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)

The occurrence in a previously healthy infant of sudden febrile encephalopathy with shock and metabolic acidosis associated with liver and renal failure and bloody watery diarrhea has been reported by several authors in recent years. We report the same association in a 3 1/2 month-old infant. We discuss the similarities between this clinical picture and malignant hyperthermia and Reye syndrome.
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PMID:[Hemorrhagic shock syndrome with encephalopathy]. 357 85

A comparative study of two patients, one affected by haemorrhagic shock and encephalopathy (HSE) and the other by heatstroke is reported. Both presented shock, disseminated intravascular coagulation, neurological damage and hepatopathy. A lowered alpha 1-antitrypsin concentration as well as a slightly increased circulating immune complexes and complement consumption were observed in the HSE patient but not in the heatstroke one. In both, cultures for bacteria were negative, the viral serology was non-specific and hepatitis A and B studies were negative. HSE patient died. A possible relationship between HSE, heatstroke, malignant hyperthermia and halothane hepatitis is postulated. Fever, potentially hepatotoxic drugs or unknown agents (HSE) might trigger this clinical picture.
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PMID:[Hemorrhagic shock and encephalopathy. Its possible relation with heat stroke]. 407 88

An infant with haemorrhagic shock encephalopathy syndrome (HSES) who in addition presented with hyperpyrexia and myoglobinuria is reported. As rhabdomyolysis is a feature of heat stroke and malignant hyperthermia, the association of HSES with myoglobinuria supports the hypothesis that HSES may be a form of hypermetabolic state triggered by hyperthermia.
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PMID:Haemorrhagic shock encephalopathy syndrome presenting with myoglobinuria. 866 84

MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) is one of the classic mitochondrial encephalomyopathies with variable clinical presentation and multisystem involvement. Enhanced sensitivity to neuromuscular blockade or anesthetic agents and susceptibility to malignant hyperthermia in these patients have ever been reported, all of which complicate the management of general anesthesia. To avoid these appalling troubles in general anesthesia, we chose spinal anesthesia for a patient with MELAS syndrome receiving appendectomy. The patient obtained adequate anesthesia and good recovery without neurologic sequelae. Although there is little information about the application of regional anesthesia in MELAS patients, we demonstrate that it may be a satisfactory choice. However, it is suggested that regional anesthesia is performed only when neurological abnormalities of spinal cord or peripheral nerves are definitely ruled out.
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PMID:Spinal anesthesia in MELAS syndrome: a case with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes. 1100 Jun 76

A 6-year-old boy with a rare mitochondrial disease (MELAS: mitochondrial encephalopathy, lactic acidosis, stroke-like episodes) was presented to undergo adenoid resection and bilateral paracentesis. ENT surgery was performed without complications under general anaesthesia using propofol, fentanyl, and ventilation with nitrous oxide and oxygen. Routine intraoperative monitoring (ECG, noninvasive blood pressure, oxymetry and capnometry) was supplemented by frequent body temperature measurements and repeated laboratory analysis of venous blood gases, lactate, and glucose. Clinically, the postoperative course was uneventful and the boy was discharged from hospital on the first postoperative day. Signs or symptoms of malignant hyperthermia never occurred. Laboratory analysis only showed a remarkable serum lactate elevation postoperatively (6 mmol/l) which decreased on the first postoperative day (3.7 mmol/l). The present anaesthesiologic experiences with MELAS-syndrome are limited, and recommendations are mainly based on case reports. Careful preoperative physical examination with special regard to all available medical records, and anaesthetic management comparable with that in malignant hyperthermia susceptible resulted in an uneventful course in our patient. Pathogenetic aspects of mitochondrial diseases focussing on anaesthetic considerations are briefly discussed.
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PMID:[Anesthesia in mitochondrial encephalomyopathies]. 1149 20

A 23-year-old woman with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) underwent a laparoscopy-assisted appendectomy. MELAS syndrome is a multisystemic disease caused by mitochondrial dysfunction. General anesthesia has several potential hazards to patients with MELAS syndrome, such as malignant hyperthermia, hypothermia, and metabolic acidosis. In this case, anesthesia was performed with propofol, remifentanil TCI, and atracurium without any surgical or anesthetic complications. We discuss the anesthetic effects of MELAS syndrome.
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PMID:Total intravenous anesthesia with propofol and remifentanil in a patient with MELAS syndrome -A case report-. 2050 2

There are several problems in anesthetic management for patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS); susceptibility to malignant hyperthermia, metabolic disorders such as lactic acidosis and diabetes, and dysfunction of vital organs such as cardiomyopathy. Here we report an anesthetic management of emergency laparotomy in a 58-year-old woman with MELAS and systemic inflammatory response syndrome (SIRS). Pre-operative examinations revealed lactic acidosis, hyperglycemia, moderate cardiac depression, and slightly decreased renal function. We chose total intravenous anesthesia to avoid risks of malignant hyperthermia. Anesthesia was induced by rapid-sequence fashion and maintained using midazolam, propofol, ketamine, fentanyl and vecuronium. Based on arterial blood gas analyses, we adjusted ventilator settings, restored blood volume using acetated-Ringer's solution and alubumin preparation with transfusion, and administered sodium bicarbonate and catecholamines, to keep adequate oxygen demand/supply balance and improve acid-base balance. We applied a patient warming system to avoid the progression of hypothermia. After the surgery, the patient was transferred to the intensive care unit, and underwent the endotoxin absorption therapy as well as antibiotics therapy for the treatment of SIRS. The post-operative course was almost uneventful. We consider that careful anesthetic management was essential for the uneventful peri-operative course of this patient.
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PMID:[Anesthetic management of emergency total gastrectomy in a patient with mitochondrial encephalomyopathy: a case of gastric perforation accompanied by systemic inflammatory response syndrome]. 2056 Mar 85