1 / 17

Malignant Hyperthermia

Malignant Hyperthermia. Ri 劉家宏 劉志中. Definition. An uncommon pharmacogenetic disorder of skeletal muscle autonomic dominant inheritance 1:40,000 in adults 1:15,000 in children

razi
Télécharger la présentation

Malignant Hyperthermia

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Malignant Hyperthermia Ri 劉家宏 劉志中

  2. Definition • An uncommon pharmacogenetic disorder of skeletal muscle • autonomic dominant inheritance • 1:40,000 in adults • 1:15,000 in children • From Basic of anesthesia 3th edition , Robert K. Stoelting

  3. Background • MH was first described by Dr Michael Denbo-rough in 1960. • Manawatu-Horowhenua Region in New Zealand • In the 1840s when a whaler landed in Wellington • eight generations with at least 1500 family members From www.pnh.org

  4. Background • MH affects other species. • In 1966 a veterinarian submitted an article to the British Medical Journal entitled "Unusual reaction to Suxamethonium, Thiopentone and Halothane" • Mortality rate has reduced from 80% to less than 5% world wide in the last twenty years From www.pnh.org

  5. Aetiology • The anaesthetic drugs that have been definitely • succinylcholine • all of the anaesthetic gases • halothane, enflurane, isoflurane sevoflurane and desflurane- not nitrous oxide • Central core disease and King denborough syndrome are the only known associated disorders.

  6. Clinical picture • rigidity of the muscles • HR , RR • Temp. 39-40oC ( 46oC.) • From www.faseb.org

  7. Clinical picture • Capnogram • Gradually increasing EtCO2

  8. Pathophysiology An abnormal contraction state of the muscles • From www.mdausa.com

  9. Pathophsiology • An increase in metabolic needs • Muscle cell die and release chemicals into the blood stream • an unstable heart rhythm and cardiac arrest • an inability of the lungs to oxygenate the blood properly • kidney failure requiring dialysis. From Ultrastructural Studies of skeletal muscle of the pig with Malignant Hyperthermia

  10. Differential Diagnosis • Neuroleptic malignant syndrome • For sedation , psychiatric practice and also for nausea and vomiting • Heat-stroke or exercise-induced muscle breakdown

  11. Differential Diagnosis • MDMA(搖頭丸/快樂丸) • 在安全劑量時，其抑制病況強度不佳，故沒有多大的醫學價值 • 害怕、憂鬱或產生妄想之精神病症狀 • 惡性高溫症候群(全身性血液凝結，肌肉崩解，腎衰竭及肝毒性) • 這些症狀會因使用者體力透支而惡化，特別是過度的跳舞及運動後 From www.homepage.renren.com

  12. Diagnosis • Muscle biopsy test • a fascicle of quadriceps muscle • halothane and caffeine • contracture status of the muscle From www.faseb.org

  13. Diagnosis • Biochemical tests • the specificity and sensitivity was unacceptable

  14. Diagnosis • DNA-based tests • the gene encoding the formation of the calcium release channel • Ryanodine receptor gene (RYR1) • the chromosomal region 19q13.1 • altered amino acid residues in the skeletal muscle ryanodine receptor • Over 30 mutations have been described affecting <50% of all known susceptible families. From A complex satellite DNA polymorphism fanking the ryanodine receptor gene (RYR1)

  15. Reference • Book • Basic of anesthesia 3th edition , Robert K. Stoelting • Clinical Anesthesia 2nd edition , G. Edward morgan, Jr. Maged , S.Mikhail • Website • www.pnh.org • www.homepage.renren.com • www.oridin.com • www.mdausa.com • www.faseb.org

  16. Reference • Original article • A complex satellite DNA polymorphismflanking the ryanodine receptor gene (RYR1) • Werner Wolz, Ute Wendelmuth, Sylvie Rouquier#, Dominique Giorgi# and Clemens R. M • Ultrastructural Studies of skeletal muscle of the pig with Malignant Hyperthermia • David L. Liu, Stefan Andersson-Engels, Katarina Svanberg, Ingrid Wang, Christian Sturesson, Carl H. Håkansson and Sune Svanberg

  17. Thanks to your attention

More Related