in porcine ryanodine receptor associated with malignant hyerthermia. Science 253:448–451

    Article  PubMed  CAS  Google Scholar 

  11. Galloway GJ, Denborough MA (1986) Suxamethonium chloride and malignant hyperthermia. Br J Anaesth 58:447–450

    Article  PubMed  CAS  Google Scholar 

  12. Gillard EF, Otsu K, Fujii J et al. (1991) A substitution of cysteine for arginine 614 in the ryanodine receptor is potentially causative of human malignant hyperthermia. Genomics 11:751–755

    Article  PubMed  CAS  Google Scholar 

  13. Harrison GG (1975) Control of malignant hyperpyrexia syndrome in MHS swine by dantrolene sodium. Br J Anaesth 47:62–65

    Article  PubMed  CAS  Google Scholar 

  14. Härtung E, Horbaschek H, Olthoff D et al. (1998) Die regionale Verbreitung der Maligne Hyperthermie Veranlagung in Deutschland: Stand 1997.Anasthesiol Intensivmed Notfallmed Schmerzther 33:238–243

    Article  PubMed  Google Scholar 

  15. Hopkins PM, Ellis FR, Halsall PJ (1991) Ryanodine contracture: a potentially specific in vitro diagnostic test for malignant hyperthermia. Br J Anaesth 66:611–613

    Article  PubMed  CAS  Google Scholar 

  16. Isaacs H, Badenhorst M (1993) False-negative results with muscle caffeine halothane contracture testing for malignant hyperthermia. Anesthesiology 79:5–9

    Article  PubMed  CAS  Google Scholar 

  17. Kalow W, Britt BA, Terrau ME, Haist C (1970) Metabolic error of muscle metabolism after recovery from malignant hyperthermia. Lancet 11:895–898

    Article  Google Scholar 

  18. Lai FA, Erickson HP, Rousseau E, Liu QY, Meissner G (1988) Purification and reconstitution of the calcium release channel from skeletal muscle. Nature 331:315–319

    Article  PubMed  CAS  Google Scholar 

  19. Larach MG, Localio AR, Allen GC (1994) A clinical grading scale to predict malignant hyperthermia susceptibility. Anesthesiology 80:771–779

    Article  CAS  Google Scholar 

  20. Löscher W, Witte U, Fredow G, Ganter M, Bickhardt K (1990) Pharmacodynamic effects of serotonin (5-HT) receptor ligands in pigs: stimulation of 5-HT2 receptors induces malignant hyperthermia. Naunyn-Schmiedeberg’s Arch Pharmacol 34:483–493

    Google Scholar 

  21. MacLennan DH, Phillips MS (1992) Malignant hyperthermia. Science 256:789–794

    Article  PubMed  CAS  Google Scholar 

  22. MacLennan DH, Duff C, Zorzato F et al.(1990) Ryanodine receptor gene is a candidate for predisposition to malignant hyperthermia. Nature 343:559–561

    Article  PubMed  CAS  Google Scholar 

  23. Mauritz W, Sporn P, Steinbreitner K (1986) Mafigne Hyperthermie in Österreich, I. Epidemiology und Klinik. Anaesthesist 5:639–650

    Google Scholar 

  24. McCarthy TV, Healy JMS, Heffron JJA et al. (1990) Localization of the malignant hyperthermia susceptibility locus to human chromosome 19q12–13.2. Nature 343:562–564

    Article  PubMed  CAS  Google Scholar 

  25. McCarthy TV, Quane KA, Lynch PJ (2000) Ryanodine receptor mutations in malignant hyperthermia and central core disease. Hum Mutat 15:410–417

    Article  PubMed  CAS  Google Scholar 

  26. Meier-Hellmann A, Römer M, Hannemann L, Kersting T, Reinhart K (1990) Früherkennung einer malignen Hyperthermie durch Capnometrie. Anaesthesist 39:41–43

    PubMed  CAS  Google Scholar 

  27. Meissner G (1994) Ryanodine receptor/Ca2+release channel and their regulation by endogenous effectors. Annu Rev Physiol 56:485–508

