Cases reported "Liver Cirrhosis"

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1/1533. Giant hyperplasia of the caudate lobe of the cirrhotic liver: correlation with an anomaly of the caudate portal branch.

    Different imaging appearances of giant hyperplastic change of the caudate lobe of the liver are presented in a patient with liver cirrhosis. The mass like caudate lobe was isoechoic on ultrasound, hypodense on postcontrast computed tomography (CT), hyperintense on T1-weighted magnetic resonance, images and isointense on T2-weighted images. These imaging findings are similar to those of dysplastic nodule in cirrhotic liver. The caudate lobe received normal portal flow on CT during arterial portography, but superior mesenteric arteriography showed precocious or early division of the caudate portal branch. We suspect that caudate hyperplastic change may be correlated to anomalous caudate portal vein branch. ( info)

2/1533. Ferumoxide-enhanced MRI of sideronecrosis superimposed on genetic hemochromatosis.

    Genetic hemochromatosis is an autosomal recessive disorder characterized by excessive iron absorption from the gut, resulting in increased total body iron stores, multisystem organ dysfunction, and an increased risk of hepatocellular carcinoma. The magnetic susceptibility effects of excess hepatocellular iron generally cause diffuse hepatic signal loss on T2- or T2*-weighted MR images. Although hepatic iron deposition is usually diffuse, focal areas of iron sparing can occur, and, when present, superimposed neoplasm is a consideration. We describe a patient with cirrhosis, hemochromatosis, and multiple small benign relatively hyperintense iron-poor foci consisting of piecemeal sideronecrosis. ( info)

3/1533. liver transplantation for endstage hepatitis c cirrhosis in a patient with primary hypogammaglobulinaemia.

    liver transplantation was performed in a patient with primary hypogammaglobulinaemia, chronic hepatitis c and hepatic failure. The immediate posttransplant period was uncomplicated. Owing to a stricture of the choledochojejunostomy the patient was reoperated with construction of a hepaticojejunostomy 11 months posttransplant. The patient remained hepatitis c virus (HCV) rna-positive, with high and increasing levels of HCV. Liver biopsies demonstrated the recurrence of HCV. 14 months after the transplantation the patient developed severe diarrhoea caused by cryptosporidium parvum. The infection did not respond to available therapeutic measures. He deteriorated with development of liver failure and died 18 months after the transplantation. The present case report illustrates the difficulties associated with organ transplantation in patients with primary hypogammaglobulinaemia. ( info)

4/1533. Cirrhotic ascites, ovarian carcinoma, and CA-125.

    We describe two postmenopausal women with ascites and elevated CA-125 level, a serologic marker used to detect ovarian cancer. Both patients had unrecognized liver disease but underwent surgical exploration for suspected ovarian disease, which subsequently revealed benign pelvic organs. Elevated serum CA-125 levels have been reported in many patients with ascites due to liver disease and cirrhosis. Thus, the presence of both ascites and an elevated CA-125 level mandates a thorough elevation for liver disease as well as for a possibility of ovarian carcinoma. These cases outline the common finding and provide insight into the management of patients with ascites and elevated CA-125 values. ( info)

5/1533. Markedly elevated CA125 in hepatic cirrhosis: two case illustrations and review of the literature.

    CA125 is the most widely used tumor marker presently available for use in patients with epithelial ovarian cancer. Although elevated in a high percentage of patients with ovarian cancer, serum CA125 levels have also been detected in patients with numerous benign and malignant nongynecologic disorders, including various diseases of the liver. Despite this well-publicized fact, it has become apparent that the association between CA125 elevation, particularly the degree of elevation, and liver disease may not be as widely recognized as one would suspect. When marked CA125 elevations occur, diagnostic confusion is common. We describe two cases illustrative of this point. Both cases involve middle-aged women who presented with massive ascites and due to markedly elevated serum CA125 levels underwent exploratory laparotomy with hysterectomy and/or bilateral salpingo-oopherectomy before their referral to our center. Because preservation of a woman's reproductive organs is a significant concern, it is imperative that both primary care physicians and specialists are aware of such associations and the proper use of tumor markers. ( info)

6/1533. Fibropolycystic disease of the hepatobiliary system and kidneys.

    This complicated case of fibropolycystic disease of the hepatobiliary system and kidneys was ably and incisively analyzed by Professor Sheila Sherlock. Her clinical acumen was revealed by her ability to differentiate congenital hepatic fibrosis, Caroli's disease, and adult polycystic disease of the liver and kidney. Interesting histologic features of this case included hepatic fibrosis with intact limiting plates anc central veins and the presence of bile plugs in the ducts, but the absence of bile statsis in the parenchyma. A percutaneous transhepatic cholangiogram demonstrated the dilated intrahepatic and extrahepatic ducts. Washing out the "gunk" from the biliary tract by T-tube drainage has great limitations in this type of case. Therefore, Dr. Adson suggested irrigation of the biliary ductal system using tubed placed transhepatically, plus a wide choledojejunostomy. Dr. Sherlock questioned this surgical approach. The use of chenodeoxycholic acid for this "gunk" was suggested. In spite of the dilated ducts and pathologic changes in the liver, the patient was not jandiced and did not have stones in her biliary tract. The genetics of this patient's problems was discussed. ( info)

7/1533. Heteropolymerization of S, I, and Z alpha1-antitrypsin and liver cirrhosis.

