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Yeungnam Univ J Med > Volume 25(1); 2008 > Article
Yeungnam University Journal of Medicine 2008;25(1):78-83.
DOI:    Published online June 30, 2008.
General Anesthesia for Extracorporeal Shockwave Lithotripsyin Child with Lesch-Nyhan Syndrome.
Sang Jin Park, Il chi Kwon, Won Ki Lee, Deok Hee Lee
Department of Anesthesiology and Pain Medicine, College of Medicine, Yeungnam University, Daegu, Korea.
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.
Key Words: Lesch-Nyhan syndrome, General anesthesia, Extracorporeal shockwave lithotripsy
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