Cases reported "Muscular Dystrophies"

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1/14. Suxamethonium-induced rhabdomyolysis in a healthy middle-aged man.

    A 43-year-old man developed rhabdomyolysis after uvulo- palatopharyngoplasty. After induction with thiopentone and suxamethonium the anesthesia was maintained with halothane. The patient responded to treatment and made an uneventful recovery. In earlier reports of rhabdomyolysis after general anaesthesia with halothane and suxamethonium almost all the patients had malignant hyperthermia (MH) or muscular dystrophy. About 50% of malignant hyperthermia patients carry a mutation in the RYR1 gene. Our patient did not have mutations in the four MH-associated genes tested, but the total amount of different mutations is by now about twenty. Therefore, despite these negative tests rhabdomyolysis may be a sign of subclinical malignant hyperthermia which cannot be ruled out by our investigations. This rare case of rhabdomyolysis in a healthy man suggests careful monitoring of the patient when-ever suxamethonium is used.
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ranking = 1
keywords = anaesthesia
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2/14. General anaesthesia for a patient with progressive muscular dystrophy.

    The case is reported of a four-year-old boy with progressive muscular dystrophy who underwent anal fistulectomy under neuroleptanaesthesia with Thalamonal. Recovery was uneventful. Anaesthetic problems associated with the disease include sudden death due to myocardial involvement and high postoperative mortality due to pulmonary infection. Mental retardation and ECG abnormalities may be present and acute postoperative gastric dilatation has been reported. narcotics or minor tranquilizers and hyoscine are recommended for premedication. light anaesthesia should be maintained throughout surgery and minimal doses of non-depolarizing muscle relaxants may be used if necessary. Suxamethonium chloride should be avoided because muscle damage and cardiac arrest might occur.
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ranking = 6
keywords = anaesthesia
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3/14. Fatal rhabdomyolysis complicating general anaesthesia in a child with Becker muscular dystrophy.

    A 6-yr-old boy who presented with brown urine due to myoglobinuria and who was otherwise virtually asymptomatic was diagnosed as having Becker muscular dystrophy on the basis of a greatly elevated creatine kinase, muscle biopsy, dystrophin analysis, and a deletion of exons 3-7 in the dystrophin gene. Fifteen months later, during a general anaesthetic for dental treatment, he had a cardiac arrest associated with acute rhabdomyolysis, hyperkalaemia and hypocalcaemia. He died 4 days later. This case is reported to highlight this rare but potentially fatal complication of anaesthesia in muscular dystrophy, and to discuss possible ways of preventing such a catastrophe.
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ranking = 5
keywords = anaesthesia
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4/14. Cardiac arrest after isoflurane anaesthesia in a patient with Duchenne's muscular dystrophy.

    An 8-year-old boy known to have Duchenne's muscular dystrophy suffered a cardiac arrest 10 minutes after he regained consciousness after isoflurane anaesthesia for an orchidopexy procedure. Resuscitation was successful 2 hours after the start of external cardiac compression and after correction of hyperkalaemia and the administration of dantrolene. He later developed myoglobinuria elevated creatine kinase and a metabolic and respiratory acidosis. He demonstrated a delayed increase in rectal temperature.
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ranking = 5
keywords = anaesthesia
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5/14. Complications during anaesthesia in patients with Duchenne's muscular dystrophy (a retrospective study)

    The purpose of this retrospective study was to estimate the frequency and severity of anaesthetic complications in patients with Duchenne's muscular dystrophy (DMD). Forty-four boys with DMD were exposed to anaesthesia and surgery 84 times during a period of 22 years (1965-86). The procedures took place at 15 different hospitals. Retrospective examination of the case records showed: 19 cases with local analgesia without any complications, and 18 of 65 general anaesthetics with minor or more serious complications. In ten cases an increase in body temperature above 37.5 degrees C was seen, five had abdominal pain and dark-coloured urine after surgery, and three had a critical perioperative course with a resemblance to malignant hyperthermia. The complications were almost exclusively related to the use of succinylcholine. The use of succinylcholine was dispersed through all ages. Three out of the eight patients with severe complications occurred 1.5, 2.5 and 4 years before the neuromuscular disease was diagnosed. Thus an unusual course of anaesthesia in male children calls for further investigation. Although it has been stated before that succinylcholine is contraindicated in patients with Duchenne's muscular dystrophy, the drug continues to be used.
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ranking = 6
keywords = anaesthesia
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6/14. serum creatine kinase levels after succinylcholine in children with "muscle, eye and brain disease".

