Paediatric anaesthesia
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Paediatric anaesthesia · Sep 2013
ReviewPerioperative temperature elevation: not all hyperthermia is malignant hyperthermia.
The objective of this review is to assist the readers, anesthesiologists, intensivists, and emergency physicians in making a more accurate diagnosis of perioperative fever or hyperthermia and subsequently choose the proper course of treatment. ⋯ A literature search was performed which identified many possible common and uncommon sources of fever. Some of these sources are quite relevant to the anesthesiologist. Other sources had potential relevance in obscure cases.
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Paediatric anaesthesia · Sep 2013
ReviewPerioperative respiratory management of pediatric patients with neuromuscular disease.
Patients with neuromuscular disorders undergoing general anesthesia present a special set of respiratory problems for perioperative management. While there are disease-specific concerns, there are many common themes in the respiratory management of patients with neuromuscular disorders. ⋯ Such common perioperative concerns include upper airway obstruction, chest wall restriction, postoperative hypoventilation, inadequate airway clearance, and chronic lower airway disease. Each of these challenges has an effective management approach, and careful planning can help avoid perioperative respiratory complications.
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Paediatric anaesthesia · Sep 2013
ReviewUnexpected MH deaths without exposure to inhalation anesthetics in pediatric patients.
Children, later found to have ryanodine receptor type one variants (RYR1), died without exposure to inhalation anesthetics. Family members with the same RYR1 variants had contracture tests consistent with susceptibility to malignant hyperthermia or in vitro testing showed increased sensitivity to RYR1 agonist.
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Paediatric anaesthesia · Sep 2013
ReviewCore myopathies and malignant hyperthermia susceptibility: a review.
The core myopathies are a subset of myopathies that present in infancy with hypotonia and muscle weakness. They were formerly considered a rare type of congenital myopathy but are now recognized as being more prevalent. Due to their genetic linkage to mutations in the ryanodine receptor gene (RYR1), core myopathies (in particular, central core disease) carry a high risk of malignant hyperthermia susceptibility. ⋯ We also review the level of suspicion a clinician should exhibit with a child who has a possible core myopathy or other congenital myopathy presenting for an anesthetic prior to a definitive genetic analysis. For this review article, we performed literature searches using the key words anesthesiology, core myopathies, pediatric neurology, malignant hyperthermia, genetics, ryanodine receptor, and molecular biology. We also relied on literature accumulated by the two authors, who served as hotline consultants for the Malignant Hyperthermia Hotline of the Malignant Hyperthermia Association of the United States (MHAUS) for the past 12 years.
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Paediatric anaesthesia · Sep 2013
ReviewAnesthetic considerations in patients with mitochondrial defects.
Mitochondrial disease, once thought to be a rare clinical entity, is now recognized as an important cause of a wide range of neurologic, cardiac, muscle, and endocrine disorders. The incidence of disorders of the respiratory chain alone is estimated to be about 1 per 4-5000 live births, similar to that of more well-known neurologic diseases. High-energy requiring tissues are uniquely dependent on the energy delivered by mitochondria and therefore have the lowest threshold for displaying symptoms of mitochondrial disease. ⋯ Mitochondrial disease represents probably hundreds of different defects, both genetic and environmental in origin, and is thus difficult to characterize. The specter of possible delayed complications in patients caused by inhibition of metabolism by anesthetics, by remaining in a biochemically stressed state such as fasting/catabolism, or by prolonged exposure to pain is a constant worry to physicians caring for these patients. Here, we review the considerations when caring for a patient with mitochondrial disease.