Cases reported "Cholestasis, Intrahepatic"

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1/16. Multidisciplinary approach to palliation of obstructive jaundice caused by a central hepatocellular carcinoma.

    BACKGROUND/AIMS: Obstructive jaundice due to intraductal tumour growth is a rare symptom in association with hepatocellular carcinoma (HCC). methods: We report a 65-year-old white male who was admitted to our department with a 2-week history of progressive jaundice. At laparotomy, the liver showed advanced cirrhosis due to long-standing biliary obstruction. cholangiography confirmed total obstruction of the main bifurcation of the hepatic duct by intraductal tumour growth. Combination treatment with surgical segment III drainage, transcatheter arterial embolization and radioembolization with yttrium-90 resin particles and endoscopic stenting was performed. This form of treatment has never been reported before. RESULTS: With these combined procedures, relief of jaundice and a survival time of 32 months could be achieved. CONCLUSION: The combination of palliative methods may relieve jaundice, ensure a good quality of life and possibly prolong survival in patients with mechanical tumour obstruction of the biliary tree by HCC.
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2/16. Hereditary haemorrhagic telangiectasia simulating biliary tree obstruction secondary to malignant porta hepatis lymphadenopathy.

    Hereditary telangiectasis is an uncommon condition and is thus not usually considered in the differential diagnosis of porta hepatis lymphadenopathy causing biliary tree obstruction. The present case illustrates a patient in whom tortuous vessels in the porta hepatis simulated masses on delayed contrast-enhanced CT with subsequent referral for biopsy.
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3/16. Prolonged cholestasis and ductopenia associated with tenoxicam.

    Cholestatic liver diseases leading to progressive destruction of intra-hepatic bile ducts and ductopenia encompass multiple etiologies. Pathophysiology and natural history of drug-induced cholangiopathies remain unclear. We report a case of prolonged ductopenia attributed to Tenoxicam (Tilcotil o--a non-steroidal anti-inflammatory drug of the oxicam family) ingested at therapeutic dose. A 36 year-old male patient was admitted for jaundice and Lyell syndrome starting 1 week after the ingestion of Tenoxicam. Liver biopsy showed cholestasis, non-suppurative cholangitis and polymorphous inflammatory infiltrate of the portal tracts (round cells, macrophages an eosinophils). Treatment with ursodesoxycholic acid and cholestyramine was instituted and the patient was asymptomatic 1 year after. Three years later mild biological cholestasis persisted and ductopenia was evidenced on liver biopsy. In this report we found that: (1) The toxicity of tenoxicam was probably mediated by an immunoallergic mechanism (Lyell syndrome and eosinophils on histology); (2) ductopenia was secondary to inflammatory cholangitis. Factors responsible for this chronic evolution are still unknown (genetic predisposition, vascular factors, etc.); and (3) the presence of ductopenia contrasted with the "clinical recovery" of the disease suggesting accessory bile drainage by cholangioles or partial reconstruction of the biliary tree.
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4/16. Restoration of bile ducts in drug-induced vanishing bile duct syndrome due to zonisamide.

    Prolonged drug-induced cholestasis may be due to destruction and disappearance of bile ducts, sometimes referred to as vanishing bile duct syndrome. Although some of these cases progress to fibrosis, cirrhosis, and liver failure, others improve with time. We report a case of a 35-year-old man who developed vanishing bile duct syndrome after ingestion of zonisamide, an antiepileptic drug that is also prescribed for weight loss. His liver biopsy showed complete absence of bile ducts 3 weeks after starting treatment. There was no ductular reaction. The drug was stopped and a follow-up biopsy 3 months later showed strands of intermediate hepatobiliary cells at the periphery of the portal tracts that extended into lobules; these structures lacked lumina and expressed biliary cytokeratins, CK7 and CK19. A third biopsy, 7 months later, showed the presence of ductules with lumina located within portal tracts. Intermediate hepatobiliary cells were rare; although sparse clusters of hepatocytes with membrane staining for CK7 were present. cholestasis and levels of bilirubin improved over time. The histologic features in this case document the sequence of events in restoration of the biliary tree after loss of bile ducts, which seems to be a process of maturation of intermediate hepatobiliary cells that arise from a proliferative compartment at the porto-hepatic interface.
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5/16. biliary tract obstruction in the acquired immunodeficiency syndrome.

