Journal of clinical anesthesia
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Case Reports
A rare genetic variant of the ryanodine receptor in a suspected malignant hyperthermia susceptible patient.
Malignant hyperthermia (MH) remains a diagnostic challenge. This case report describes the anesthetic management of a suspected intraoperative MH episode and the subsequent, genetic sequence analysis of 3 genes associated with MH. ⋯ Ala4185Thr), in the RYR1 gene encoding the ryanodine receptor. Although the RYR1 gene has previously been implicated in the pathogenesis of MH, (1) this particular variant has only been reported in one other case of MH; (2) the role for diagnostic genetic testing in the diagnosis of MH will be examined.
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Characterize the incidence of elevated aPTT results in patients treated with prophylactic, subcutaneous unfractionated heparin (UFH). ⋯ Treatment with UFH resulted in a small, but significant, increase in aPTT.
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To determine the incidence of postoperative airway complications in infants <5kg in weight undergoing cardiac surgery intubated with Microcuff (Kimberley-Clark, Roswell, GA) endotracheal tubes (ETTs). ⋯ Microcuff ETTs in infants <5.0 kg in weight undergoing cardiac surgery are associated with a low incidence of severe postextubation stridor. Because cuffed ETTs allow for improved control of ventilation/oxygenation and decreased risk of aspiration, they should be considered for use in this high-risk population. Larger studies are needed to confirm these results.
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Case Reports
Reversal of profound neuromuscular blockade with sugammadex in an infant after bronchial foreign body removal.
Sugammadex is a selective chemical agent that can reverse neuromuscular blockade induced by vecuronium and rocuronium. The aim of this report is to discuss the effectiveness of sugammadex in the reversal of neuromuscular blockade in children younger than 2 years. A 16-month-old boy, weighing 10 kg, was admitted to the pediatric emergency department due to choking, cyanosis, and severe respiratory distress that occurred while he was eating peanuts. ⋯ Clinical use of sugammadex in children younger than 2 years is not recommended because of the lack of clinical studies. In this case report, the profound neuromuscular blockade was successfully reversed with a dose of 2 mg/kg sugammadex in a 16-month-old boy. However, more prospective clinical studies are required for the safe use of this agent in children.
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Allgrove syndrome (AS) is a rare autosomal recessive disorder characterized by achalasia cardia, alacrimia, and adrenocorticotropic hormone-resistant adrenal insufficiency which is sometimes associated with autonomic dysfunction. It has also been referred to as the triple A syndrome in view of the cardinal symptoms described above. First described by Allgrove et al in 1978, the disorder usually presents mostly during the first decade of life. These patients have the threat of adrenal crisis, shock, and hypoglycemia and are usually on steroid supplementation. ⋯ Being aware of the pathophysiology of AS gives useful insight about the disease and successful perioperative management in the form of the triple S strategy, that is, stress dose of steroids, slow induction and positioning, and finally maintenance of stable hemodynamics and euglycemia.