Cases reported "Malignant Hyperthermia"

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1/45. survival of an oral surgery patient with malignant hyperthermia.

    malignant hyperthermia is a life-threatening complication of general anesthesia. Its cause is not precisely known but it appears to be related to a genetic defect that allows increased release or decreased reaccumulation of calcium by the sarcoplasmic reticulum whech then results in a hypermetabolic state. As with any unexpected complication when a patient is under general anesthesia, early diagnosis and treatment are essential. The early clinical signs that the surgeon and anesthesiologist shoulc be alert to are unexplained tachycardia, unexplained tachypnea, muscular rigidity, and increased temperature. Therapy should be accurate and immediate. The essentials of therapy are discontinuance of the anesthetic agent; immediate, active, and aggressive cooling; administration of procaine or procaine amide, 1 mg/kg/min until the pulse slows; correction of electrolyte and acid-base imbalances; maintenance of urinary output with furosemide and large volumes of fluids, intravenously; and supportive care. A thorough knowledge of the management of malignant hyperthermia ahd the pathophysiology of the complications that may occur with general anesthesia will allow the oral surgeon to fully meet his obligations to his patients.
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2/45. malignant hyperthermia in a patient with Graves' disease during subtotal thyroidectomy.

    We report the case of a 31-year-old man with Graves' disease who manifested malignant hyperthermia during subtotal thyroidectomy. His past medical history and family history were unremarkable. Before surgery, his condition was well controlled with propylthiouracil, beta-adrenergic blocker and iodine. During the operation, anesthesia was induced by intravenous injection of vecuronium and thiopental, followed by suxamethonium for endotracheal intubation. anesthesia was maintained with nitrous oxide and sevoflurane. One hour after induction of anesthesia, his end tidal carbon dioxide concentration (ET(CO2)) increased from 40 to 50 mmHg, heart rate increased from 90 to 100 beats per min and body temperature began to rise at a rate of 0.3 degrees C per 15 min. Suspecting thyroid storm, propranolol 0.4 mg and methylprednisolone 1,500 mg were administered, which, however, had little effect. Despite the lack of muscular rigidity, the diagnosis of malignant hyperthermia was made based on respiratory acidosis. Sevoflurane was discontinued and dantrolene was given by intravenous bolus. Soon after the treatment, ET(CO2), heart rate and body temperature started to fall to normal levels. His laboratory findings showed abnormally elevated serum creatine phosphokinase and myoglobin but normal thyroid hormone levels. Since dantrolene is efficacious in thyrotoxic crisis and malignant hyperthermia, an immediate intravenous administration of dantrolene should be considered when a hypermetabolic state occurs during anesthesia in surgical treatment for a patient with Graves' disease.
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3/45. Fatal malignant hyperthermia--delayed onset and atypical course.

    A case of malignant hyperthermia (mh) in a 27-year-old man is described. In a first anaesthesia using isoflurane and succinylcholine, the end-tidal CO(2) rose from 39 to 49 mmHg 2.75 h post-intubation and the body temperature rose to 39.8 degrees C 14 h post-intubation but was normal again the next day. In a second anaesthesia using the same medication, the maximal end-tidal CO(2) was 44 mmHg and the body temperature rose to 39 degrees C after 9 h. After 4 days, the fever rose to 40 degrees C, and to 42 degrees C when death occurred 10 days after the second anaesthesia. Masseter spasms or muscle rigidity were never present. According to the death certificate, death was due to multi-organ failure from sepsis. At autopsy, the skeletal muscles were pale and oedematous. histology demonstrated focal necroses in the skeletal muscles, shock kidneys with myoglobin excretion and myoglobin clots in small blood vessels of the lungs. Hence, the postmortem diagnosis "malignant hyperthermia" was established but accusations of medical maltreatment were rejected because of the atypical and protracted clinical course and because uncharacteristic signs of malignant hyperthermia were attributable to the clinically suspected sepsis.
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4/45. Chronic myopathy in a patient suspected of carrying two malignant hyperthermia susceptibility (MHS) mutations.

    malignant hyperthermia (MH) is a pharmacogenetic myopathy triggered by a variety of anaesthetic agents and muscle relaxants. In humans, susceptibility to MH is inherited as an autosomal dominant trait, and susceptible patients do not show a clinically relevant myopathy unless having suffered from a MH crisis. Homozygosity for the MHS trait is thought to be an uncommon finding, and so far only a few cases of patients suggested to be homozygous for MH on the basis of pedigree information were reported and described as having a more severe form of this condition resulting in clinical symptoms also in the absence of triggering agents. We report clinical findings in a patient with chronic myopathy beginning at the age of 2 yr and associated with a number of unique features, the most important being a family history of MHS present in both parents. She became symptomatic with marked muscular weakness and elevated serum CK levels. A muscle biopsy showed a distinct enlargement and increase of muscle mitochondria. In the in vitro contracture test the patient's muscle responded with unusually high contractures already at basal levels of triggering agents indicating a particularly severe MHS condition. dna markers for the MHS1 locus, described previously on chromosome 19q12-13.2 in Irish and Canadian pedigrees, could not be used to confirm her homozygous state because our molecular genetic studies had previously excluded the MHS trait in this pedigree from this locus.(ABSTRACT TRUNCATED AT 250 WORDS)
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5/45. malignant hyperthermia.

