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

This paper presents a patient showing both the unusual syndrome of catamenial pneumothorax and a strong family history of malignant hyperthermia. The anaesthetic management is described and discussed.
...
PMID:Catamenial pneumothorax and malignant hyperthermia. 62 7

We studied 27 Duchenne muscular dystrophy patients having spinal fusion for scoliosis. One patient died intraoperatively of cardiac arrest; all others have done well with no instances of malignant hyperthermia, postoperative ventilatory system dependence, pneumothorax, persisting infection, neurologic damage, nonunion, or pain. The anesthetic management included primarily intravenous general anesthetics with minimal myocardial depressant effects, avoiding succinylcholine and inhalation agents. Preoperative cardiac studies aided anesthetic management intra-operatively. There was an almost universal sinus tachycardia. Holter monitoring defined 4 of 16 with ventricular premature beats, 4 of 16 with atrial premature beats, and no ventricular tachycardia or atrial flutter or fibrillation. Echocardiogram demonstrated mitral prolapse in 2 of 22, frequent abnormal systolic performance with abnormal shortening fraction less than 28% in 7 of 16, and reduced rate-corrected velocity of fiber shortening in 9 of 15. Afterload was elevated in 7 of 15. The mean forced vital capacity (FVC) preoperatively was 45.3 +/- 15.9% with continuing diminution to 28.7 +/- 14.9% at 3.3 +/- 2.2 years after surgery. The main benefit of surgical stabilization is the relative ease and comfort of wheelchair seating compared with those nonoperated patients who develop progressive deformity. We have not seen lasting improvement or stabilization in FVC following surgery as decreasing function is related primarily to muscle weakness.
...
PMID:Spinal fusion in Duchenne muscular dystrophy: a multidisciplinary approach. 158 53

Anaesthetists are called upon to manage complex life-threatening crises at a moment's notice. As there is evidence that this may require cognitive tasking beyond the information-processing capacity of the human brain, it was decided to try and develop a generic crisis management algorithm analogous to the "Phase I" immediate response routine used by airline pilots. Such an algorithm, based on the mnemonic "COVER ABCD, A SWIFT CHECK", was developed and refined over 3 meetings, each attended by 60-100 anaesthetists and aviation psychologists. It was validated against 1301 relevant incidents among the first 2000 incidents reported to the Australian Incident Monitoring Study. It proved sufficiently robust and safe to recommend its general use as an initial response to any incident or crisis which occurs when a patient is breathing gas from an anesthetic machine. It requires a limited knowledge base and is easily learnt and rehearsed during the anaesthetist's working day. It will provide a functional diagnosis in over 99% of cases and will correct 62% of the problems in 40-60 seconds. In the remaining 37% it will allow the anaesthetist to proceed with a "sub-algorithm", confident in the knowledge that some important step has not been missed. In just over 30% of incidents this will be for a problem familiar to all anaesthetists (e.g. laryngospasm, bradycardia); in just over 6% it will be for a less common, more complex, but finite, set of problems (3% cardiac arrest, 1% air embolism, 1% anaphylaxis, 1% for the remaining desaturations); in less than 1% diagnosis and correction will require a more complex checklist (e.g. for malignant hyperthermia, pneumothorax). The next stage, the development of specific sub-algorithms and a structured team approach for ongoing problems, is in progress.
...
PMID:The Australian Incident Monitoring Study. Crisis management--validation of an algorithm by analysis of 2000 incident reports. 827 79

The pathological investigation of deaths following surgery, anaesthesia, and medical procedures is discussed. The definition of "postoperative death" is examined and the classification of deaths following procedures detailed. The review of individual cases is described and the overall approach to necropsy and interpretation considered. There are specific sections dealing with the cardiovascular system (including air embolism, perioperative myocardial infarction, cardiac pacemakers, central venous catheters, cardiac surgery, heart valve replacement, angioplasty, and vascular surgery); respiratory system (postoperative pneumonia, pulmonary embolism, pneumothorax); central nervous system (dissection of cervical spinal cord), hepatobiliary and gastrointestinal system; musculoskeletal system; and head and neck region. Deaths associated with anaesthesia are classified and the specific problems of epidural anaesthesia and malignant hyperthermia discussed. The article concludes with a section on the recording of necropsy findings and their communication to clinicians and medicolegal authorities.
...
PMID:Acp. Best practice no 155. Pathological investigation of deaths following surgery, anaesthesia, and medical procedures. 1096 Nov 86

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.
...
PMID:[The surgical treatment of pulmonary and associated echinococcosis in childhood]. 1083 13