    The association between Z alpha1-antitrypsin deficiency and juvenile cirrhosis is well-recognized, and there is now convincing evidence that the hepatic inclusions are the result of entangled polymers of mutant Z alpha1-antitrypsin. Four percent of the northern European Caucasian population are heterozygotes for the Z variant, but even more common is S alpha1-antitrypsin, which is found in up to 28% of southern Europeans. The S variant is known to have an increased susceptibility to polymerization, although this is marginal compared with the more conformationally unstable Z variant. There has been speculation that the two may interact to produce cirrhosis, but this has never been demonstrated experimentally. This hypothesis was raised again by the observation reported here of a mixed heterozygote for Z alpha1-antitrypsin and another conformationally unstable variant (I alpha1-antitrypsin; 39Arg-->Cys) identified in a 34-year-old man with cirrhosis related to alpha1-antitrypsin deficiency. The conformational stability of the I variant has been characterized, and we have used fluorescence resonance energy transfer to demonstrate the formation of heteropolymers between S and Z alpha1-antitrypsin. Taken together, these results indicate that not only may mixed variants form heteropolymers, but that this can causally lead to the development of cirrhosis. ( info)

8/1533. Refractory hepatic hydrothorax treated with transjugular intrahepatic portosystemic shunt.

    A 66-year-old cirrhotic woman was referred to our hospital for evaluation of refractory pleural effusion and dyspnea. Massive right sided-pleural effusion but no ascites was detected. She had been treated with diuretics and albumin, repeated thoracenteses, and pleural drainage with an intercostal catheter, all of which had failed to relieve her symptoms. The diagnosis of hepatic hydrothorax without ascites was made by injection of technetium-99m-sulfur colloid into the peritoneal cavity. A transjugular intrahepatic portosystemic shunt was placed and successfully reduced the pleural effusion, resulting in complete relief of her symptoms. The patient has been free of symptoms for 18 months after the procedure. ( info)

9/1533. Immunohistochemical study on transforming growth factor-beta1 expression in liver fibrosis of Down's syndrome with transient abnormal myelopoiesis.

    A case of Down's syndrome associated with liver fibrosis is reported. The fibrosis was diffusely distributed along sinusoids, and an excess of megakaryocytes was also found in the liver. To determine the mechanism of liver fibrosis in Down's syndrome, we immunohistochemically stained the liver with markers of myofibroblast-like cells, antialpha smooth muscle actin antibodies and antidesmin antibodies. The myofibroblast-like cells were found along sinusoids, suggesting that liver fibrosis in Down's syndrome is caused by the myofibroblast-like cells derived from Ito cells/lipocytes. The expression of transforming growth factor (TGF)-betal, which is an important mediator of the activation of lipocytes, was immunohistochemically examined. The accumulation of TGF-betal was observed in cells in the sinusoidal spaces, which involve the intracellular expression of megakaryocytes. Together, these findings suggest that megakaryocyte-derived TGF-betal is one of the likely candidates in the lipocyte activation of liver fibrogenesis in Down's syndrome. ( info)

10/1533. Ileal varices associated with recurrent bleeding in a patient with liver cirrhosis.

    We report a rare case of massive and recurrent bleeding from ileal varices in a patient with hepatitis c virus-positive liver cirrhosis. A 66-year old woman, who had undergone laparotomy and blood transfusion 36 years before (because of an extrauterine pregnancy) and endoscopic sclerotherapy for esophageal varices 1 year previously, was admitted to our hospital with loss of bright red blood per rectum. The bleeding was massive and recurrent, and frequent blood transfusions were required. Endoscopic studies failed to find the bleeding site. In the venous phase of selective superior mesenteric angiography, mesenteric varices in the lower part of the abdominal cavity were observed. laparotomy was performed to control the repeated bleeding which had lasted for more than 1 month. Varices communicating with the right ovarian vein were found on the ileal wall and segmental resection of the ileum was performed. Histological examination demonstrated a massive varicose vein and several dilated veins in the submucosa. The patient's postoperative course was favorable, with no hemorrhagic events during a follow-up of more than 6 months after surgery. Ileal varices should be considered in the diagnosis of a patient who presents with lower gastrointestinal bleeding and portal hypertension. ( info)
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