초록

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1988년 9월부터 1999년 4월까지 가톨릭 대학교 의과대학 부속병원 외과에서 황달, 무담즙성 변 및 복부 종괴를 주소로 내원하여 간 외 담관낭이 동반된 담도폐쇄증으로 진단받은 7예의 환자의 임상상을 검토하였다. 전체 선천성 담도폐쇄증 환아 중 9.6 %에서 간 외 담관낭을 동반한 III 형의 담도폐쇄증을 보였다. 남여 성비는 2:5로 여아에서 높았다. 모두 카사이 수술을 시행하였으며 수술 시 평균연령은 57일이었다. 7예 중 3예 (42.9 %)에서 긴 생존기간을 보였다. 예후에 미치는 인자에 대해서는 지속적인 연구관찰이 필요할 것으로 사료된다.

Abstract ▼ AI-Helper

Biliary atresia (BA) with extrahepatic biliary cysts (EHBC) is a rare disease. It has been generally recognized as type I (correctable with cystic dilatation), which means a good prognosis. From a total of 73 patients with BA who underwent operation from September 1988 to September 2003 at our insti...

Biliary atresia (BA) with extrahepatic biliary cysts (EHBC) is a rare disease. It has been generally recognized as type I (correctable with cystic dilatation), which means a good prognosis. From a total of 73 patients with BA who underwent operation from September 1988 to September 2003 at our institute, 7 (9.6 %) cases of type III BA with EHBC (uncorrectable with cystic dilatation) are reviewed. Clinical findings, laboratory data, radiologic findings, treatment methods and outcomes were reviewed. Female was more prevalent (male to female ratio; 2:5). All cases were type III with EHBC according to the intraoperative cholangiography, and underwent Kasai' s portoenterostomy. The mean age was 57 days at operation. Three patients(42.9 %) are long term survivors. Further evaluation is needed to determine the correlation between prognostic factors and outcome for.

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