Cases reported "Cholestasis, Intrahepatic"

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11/16. Tubulointerstitial nephropathy associated with arteriohepatic dysplasia.

    An infant with a variant of arteriohepatic dysplasia (characteristic facies, valvular and peripheral pulmonic stenosis, hypercholesterolemia, intrahepatic cholestasis) and a severe tubulointerstitial nephropathy is described. liver biopsy at 2 mo and at 10 mo of age revealed marked hepatocellular cholestasis and a slight decrease in the number of interlobular bile ducts per portal triad. Severe fibrosis was noted at 10 mo. Renal findings included tubular atrophy, microcystic tubular dilatation, and interstitial fibrosis. death at 10 mo was associated with renal failure and pneumonia. The case suggests an additional association between renal cystic disease and abnormalities of the biliary tree.
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12/16. Intrahepatic cholestasis and sicca complex after thiabendazole. Report of a case and review of the literature.

    thiabendazole is a relatively safe and effective agent with a wide range of activity against nematodes infesting the gastrointestinal tract. A 55-yr-old man developed prolonged jaundice and sicca complex after a course of thiabendazole therapy. Endoscopic retrograde cholangiopancreatography demonstrated normal biliary tree and pancreatic ducts. liver biopsy was consistent with a drug-induced intrahepatic cholestatic reaction. The patient recovered completely from his liver injury, but the sicca complex persisted 1 yr after the drug was given. The literature on thiabendazole-induced cholestasis and its association with sicca complex is reviewed.
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13/16. Tc-99m HIDA scintigraphy in segmental biliary obstruction.

    Segmental biliary obstruction as a result of primary or secondary hepatic malignancy has been reported with increasing frequency. For two representative patients, the clinical and Tc-99m HIDA scintigraphic findings in segmental biliary obstruction are described. The presence of photon-deficient dilated bile ducts in one segment of the biliary tree is highly suggestive of localized biliary obstruction and should be considered in the patient with suspected or proven hepatic malignancy despite the absence of jaundice.
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14/16. Ultrasound demonstration of anicteric dilatation of the biliary tree.

    This report presents eight patients with dilatation of the biliary tree, six of whom had no clinical or laboratory evidence of biliary tract disease except for elevated levels of serum alkaline phosphatase. In two patients, dilatation was caused by obstruction of only one hepatic duct; in three, it was caused by a pancreatic tumor; and in three, it was associated with gallstones which exercised a ball-valve effect, producing intermittent obstruction. The elevation of the alkaline phosphatase level in six of these patients suggests that it is a more sensitive indicator of biliary tract obstruction than the serum bilirubin level is. The recognition of biliary dilatation in one or both lobes of the liver should lead to a careful examination, with ultrasound or another modality, of the porta hepatis, common bile duct, and pancreas.
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15/16. Benign recurrent intrahepatic cholestasis.

    Benign recurrent intrahepatic cholestasis is a rare disorder of unknown etiology and has not yet been reported in taiwan. We report a case with a typical clinical course. A 17-year-old Taiwanese boy had three episodes of pruritus and jaundice from February 1993 to July 1995, each lasting 3 to 4 months. jaundice spontaneously subsided and he was symptom-free during periods of remission. A fourth episode of pruritus began in July 1995, with jaundice developing later and lasting for 3 months. Laboratory tests revealed direct hyperbilirubinemia. Endoscopic retrograde cholangiopancreatography showed normal intra- and extrahepatic biliary trees. light microscopy of a liver biopsy sample revealed hepatocellular and canalicular cholestasis with bile retention in the kupffer cells. Benign recurrent intrahepatic cholestasis was diagnosed after exclusion of other possible causes of jaundice. The patient made an uneventful recovery.
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16/16. Prolonged cholestatic jaundice after endoscopic retrograde cholangiography.

    The main complications of endoscopic retrograde cholangiography and sphincterotomy are bleeding, pancreatitis, perforation and sepsis. Two cases of unexplained prolonged cholestatic jaundice in patients who underwent endoscopic retrograde cholangiography (ERC) for biliary obstruction due to choledocholithiasis are reported. The patients were admitted because of right upper quadrant pain, vomiting and jaundice. Laboratory tests showed increased levels of total and conjugated serum bilirubin and increased alkaline phosphatase. Ultrasound examination showed cholelithiasis and choledocholithiasis with bile duct dilatation. ERC with sphincterotomy was performed and gallstones obstructing the common bile duct were removed endoscopically. Following ERC and despite complete patency of the biliary tree, a progressive increase of total and conjugated bilirubin and of alkaline phosphatase was noted, associated with itching and total stool discoloration. The insertion of nasobiliary drain did not improve the jaundice. prednisolone treatment for 12 days was associated with progressive restoration of serum bilirubin alkaline phosphatase to normal levels. It was postulated that the radiocontrast material used may have acted toxically on the liver with disruption of the canalicular plasma membrane. It is proposed that intrahepatic cholestasis should be added in the list of complications of endoscopic retrograde cholangiography.
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