• A & A case reports · Oct 2013

    Perioperative management of a child with glutaric aciduria type I undergoing cardiac surgery.

    • Stefan Kölker, Joachim Eichhorn, Christian Sebening, Berthold Klein, Wolfgang Springer, Christian Bopp, and Helmut Rauch.
    • From the Departments of *General Pediatrics, Division of Inherited Metabolic Diseases, †Pediatric Cardiology, ‡Pediatric Cardiac Surgery, and §Anesthesiology, University Hospital Heidelberg, Heidelberg, Germany.
    • A A Case Rep. 2013 Oct 1;1(1):5-7.

    AbstractPatients 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.

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