Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024591 (malignant hyperthermia)
 2,353 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Myotonia is defined as a persistent contraction of skeletal muscles after their stimulation. This contracture is not prevented or relieved by regional anaesthesia or muscle relaxants. The sensitivity to non-depolarizing muscle relaxants is usually normal. Suxamethonium, neostigmine, hypothermia, a rise in kalaemia should be avoided. There have been case reports of malignant hyperthermia in patients with myotonia congenita. Dystrophia myotonica is the second most frequent of the inherited muscle diseases, after Duchenne's dystrophy. The severity of the disease is due more to the muscular atrophy and the multiple organ involvement than to the abnormal contraction. Atrioventricular heart block and dysrhythmias are more common than heart failure. Prolonged apnoea and pneumonia are the main risks of anaesthesia. In severe cases, exists a restrictive respiratory insufficiency which is preceded by a fall in the maximum expiratory pressure. Dysphagias and inefficient coughing may occur early. An increased susceptibility to hypnotic drugs and opiates is a common feature. Spontaneous sleep apnoeas should be sought before anaesthesia, especially by using pulse oximetry. The anaesthetic implications are reemphasized.
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PMID:[Anesthesia in myotonia]. 253 24

Induction, emergence and recovery characteristics were compared during sevoflurane or halothane anaesthetic in a large (428) multicentre, international study of children undergoing elective inpatient surgical procedures. Two hundred and fourteen children in each group underwent inhalation induction with nitrous oxide/oxygen and sevoflurane or halothane. Incremental doses of either study drug were added until loss of eyelash reflex was achieved. Steady state concentrations of anaesthesia were maintained until the end of surgery when anaesthetic agents were terminated simultaneously. Time variables were recorded for induction, emergence and the first need for analgesia in the recovery room. In addition, in 86 of the children in both groups, venous blood samples were drawn for plasma fluoride levels during and after surgery. There was a trend toward smoother induction (induction of anaesthesia without coughing, breath holding, excitement laryngospasm, bronchospasm, increased secretion, and vomiting) in the sevoflurane group with faster induction (2.1 min vs 2.9 min, P = 0.037) and rapid emergence times (10.3 min vs 13.9 min, P = 0.003). Among the children given sevoflurane, 2% developed bradycardia compared with 11% in the halothane group. Postoperatively, 46% of the children in the halothane group developed nausea and or vomiting versus 31% in the sevoflurane group (P = 0.002). Two children in the halothane group developed cardiac dysrhythmia and were dropped from the study. In addition, a child in the halothane group developed malignant hyperthermia, received dantrolene, and had an uneventful recovery. Mean maximum inorganic fluoride concentration was 18.3 microM.l-1. The fluoride concentrations peaked within one h of termination of sevoflurane anaesthetic and returned rapidly to baseline within 48 h. This study suggests that sevoflurane may be the drug of choice for the anaesthetic management of children.
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PMID:A comparison of sevoflurance to halothane in paediatric surgical patients: results of a multicentre international study. 882 44

Over a nine-year period (1990-1998), a total of 196 children with pulmonary and associated hydatid disease, aged 2 to 16 years, undergo treatment in the Department of Pediatric Thoracic Surgery--Emergency Medicine Institute "N. I. Pirogov"--Sofia. One hundred thirty children (62.1%) present isolated, and sixty-six (38.8%)--associated hydatid disease, of which with localization in the liver (63 cases), brain (1), kidney (1) and spleen (1). The pulmonary cysts are located in the left lung in 96 cases (48.9%), right lung--79 (40.3%) and bilateral involvement--21 (10.8%). In 74 children the disease runs an asymptomatic course, in 128 (65.3%)--with fever and cough, and in 65 (33.1%)--with thoracic pain. On admission 183 children (94.4%) are in a satisfactory and good general condition; 13 with complications in the cyst and pleural cavity are in a serious general condition accompanied by respiratory failure. In 129 children diagnosis is made on the ground of x-ray study, in 40 CAT study is additionally performed, and in 24--ultrasonography. Of 22 children with bilateral pulmonary location, 13 (59.9%) undergo operation in two stages, and nine (40.1%)--in one stage, with bilateral thoracotomy done in seven, and sternotomy in two instances. Intraoperatively more than one echinococcus cysts are discovered in 23 cases (11.7%). In 105 children (53.5%) echinococcotomy is performed, in 67 (34.2%)--atypical resection, in 8 (4.1%)--lobectomy, in 15 (7.6%)--segmentectomy and in one--pneumonectomy. In the early postoperative period, one child develops hemothorax from a bleeding intercostal artery, and another one--pneumothorax from unsutured bronchus in the cyst bed necessitating emergency re-thoracotomy. The outcome is fatal in one patient with malignant hyperthermia, and the remainder are discharged clinically cured.
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PMID:[The surgical treatment of pulmonary and associated echinococcosis in childhood]. 1083 13