Paediatric anaesthesia
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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.
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Spinal muscle atrophy (SMA) is autosomal recessive and one of the most common inherited lethal diseases in childhood. The spectrum of symptoms of SMA is continuous and varies from neonatal death to progressive symmetrical muscle weakness first appearing in adulthood. The disease is produced by degeneration of spinal motor neurons and can be described in three or more categories: SMA I with onset of symptoms before 6 months of age; SMAII with onset between 6 and 18 months and SMA III, which presents later in childhood. Genetics: The disease is in more than 95% of cases caused by a homozygous deletion in survival motor neuron gene 1 (SMN1). ⋯ The perioperative risks can be considerable and are mainly related to the respiratory system, from respiratory failure to difficult/impossible intubation.
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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
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.