대한장애인치과학회지 (The Journal of Korea Assosiation for Disability and Oral Health)

  • 제9권2호
  • /
  • Pages.107-110
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  • 2013
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  • 1738-8813(pISSN)
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  • 2287-7142(eISSN)
대한장애인치과학회 (Korean Association for Disability and Oral Health)
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부신백질이영양증 환자의 전신마취 하 치과 치료 : 증례보고

DENTAL TREATMENT OF A 11-YEAR-OLD MALE PATIENT WITH ADRENOLEUKODYSTROPHY UNDER GENERAL ANESTHESIA : A CASE REPORT

  • 장준혁 (서울대학교 치의학대학원 소아치과학교실) ;
  • 신터전 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김영재 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김정욱 (서울대학교 치의학대학원 소아치과학교실) ;
  • 장기택 (서울대학교 치의학대학원 소아치과학교실) ;
  • 이상훈 (서울대학교 치의학대학원 소아치과학교실) ;
  • 김종철 (서울대학교 치의학대학원 소아치과학교실) ;
  • 현홍근 (서울대학교 치의학대학원 소아치과학교실)
  • Jang, Jun-Hyuk (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Shin, Teo-Jeon (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Young-Jae (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Jung-Wook (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Jang, Ki-Taeg (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Lee, Sang-Hoon (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Kim, Chong-Chul (Department of Pediatric Dentistry, School of Dentistry, Seoul National University) ;
  • Hyun, Hong-Keun (Department of Pediatric Dentistry, School of Dentistry, Seoul National University)
  • 투고 : 2013.11.20
  • 심사 : 2013.12.10
  • 발행 : 2013.12.31
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초록

Adrenoleukodystrophy (ALD) is a rare, inherited disorder that leads to progressive brain damage and failure of the adrenal glands. It is passed down from parents to their children as an X-linked genetic trait. Therefore, it affects primarily males especially under the age of 10. People with ALD have excessive accumulation of very long chain fatty acids (VLCFAs) in their brain because they do not have the enzyme which break down these fatty acids. Special method for treatment of ALD is not available. Eating a diet low in VLCFAs and taking special oils, called Lorenzo's oil, can lower the blood levels of VLCFAs. But this oil cannot stop the destruction of nerve cell. This article presents a case report: Dental treatment of a 11-year-old male patient with ALD under general anesthesia. Careful management must be required during treatment procedure because of difficulty in cooperation, seizure disorders, gastroesophageal reflux, pulmonary aspiration and airway complications.

키워드

참고문헌

  1. Moser HW, Mahmood A, Raymond GV: X-linked adrenoleukodystrophy. Nat Clin Pract Neurol 3(3):140-151, 2007. https://doi.org/10.1038/ncpneuro0421
  2. Moser H, Dubey P, Fatemi A: Progress in Xlinked adrenoleukodystrophy. Curr Opin Neurol 17(3):263-269, 2004. https://doi.org/10.1097/00019052-200406000-00005
  3. Bezman L, Moser HW: Incidence of X-linked adrenoleukodystrophy and the relative frequency of its phenotypes. Am J Med Genet 76(5):415-419, 1998. https://doi.org/10.1002/(SICI)1096-8628(19980413)76:5<415::AID-AJMG9>3.0.CO;2-L
  4. Aubourg P, Blanche S, Jambaque I, et al.: Reversal of early neurologic and neuroradiologic manifestations of X-linked adrenoleukodystrophy by bone marrow transplantation. N Engl J Med 322(26):1860-1866, 1990. https://doi.org/10.1056/NEJM199006283222607
  5. Shapiro E, Krivit W, Lockman L, et al.: Longterm effect of bone-marrow transplantation for childhood-onset cerebral X-linked adrenoleukodystrophy. Lancet 356(9231):713-718, 2000. https://doi.org/10.1016/S0140-6736(00)02629-5
  6. Brown FR 3rd, Van Duyn MA, Moser AB, et al.: Adrenoleukodystrophy: effects of dietary restriction of very long chain fatty acids and of administration of carnitine and clofibrate on clinical status and plasma fattyacids. Johns Hopkins Med J 151(4):164-172, 1982.
  7. Igarashi M, Schaumburg HH, Powers J, et al.: Fatty acid abnormality in adrenoleukodystrophy. J Neurochem 26(4):851-860, 1976. https://doi.org/10.1111/j.1471-4159.1976.tb04462.x
  8. Moser HW: Adrenoleukodystrophy: phenotype, genetics, pathogenesis and therapy. Brain 120(8):1485-1508, 1997. https://doi.org/10.1093/brain/120.8.1485
  9. Powers JM, DeCiero DP, Ito M, et al.: Adrenomyeloneuropathy: a neuropathologic review featuring its noninflammatory myelopathy. J Neuropathol Exp Neurol 59(2):89-102, 2000. https://doi.org/10.1093/jnen/59.2.89
  10. Powers JM, DeCiero DP, Cox C, et al.: The dorsal root ganglia in adrenomyeloneuropathy: neuronal atrophy and abnormal mitochondria. J Neuropathol Exp Neurol 60(5):493-501, 2001. https://doi.org/10.1093/jnen/60.5.493
  11. Schaumburg HH, Powers JM, Raine CS, et al.: Adrenoleukodystrophy. A clinical and pathological study of 17 cases. Arch Neurol 32(9):577-591, 1975. https://doi.org/10.1001/archneur.1975.00490510033001
  12. Ito M, Blumberg BM, Mock DJ, et al.: Potential environmental and hostparticipants in the early white matter lesion of adrenoleukodystrophy: morphologic evidence for CD8 cytotoxic T cells, cytolysis of oligodendrocytes, and CD1-mediated lipid antigen presentation. J Neuropathol Exp Neurol 60(10):1004-1019, 2001. https://doi.org/10.1093/jnen/60.10.1004
  13. Mosser J, Douar AM, Sarde CO, et al.: Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters. Nature 361(6414):726-730, 1993. https://doi.org/10.1038/361726a0
  14. Higgins CF: ABC transporters: from microorganisms to man. Annu Rev Cell Biol 8(1):67-113, 1992. https://doi.org/10.1146/annurev.cb.08.110192.000435
  15. Moser AB, Kreiter N, Bezman L, et al.: Plasma very long chain fatty acids in 3,000 peroxisome disease patients and 29,000 controls. Ann Neurol 45(1):100-110, 1999. https://doi.org/10.1002/1531-8249(199901)45:1<100::AID-ART16>3.0.CO;2-U
  16. Moser AB, Moser HW: The prenatal diagnosis of X-linked adrenoleukodystrophy. Prenat Diagn 19(1):46-48, 1999. https://doi.org/10.1002/(SICI)1097-0223(199901)19:1<46::AID-PD501>3.0.CO;2-E
  17. Bezman L, Moser AB, Raymond GV, et al.: Adrenoleukodystrophy: incidence, new mutation rate, and results of extended family screening. Ann Neurol 49(4):512-517, 2001. https://doi.org/10.1002/ana.101
  18. Aubourg P, Chaussain JL: Adrenoleukodystrophy: the most frequent genetic cause of Addison's disease. Horm Res 59(Suppl 1):104-105, 2003. https://doi.org/10.1159/000067833
  19. Schwartz RE, Stayer SA, Pasquariello CA, et al.: Anaesthesia for the patient with neonatal adrenoleukodystrophy. Can J Anaesth 41(1):56-58, 1994. https://doi.org/10.1007/BF03009663