로터 증후군(Rotor’s syndrome) 1례
A Case of Rotor’s Syndrome
김성수, 박재옥, 고은석,
소속 상세정보
김성수 ( Kim Sung-Soo )
순천향대학교 의과대학 소아과학교실
박재옥 ( Park Jae-Ock )
순천향대학교 의과대학 소아과학교실
고은석 ( Koh Eun-Seok )
순천향대학교 의과대학 병리학교실
KMID : 0816120040070020243
Abstract
저자 등은 정계정맥류 수술을 위해 본원 비뇨기과에 입원한 14세 남아가 공막의 황달과 직접형 빌리루빈의 증가 소견을 보였고 간생검 등으로부터로터 증후군으로 증명되어 참고문헌과 아울러 보고하는 바이다.
Rotor’s syndrome is a hereditary disorder characterized by predominantly conjugated hyperbilirubinemia with normal hepatic histology. It resembles Dubin-Johnson syndrome but the main differences are no dark brown pigmentation in the hepatic cells and visualization of the gallbladder in oral cholangiography. We experienced a 14 year-old male patient who had icteric sclerae and predominantly conjugated hyperbilirubinemia when he was hospitalized for varicocelectomy. His liver biopsy specimen showed no dark brown pigmentation and any other pathologic abnormalities in the hepatic cells. Hepatobiliary scan shows no evidence of obstructive lesions. His urinary excretion of total coproporphyrin was markedly increased.
키워드
Rotor’s syndrome;Hyperbilirubinemia
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