4세 여아에서 자연 완해된 담즙 마개 증후군 1례
A Case of Spontaneous Resolution of Bile Plug Syndrome in a 4-year-old Girl
지금봉, 김덕하, 민기식, 유기양, 이관섭, 송준영,
소속 상세정보
지금봉 ( Jee Keum-Bong )
한림대학교 의과대학 소아과학교실
김덕하 ( Kim Dug-Ha )
한림대학교 의과대학 소아과학교실
민기식 ( Min Ki-Sik )
한림대학교 의과대학 소아과학교실
유기양 ( Yoo Ki-Yang )
한림대학교 의과대학 소아과학교실
이관섭 ( Lee Kwan-Seop )
한림대학교 의과대학 방사선과학교실
송준영 ( Song Joon-Young )
한림대학교 의과대학 소아과학교실
KMID : 0816119990020020262
Abstract
Obstruction of the extrahepatic bile ducts is the most common cause of conjugated
hyperbilirubinemia in early infancy. More than 90% of such obstructive lesions are
accounted for by extrahepatic biliary atresia. A rare lesion is obstruction of the common
duct by impacted, thickened secretions and bile. Bile plug syndrome is defined as
extrahepatic obstruction of the bile ducts by bile sludge in term infants without
anatomic abnormalities, congenital chemical defects of bile, or hepatocellular lesions.
Obstruction of extrahepatic ducts by plugs of biliary material apperas to be due to the
inspissation and precipitation of bile and mucus within the lumen of the ducts.
Cholestasis and precipitation of bile develop in association with abnormal composition of
bile in cystic fibrosis, hepatocellular damage, prolonged erythroblastic jaundice, altered
biliary dynamics with total parenteral nutrition, gut dysfunction, diuretic therapy,
exchange transfusions and perinatal hemolysis. In those cases, the term inspissated bile
syndrome is used. The clinical and laboratory findings in bile plug syndrome are
identical to those observed in biliary atresia and choledochal cyst. The diagnosis can be
suspected based on the findings of clinical and laboratory examinations together with
hepatobiliary imaging, ultrasonography, radionuclide scan and liver biopsy. We
experienced a case of spontaneous resolution of bile plug syndrome in a 4-year-old girl.
We report this case with brief review related literatures.
키워드
Bile Plug Syndrome;
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