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.
Highlights
Congenital disorders of glycosylation (CDGs) are group of genetic defects in the assembly and processing pathway of protein glycosylation
This paper describes the anesthetic management of 6 years old boy with CDG type I-x for upper airway surgery
We describe the anesthetic management of a six years old boy suffering from CDG Type I-x, which was referred for an upper airway surgery under general anesthesia
Summary
Congenital disorders of glycosylation (CDGs) are group of genetic defects in the assembly and processing pathway of protein glycosylation. This causes a phenotype of which growth, cardiac, neurological, hematological, gastrointestinal, renal, and dermatological and ophthalmological systems are affected in various penetration. The literature concerning information on the anesthetic management of CDG patients is very limited. The purpose of our report is to raise the awareness of anesthesiologists to this group of disorders, with emphasis on the CDG I-x subtype, because specific diagnosis of any entity is important for preventive measures. We describe the anesthetic management of a six years old boy suffering from CDG Type I-x, which was referred for an upper airway surgery under general anesthesia
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