    Three patients with the acquired immunodeficiency syndrome had biliary obstruction resulting from benign strictures of the biliary tract. Stenosis of the distal common bile duct with differing degrees of irregularity of the smaller intrahepatic and extrahepatic ducts was seen in association with either cryptosporidial or cytomegaloviral infection of the biliary tree. We review cytomegaloviral and cryptosporidial infections of the biliary system, as well as possible relationships with idiopathic primary sclerosing cholangitis. Stenotic biliary tract disease appears to be yet another complication of the acquired immunodeficiency syndrome.
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6/16. Intrahepatic cholestasis associated with an enlarged gall-bladder.

    A 60-year-old man with a history of ingesting herbal medication and a 59-year-old woman with malignant lymphoma presented with painless jaundice and palpably enlarged gall-bladders. Abdominal ultrasonography confirmed that the gall-bladders were enlarged, but showed normal-sized biliary trees with no stones. The final diagnoses for these patients were drug-induced hepatitis with intrahepatic cholestasis and lymphomatous infiltration of the liver, respectively. A palpable gall bladder in cholestatic jaundice may not always be caused by extrahepatic biliary obstruction, and ultrasonography is very useful in ruling this out.
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7/16. Caustic sclerosing cholangitis. A complication of the surgical treatment of hydatid disease of the liver.

    In five patients, sclerosing cholangitis developed after the surgical treatment of hydatid cyst of the liver. The cyst communicated with the biliary tree, and a scolicidal solution (2% formaldehyde in two patients and 20% sodium chloride in three) was injected into the cyst. cholangiography showed strictures affecting the intrahepatic biliary tree in two and both the intrahepatic and extrahepatic biliary tree in three. Sclerosing cholangitis in these patients was likely to result from the caustic effect of the scolicidal solution having diffused from the cyst into the biliary tree. We propose to designate this entity "caustic sclerosing cholangitis". Because of the risk of this complication, and the unproved efficacy of intracystic injection of a scolicidal solution in preventing the dissemination of the parasite, we recommend that this maneuver be abandoned in the surgical treatment of hydatid disease of the liver.
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8/16. atrophy and ductopenia of the right hepatic lobe in a patient with choledocholithiasis.

    An extremely rare case of atrophy and extensive ductopenia of the right hepatic lobe is presented. The surgically resected atrophic right lobe (100 g) did not show cholestasis or cirrhosis. The right hepatic bile duct revealed sclerosis with luminal obliteration and marked diminution of its branches. The extrahepatic bile duct contained a single cholesterol stone. The right portal vein branches showed luminal narrowing. Disturbance of biliary drainage following sclerosing cholangitis and impediment of portal venous flow in the right hepatic lobe were considered responsible for atrophy of the lobe. Although choledocholithiasis presumably played an important part in the pathogenesis of the sclerosing cholangitis and ductopenia, the reason for selective involvement of the right biliary tree remains nuclear.
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9/16. Familial intrahepatic cholestatic cirrhosis in young adults.

    Two siblings with intrahepatic cholestatic cirrhosis and their brother, who had a potentially related disease at the time of accidental death, are presented. The onset of disease occurred during adolescence in all 3 cases. The initial sign was mild jaundice or portal hypertension. There was no abnormality in the countenance, cardiovascular system, or vertebral column. Except for the brother who died from an accident, jaundice gradually increased. death followed due to cirrhosis. Liver biopsy specimens of these 2 patients showed diminution of interlobular bile ducts with no significant cholangitis. At autopsy, the livers of the 2 patients showed biliary cirrhosis without extrahepatic biliary obstruction. In both cases there was an accessory lobe on the right hepatic lobe. Histologically, septal bile ducts showed pronounced papillary proliferations of the epithelium; there was also a decrease in the number of small interlobular bile ducts. Excess copper accumulation in the liver was ascertained. It is suggested that the disease in the 2 autopsied cases is intrahepatic cholestatic cirrhosis due to hypoplasia of the intrahepatic biliary trees.
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10/16. 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.
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