General Anesthesia for Extracorporeal Shockwave Lithotripsyin Child with Lesch-Nyhan Syndrome.
10.12701/yujm.2008.25.1.78
- Author:
Sang Jin PARK
;
Il chi KWON
;
Won Ki LEE
;
Deok Hee LEE
- Publication Type:Case Report
- Keywords:
Lesch-Nyhan syndrome;
General anesthesia;
Extracorporeal shockwave lithotripsy
- MeSH:
Anesthesia, General;
Calculi;
Child;
Death, Sudden;
Humans;
Incidence;
Intubation;
Lesch-Nyhan Syndrome;
Lithotripsy;
Movement Disorders;
Pneumonia, Aspiration;
Preschool Child;
Purines;
Renal Insufficiency;
Self-Injurious Behavior;
Uric Acid;
Urinary Tract
- From:Yeungnam University Journal of Medicine
2008;25(1):78-83
- CountryRepublic of Korea
- Language:Korean
-
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.
