Lowe 증후군을 동반한 소아치과 환자의 전신 마취 경험 : 증례보고
An Anesthetic Management in a Pedodontic Patient with Lowe Syndrome : A case report
최영규, 오재열, 김동옥, 신옥영, 이긍호,
소속 상세정보
최영규 ( Choi Young-Kyu ) - 경희대학교 의과대학 마취통증의학교실
오재열 ( Oh Jae-Yeol ) - 경희대학교 의과대학 마취통증의학교실
김동옥 ( Kim Dong-Ok ) - 경희대학교 의과대학 마취통증의학교실
신옥영 ( Shin Ok-Young ) - 경희대학교 의과대학 마취통증의학교실
이긍호 ( Lee Keung-Ho ) - 경희대학교 치과대학 소아치과학교실
KMID : 0980220020020010033
Abstract
The Oculo-cerebro-renal syndrome of Lowe (Lowe syndrome) is an X-linked recessive disorder involving the eyes, nervous systems, and kidneys. The clinical manifestation of this syndrome is characterized by congenital cataracts, glaucoma, seizure disorder, psychomotor growth retardation, hypotonia, renal tubular acidosis, aminoaciduria, rickets, and osteoporosis. We report a 5-year old boy underwent general anesthesia for the treatment of multiple dental carries. During intraoperative period, marked metabolic acidosis was noted and such acidosis was partially corrected by hyperventilation. We suggest that patients with Lowe´s syndrome should be attention and treated to possible anesthetic hazards such as metabolic acidosis due to renal tubular dysfunction, rise of intraocular pressure in patient with glaucoma, the fragility of the bone structures due to rickets and osteoporosis.
키워드
Oculo-cerebro-renal syndrome of Lowe
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