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dc.contributor.authorDukova, Blagicaen_US
dc.contributor.authorIlievski, Boroen_US
dc.contributor.authorDuganovska, Snezanaen_US
dc.contributor.authorChadikovski, Vladimiren_US
dc.contributor.authorKostovski, Acoen_US
dc.description.abstractIntroduction: Caroli's syndrome (CS) is a rare congenital disorder characterized by intrahepatic bile duct dilatation and congenital hepatic fibrosis. The clinical features of this condition include signs of portal hypertension, cholangitis and lithiasis. Liver transplantation is the ultimate treatment in most patients with liver failure. Case Report: A three month old infant treated with the diagnosis of biliary atresia, after two liver biopsies presented with distended abdomen, hepatosplenomegaly and signs of portal hypertension. Liver transplantation was preformed after four months. We found ectatic hilar bile ducts and intrahepatic bile duct dilatation. The pathologic finding of congenital hepatic fibrosis and proliferated dilated bile ducts suggested the diagnosis of Caroli's syndrome. Conclusion: Caroli's disease and Caroli's syndrome may represent single disorder distinguished by congenital hepatic fibrosis. Fibrosis itself leads to portal hypertension appearing late in patients with Caroli's disease while it's dynamic and progressive in CS. Elevated white blood cell count is due to recurrent cholangitis, cholestasis and hepatolithiasis. Caroli's disease can be associated with extrahepatic bile duct dilatation, but the exact incidence is not known. CS often is associated with kidney lesions and cardiac disease. Liver transplantation should be preformed early. Symptoms are presented early in life due to congenital and progressive hepatic fibrosis. Caroli's syndrome must be considered in differential diagnosis in neonates with jaundice, ascites and hepatosplenomegaly. The first child with liver transplantation in Republic of Macedonia was diagnosed as Caroli's syndrome.en_US
dc.publisherEdorium Journalsen_US
dc.relation.ispartofInternational Journal of Hepatobiliary and Pancreatic Diseasesen_US
dc.subjectCaroli's syndromeen_US
dc.subjectcongenital hepatic fibrosisen_US
dc.subjectIntrahepatic bile duct dilatationen_US
dc.titleLiver pathology findings in infant with Caroli's syndromeen_US
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Appears in Collections:Faculty of Medicine: Journal Articles
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