• A&A practice · Oct 2021

    Anesthetic Management of Children and Adolescents With Giant Axonal Neuropathy: A Large Case Series.

    • Muhammad Yousef, Diana Bharucha-Goebel, Dimah Saade, Gilberto Averion, Carsten G Bönnemann, and QuezadoZenaide M NZMNFrom the Pediatric Anesthesia and Critical Care Section, Department of Perioperative Medicine, National Institutes of Health Clinical Center..
    • From the Pediatric Anesthesia and Critical Care Section, Department of Perioperative Medicine, National Institutes of Health Clinical Center.
    • A A Pract. 2021 Oct 25; 15 (10): e01539.

    AbstractGiant axonal neuropathy (GAN) is a rare autosomal recessive neurodegenerative disorder caused by mutations in the GAN gene, which encodes for gigaxonin, a protein involved in intermediate filament processing in neural cells and fibroblasts. We report on 14 GAN patients who underwent 77 anesthetics during the conduct of an intrathecal gene transfer clinical trial from April 2015 to August 2020. We observed only a few nonsignificant perianesthetic complications. Our data expand the knowledge regarding safety of anesthesia for patients with this rare and potentially fatal disease and highlights the tolerability of shorter procedural sedation and anesthesia.Copyright © 2021 International Anesthesia Research Society.

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