Cases reported "Cholestasis, Intrahepatic"

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1/159. Are expandable metallic stents better than conventional methods for treating difficult intrahepatic biliary strictures with recurrent hepatolithiasis?

    BACKGROUND: Conventional methods for treating patients with recurrent hepatolithiasis associated with complicated intrahepatic biliary strictures include balloon dilatation of the intrahepatic biliary strictures, lithotripsy, and the clearance of difficult stones as completely as possible, with the placement of an external-internal stent for at least 6 months. After these modalities are used, symptomatic refractory strictures remain. Recently we used internal Gianturco-Rosch metallic Z stents to treat patients who had refractory strictures. OBJECTIVE: To compare therapeutic results and complications of an internal expandable metallic Z stent with those of repeated external-internal stent placement. STUDY DESIGN: Case-control study. SETTING: A referral center. patients: From January 1992 to December 1996, 18 patients with recurrent hepatolithiasis and complicated intrahepatic biliary strictures underwent percutaneous dilatation of stricture and transhepatic percutaneous cholangioscopic lithotomy for recurrent stones. After their stones were completely cleared, their biliary strictures failed to dilate satisfactorily. The patients were randomly enrolled into 2 groups: group A (7 patients), who received an expandable metallic Z stent, and group B (11 patients), who had repeated placement of external-internal stents. INTERVENTIONS: Percutaneous stricture dilatation, electrohydraulic lithotripsy, balloon dilatation, percutaneous transhepatic cholangioscopic lithotomy, and biliary stenting by a Silastic external-internal catheter or a modified Gianturco-Rosch expandable metallic Z stent (for an internal stent). MAIN OUTCOME MEASURES: The number of procedures, days in hospital, procedure-related complications, incidents of stone recurrence and recurrence of cholangitis, readmissions to the hospital, treatment sessions required, and mortality rate. patients' limitations in ordinary activities were also compared. RESULTS: The follow-up period ranged from 28 to 60 (40.7 /-12.7 [mean /- SD]) months in group A and from 28 to 49 (36.0 /-7.2) months in group B. Fewer group A patients (3 [43%]) than group B patients (8 [73%]) tended to have recurrent cholangitis and to require readmission to the hospital, but this was not statistically significant (P = .33). When their cumulative probability of a first episode of cholangitis during follow-up was compared, however, it was significantly lower in patients treated with a metallic stent (P = .04). Compared with group B patients, group A patients had less frequent recurrence of stones (0% vs 64%; P = .01), fewer procedures for the clearance of biliary stones or sludge (1.7 /-2.2 vs 6.4 /-4.3; P = .03), and shorter hospital stays (8.0 /-11.5 days vs 17.0 /-12.0 days; P = .07). No patients in group A experienced limitation in ordinary activities, whereas 7 patients in group B did (P<.02). CONCLUSIONS: Compared with the repeated placement of external-internal stents, the use of a metallic internal stent effectively decreases stone recurrence, simplifies further procedures, and is more convenient. Its use is suggested as an alternative choice in the treatment of recurrent hepatolithiasis with refractory intrahepatic biliary strictures.
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keywords = stone
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2/159. Fibrosing cholestatic hepatitis in renal transplant recipients with hepatitis c virus infection.

