Cases reported "Cholestasis"

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1/21. Metastatic prostate cancer (with prostate-specific antigen of 9996) presenting as obstructive jaundice.

    A 78-year-old man admitted with clinical jaundice and pelvic pain had a total bilirubin level of 6.56 mg/dL, an alkaline phosphatase level of 855 U/L, and a prostate specific antigen (PSA) level of 9996 ng/mL. A computed tomogram demonstrated marked retroperitoneal, peripancreatic, periceliac, and periaortic lymphadenopathy. A bone scan revealed increased radiolabeled technetium uptake in the pelvis, vertebral column, parietooccipital region, ribs, and appendiceal skeleton. A biopsy of one pelvic lesion revealed metastatic prostate cancer. This man's obstructive jaundice and bone pain had a dramatic response to treatment with a gonadotropin-releasing hormone analog (leupro lide) and antiandrogen (bicalutamide). All bone pair and clinical signs of jaundice disappeared in 1 week His total bilirubin decreased to 0.84 mg/dL by 2 weeks His PSA values reflected this clinical response, decreasing to 4022 ng/mL in 1 week, 2680 ng/dL after 2 weeks and 1028 ng/mL after 1 month of the above therapy.
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2/21. amputation neuroma mimics common hepatic duct carcinoma.

    Most amputation neuromas of the biliary tract occur in the cystic duct stump after cholecystectomy and are asymptomatic. However, when they arise in the main hepatic duct and are associated with obstructive jaundice, it is difficult to distinguish them from carcinoma. We describe a case in which preoperative differential diagnosis was difficult. A 60-year-old man was admitted to the Institute of clinical medicine, University of Tsukuba, with a chief complaint of jaundice. cholangiography showed an irregularly elevated nodular lesion on the lateral wall of the common hepatic duct and multiple floating stones in the choledochus. ultrasonography and computed tomography revealed one-sided regional thickening of the common hepatic duct associated with dilatation of the intrahepatic and extrahepatic bile ducts. Carbohydrate antigen 19-9 level was markedly elevated to 11,200 IU/mL in the bile juice, but was only 38 IU/mL in the serum, below the limit of abnormality. Cholangioscopy showed papillary tumor with coarse granular surface mimicking papillary carcinoma, but biopsy revealed no malignancy. The patient underwent hepaticocholedochus resection. Although the macroscopic finding from the surgical specimens was papillary carcinoma of the common hepatic duct penetrating to the hepatoduodenal ligament, histopathological examination revealed an amputation neuroma consisting of hypertrophic nerve tissues and giant cells containing foreign bodies, probably as a consequence of a previous cholecystectomy. The postoperative course was uneventful and the patient has been living well for the 5 years since the resection.
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3/21. Drug-induced cholestasis.

    The spectrum of drug-induced cholestasis ranges from 'bland' reversible cholestasis to chronic forms due to the vanishing bile duct syndrome. Agents known for many years to cause cholestasis include estrogens and anabolic steroids, chlorpromazine, erythromycin, and the oxypenicillins; structurally similar congeners of these drugs (tamoxifen, newer macrolides) may also cause cholestasis. Contemporary drugs linked to cholestastic liver injury include ticlopidine, terfenadine, terbinafine, nimesulide, irbesartan, fluoroquinolones, cholesterol-lowering 'statins,' and some herbal remedies (greater celandine, glycyrrhizin, chaparral). Amoxillin-clavulanate, ibuprofen, and pediatric cases of the vanishing bile duct syndrome are recent additions to a long list of drugs associated with the vanishing bile duct syndrome. Particular human leukocyte antigen profiles have recently been identified among those who have developed cholestasis with specific drugs (tiopronin and amoxicillin-clavulanate), and the mechanistic relevance of these genetic associations is being explored. The treatment of drug-induced cholestasis is largely supportive. The offending drug should be withdrawn immediately. Cholestyramine or ursodeoxycholic acid are used to alleviate pruritus, with rifampicin and opioid antagonists being reserved for those who fail first line therapy. nutritional support is essential for those with prolonged cholestasis, a subgroup who are at risk of developing biliary cirrhosis and liver failure. Timely referral for liver transplant assessment is crucial in these patients.
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4/21. Fatal reactivation of hepatitis b virus following cytotoxic chemotherapy for acute myelogenous leukemia: fibrosing cholestatic hepatitis.

    hepatitis b virus (HBV) is a well known pathogen that sometimes causes fulminant hepatitis in patients undergoing cytotoxic chemotherapy. Fibrosing cholestatic hepatitis (FCH) is a recently recognized unique variant of viral hepatitis, which has been occasionally reported in HBV-infected recipients of liver, renal, or bone marrow transplantation. We present here a 48-yr-old male in whom HBV was reactivated during post-remission chemotherapy for acute myelogenous leukemia, which resulted in rapidly fatal outcome. He manifested with deterioration of liver function in association with enormous replication of HBV. Liver biopsy showed marked ballooning of hepatocytes, cholestasis, and periportal fibrosis with minimum infiltrates. Immunostaining revealed that hepatocytes were strongly positive for hepatitis B surface antigen. Under the diagnosis of FCH, he was treated with lamivudine and interferon beta, which was not effective. autopsy showed severe atrophy of the liver and marked degeneration of hepatocytes. Hematologists should be aware that FCH is a fatal complication that can develop under post-chemotherapy immunosuppressed conditions. Although there is no convincing evidence, prophylactic administration of lamivudine seems to be a reasonable strategy.
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5/21. Endothelial cell damage in primary biliary cirrhosis: influence of cholestasis and immunological mechanisms.

