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KMID : 1144020130090020107
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Journal of Korean Association for Disability and Oral Health
2013 Volume.9 No. 2 p.107 ~ p.110
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DENTAL TREATMENT OF A 11-YEAR-OLD MALE PATIENT WITH ADRENOLEUKODYSTROPHY UNDER GENERAL ANESTHESIA : A CASE REPORT
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Jang Jun-Hyuk
Shin Teo-Jeon
Kim Young-Jae
Kim Jung-Wook
Jang Ki-Taeg
Lee Sang-Hoon
Kim Chong-Chul
Hyun Hong-Keun
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Abstract
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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.
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KEYWORD
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Adrenoleukodystrophy, Dental caries, General anesthesia
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