Anästhesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie : AINS
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Anasthesiol Intensivmed Notfallmed Schmerzther · Sep 2019
[Pharmacological Treatment of Malignant Hyperthermia: Update 2019].
Malignant hyperthermia (MH) is a pharmacogenetic disorder of skeletal muscle presenting as a hypermetabolic response to potent volatile anesthetics such as sevoflurane, desflurane, isoflurane and the depolarizing muscle relaxant succinylcholine. Following introduction of the hydantoin derivative dantrolene, the previously high mortality of fulminant MH episodes could be reduced from > 80% to less than 10%. For treatment of MH an initial intravenous bolus of 2.5 mg/kg dantrolene based on the actual body weight should be applied. ⋯ If none of these agents are ever used in the facility, the stockage of dantrolene is dispensable. Since dantrolene is not easily dissoluble, preparation requires time and manpower. Due to its pharmacological characteristics, ryanodex, a modern nanocrystalline dantrolene sodium suspension, might be a promising alternative in the treatment of MH.
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Anasthesiol Intensivmed Notfallmed Schmerzther · Sep 2019
[Malignant Hyperthermia - Update on Pathophysiology, Diagnostics and Treatment].
Malignant hyperthermia (MH) is a rare, heterogenic syndrome leading to potentially life-threatening skeletal muscle hypermetabolism following exposure to inhalational anesthetics and succinylcholine. In more than 50% of affected individuals a pathogenic variant in the RYR1 gene coding for the sarcoplasmic reticulum calcium channel is responsible for the underlying pathology of uncontrolled calcium liberation. ⋯ A predisposition to MH should be investigated in patients following a suspected MH crisis, in relatives from MH-families, after exertional or unexplained perioperative rhabdomyolysis and in patients with idiopathic hyper-CK-aemia. According to recent European guidelines, initial DNA screening is an alternative to muscle biopsy and in-vitro contracture testing, although in cases where no diagnostic variants are found, only contracture testing can safely exclude predisposition to MH.
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Anasthesiol Intensivmed Notfallmed Schmerzther · Sep 2019
[Malignant Hyperthermia - Diagnosis in Practice].
Testing for malignant hyperthermia (MH) susceptibility is usually performed either in relatives of MH susceptible persons or in patients with an MH suspicious clinical event. There are two diagnostic options: muscle biopsy with in-vitro contracture testing and molecular genetic diagnosis. Patients with familial MH mutations are usually tested genetically. ⋯ MH susceptibility can be confirmed by presence of a diagnostic MH mutation. Absence of MH mutations cannot exclude MH. The only option to exclude MH susceptibility is contracture testing.
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Anasthesiol Intensivmed Notfallmed Schmerzther · Sep 2019
["Status Thymolymphaticus" - a Risk of Anaesthesia?]
The administration of narcotics such as ether and chloroform to combat pain during surgery was overshadowed from the beginning by serious, often fatal incidents. Even the use of local anaesthetics, which were praised as being less dangerous, was not as uncomplicated as had initially been hoped for and was often fatal. Investigations into the various causes of anaesthetic incidents were carried out at an early stage, but usually did not lead to clear results. ⋯ If, in case of a fatal anaesthetic incident, the event was attributed to the presence of this anomaly, one also spoke of "thymus death" and usually refrained from further legal investigations. Corresponding statements therefore acquired a great forensic significance, since now also in cases of a "mors subita" in infants or small children in the domestic sphere unjustly accused persons in the presence of a verifiable thymus hyperplasia did not have to be prosecuted further under criminal law. In operative medicine, the "status thymolymphaticus" has become a topic for decades, especially among the persons performing the anaesthesia, so that a historical review of the feared alleged syndrome appears justified.
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An indication for a medical intervention alone is not sufficient to justify its implementation. In addition, consent has to be obtained from the patient who has been given relevant information by a doctor. If, instead of the patient, other persons are entitled to decide for him (parents for children incapable of consent, authorised representatives, carers), they must be informed. ⋯ Consent and clarification are also verbally effective, written form is strongly recommended for reasons of preserving evidence. However, the patient's right to self-determination also means that the patient can expressly dispense with more detailed information. Such a waiver should be carefully documented.