    Fibrosing cholestatic hepatitis (FCH) has been described as a specific manifestation of hepatitis b virus (HBV) infection in liver allograft recipients characterized by a rapid progression to liver failure. Only sporadic cases have been reported in other immunocompromised groups infected with HBV and in a few transplant recipients with hepatitis c virus (HCV) infection. We present the occurrence of FCH in 4 HCV-infected renal transplant recipients within a series of 73 renal transplant recipients with HCV infection followed up closely serologically and with consecutive liver biopsies. All 4 patients received the triple-immunosuppressive regimen (azathioprine, cyclosporine A, methylprednisolone). The interval from transplantation to the appearance of liver dysfunction was 1 to 4 months and to histological diagnosis, 3 to 11 months. The biochemical profile was analogous to a progressive cholestatic syndrome in 3 patients, whereas the fourth patient had only slightly increased alanine aminotransferase and gamma-glutamyl transferase (gammaGT) levels. Liver histological examination showed the characteristic pattern of FCH in 2 patients, whereas the other 2 patients had changes compatible with an early stage. All patients were anti-HCV negative at the time of transplantation, whereas 2 patients, 1 with incomplete and 1with complete histological FCH features, seroconverted after 3 and 31 months, respectively. The patients were HCV rna positive at the time of the first liver biopsy and showed high serum HCV rna levels (14 to 58 x 10(6) Eq/mL, branched dna). HCV genotype was 1b in 3 patients and 3a in 1 patient. After histological diagnosis, immunosuppression was drastically reduced. Two patients died of sepsis and liver failure 16 and 18 months posttransplantation, whereas the seroconverted patients showed marked improvement of their liver disease, which was histologically verified in 1 patient. In conclusion, FCH can occur in HCV-infected renal transplant recipients. It seems to develop as a complication of a recent HCV infection during the period of maximal immunosuppression and is associated with high HCV viremia levels. There are indications that drastic reduction of immunosuppression may have a beneficial effect on the outcome of the disease.
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ranking = 2.0437575834712
keywords = duct
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3/159. Familial idiopathic adulthood ductopenia: a report of five cases in three generations.

    BACKGROUND/AIMS: Idiopathic adulthood ductopenia is a cholestatic liver disease of unknown etiology. Although most cases are sporadic, familial cases do occur. methods: We describe a series of adult-onset bile duct depletion involving five members of an extended family spanning three generations. The proband, a 49-year-old man, presented in 1989 with asymptomatic elevation of liver enzyme tests. Investigations for chronic liver disease, including endoscopic retrograde cholangiopancreatography, were negative. Findings on liver biopsy progressed from normal in 1989 to striking loss of interlobular bile ducts in 1992. ursodeoxycholic acid has resulted in improvement of liver enzyme tests. The proband's brother required a liver transplant at age 35 for cryptogenic cirrhosis. The proband's sister, age 42, has had intermittent jaundice and elevation of liver enzyme tests since 1971. Her liver biopsy findings progressed from normal in 1975, to striking bile duct damage by 1997. The proband's 21-year-old son has elevated liver enzyme tests and a liver biopsy consistent with idiopathic adulthood ductopenia. The proband's father had a liver biopsy at age 70 for investigation of a liver mass. It revealed extensive fibrosis and striking bile duct destruction. RESULTS/CONCLUSIONS: This is the largest series of familial idiopathic adulthood ductopenia reported, and the first with multiple generations described. genetics appears to play a role in some cases of adulthood ductopenia. ursodeoxycholic acid may be beneficial in the treatment of this condition.
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ranking = 24.525091001655
keywords = duct
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4/159. Idiopathic adulthood ductopenia: a diagnosis: two clinicopathologic courses.

    Idiopathic adulthood ductopenia is a chronic cholestatic entity of unknown origin that was discovered in the past decade. Although it is classically a progressive and severe disease, a benign and responsive-to-therapy form has been reported recently. We present two middle-aged females that represent the extremes of the clinicopathologic spectrum: One with a severe form of the disease for whom liver transplantation was considered and the other with a benign form.
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ranking = 10.218787917356
keywords = duct
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5/159. Biliary migration of hepatocellular carcinoma fragment after transcatheter arterial chemoembolization therapy.

    Occasional side-effects of transcatheter arterial chemoembolization therapy in hepatocellular carcinoma are essentially related to tissue necrosis. We report the case of a patient with hepatocellular carcinoma who experienced an acute common bile duct obstruction a few weeks after such a procedure, in the absence of obvious biliary tract invasion. An endoscopic sphincterotomy relieved the obstruction. At histology, the intra-biliary material was identified as a fragment of hepatocellular carcinoma. We discuss the causes of obstructive jaundice in the setting of hepatocellular carcinoma as well as in the specific situation of transcatheter arterial chemoembolization therapy.
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ranking = 2.0437575834712
keywords = duct
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6/159. Idiopathic adulthood ductopenia: case report and review of the literature.

