TITLE:
Anesthetic Management of a Boy with Congenital Disorder of Glycosylation (CDG) I-x
AUTHORS:
Amit Lehavi, Hanna Mandel, Yeshayahu (Shai) Katz
KEYWORDS:
Anesthesia, Pediatric, Metabolic Disorders, Congenital Disorders of Glycosylation, CDG
JOURNAL NAME:
International Journal of Clinical Medicine,
Vol.2 No.3,
July
21,
2011
ABSTRACT: Congenital disorders of glycosylation (CDGs) are group group of genetic defects in the assembly and processing pathway of protein glycosylation, which cause a wide range of multi system dysfunction. This paper describes the anesthetic management of 6 year old boy with CDG type I-x for upper airway surgery. We used a sevoflurane-nitrous oxide-remifentanil regime with no complications and good results. As for now, the literature lacks reports of anesthetic management of children with CDG I-x, and this report may provide clinicians an option for safe anesthetic management.