Literature DB >> 33393605

Anesthetic Management for an Adult With Glycogen Storage Disease Type 0.

Airi Sakamizu1, Erika Yaguchi1, Shinsuke Hamaguchi2.   

Abstract

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.
© 2020 by the American Dental Society of Anesthesiology.

Entities:  

Keywords:  Glycogen storage disease type 0; Hypoglycemia; Malignant hyperthermia; Total intravenous anesthesia

Mesh:

Substances:

Year:  2020        PMID: 33393605      PMCID: PMC7780254          DOI: 10.2344/anpr-67-02-06

Source DB:  PubMed          Journal:  Anesth Prog        ISSN: 0003-3006


  5 in total

1.  Muscle glycogen storage disease 0 presenting recurrent syncope with weakness and myalgia.

Authors:  Sayuri Sukigara; Wen-Chen Liang; Hirofumi Komaki; Tokiko Fukuda; Takeshi Miyamoto; Takashi Saito; Yoshiaki Saito; Eiji Nakagawa; Kenji Sugai; Yukiko K Hayashi; Hideo Sugie; Masayuki Sasaki; Ichizo Nishino
Journal:  Neuromuscul Disord       Date:  2011-09-29       Impact factor: 4.296

2.  The association of malignant hyperthermia and unusual disease: when you're hot you're hot or maybe not.

Authors:  Peter J Davis; Barbara W Brandom
Journal:  Anesth Analg       Date:  2009-10       Impact factor: 5.108

3.  Cardiomyopathy and exercise intolerance in muscle glycogen storage disease 0.

Authors:  Gittan Kollberg; Már Tulinius; Thomas Gilljam; Ingegerd Ostman-Smith; Gun Forsander; Peter Jotorp; Anders Oldfors; Elisabeth Holme
Journal:  N Engl J Med       Date:  2007-10-11       Impact factor: 91.245

4.  Mutations in the liver glycogen synthase gene in children with hypoglycemia due to glycogen storage disease type 0.

Authors:  M Orho; N U Bosshard; N R Buist; R Gitzelmann; A Aynsley-Green; P Blümel; M C Gannon; F Q Nuttall; L C Groop
Journal:  J Clin Invest       Date:  1998-08-01       Impact factor: 14.808

5.  Patients with glycogen storage diseases undergoing anesthesia: a case series.

Authors:  Carmelina Gurrieri; Juraj Sprung; Toby N Weingarten; Mary E Warner
Journal:  BMC Anesthesiol       Date:  2017-10-06       Impact factor: 2.217

  5 in total

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