The malignant hyperthermia represents seldom found disease which cornerstone the genetic predisposition is and which is characterized by uncontrollable fervescence of the patient in response to hit in a drug trigger organism. The patient has strengthening of oxidizing metabolism in cross-striped muscles that leads to sharp increase of consumption of oxygen, education in excess amounts of carbonic acid and to fervescence. This state is followed by very high lethality if remains not distinguished and timely events for its stopping are not held.
The predisposition to this pathology is descended, the principle of inheritance — autosomal and dominant. The clinical picture of a malignant hyperthermia usually develops or during the anesthesia, or after its end.
Substances which can be carried to triggers of a malignant hyperthermia quite a lot, but the main role in this list belongs to inhalation anesthetics and a suktsinilkholin (Dithylinum). The prevalence of a malignant hyperthermia is about one case on 4,500-60,000 general anesteziya. It should be noted that this pathology meets irrespective of race and it is widespread everywhere.
The pathogeny of a malignant hyperthermia consists in a mutation of a receptor of a rianodin owing to what there is an emission of calcium in much bigger quantity at depolarization. It is shown that various anomalies of skeletal muscles, such as hernias, scoliosis, squint and so on, do not result in the increased risk of emergence of a malignant hyperthermia. There is a weak communication between risk of emergence of a malignant hyperthermia and muscular dystrophies.
The clinical picture of a malignant hyperthermia can be both very typical, and atypical. In the first case diagnosis usually does not cause difficulties, but in the second can be considerably complicated. Specific symptoms of this state do not exist. The malignant hyperthermia, as a rule, is followed by sharp increase of concentration of carbon dioxide on an exhalation that usually is registered by means of a kapnografiya. Besides, fervescence to very high figures is often noted. This state is usually accompanied by tachycardia and the mixed respiratory and metabolic acidosis. But it is necessary to remember that the rigidity of masseters, separate groups of muscles or muscles as all body can become the only sign of a malignant hyperthermia. Fervescence usually is late sign of this state.
The spasm of chewing muscles allows to suspect development of a malignant hyperthermia even if it in the anamnesis is absent kakaye-or data on a similar state at the patient's relatives. At the same time it is necessary to distinguish the expressed spasm from short compression of jaws which sometimes meets at patients. Emergence of a long spasm of chewing muscles dictates immediate refusal of use of drugs triggers and also the beginning of implementation of the protocol of stopping of a malignant hyperthermia. In the absence of the emergency indications operation is postponed. After awakening of the patient to it and members of his family appoint the corresponding inspection regarding identification of predisposition to a malignant hyperthermia.
At emergence of suspicion on development in the patient of a malignant hyperthermia it is necessary to be guided by an algorithm of its stopping. First, it is necessary to stop immediately supply of inhalation anesthetics and to replace the narcotic device to get rid of impurity of inhalation anesthetic in a contour. Provide a hyperventilation of 100% with oxygen for the purpose of removal of carbon dioxide. The stream of fresh gas thus has to exceed minute ventilation of the patient and to be 10 l/min and more. Secondly, the patient should enter dantrolen as soon as possible — the only drug for treatment of a malignant hyperthermia. Its molecular mechanism of action is not clear, but dantrolen influences receptors of a rianodin, reducing release of calcium from a sarcoplasmic reticulum of skeletal muscles. It prevents increase in intracellular concentration of calcium. Drug is badly soluble and for its cultivation in a critical situation the additional help will be necessary. Thirdly, it is necessary to begin active cooling of the patient at fervescence higher than 39 degrees: infusion of cold solutions, cold on area of large vessels and other ways of cooling. Cooling is stopped at decrease in body temperature below 38 degrees.
At successful stopping of an attack in the subsequent carry out treatment of consequences of a malignant hyperthermia: rabdomioliza, hyperpotassemias, IDCS and so on.