    malignant hyperthermia is a rare syndrome that occurs in genetically susceptible individuals who are exposed to frequently used inhalation anesthetics. The disorder is most common in children and young adults. It is triggered through a defect in the ability of skeletal muscles to concentrate and release calcium. Signs of malignant hyperthermia include hypercarbia, muscle rigidity and tachycardia. Temperature elevation is often a late sign of the syndrome. Treatment begins with stopping all inhaled anesthetics at the earliest sign of the syndrome. The use of dantrolene has significantly reduced mortality from malignant hyperthermia. No simple screening test exists. family members or those with a suspicious history need to be counseled and should consider muscle biopsy and testing prior to surgery.
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6/45. Desflurane, malignant hyperthermia, and release of compartment syndrome.

    We describe a case of suspected malignant hyperthermia in a healthy 20-yr-old man. The patient underwent urgent release of upper extremity compartment syndrome as a result of traumatic vascular injury. After 3 h of general anesthesia with desflurane, he developed a hypermetabolic state (hypercarbia, hyperthermia, hyperkalemia, and metabolic acidosis), consistent with the diagnosis of malignant hyperthermia. Cardiovascular instability coincided with reperfusion of the injured extremity. Treatment with dantrolene and supporting measures restored cardiovascular stability. Three days later he underwent a successful second surgery under regional block with total IV anesthesia.
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7/45. A case report of malignant hyperthermia in a dental clinic operating room.

    A healthy 5-year-old boy presented for arch bar placement under general anesthesia in an operating room in a dental school. The patient had previously undergone general anesthesia without complication, and no family history of anesthetic problems were reported. halothane mask induction, intravenous catheter placement, and nasal intubation proceeded uneventfully without the aid of a muscle relaxant. Halfway through the procedure, signs and symptoms of malignant hyperthermia, including muscle rigidity, hypercarbia, tachypnea, and tachycardia were noted. Immediate treatment, including discontinuation of the triggering agent, dantrolene administration, and cooling measures were applied, and once stable, the child was transferred to Columbus Children's Hospital for further management. The patient experienced no postoperative complications. Further discussion regarding the pathophysiology and management of malignant hyperthermia is provided.
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8/45. neuroleptic malignant syndrome induced by domperidone.

    A 47-year-old woman with diabetic gastroparesis, on treatment with domperidone, a dopamine-receptor antagonist, was admitted to the hospital in coma, with high blood pressure and nonreactive pupils. She then developed high fever. Her condition progressively worsened for two days, when muscle rigidity was noted and creatine phosphokinase was greater than 2000 U/liter. A diagnosis of neuroleptic malignant syndrome was made, and the patient was given dantrolene with prompt and complete resolution of all signs and symptoms. Subsequent inquiry revealed a distant past history of positive muscle biopsy for malignant hyperthermia, obtained after the diagnosis had been made in a family member. This case suggests that domperidone may induce neuroleptic malignant syndrome and that patients with malignant hyperthermia are at increased risk for this complication.
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9/45. succinylcholine-induced masseter muscle rigidity during bronchoscopic removal of a tracheal foreign body.

    masseter muscle rigidity during general anesthesia is considered an early warning sign of a possible episode of malignant hyperthermia. The decision whether to continue or discontinue the procedure depends on the urgency of the surgery and severity of masseter muscle rigidity. Here, we describe a case of severe masseter muscle rigidity (jaw of steel) after succinylcholine (Sch) administration during general anesthetic management for rigid bronchoscopic removal of a tracheal foreign body. anesthesia was continued uneventfully with propofol infusion while all facilities were available to detect and treat malignant hyperthermia.
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10/45. Repeat episodes of severe muscle rigidity in a child receiving sevoflurane.

    We report on a patient who developed two episodes of severe muscle rigidity, increased endtidal CO2 and increased creatine phosphate kinase associated with sevoflurane anesthesia. Dysrhythmias and hyperthermia were not observed and dantrolene was not administered. genetic testing for the 17 known mutations associated with malignant hyperthermia (MH) was negative. Although we cannot rule out MH or other neuromuscular diseases we suggest that this rare event may be a direct effect of sevoflurane.
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