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Perioperative management of children with glycogen storage disease type II—Pompe disease

Paediatric Anaesthesia Apr 05, 2018

Bosman L, et al. - Researchers determined the incidence of perioperative complications in children with Pompe disease treated in their hospital (the Erasmus MC—Sophia Children's Hospital, University Medical Centre, Rotterdam, The Netherlands) since the introduction of enzyme replacement therapy. Relatively low incidence of perioperative complications was documented, however, a potentially high anesthetic risk was reported for children with Pompe disease under enzyme replacement therapy. The relative safety of general anesthesia in children with Pompe disease could be ensured with proper precautionary measures and a critical choice of timing of the operation.
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