    The clinical and pathological findings of idiopathic ductopenia were studied in a 30-year-old woman who initially manifested jaundice and pruritus. serum biochemical tests of liver function indicated severe and progressive cholestasis. Viral hepatitis markers and circulating autoantibodies were absent. The patient had a normal cholangiogram and lacked evidence of inflammatory bowel disease. Histological examination of a liver specimen showed severe cholestasis and absence of interlobular bile ducts. Severe jaundice and intractable pruritus developed in the patient and served as the indications for liver transplantation 4 months after initial examination. Transplantation resulted in prompt and complete resolution of the jaundice and pruritus. Two types of idiopathic adulthood ductopenia associated with different prognoses are recognized. patients with type 1 idiopathic adulthood ductopenia are asymptomatic or manifest symptoms of cholestatic liver disease. They tend to have less destruction of the intrahepatic bile ducts on liver biopsy specimens. Their clinical course ranges from spontaneous improvement to progression to biliary cirrhosis. In contrast, patients with type 2 idiopathic adulthood ductopenia generally manifest initial symptoms of decompensated biliary cirrhosis, have extensive destruction of the intrahepatic bile ducts on liver biopsy, and frequently require orthotopic liver transplantation.
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ranking = 22.481333418184
keywords = duct
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7/159. Doppler sonography in hepatolithiasis: a case report.

    In the treatment of hepatolithiasis, it is important to not only remove all stones and eliminate bile stasis in the biliary tract, but also to remove atrophic hepatic tissue, as such tissue may cause recurrent cholangitis as well as latent cholangiocarcinoma. A 75-year-old woman was diagnosed as having hepatolithiasis with stenosis at the branching of the bile ducts in segment II and segment III by sonography, computed tomography, endoscopic retrograde cholangiography, and magnetic resonance cholangiography. Portograms did not reveal portal branches in segment III but did demonstrate the portal branches in segment II. During the operation, Doppler sonography was done. Doppler sonography did not reveal the portal blood flow in segment III, but did demonstrate a "to-and-fro" pattern in the portal branches in segment II, indicating atrophic hepatic tissue with disrupted portal blood flow. Lateral segmentectomy (resection of segments II and III and lithotomy with choledo-chotomy were done. The patient is in good health 2 years 6 months after the operation. Doppler sonography is useful in the detection of disrupted portal blood flow and the diagnosis of hepatic atrophy. This is clearly advantageous in the decision-making about whether to perform a liver resection with hepatolithiasis.
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ranking = 2.1866147263284
keywords = duct, stone
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8/159. 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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ranking = 6.2062671089251
keywords = duct, ductal
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9/159. Effective percutaneous transhepatic biliary drainage for cystic dilatation of the intrahepatic biliary system in biliary atresia: a case report.

    We report on a 16-year old girl with postoperative biliary atresia developing cystic dilatation of the intrahepatic biliary system (CDIB) with abdominal pain, which was relieved after percutaneous transhepatic biliary drainage (PTBD). A cyst was detected in segment 4 of the liver at the age of 14 years. She developed severe right hypochondralgia, subfever and liver dysfunction. Percutaneous cholangiogram showed a solitary cyst without communication and a PTBD tube was placed within the cyst. After lavage of the cyst through PTBD, CDIB was re-communicated with the intestine and the pain was relieved with reduction of the cyst size. From this experience, PTBD may be a less invasive treatment as the first choice for non-communicating CDIB and may alleviate the symptoms when recommunication with the intestine is obtained.
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ranking = 2.0437575834712
keywords = duct
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10/159. Benign biliary stricture associated with atherosclerosis.

    We report a case of benign bile duct stricture that could not be differentiated from intrahepatic bile duct carcinoma preoperatively. The patient was a 79-year-old man. Computed tomography showed dilatation of the intrahepatic bile duct in the left lobe. Direct cholangiography showed segmental stricture of the left bile duct. Angiography showed narrowing of the left hepatic artery. Although bile cytology did not show malignant cells, we suspected intrahepatic bile duct carcinoma preoperatively. We performed extended left hepatic lobectomy. Histopathologic examination of the resected duct also showed no malignant cells; fibrosis with infiltration by lymphocytes was seen at the bile duct stricture. In addition, the resected liver specimen showed sclerotic change in the intrahepatic arteries. The postoperative course was uneventful for more than 26 months, without recurrence or cholangitis. We encountered a very rare case of benign segmental bile duct stricture, which was difficult to differentiate from bile duct carcinoma. We think the biliary stricture was secondary to atherosclerosis which may have caused bile duct ischemia.
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ranking = 20.437575834712
keywords = duct
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