title: Perioperative management of a child with glutaric aciduria type I undergoing cardiac surgery.
abstract: Patients with glutaric aciduria type I are at risk for acute striatal injury precipitated by catabolic stress. Here, we report the successful interdisciplinary anesthetic and perioperative management of a child with glutaric aciduria type I undergoing cardiac surgery with extracorporeal circulation. Given the central focus on prevention of acute striatal injury, our anesthetic strategy emphasized avoiding a high protein load, high-dose inotropics, especially epinephrine (associated with impaired glucose utilization), deliberate hyperventilation, and other interventions associated with systemic inflammatory response.
authors.affiliation: From the Departments of *General Pediatrics, Division of Inherited Metabolic Diseases, †Pediatric Cardiology, ‡Pediatric Cardiac Surgery, and §Anesthesiology, University Hospital Heidelberg, Heidelberg, Germany.
authors.forename: Stefan
authors.initials: S
authors.lastname: Kölker
authors.forename: Joachim
authors.initials: J
authors.lastname: Eichhorn
authors.forename: Christian
authors.initials: C
authors.lastname: Sebening
authors.forename: Berthold
authors.initials: B
authors.lastname: Klein
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Springer
authors.forename: Christian
authors.initials: C
authors.lastname: Bopp
authors.forename: Helmut
authors.initials: H
authors.lastname: Rauch