Anesthetic Management for an Adult With Glycogen Storage Disease Type 0 | Zendy

Airi Sakamizu | Zendy; Erika Yaguchi | Zendy; Shinsuke Hamaguchi | Zendy

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Anesthetic Management for an Adult With Glycogen Storage Disease Type 0

Author(s) -

Airi Sakamizu,

Erika Yaguchi,

Shinsuke Hamaguchi

Publication year - 2020

Publication title -

anesthesia progress

Language(s) - English

Resource type - Journals

eISSN - 1878-7177

pISSN - 0003-3006

DOI - 10.2344/anpr-67-02-06

Subject(s) - medicine , remifentanil , anesthesia , propofol , velopharyngeal insufficiency , palatoplasty , glycogen storage disease , anesthetic , surgery , adverse effect , glycogen

A 20-year-old woman with glycogen storage disease type 0 (GSD-0) underwent velopharyngeal closure for velopharyngeal insufficiency following palatoplasty. To reduce the risk of complications attributed to GSD-0, general anesthesia was administered using a total intravenous anesthesia (TIVA) technique with propofol and remifentanil, along with supplemental glucose-containing intravenous fluids. Her blood glucose remained stable, intraoperative body temperature ranged from 36.5 to 37.2°C, and the velopharyngeal closure was completed without any adverse events.

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