Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We encountered a 53-year-old woman with myotonic dystrophy for a total abdominal hysterectomy. In patients with myotonic dystrophy, hypersensitivity to anesthetic drugs, especially muscle relaxants and opioids, may complicate postoperative management. Combined spinal and epidural block was used in this patient to prevent the occurrence of potential postoperative complications associated with general anesthesia, including respiratory depression. In addition, dexmedetomidine was used for sedation during surgery, because the patient had mental retardation associated with this disease. Airway obstruction was observed after the initial administration of dexmedetomidine at 2 microg x kg(-1) x hr(-1). Therefore, the dose of the drug was reduced to a maintenance dose of 0.2% microg x kg(-1) x hr(-1), resulting in adequate sedation. Dexmedetomidine was proved to be useful in this case; however, use of the drug should be carefully started at a low initial dose in patients with myotonic dystrophy.
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PMID:[Anesthetic management in a case of myotonic distrophy with dexmedetomidine]. 1970 15