소아에서 발생한 전신성 모세혈관 누출 증후군(Systemic Capillary Leak Syndrome) 1례

A Case of Systemic Capillary Leak Syndrome in a Child

전신성 모세혈관 누출 증후군(Systemic Capillary Leak Syndrome)은 Clarkson 등이 처음 기술한 이래 50여명의 증례가 보고된 매우 드문 질환이며, 소아에서는 1995년 Ivan Foeldvari 등이 보고한 예가 유일하다. 저자들은 빈호흡, 청색증, 부종, 소변량 감소를 주소로 내원한 6세 여아에서 저혈압, 혈액농축, 저알부민혈증의 소견을 확인하고 전신성 모세혈관 누출 증후군으로 진단한 예를 문헌 고찰과 함께 보고하는 바이다.

Systemic capillary leak syndrome(SCLS) is a rare disorder of unknown etiology, which is characterized by recurrent attacks of hypotension, hemoconcentration, and hypoalbuminemia. Urinary or enteric loss of protein is not demonstrated. It is often associated with monoclonal gammopathy, but does not manifest multiple myeloma. Since Clarkson et al. described the first case in a 34-year-old woman, about 50 cases have been reported in the literature. However, most of the cases were of adult age, and the mean age of onset in the reported cases was 42.6 years. In literature review, we could refer only one pediatric case of SCLC by Foeldvari et al. in 1995. We report another pediatric case of SCLS.