Anesthetic Management of a Boy with Congenital Disorder of Glycosylation (CDG) I-x英文文献资料.docVIP

International Journal of Clinical Medicine, 2011, 2, 325-327 325 doi:10.4236/ijcm.2011.23056 Published Online July 2011 (http://www.SciRP.org/journal/ijcm) Anesthetic Management of a Boy with Congenital Disorder of Glycosylation (CDG) I-x Amit Lehavi , Hanna Mandel , Yeshayahu (Shai) Katz 1,3* 2,3,4 1,3,4 1 Department of Anesthesiology, Rambam Healthcare Campus, Haifa, Israel; 2 Metabolic Unit, Meyer Children’s Hospital, Haifa, Israel; Rambam Health Care Campus, Haifa, Israel; Bruce Rappaport Faculty of Medicine, Technion (Israel Institute of Technol- 3 4 ogy), Haifa, Israel. * Email: amit.lehavi@ Received April 16 , 2011; revised April 27 , 2011; accepted May 27 , 2011. th th th ABSTRACT Congenital disorders of glycosylation (CDGs) are group group of genetic defects in the assembly and processing path- way of protein glycosylation, which cause a wide range of multi system dysfunction. This paper describes the anesthetic management of 6 years old boy with CDG type I-x for upper airway surgery. We used a sevoflurane-nitrous ox- ide-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 manage- ment. Keywords: Anesthesia, Pediatric, Metabolic Disorders, Congenital Disorders of Glycosylation, CDG 1. Introduction years. The boy, weighing 16.2 kg (second per- centile), had no cognitive impairment, and physical ex- amination did not reveal any abnormal findings. He was put on oral captopril 2.5 mg twice