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http://dx.doi.org/10.12655/KADH.2013.9.2.107

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

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)
Publication Information
The Journal of Korea Assosiation for Disability and Oral Health / v.9, no.2, 2013 , pp. 107-110 More about this Journal
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.
Keywords
Adrenoleukodystrophy; Dental caries; General anesthesia;
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