Journal of Yeungnam Medical Science
- Volume 25 Issue 1
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- Pages.78-83
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- 2008
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- 2799-8010(eISSN)
General Anesthesia for Extracorporeal Shockwave Lithotripsyin Child with Lesch-Nyhan Syndrome
Lesch-Nyhan 증후군을 가진 소아의 체외충격파신쇄석술을 위한 전신마취 경험
- Park, Sang-Jin (Department of Anesthesiology and Pain Medicine, College of Medicine, Yeungnam University) ;
- Kwon, Il-Chi (Department of Anesthesiology and Pain Medicine, College of Medicine, Yeungnam University) ;
- Lee, Won-Ki (Department of Anesthesiology and Pain Medicine, College of Medicine, Yeungnam University) ;
- Lee, Deok-Hee (Department of Anesthesiology and Pain Medicine, College of Medicine, Yeungnam University)
- 박상진 (영남대학교 의과대학 마취통증의학교실) ;
- 권일치 (영남대학교 의과대학 마취통증의학교실) ;
- 이원기 (영남대학교 의과대학 마취통증의학교실) ;
- 이덕희 (영남대학교 의과대학 마취통증의학교실)
- Published : 2008.06.30
Abstract
Lesch-Nyhan syndrome is an inborn error of purine metabolism resulting from hypoxanthine-guanine-phosphoribosyltransferase (HGPRT) deficiency and leading to excess purine production and uric acid over-production. It is a very rare X-linked recessive disorder, characterized by movement disorder, cognitive deficits, and self-injurious behavior. However, because of the high incidence of calculi, patients may present for surgery of urinary tract, and have increased risk of difficult intubation, aspiration pneumonia, renal insufficiency or sudden death. We report the case of a 5-year-old boy with Lesch-Nyhan syndrome who underwent successive extracorporeal shockwave lithotripsy under general anesthesia.