Malignant Hyperthermia in Duchenne Muscular Dystrophy

Brownell, A. Keith W.; Paasuke, R. T.; Elash, A; Fowlow, S. B.; Seagram, C. G. F.; Diewold, R. J.; Friesen, Cliff

doi:10.1097/00000542-198302000-00013

Cited by 87 publications

(23 citation statements)

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“…Evidence for a relation to the sudden infant death syndrome 27,131 is rather weak. 33 MH-like crises have also been observed in patients with myopathies such as myotonia fluctuans, 141,168 Duchenne and Becker dystrophy, 15,78,120 myotonia congenita, 62 and myotonic dystrophy. It seems very likely that the molecular mechanisms underlying these MH-like events differ from those of true MH susceptibility, i.e., increased sensitivity of skeletal muscle to the triggering agents by an increased resting calcium level 158 or increased myotonic reactions to anesthetic agents.…”

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confidence: 98%

Genetics and pathogenesis of malignant hyperthermia

2000

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Malignant hyperthermia (MH) is a potentially life-threatening event in response to anesthetic triggering agents, with symptoms of sustained uncontrolled skeletal muscle calcium homeostasis resulting in organ and systemic failure. Susceptibility to MH, an autosomal dominant trait, may be associated with congenital myopathies, but in the majority of the cases, no clinical signs of disease are visible outside of anesthesia. For diagnosis, a functional test on skeletal muscle biopsy, the in vitro contracture test (IVCT), is performed. Over 50% of the families show linkage of the IVCT phenotype to the gene encoding the skeletal muscle ryanodine receptor and over 20 mutations therein have been described. At least five other loci have been defined implicating greater genetic heterogeneity than previously assumed, but so far only one further gene encoding the main subunit of the voltage-gated dihydropyridine receptor has a confirmed role in MH. As a result of extensive research on the mechanisms of excitation-contraction coupling and recent functional characterization of several disease-causing mutations in heterologous expression systems, much is known today about the molecular etiology of MH.

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confidence: 98%

Genetics and pathogenesis of malignant hyperthermia

2000

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“…To our knowledge this is the first case of ventricular fibrillation occurring in the prone position secondary to the cardiomyopathy of DMD. Numerous other cases of ventricular fibrillation have been described in patients with DMD undergoing general anaesthesia, but they were caused by hyperkalaemia secondary to suxamethonium use [7], malignant hyperpyrexia [8, 9] or rhabdomyolysis secondary to volatile agents [10]. The two postoperative cases described by Boba in 1970 [11] seem to have been precipitated by postoperative hypoxia/hypercarbia.…”

Section: Discussionmentioning

confidence: 99%

A case of ventricular fibrillation in the prone position during back stabilisation surgery in a boy with Duchenne's muscular dystrophy

Reid

Appleton

1999

Anaesthesia

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“…USA. Brownwall et al 1983;Kelfer, Singer & Reynolds 1983). Although non-depolarizing agents are considered safe in patients with DMD, prolonged duration of action and an increased sensitivity have been reported (Buzello & Huttarsch 1988;Huang et al 1990), so that postoperative ventilation may be required.…”

Section: Introductionmentioning

confidence: 99%

Mivacurium in children with Duchenne muscular dystrophy

Tobias

Atwood

1994

Pediatric Anesthesia

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The authors retrospectively reviewed their experience with mivacurium for neuromuscular blockade in seven children with Duchenne muscular dystrophy. Mivacurium was administered to seven children ranging in age from 8.3 to 14.4 years and in weight from 29 kg to 68 kg during either posterior spinal fusion or lower extremity release. An initial bolus dose of 0.2 mgkg-' was followed by a continuous infusion. Neuromuscular blockade was monitored with a standard twitch monitor and the TOF (2 Hz for 2 s). Complete suppression of all four twitches occurred in 1.5 to 2.6 min. The continuous infusion was started with the return of the first twitch and adjusted to maintain one twitch. Time to recovery of the first twitch varied from 12 to 18 min. Continuous infusion requirements varied from 3 to 20 Fgkg-' with an average for the case of less than 10 pg-kg-' min-' in five of the seven patients. A moderate increase in sensitivity to mivacurium in this patient population is suggested by a decrease in infusion requirements and a prolonged effect following the initial dose.

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Malignant Hyperthermia in Duchenne Muscular Dystrophy

Cited by 87 publications

References 0 publications

Genetics and pathogenesis of malignant hyperthermia

Genetics and pathogenesis of malignant hyperthermia

A case of ventricular fibrillation in the prone position during back stabilisation surgery in a boy with Duchenne's muscular dystrophy

Mivacurium in children with Duchenne muscular dystrophy

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