1/12. Case report: two cases of biliary papillomatosis with unusual associations.Papillomatosis arising from the biliary tree is a well recognized but rare entity. We encountered two patients with this condition. However, one of them had associated hepatocellular carcinoma and cirrhosis and the other had concomitant recurrent pyogenic cholangitis. To our knowledge, these associations have not been reported before. We, therefore, present these clinical problems and highlight the added difficulty in the management of these patients.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
2/12. Recurrent bacteremia with enteric pathogens in recessive polycystic kidney disease.Eight children with autosomal recessive polycystic kidney disease (ARPKD) and recurrent bacteremia with enteric pathogens are described. Typical clinical features of bacterial cholangitis were absent, although in five patients histological and/or microbiological data indicated that the bacteremic episodes originated in the biliary tree. bacteremia with enteric pathogens or recurrent culture-negative febrile illness in a child with ARPKD should raise suspicion of cholangitis, even in the absence of typical clinical findings.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
3/12. Caroli's disease and congenital hepatic fibrosis associated with polycystic kidney disease. A case presenting with acute focal bacterial nephritis.Congenital cystic dilatation of the intrahepatic biliary ducts (Caroli's disease), until recently, has been infrequently recognized. It is often associated with autosomal recessive polycystic kidney disease (ARPKD) and congenital hepatic fibrosis (CHF). We hereby report a case with Caroli's disease, polycystic kidney disease (PKD), and CHF: This 24-year-old female patient initially presented with acute bacterial nephritis (ABN). Renal ultrasonography revealed bilateral enlarged kidneys with multiple cysts. Because her parents showed no renal cyst on ultrasonographic examination, she received further studies. Abdominal ultrasonography showed cystic dilatation of the biliary tree. Computed tomography (CT) with meglumine lotroxinate (biliscopin) infusion study and hepatobiliary scintigraphy confirmed the diagnosis of Caroli's disease. Liver biopsy revealed CHF: The radiographic and scintigraphic pictures are hereby illustrated and CT with biliscopin infusion study is emphasized. We conclude that if radiologic evidence of renal cystic lesions is absent in the parents of patients with PKD, the coexistence of Caroli's disease and CHF should be considered. The clinical pictures of ABN in this patient are also discussed. As far as we know, this is the first reported case of ABN in a patient with PKD and Caroli's disease, and it showed good response to antibiotic therapy.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
4/12. pathology of hepatolithiasis associated with biliary malformation in japan.A national survey of the morphologic features of hepatolithiasis in japan disclosed that 13 (4.8%) of the 273 cases examined were associated with obvious biliary malformations: Caroli's disease with congenital hepatic fibrosis (one case), adult polycystic disease of the liver and kidneys (one case), choledochal cyst (nine cases), and anomalous pancreaticobiliary ductal union (four cases). The latter two malformations overlapped in two cases. Intrahepatic calculi in all of these cases were of brown pigment stones, suggesting that focal bile stasis as well as bacterial infection might have played an important role in their formation and growth. It seems likely that such phenomena occur in the congenitally dilated segments of the intrahepatic biliary tree. These data imply that biliary malformation is one of the lithogenic factors in hepatolithiasis in japan.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
5/12. The spectrum of hepatic dysfunction in inflammatory bowel disease.The spectrum and incidence of liver disease is described among a large series of patients with inflammatory bowel disease. The incidence of significant liver disease identified by the presence of serial biochemical abnormalities of liver function was 8.2 per cent. Transient peri-operative changes in liver function tests are common and usually relate to underlying intra-abdominal sepsis. Percholangitis, sometimes termed portal triaditis, is one of the commoner lesions, and is usually associated with extensive colitis and improves with resection of the underlying bowel disease. Cirrhosis of the liver is an important but uncommon complication and is usually associated with extensive long-standing disease. Stenosing cholangitis and biliary tract carcinoma are both important though rare associations. They are both associated with extensive disease of long-standing, but resection of the underlying inflammatory bowel disease does not necessarily protect the individual from these complications. Although stenosing cholangitis is a diffuse lesion of the biliary tree it is important to exclude strictures of the extra-hepatic biliary tree which may be amenable to surgical correction. Hepatic dysfunction is rarely the sole indication for advising surgery for the underlying bowel disease but the identification of the nature of the hepati- dysfunction provides a rational basis for such a decision and opportunities for the surgical correction of the hepatic lesion itself.- - - - - - - - - - ranking = 2keywords = tree (Clic here for more details about this article) |
