Cases reported "Sclerosis"

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1/12. Percutaneous transhepatic cholangiography. Problems in interpretation.

    The patient who presents with jaundice, regardless of the etiology, cannot be adequately examined by a gallbladder series and intravenous cholangiography. Clinical evaluation aided by laboratory analysis will not always differentiate between primary liver parenchymal disease and biliary tract obstruction. Percutaneous transhepatic cholangiography, when successfully performed, answers the question of whether the jaundice is due to primary liver parenchymal disease or due to biliary tract obstruction. The point to emphasize is that under no circumstances is it appropriate to presume any information if the biliary system is not entered and visualized successfully. The proper interpretation of the level of block can fall prey to the mistake of incomplete aspiration of the thick, inspissated bile in the obstructed biliary tree before injection of contrast material. The problem will be worsened by a peripheral entrance into the biliary system rather than a more central one. Gastrointestinal series should always be available to aid in identifying pathology at the entrace of the common bile duct into the duodenum. Lastly, identifying the etiology at the site of the block will require additional procedures. Selective visceral angiography has contributed greatly in this area. During the 20 years of clinical usage of percutaneous transhepatic cholangiography, we feel it has become the single examination capable of preventing unnecessary exploration of the jaundiced patient with primary liver parenchymal disease and the most useful potential source of practical information if laparotomy is necessary to correct biliary tract obstruction.
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2/12. Sclerosing cholangitis after continuous hepatic artery infusion of FUDR.

    Eight of 46 (17.4%) patients treated in our trial of continuous hepatic artery infusion (CHAI) of fluorodeoxyuridine (FUDR) by Infusaid pump developed biliary strictures. The lesions were clinically, radiographically, and pathologically identical to the idiopathic sclerosing cholangitis frequently seen in association with inflammatory bowel disease. Treatment included immediate cessation of intraarterial FUDR, and surgical or percutaneous drainage of the biliary tree if it was dilated. Two of the eight patients died of the complication. Three patients stabilized after biliary system drainage, and two patients improved on observation only. The pathogenesis of this complication is not understood. This report details the clinical and pathological features of this entity.
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3/12. Acalculous inflammatory disease of the biliary tree in acquired immunodeficiency syndrome: CT demonstration.

    Acalculous inflammatory disease of the biliary tree is a recently recognized complication of acquired immunodeficiency syndrome (AIDS). Two men with AIDS and elevated serum alkaline phosphatase levels are reported. Computed tomography and cholangiography demonstrated biliary disease resembling sclerosing cholangitis.
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4/12. 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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5/12. Primary sclerosing cholangitis and pregnancy.

    Primary sclerosing cholangitis is a chronic, fibrosing, inflammatory disorder of unknown etiology affecting the biliary tree. We describe a case of a pregnancy complicated by this condition. Remarkably, maternal cholestasis improved with advancing gestation. Despite a marked elevation of bile acid levels in cord blood, the patient was delivered of a healthy term infant. The principles of management and potential effects of primary sclerosing cholangitis on pregnancy care are discussed.
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6/12. Primary sclerosing cholangitis.

    Primary sclerosing cholangitis is a chronic, cholestatic disease affecting the biliary tree. Recent data suggest an autoimmune etiology. Clinical findings, roentgenographic characteristics, and compatible liver histology will help in establishing the diagnosis. There is no known treatment for cure, though relief of symptoms may be accomplished with certain drugs, such as antibiotics for cholangitis and cholestyramine for pruritus. death usually ensues within five to seven years after diagnosis, as a consequence of liver failure, cholangitis, and cholangiocarcinoma.
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7/12. Sclerosing cholangitis associated with autoimmune hemolytic anemia and hyperthyroidism.

    A patient presented with autoimmune hemolytic anemia and sclerosing cholangitis. Six years later he was found to be hyperthyroid. Although sclerosing cholangitis has been associated with other autoimmune disorders, it has not been reported in association with either autoimmune hemolytic anemia or hyperthyroidism. Sclerosing cholangitis is a chronic fibrosing inflammatory disease process which may include parts or all of the biliary tree including the gallbladder. Although this disorder has been reported to occur with a variety of autoimmune disorders, it has not been previously associated with autoimmune hemolytic anemia and hyperthyroidism.
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8/12. Localized sclerosing cholangitis?

    Primary sclerosing cholangitis presenting as a localized stricture affecting a segment of the extrahepatic biliary tree is seldom found. This article describes the case of a 56-year-old male admitted with obstructive jaundice, in whom endoscopic retrograde cholangiography showed a localized and smooth stricture at the common hepatic duct, initially diagnosed as cholangiocarcinoma. However, the histological examination of the resected stricture, common bile duct and gallbladder, and a liver biopsy performed 3.5 years later were consistent with primary sclerosing cholangitis, affecting both the extrahepatic and intrahepatic biliary tree.
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9/12. Sclerosing cholangitis and histiocytosis X.

    Three patients with biopsy proven histiocytosis X who developed a clinical and pathological picture compatible with sclerosing cholangitis are reported. In one patient, operative biopsy of the common bile duct revealed histiocytosis X in the granulomatous/xanthomatous phase. At necropsy, however, only fibrosis of the biliary tree was seen, a picture consistent with sclerosing cholangitis. Fibrotic obstruction of the biliary tree led to death from liver failure in all three patients. The aetiology of primary sclerosing cholangitis is unknown and may be multifactorial. Perhaps involvement of the biliary tree by histiocytosis X is one cause.
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10/12. Primary sclerosing cholangitis: CT findings.

    Computed tomographic (CT) findings in six cases of primary sclerosing cholangitis (PSC) are described. Computed tomographic findings correlate with cholangiographic findings and show ductal dilatations and distortions in the porta hepatis and in the liver parenchyma. The CT features are round, oval, or irregular, sometimes branching, focal dilatations of the biliary tree. When subtle, irregular dilatations of the intrahepatic biliary tree are observed, the presumptive diagnosis of sclerosing cholangitis can be made from CT. Confirmation of this diagnosis with cholangiography is essential in all patients. Recognition of this entity by a noninvasive method will avoid long delays in diagnosis and will expedite the use of the more definitive procedures such as endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography, or liver biopsy.
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