Pelizaeus-Merzbacher 병 환자의 외래전신마취 하 치과치료 -증례보고-Dental Treatment of a Patient with Pelizaeus-Merzbacher Disease under Outpatient General Anesthesia -A Case Report-
김태경, 서광석, 신차욱, 김현정, 염광원,
소속 상세정보
김태경 ( Kim Tae-Kyung ) - 서울대학교병원 마취통증의학과
서광석 ( Seo Kwang-Suk ) - 서울대학교 치의학대학원 치과마취과학교실
신차욱 ( Shin Cha-Uk ) - 서울대학교 치의학대학원 소아치과학교실
김현정 ( Kim Hyun-Jeong ) - 서울대학교 치의학대학원 치과마취과학교실
염광원 ( Yum Kwang-Won ) - 서울대학교 치의학대학원 치과마취과학교실
KMID : 0980220070070010018
Abstract
Pelizaeus-Merzbacher disease (PMD) can be defined as an X-linked recessive leukodystrophy that is caused by a mutation in the proteolipid protein gene on chromosome Xq22. PMD is one of a group of progressive, degenerative disorders of the cerebral white matter known as the leukodystrophies. Due to the progressive nature of the disorders and their devastating effects on the central nervous system, these children frequently require anesthesia during imaging procedures such as MRI or during various surgical procedures. Anesthetic concerns in theses cases include high prevalence of seizure disorders, gastroesophageal reflux with the risk of aspiration, airway complications related to poor pharyngeal muscle control and copious oral secretions, and mental retardation. We report a successful anesthetic management in a patient with PMD for dental procedures.
키워드
Dental procedure;General anesthesia;Leukodystrophy;Pelizaeus-Merzbacher disease
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