Lowe 증후군 환자의 전신 마취 관리

Lowe syndrome (the oculo-cerebro-renal syndrome of Lowe, OCRL) is a multi-system disorder that affects the eyes, nervous system, and kidney. OCRL is a rare X-linked recessive disease with a prevalence of approximately 1:500,000. The clinical features of OCRL include congenital cataracts, growth and...

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Bibliographic Details
Published in:Korean journal of anesthesiology 2009, 56(1), , pp.112-115
Main Authors: 정진용, Jin Yong Chung, 권재현, Jae Hyoun Kwon, 서귀주, Kwi Chu Seo, 송석영, Seok Young Song, 노운석, Woon Seok Roh, 김봉일, Bong Il Kim
Format: Article
Language:Korean
Subjects:
General anesthesia
Hypotonia
Lowe syndrome
Metabolic acidosis
마취과학
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Summary:Lowe syndrome (the oculo-cerebro-renal syndrome of Lowe, OCRL) is a multi-system disorder that affects the eyes, nervous system, and kidney. OCRL is a rare X-linked recessive disease with a prevalence of approximately 1:500,000. The clinical features of OCRL include congenital cataracts, growth and mental retardation, areflexia, hypotonia, and renal tubular dysfunction (Fanconi-type). Chronic metabolic acidosis and hypotonia may be the most important component affecting management of the peri-anesthetic period during general anesthesia. However, problems such as electrolyte imbalance, seizure, fragility of the bone structures, and increased intraocular pressure should also be considered during the perioperative period. We report here the perioperative management of a patient with Lowe syndrome during the removal of multiple scalp cysts under general anesthesia. (Korean J Anesthesiol 2009;56:112~5)
ISSN:2005-6419
2005-7563