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, 신터전, 김영재, 김정욱, 장기택, 이상훈, 김종철, 현홍근,
소속 상세정보
장준혁 ( Jang Jun-Hyuk ) - 서울대학교 치의학대학원 소아치과학교실
신터전 ( Shin Teo-Jeon ) - 서울대학교 치의학대학원 소아치과학교실
김영재 ( Kim Young-Jae ) - 서울대학교 치의학대학원 소아치과학교실
김정욱 ( Kim Jung-Wook ) - 서울대학교 치의학대학원 소아치과학교실
장기택 ( Jang Ki-Taeg ) - 서울대학교 치의학대학원 소아치과학교실
이상훈 ( Lee Sang-Hoon ) - 서울대학교 치의학대학원 소아치과학교실
김종철 ( Kim Chong-Chul ) - 서울대학교 치의학대학원 소아치과학교실
현홍근 ( Hyun Hong-Keun ) - 서울대학교 치의학대학원 소아치과학교실
KMID : 1144020130090020107
Abstract
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
Adrenoleukodystrophy; Dental caries; General anesthesia
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