    Article  PubMed  CAS  Google Scholar 

  28. Mignery GA, Südhof TC, Takei K, Camilli PD (1989) Putative receptor for inositol 1,4,5-trisphosphate similar to ryanodine receptor. Nature 342:192–195

    Article  PubMed  CAS  Google Scholar 

  29. Nelson TE (1983) Abnormality in calcium release from skeletal sarcoplasmic reticulum of pigs susceptible to malignant hyperthermia. J Clin Invest 72:862–872

    Article  PubMed  CAS  Google Scholar 

  30. Ording H (1985) Incidence of malignant hyperthermia in Denmark. Anesth Analg 64:700–704

    PubMed  CAS  Google Scholar 

  31. Ording H, Brancadoro V, Cozzolino S et al. (1997) In vitro contracture test for diagnosis of malignant hyperthermia following the protocol of the European MH Group: Results of testing patients surviving fulminant MH and unrelated low-risk subjects. Acta Anaesthesiol Scand 41:955–966

    Article  PubMed  CAS  Google Scholar 

  32. Parness J, Palnitkar SS (1995) Identification of dantrolene binding sites in porcine skeletal muscle sarcoplasmic reticulum. J Biol Chem 270:18465–18472

    Article  PubMed  CAS  Google Scholar 

  33. Quane KA, Healy JMS, Keating KE et al. (1993) Mutations in the ryanodine receptor gene in central core disease and malignant hyperthermia. Nature Genet 5:51–55

    Article  PubMed  CAS  Google Scholar 

  34. Reber A, Schumacher P, Urwyler A (1993) Effects of three different types of management on the elimination kinetics of volatile anaesthetics. Anaesthesia 48:862–865

    Article  PubMed  CAS  Google Scholar 

  35. Richthofen V von, Wappler F, Scholz J, Fiege M, Schulte am Esch J (1998) Evaluierung von Maligne Hyperthermie-Episoden mit der Clinical Grading Scale. Anasthesiol Intensivmed Notfallmed Schmerzther 33:244–249

    Article  Google Scholar 

  36. Roewer N (1991) Maligne Hyperthermie heute. Anasthesiol Intensivmed Notfallmed Schmerzther 26:431–449

    Article  PubMed  CAS  Google Scholar 

  37. Sambuughin N, Sei Y, Gallagher KL et al. (2001) North american malignant hyperthermia population: Screening of the ryanodine receptor gene and identification of novel mutations. Anesthesiology 95:594–599

    Article  PubMed  CAS  Google Scholar 

  38. Steinfath M, Scholz J, Singh S, Wappler F (1996) Welche Bedeutung haben Genotypveränderungen in der Diagnostik der malignen Hyperthermia? Anasthesiol Intensivmed Notfallmed Schmerzther 31:334–343

    Article  PubMed  CAS  Google Scholar 

  39. Urwyler A, Hartung E (1994) Die Maligne Hyperthermie. Anaesthesist 43:557–569

    Article  PubMed  CAS  Google Scholar 

  40. Wappler F (2001) Malignant hyperthermia. Eur J Anaesthesiol 18:632–652

    PubMed  CAS  Google Scholar 

  41. Wappler F, Roewer N, Köchling A et al. (1995) Effekte von Serotonin-2-Rezeptoragonisten auf Skelettmuskelpräparate von Patienten mit Disposition zu maligner Hyperthermie. Anaesthesist 44:238–244

    Article  Google Scholar 

  42. Wappler F, Roewer N, Köchling A, Scholz J, Steinfath M, Schulte am Esch J (1996) In vitro-diagnosis of malignant hyperthermia susceptibility with ryanodine-induced contractures in human skeletal muscles. Anesth Analg 82:1230–1236

