期刊
A & A PRACTICE
卷 15, 期 10, 页码 -出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1213/XAA.0000000000001539
关键词
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资金
- Intramural Research Program of the National Institutes of Health Clinical Center
- National Institute of Neurological Disorders and Stroke
A clinical trial involving 14 patients with Giant Axonal Neuropathy (GAN) undergoing anesthesia showed few nonsignificant perianesthetic complications, indicating the safety and tolerability of shorter procedural sedation and anesthesia for patients with this rare and potentially fatal disease.
Giant 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.
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