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)

A patient with McArdle disease underwent bowel surgery with general anesthesia and was successfully managed. McArdle disease is a rare skeletal muscle disorder affecting approximately 1 in 100,000 people. McArdle disease, also known as type V glycogen storage disease, is an autosomal recessive inherited condition caused by a missing or nonfunctioning enzyme called myophosphorylase C. This phosphorylase is the enzyme responsible for making glucose for energy. Individuals suffering from McArdle disease have muscles that cannot properly metabolize energy and may experience fatigue and failure during strenuous activities. When a patient with McArdle disease presents for any surgical procedure, a variety of anesthesia implications should be discussed and incorporated into the overall management of his or her care. Careful attention to adequate fluid management, appropriate neuromuscular blockade choices, normothermia maintenance, normoglycemia maintenance, blood pressure monitoring, and maintaining malignant hyperthermia precautions is critical to providing safe anesthesia to this unique patient population.
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PMID:Anesthesia considerations in a patient with mcArdle disease: a case report. 2175 93

McArdles disease (glycogen storage disease type v) is a rare condition in which energy-metabolism in the muscle is hampered. A case report is presented and the possible risk for perioperative complications including malignant hyperthermia is discussed. A checklist for the anesthesiological management of patients with McArdles disease is provided. A short overview of anesthesiological challenges and perioperative complications of other glycogen storage diseases is given.
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PMID:McArdle's disease (glycogen storage disease type V) and anesthesia--a case report and review of the literature. 2356 73

McArdle disease, known as type V glycogen storage disease, is a rare skeletal muscle disorder. Patients with McArdle disease lack skeletal muscle specific glycogen phosphorylase, and myophosphorylase. This subsequently leads to an elevation in serum creatine kinase levels, and results in suffering from exercise intolerance. They have such anesthesiological problems as follows; rhabdomyolysis, myoglobinuria, acute renal failure, and a higher risk of developing malignant hyperthermia. We report a case of general anesthesia for a patient with McArdle disease. The patient was a 38-year-old woman who underwent dilation and curettage. Anesthesia was induced by intravenous administrations of fentanyl and propofol. For maintenance of anesthesia, we used total intravenous anesthesia with propofol. We avoided the use of neuromuscular blockades throughout the operation. There were no signs of rhabdomyolysis, myoglobinuria, worsening muscle weakness, or occurrence of malignant hyperthermia in the perioperative period, and the patient recovered uneventfully.
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PMID:[Anesthesia in a Patient with McArdle Disease]. 2668 76

McArdle disease or type V glycogenosis is a rare metabolic myopathy consisting of muscle loss and weakness. These patients have risks associated with anaesthesia. They can present with hypoglycaemia, rhabdomyolysis, acute renal failure, and electrolyte changes. It has also been associated with a higher incidence of malignant hyperthermia during the anaesthetic procedure. Intermittent compression due to the measurement of non-invasive pressure, postures on the operating table that may cause muscle contractures, or tremor caused by hypothermia or anaesthesia itself, may trigger rhabdomyolysis in these patients. In this article we present our experience in submitting a patient with McArdle's syndrome to general anaesthesia for total thyroidectomy due to multinodular euthyroid goitre.
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PMID:Total thyroidectomys in patient with McArdle's syndrome: Anesthetic management. 3050 83