6/12. Multiple cysts in the hepatic hilum and their pathogenesis. A suggestion of periductal gland origin.Multiple serous cysts involving only the hepatic hilum and larger portal tracts were found incidentally in 8 autopsy cases with several underlying liver diseases. The cysts were mostly small in size, and did not communicate with the lumena of the biliary tree. The lining of the cysts consisted of a single layer of columnar or cuboidal epithelium and the surrounding fibrous tissue was scanty. These were not associated with polycystic disease of the kidneys or cystic dilatation of the biliary tree. cysts were absent in the liver parenchyma and an association with von Meyenburg complexes was rare. In the vicinity of the cysts there were many lobules of the glands which are physiologically present in the periductal connective tissue of the large intrahepatic bile ducts. It was noted that some of these glandular elements around the cysts disclosed varying degrees of cystic luminal dilatations. Almost all patients with such cysts had severe portal hypertension and over a half had occluding thromboemboli in the portal veins. These results lead us to propose the hypothesis that multiple cysts involving the hepatic hilum and the larger portal tracts are derived from the periductal glands in close association with an intrahepatic circulatory disturbance of the portal venous system.- - - - - - - - - - ranking = 2keywords = tree (Clic here for more details about this article) |
7/12. Caroli's disease: role of endoscopic retrograde cholangiopancreatography.Several modalities have been used in the diagnosis of congenital dilatation of the intrahepatic bile ducts (Caroli's disease) with varying degrees of reliability and morbidity. We report two patients with Caroli's disease where endoscopic retrograde cholangiopancreatography was used to define the anatomy of the extra and intrahepatic biliary trees. In one of the patients (BT) esophageal varices were easily visualized on endoscopy which had been previously demonstrated by a more invasive procedure (splenoportography). There were no complications of retrograde cholangiopancreatography in these two patients. We believe that retrograde cholangiopancreatography is a safe and effective method of evaluating the biliary tree and status of portal hypertension in patients with Caroli's disease as well as providing a reliable means of following the progression of their disease.- - - - - - - - - - ranking = 2keywords = tree (Clic here for more details about this article) |
8/12. Florid duct lesions and extensive bile duct loss of the intrahepatic biliary tree in chronic liver diseases other than primary biliary cirrhosis.Intrahepatic biliary tree with either florid duct lesions or a moderate to severe degree of the duct loss in four livers with chronic hepatic diseases other than primary biliary cirrhosis were studied with histometric and serial section observations. Florid duct lesions, distributed segmentally in the liver, were found in one case with incomplete septal cirrhosis and one case with idiopathic portal hypertension. The florid duct lesions including marked plasma cell infiltration and occasional periductal granulomas, were not associated with any bile duct loss in the two cases. The duct lesions were reversible in one case during a long clinical course. On the other hand, a moderate to severe bile duct loss with biliary epithelial degeneration and necrosis was associated with no or little periductal inflammatory cell infiltration in one other case with chronic intrahepatic cholestasis, probably drug-induced, and in one case with idiopathic portal hypertension. Although florid duct lesions and bile duct loss were important diagnostic features of primary biliary cirrhosis, one of them was observed to develop independently in severely diseased livers, not consistent with a diagnosis of primary biliary cirrhosis, sclerosing cholangitis or intrahepatic bile duct paucity syndrome.- - - - - - - - - - ranking = 5keywords = tree (Clic here for more details about this article) |
9/12. Congenital hepatic fibrosis. A case report with study of three dimensional reconstruction of serial sections of the liver.A patient who developed pancytopenia and hepatosplenomegaly with portal hypertension is described. Liver wedge biopsy at the time of operation showed typical histology of congenital hepatic fibrosis. Renal surgical biopsy revealed tubular ectasia. A continuous anastomosing network of the biliary tree was observed by reconstruction of serial sections of the liver. Some parts of the network were dilated and no isolated bile ducts were present.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
10/12. Noninvasive diagnosis of Caroli syndrome associated with congenital hepatic fibrosis using hepatobiliary scintigraphy.A patient who was studied for portal hypertension had an enlarged liver and multiple hepatic cysts on abdominal CT scan. He underwent hepatobiliary scintigraphy using Tc-99m mebrofenin which documented communication of the cysts with the main biliary tree and allowed a noninvasive diagnosis of Caroli syndrome associated with congenital hepatic fibrosis.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
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