    In a study looking for evidence of endothelial cell damage in primary biliary cirrhosis, serum from patients was found to be significantly more cytotoxic to cultured endothelial cells in vitro than normal control serum (P less than 0.001). serum also contained higher levels of von willebrand factor antigen (vWFAg, a specific product of the endothelium) than control serum (P less than 0.001). Cytotoxicity correlated with serum levels of vWFAg (P less than 0.05) and with serum bilirubin (P less than 0.05). No correlations were found between cytotoxicity and circulating immune complexes, c-reactive protein or CH50. The study was controlled by serum from patients with other liver diseases. In these samples vWFAg (P less than 0.003), and bilirubin levels (P less than 0.001) were also raised relative to normal controls. vWFAg levels again correlated with levels of bilirubin (P less than 0.05). Tissue culture experiments showed that purified bilirubin was cytotoxic to human umbilical vein endothelial cells and induced the release of vWFAg. The case report of a patient with obstructive jaundice again indicated that levels of bilirubin and vWFAg were related. These results suggest that endothelial cell damage occurs in both primary biliary cirrhosis and to a lesser extent in other liver diseases, and may be mediated by cholestasis, not by humoral immunological mechanisms.
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6/21. Cholangiographic appearances of ductular rejection of ABO-incompatible liver transplants.

    Two patients, after ABO-incompatible liver transplant, developed a picture of chronic cholestasis that was associated with unique ductular appearances within the donor graft on retrograde cholangiography. We believe that the appearance of progressive ductular ectasia within liver grafts indicates a specific immunologically mediated injury to ductular epithelium, probably because of ductular epithelial expression of ABH antigens.
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7/21. Elevated CA19-9 in a case of Mirizzi's syndrome.

    At levels greater than 1000 U/ml, the tumor-associated antigen CA19-9 has a specificity approaching 100% for malignant disease. A 67-yr-old woman with known cholelithiasis presented with painless jaundice and a CA19-9 of 1320 U/ml. The diagnosis of Mirizzi's syndrome was made at laparotomy, and the postoperative level fell rapidly to normal. Mirizzi's syndrome should be included among the few benign disorders which can yield such elevations in the CA19-9.
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8/21. Cardiolipin-fluorescent (M1) antimitochondrial antibody and cholestatic hepatitis in secondary syphilis.

    A 27-year-old black male with secondary syphilis and cholestatic jaundice is presented. The liver biopsy was believed to be most consistent with large bile duct obstruction, but both the ultrasound and endoscopic retrograde cholangiography were normal. Prior to treatment with penicillin, his serum was positive for antimitochondrial antibody. After treatment, the antibody was no longer detectable and the jaundice gradually resolved. The patient's pretreatment serum was, after further analysis, found to be positive for the antibody to the M1 antimitochondrial antigen subtype, which is identical to cardiolipin, the antigen in both the VDRL and Wasserman tests. A review of hepatic involvement in secondary syphilis is presented.
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9/21. Benign hemangioendothelioma of the pancreas with obstructive jaundice.

    A 3-year-old boy with benign hemangioendothelioma of the pancreas is reported. He showed the presenting sign of obstructive jaundice. The boy was treated with temporary choledochojejunal bypass without additional treatment. In a 16-month follow-up the child has been well and asymptomatic. Laboratory data showed no abnormality. Biopsied specimen of pancreatic head showed hemangiomatous pattern histologically. Immunohistochemical studies of the biopsy revealed positive reaction to factor viii-related antigen (FVIII-RAG), and endothelial cell marker, supporting an endothelial origin. hemangioendothelioma has a favorable prognosis and spontaneous regression can be expected. So the first treatment of the tumor should be conservative.
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10/21. Studies on drug-induced allergic intrahepatic cholestasis--hepatic cholestasis induced in dogs by lymphocyte culture supernatant.

    When peripheral lymphocytes from patients with drug-induced allergic intrahepatic cholestasis were stimulated with a specific drug in vitro in the presence of a liver microsome fraction or soluble liver specific antigen fraction, lympholine production was seen in many cases. By the injection of culture supernatant of stimulated lymphocytes into the mesentery vein of dogs, cholestasis was induced in the liver, chiefly in the central zones of lobules. However, no cholestasis could be observed in dogs administered the supernatants of lymphocyte cultures prepared from normal individuals in the presence of drugs. Moreover, only slight swelling of the hepatocytes was observed in the liver when normal lymphocytes were stimulated with PHA-P and culture supernatant was injected into the mesentery vein of dogs. These results suggest that sensitized lymphocytes may produce a factor (or factors) by stimulation with a specific drug-carrier and this factor (or factors) causes cholestasis in the liver.
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