[Immunohistochemical findings in rhabdomyosarcoma].
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Biomedical subjects
Publications and source records attributed to R Moeller.
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Malignant hyperthermia (MH) is a pharmacogenetic disorder. It is classically described as a hypermetabolic state triggered by halogenated anaesthetics and/or depolarizing muscle relaxants. In fact, since Denborough and Lovel's case, it has been shown that MH has a great number of clinical forms. The overwhelming picture of muscular hypercatabolism with fulminating hyperthermia and generalized rigidity is becoming rare. A better knowledge of the first symptoms explains in part the better prognosis: masseter spasm after suxamethonium, an increase in expired CO2 concentration, unexplained tachycardia, ventricular arrhythmias. The use of dantrolene reduced the mortality of MH. The different types of clinical manifestations are due to genetic differences, the concentration of the anaesthetic agent, and the length of time of exposure to the drug. The severity of the episode is linked to environmental factors such as stress, physical exercise, ambient temperature, concomitant use of other drugs. Masseter spasm after suxamethonium is specific for MH, but not pathognomonic. It occurs in 1% of cases in children when using halothane with suxamethonium. However, in those patients who displayed such a spasm, more than 50% had a positive contracture test. Masseter spasm is often associated with severe rhabdomyolysis in patients with muscle dystrophy, especially Duchenne's dystrophy. In the latter case, major cardiac problems may occur at the time of anaesthetic induction. Even if there are no other signs of MH, all patients who have had a masseter spasm must be considered as open to doubt, and should be further explored. MH is often difficult to diagnose in medium severity types.(ABSTRACT TRUNCATED AT 250 WORDS)
The results are reported of the contracture test obtained by using the calcium ionophore A 23187 (Calcimycin) in two patients, the son (A) and the mother (B). The past history of patient A revealed the occurrence of an impending malignant hyperthermia crisis during induction of anaesthesia in 1975. The A 23187 contracture test confirmed the presence of the malignant hyperthermia trait in patient A, whose muscle biopsy had been submitted to the caffeine contracture test, according to Ellis' technique, and found to be positive in the presence of 1 mmol of caffeine in the tissue bath; the contracture test could not be completed with a halothane contracture test, due to the impaired viability of the muscle fibres obtained. On the reverse, the mother (patient B) was found to be negative to the tests applied: the caffeine contracture test, the halothane contracture test and the contracture test performed with the calcium ionophore A 23187. It is suggested the A 23187 contracture test be added to the contracture tests as defined by the European Malignant Hyperpyrexia Group. This test could be of great help in identifying the percentage of relatives of MHS ("malignant hyperthermia susceptible") patients found to be MHE ("malignant hyperthermia equivocal").
A search of the French and English language literature of the last 20 years (1964-1984) yielded 975 cases of immediate anaphylactoid reactions due to anaesthetic drugs given parenterally. The accident mechanism was confirmed in only half the patients, and nearly always at a later date. The immunoallergological tests most often used in the diagnostic process were skin tests and Prausnitz-Küstner tests. Although all drugs could be responsible for an anaphylactic reaction, the greatest number of cases was due to muscle relaxants (51%) and hypnotic drugs (42.3%). The signs most often described were cutaneous, cardiovascular, respiratory and occasionally gastro-intestinal. Whilst hypnotic drugs were responsible for cutaneous signs, muscle relaxants gave cardiovascular signs. A past history of drug allergy was found in 37% of cases, and atopy in 38%; virtually all patients had already had one or more anaesthetics.
A computer-aided arrangement is described which allows kinetic and regulative studies with enzymes, organelles and cells in an open system. This is demonstrated with some simple examples.
Eighteen grave anaphylactic accidents arising during induction or during anaesthesia, are analysed. By order of frequency, the clinical signs are cutaneous manifestations (70 p. 100 of the cases), bronchospasm (65 p. 100), cardiovascular collapse (45 p. 100). In four cases, cardiac arrest occurred. The course was always favourable, with no after-effects. Immuno-allergic tests enabled one to make the diagnosis of true anaphylaxis in eight cases (seven shocks due to succinylcholine, one due to propanidide), of non specific histamine-liberation in five cases; in the other cases, it was impossible to express an opinion.
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