    Four boys belonging to a group of children affected by a rare form of muscular dystrophy with eye and brain involvement, termed the "muscle, eye and brain disease" (MEB), were anaesthetized for various eye examinations and surgery. On some occasions succinylcholine was used during anaesthesia and the initially elevated serum creatine kinase (CK) values increased from a range of 122 to 1200 units.L-1 to a range of 4350 to 9690 units.L-1 22 hours after anaesthesia. CK values after anaesthesia without succinylcholine remained at the initially elevated levels. Rectal temperatures of the children were normal. These findings suggest that succinylcholine should be avoided in patients with MEB disease.
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ranking = 3
keywords = anaesthesia
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7/14. Duchenne muscular dystrophy and malignant hyperthermia--two case reports.

    The case histories are presented including the anaesthetic and postoperative management, of two children, a two-year-old with undiagnosed Duchenne muscular dystrophy (DMD) and a three-year-old with known DMD. The child with undiagnosed DMD had no symptoms of DMD and had received halothane twice before, without succinylcholine, with no apparent difficulty. Following an uneventful induction of anaesthesia with halothane, nitrous oxide and O2, succinylcholine resulted in bilateral masseter muscle spasm and then, in rapid sequence, ventricular tachycardia and cardiac arrest. Resuscitation was difficult, prolonged and associated with hyperkalaemia (K = 12.57 mEq X L-1), severe metabolic and respiratory acidosis, high peripheral venous pressure and massive hepatosplenomegaly, but not hyperthermia. The patient was finally resuscitated but died two days later. Skeletal muscle biopsy results were consistent with malignant hyperthermia. The second patient was known to have DMD but did not receive prophylactic or intraoperative dantrolene nor have his anaesthetic machine flushed with oxygen for an extended period prior to induction of anaesthesia. This child was anaesthetized with fentanyl and N2O and, with the exception of a high intraoperative heart rate (155-160 beats X min-1), had an uncomplicated anaesthetic and operation (intraoperative axillary temperatures ranged between 36.8-37.9 degrees C). Postoperatively his temperature rapidly increased to 38.8 degrees C and then 40.3 degrees C and he became metabolically acidotic. Intravenous administration of dantrolene for 48 hours reduced the temperature and allowed normal recovery and discharge. A postoperative muscle biopsy was consistent with DMD.(ABSTRACT TRUNCATED AT 250 WORDS)
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ranking = 2
keywords = anaesthesia
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8/14. Cardiac arrest following inhalation induction of anaesthesia in a child with Duchenne's muscular dystrophy.

    Cardiac arrest occurred in a 5 1/2-year-old child with suspected Duchenne's muscular dystrophy ten minutes following induction of anaesthesia with halothane, nitrous oxide and oxygen. No muscle relaxants were administered. The cardiac arrest was associated with hyperkalaemia, acidosis, myoglobinuria, elevated serum creatine phosphokinase and a 1.6 degrees C rise in temperature. The child made a complete recovery after receiving 90 minutes of cardiopulmonary resuscitation.
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ranking = 5
keywords = anaesthesia
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9/14. Anaesthesia and progressive muscular dystrophy.

    The presentation and features of Duchenne's progressive muscular dystrophy (Duchenne's PMD) are described and the increased risks associated with anaesthesia are considered. Hazards associated with induction of anaesthesia and immediate postoperative recovery have been stressed in recent case reports, and these are summarized. Features of a hyperpyrexia-like response including cardiac arrest, increased serum creatine phosphokinase concentration, myoglobinuria and metabolic acidosis following suxamethonium or halothane, or both, have been described in patients with Duchenne's PMD. Subsequent in vitro muscle tests have suggested that it is possible that a malignant hyperpyrexia response to general anaesthesia may occur. Six children known to have Duchenne's PMD who developed delayed respiratory insufficiency following anaesthesia and required controlled pulmonary ventilation are reported. In five of the children, cardiac arrest occurred despite apparently adequate respiratory support. Suxamethonium was common to the anaesthetic received by all six patients. In one of these patients subsequent anaesthetics, without suxamethonium, were uneventful and delayed muscle weakness did not occur.
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ranking = 4
keywords = anaesthesia
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10/14. Malignant hyperpyrexia and Duchenne muscular dystrophy: A case report.

    We report a patient with Duchenne muscular dystrophy who developed malignant hyperpyrexia during general anaesthesia. During anaesthesia bradycardia was followed by ventricular fibrillation, on which ventricular flutter supervened and a body temperature rise of 0.6 degrees C for 15 minutes, myoglobinuria and elevation of CPK level were observed. The caffeine sensitivity test of biopsied muscle fibers revealed an increase in sensitivity, although there was no sign of muscle rigidity during or after anaesthesia. diagnosis of Duchenne muscular dystrophy was first established after the development of malignant hyperpyrexia in the present case as well as in previously reported cases. Determination of serum CPK is very important before general anaesthesia.
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ranking = 4
keywords = anaesthesia
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