Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease), treated with ethinyl estradiol, multiple blood transfusions, and iron-dextran, developed hepatocellular carcinoma and acquired hepatocerebral degeneration. In addition to the carcinoma, the liver contained extensive arteriovenous maliformations, telangiectasis, and changes of Osler atypical cirrhosis. The carcinoma possibly had its genesis in the presence of an ocongenic serum hepatitis virus, or the cirrhosis, or both.
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PMID:Hereditary hemorrhagic telangiectasia. A case with hepatocellular carcinoma and acquired hepatocerebral degeneration. 16 31

Hereditary haemorrhagic teleangiectasia (Rendu-Osler-Weber disease) is an inborn error of vascular structure with multiple manifestations. Its incidence is about 1-2:100 000 in the European population. The incidence of telangiectases and/or fistula formation was estimated to be 1 in 10 carriers of the Osler trait. The findings in the family reported herewith suggest a much higher incidence if angiography is more frequently performed. Apart from the skin and mucous membrane, teleangiectases and/or arteriovenous fistulas may be present in the lungs, intestinal tract, spleen, kidney, brain, and bones. The liver apparently is more involved than was orginally suspected. The vascular derangement includes teleangiectases, arteriovenous fistulas, and connective tissue formation with fibrosis and atypical cirrhosis. In intestinal bleeding laser coagulation seems to be very efficient. The pathogenesis of teleangiectases is not known but involves several factors such as special formation of venules, capillaries and arterioles, abnormal perivascular connective tissue and endothelial cells.
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PMID:The liver in hereditary haemorrhagic teleangiectasia: an inborn error of vascular structure with multiple manifestations: a reappraisal. 30 29

Density-defined macrophages isolated from fluids of patients with liver cirrhosis mainly generated the 5-lipoxygenase products leukotriene B4 (LTB4, 16%) and 5-hydroxy-eicosatetraenoic acid (5-HETE, 24%) and the cyclooxygenase products 12-hydroxyheptadecatrienoic acid (HHT, 22%) and thromboxane B2 (TXB2, 18%). The synthesis of eicosanoids was linear with the maturity of the macrophage subpopulations, suggesting that eicosanoid production is increased in in-vivo activated macrophages.
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PMID:Eicosanoid production by density-defined human peritoneal macrophages during inflammation. 133 54

A 63-year-old female was admitted with dyspnea on exertion. She had previously suffered from a large amount of epistaxis. Chest X-ray showed the bilateral diffuse granular infiltrates. Arterial blood analysis revealed severe hypoxemia and pulmonary function test showed reduced function of diffusing capacity. Dynamic radionuclide perfusion scintigraphy performed with 99mTc-labeled MAA demonstrated passage of microspheres through the pulmonary vasculature into the systemic circulation, indicating a right-to-left shunt. Although selective pulmonary wedge angiography did not directly demonstrate pulmonary arteriovenous fistulae, the histopathological findings of TBLB specimens revealed multiple dilated alveolar vessels that microspheres could pass through. In addition, chronic hepatitis, dilatation of splenic vein and spleno-renal anastomosis had been present. This type of pulmonary arteriovenous fistula is said to be a rare dispersed telangiectasic type, associated with a part of Rendu-Osler-Weber syndrome and a cause of hypoxemia in liver cirrhosis. We consider that in this case, the chronic hepatitis may have played an important role of developing multiple small pulmonary arteriovenous fistulas.
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PMID:[A case of multiple small pulmonary arteriovenous fistulas with chronic hepatitis and abdominal vessel abnormalities]. 262 13

The hepatic changes in hereditary hemorrhagic telangiectasia (Rendu Osler's disease) involve the extra- and intrahepatic vessels (enlarged hepatic artery, arteriovenous fistulas and inhomogeneous perfusion) and the hepatic parenchyma (fibrosis, telangiectasic cirrhosis). From three observations with liver involvement and imaged with angiography and dynamic CT, the authors emphasize on the role of the latter for detection of these changes, mostly arteriovenous fistulas.
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PMID:[Angiography of hepatic angiomatosis in Rendu-Osler disease. Apropos of 3 cases]. 269 99

In a woman with hereditary hemorrhagic telangiectasia (HHT) (Osler-Weber-Rendu disease) who died of fulminant hepatitis B, autopsy revealed cirrhosis of the liver and diffuse hepatic telangiectasia. Her daughter and grandson also suffered from the hepatic involvement of HHT. Sufficient laboratory investigations were available to exclude known causes of cirrhosis. We review the relationship between Osler-Weber-Rendu disease and liver cirrhosis or fibrosis.
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PMID:Hepatic telangiectasia and cirrhosis. 335 77

A case of a visceral pattern (with primary liver injury) of the Rendu-Osler-Weber disease is described with emphasis on the diagnostic difficulties due to the absence of external symptoms of disease and hemorrhage. Stress is laid on the importance of making liver biopsy in the diagnosis of this pattern of teleangioectatic disease. It is assumed that microcirculatory disorders in the liver provoked by the development of teleangioectasia are essential factors in the formation of liver cirrhosis in visceral pattern of the Rendu-Osler-Weber disease.
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PMID:[A visceral form of Rendu-Osler-Weber syndrome]. 404 61

The laparoscopic findings observed in eight patients with hereditary haemorrhagic telangiectasia are reported. Clinical signs or laboratory data suggestive of liver involvement were present in all cases and constituted the main indication for laparoscopic examination. Characteristic vascular lesions distributed at random were found in four cases. Numerous oval, round or polygonal areas of 0.5 to 1.5 cm in diameter were observed on the external surface of the liver. They were of a reddish or pink colour, flat or slightly raised, and made up of a dense vascular meshwork. In one patient macronodular cirrhosis was associated with the vascular lesions. In the remaining four cases laparoscopic examination did not show striking vascular changes, although the liver biopsy revealed a fatty liver in three cases and micronodular cirrhosis with intense inflammatory activity, steatosis and Mallory bodies in one case. In these cases the accompanying hepatic lesions could probably not be aetiopathogenically related to Osler's disease.
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PMID:Laparoscopic findings in hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu disease). 620 71

Clinical and radiological findings of hepatogenic pulmonary angiodysplasia are reported in two cases. Myriad spidery pulmonary blood vessels are seen on plain radiographs and verified with right to left intrapulmonary shunting on pulmonary angiogram and pulmonary isotopic perfusion scan. Pathophysiology and differential diagnosis are discussed. We propose that the term "pulmonary angio-dysplasia" should include: 1) Pulmonary telangiectasia 2) Hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber syndrome) 3) Classical discrete pulmonary arteriovenous fistula 4) Hepatogenic pulmonary angiodysplasia: pulmonary arteriovenous communications with right to left shunting secondary to hepatic cirrhosis.
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PMID:Plain radiographic, nuclear medicine and angiographic observations of hepatogenic pulmonary angiodysplasia. 622 68

Hereditary hemorrhagic telangiectasia or Rendu-Osler-Weber syndrome (ROWS) is an angiodisplasia characterized clinically by the presence of telangiectasias in the skin and mucosas, particularly on the face, conjunctivas, nose, pharynx and rarely on the thorax. Visceral involvement specially hepatic cirrhosis associated with pulmonary arteriovenous fistulae have been described. We describe a 7 10/12 years old girl with the diagnosis of ROWS associated with chronic active hepatitis with positive HBAg that evolved to hepatic cirrhosis. This is the second case of ROWS associated with chronic active hepatitis reported in the literature, being however, the first one described in the childhood.
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PMID:Hereditary hemorrhagic telangiectasia associated to chronic active hepatitis. 721 36


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