    PubMed  CAS  Google Scholar 

  43. Wappler F, Scholz J, Richthofen V von, Fiege M, Steinfath M, Schulte am Esch (1997) 4-Chloro-m-Cresol induziert Kontrakturen an Skelettmuskelpräparaten von Patienten mit Disposition zu maligner Hyperthermie. Anasthesiol Intensivmed Notfallmed Schmerzther 32:541–548

    Article  PubMed  CAS  Google Scholar 

  44. Wappler F, Fiege M, Steinfath M, Agarwal K, Scholz J, Singh S, Matschke J, Schulte am Esch J (2001) Evidence for susceptibility to malignant hyperthermia in patients with exercise-induced rhabdomyolysis. Anesthesiology 94:95–100

    Article  PubMed  CAS  Google Scholar 

  45. Wedel DJ, Nelson TE (1994) Malignant hyperthermia — diagnostic dilemma: false-negative contracture responses with halothane and caffeine alone. Anesth Analg 78:787–792

    PubMed  CAS  Google Scholar 

  46. Wedel DJ, laizzo PA, Milde JH (1991) Desflurane is a trigger of malignant hyperthermia in susceptible swine. Anesthesiology 74:508–512

    Article  PubMed  CAS  Google Scholar 

  47. Wedel DJ, Gammel SA, Milde JH, laizzo PA (1993) Delayed onset of malignant hyperthermia induced by isoflurane and desflurane compared with halothane in susceptible swine. Anesthesiology 78:1138–1144

    Article  PubMed  CAS  Google Scholar 

  48. Wingard DW (1974) Malignant hyperthermia: a human stress syndrome? Lancet 11:1450–1451

    Article  Google Scholar 

  49. Zhao F, Li P, Chen SRW, Louis CF, Fruen BR (2001) Dantrolene inhibition of ryanodine receptor Ca2+release. J Biol Chem 276:13810–13816

    PubMed  CAS  Google Scholar 

  50. Zorzato F, Fujii J, Otsu K et al. (1990) Molecular cloning of cDNA encoding human and rabbit forms of the Ca2+release channel (ryanodine receptor) of skeletal muscle sarcoplasmic reticulum. J Biol Chem 265:2244–2256

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Klinik für Anästhesiologie und Operative Intensivmedizin, Universitätsklinikum Kiel, Schwanenweg 21, 24105, Kiel, Deutschland

    Priv.-Doz. Dr. Markus Steinfath

  2. Klinik für Anästhesiologie, Universitätsklinikum Hamburg-Eppendorf, Deutschland

    F. Wappler

  3. Klinik für Anästhesiologie und Operative Intensivmedizin, Universitätsklinikum Kiel, Deutschland

    Priv.-Doz. Dr. Markus Steinfath & J. Scholz

Authors
  1. Priv.-Doz. Dr. Markus Steinfath
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. F. Wappler
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. J. Scholz
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Klinik für Anästhesiologie, Universität Heidelberg, Im Neuenheimer Feld 110, D-69120, Heidelberg, Deutschland

    Hubert J. Bardenheuer

  2. Klinik für Anästhesiologie und operative Intensivmedizin Zentralklinikum, Stenglinstraße 2, D-86156, Augsburg, Deutschland

    Helmuth Forst

  3. Klinik für Anästhesiologie, Medizinische Einrichtungen der RWTH Aachen, Pauwelsstraße 30, D-52074, Aachen, Deutschland

    Rolf Rossaint

  4. Service d’Anesthésiologie Centre Hospitalier, Universitaire Vaudois - CHUV, Rue du Bugnon 46, CH-1011, Lausanne, Schweiz

    Donat R. Spahn

Rights and permissions

Reprints and permissions

Copyright information

© 2003 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Steinfath, M., Wappler, F., Scholz, J. (2003). Maligne Hyperthermie. In: Bardenheuer, H.J., Forst, H., Rossaint, R., Spahn, D.R. (eds) Weiterbildung für Anästhesisten 2002. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-10888-8_4

Download citation

  • DOI: https://doi.org/10.1007/978-3-662-10888-8_4

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-00092-1

  • Online ISBN: 978-3-662-10888-8

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation