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KMID : 0816119990020020262
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Korean Journal of Pediatric Gastroenterolology and Nutrition
1999 Volume.2 No. 2 p.262 ~ p.266
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A Case of Spontaneous Resolution of Bile Plug Syndrome in a 4-year-old Girl
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Jee Keum-Bong
Kim Dug-Ha
Min Ki-Sik
Yoo Ki-Yang
Lee Kwan-Seop
Song Joon-Young
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Abstract
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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.
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KEYWORD
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Bile Plug Syndrome,
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