Lesch-Nyhan 증후군을 가진 소아의 체외충격파신쇄석술을 위한 전신마취 경험
General Anesthesia for Extracorporeal Shockwave Lithotripsyin Child with Lesch-Nyhan Syndrome
0
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Lesch-Nyhan syndrome is an inborn error of purine metabolism resulting from hypoxanthineguanine- 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.
Abstract
Lesch-Nyhan syndrome is an inborn error of purine metabolism resulting from hypoxanthineguanine- 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.
참고문헌 (19)
- Lesch M, "A familial disorder of uric acid metabolism and central nervous system function", Am J Med, 36, pp.561~570 (1964)
- Nyhan WL, "The Lesch-Nyhan syndrome", Dev Med Child Neurol, 20(3), pp.376~380 (1978)
- Sikora P, "Acute renal failure due to bilateral xanthine urolithiasis in a boy with Lesch-Nyhan syndrome", Pediatr Nephrol, 21(7), pp.1045~1047 (2006)
- Larson LO, "Anesthesia and the Lesch-Nyhan syndrome", Anesthesiology, 63(2), pp.197~199 (1985)
- Nyhan WL, "The Lesch-Nyhan syndrome", Adv Nephrol Necker Hosp, 3, pp.59~70 (1974)
- Neychev VK, "Sudden death in Lesch-Nyhan disease", Dev Med Child Neurol, 48(11), pp.923~926 (2006)
- Jinnah HA, "Delineation of the motor disorder of Lesch-Nyhan disease", Brain, 129(Pt 5), pp.1201~1217 (2006)
- Shewell PC, "Atlantoaxial instability in Lesch-Nyhan syndrome", Spine, 21(6), pp.757~762 (1996)
- Mecca RS, "Clinical anesthesia", pp.1391~1392 (2001), Lippincott Williams & Wilkins
- Stone DJ, "Anesthesia", pp.1425~1443 (2000), Churchill Livingstone
- Yao FSF, "Anesthesiology", pp.55~58 (1998), Lippincott Williams & Wilkins
- Martyn JA, "Succinylcholineinduced hyperkalemia in acquired pathologic states: etiologic factors and molecular mechanisms", Anesthesiology, 104(1), pp.158~169 (2006)
- Savarese JJ, "Anesthesia", pp.419~426 (2000), Churchill Livingstone
- Hobbs WR, "Goodman & Gilman's the pharmacological basis of therapeutics", pp.373~380 (1996), McGraw-Hill
- Morgan GE, "Clinical anesthesiology", pp.169~173 (2006), McGraw-Hill
- Morgan GE, "Clinical anesthesiology", pp.215~224 (2006), McGraw-Hill
- Blobner M, "Effect of renal function on neuromuscular block induced by continuous infusion of mivacurium", Br J Anaesth, 74(4), pp.452~454 (1995)
- Erden IA, "Comparison of propofol", fentanyl and ketamine anesthesia in children during extracorporeal shockwave lithotripsy, 2007, pp.28(3), 364~368
- Landau EH, "Extracorporeal shock wave lithotripsy is highly effective for ureteral calculi in children", J Urol, 165(6 Pt 2), pp.2316~2319 (2001)