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

Telangiectasia-associated hepatic fibrosis (TAHF) in a 68-year-old woman with hereditary haemorrhagic telangiectasia (HHT) is described. The patient died of oat-cell carcinoma of the lung. In addition to the structural alterations which have been described previously in HHT, the liver exhibited focal midlobular hepatocytic necrosis and tumour metastases. The possibility that treatment of HHT was causally related to some of the hepatic abnormalities found in our patient and the differentiation of TAHF from true cirrhosis are discussed.
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PMID:'Pseudocirrhosis' in hereditary haemorrhagic telangiectasia. 20 9

A study of 66 primary hepatic tumours was carried out on cattle. These consisted almost entirely of adult females. Fifty hepatocellular tumours (10 adenomas and 40 carcinomas), 10 cholangiocellular tumours (three adenomas and seven carcinomas), two cavernous haemangiomas, two haemangioendothelial sarcomas, one fibroma and one Schwannoma were diagnosed. The 50 hepatocellular tumours were classified into adenomas (20 per cent) and carcinomas (80 per cent), both prevalent in the solitary macronodular form. The hepatocellular carcinomas were divided into six groups based on their distinctive histological arrangement and the morphology of the neoplastic cells: trabecular (55 per cent), pseudoglandular (10 per cent), solid (12 per cent), scirrhous (12 per cent), pleomorphic (7 per cent) and fibrolamellar (2 per cent). Twenty-seven of 50 hepatocellular tumours (54 per cent) were associated with features of blood-filled lakes resembling telangiectasis within the neoplastic tissue. Metastasis was uncommon (10 per cent of hepatocellular carcinomas) and grading according to Edmondson's system (1958) showed a low level of anaplasia in most carcinomas (55 per cent). An association with cirrhosis was never found.
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PMID:Primary hepatic tumours in cattle. A classification of 66 cases. 133 Dec 8

We report a Mexican family with 15 members affected with hereditary hemorrhagic telangiectasia (HHT), all of them suffering from severe hemorrhagic episodes treated with multiple blood transfusions; one patient had a cerebral arteriovenous fistula that required surgical treatment and two others had severe cirrhosis of the liver. Clinical findings in this family illustrate the variable expressivity in HHT, which should be taken into account for the purpose of genetic counselling and monitoring for early detection of systemic complications.
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PMID:Hereditary hemorrhagic telangiectasia: report of 15 affected cases in a Mexican family. 203 43

24 patients with alcoholic intake were classified according to the amount of alcohol ingestion; clinical symptoms and signs, liver function tests (bilirubin, aminotransferases and prothrombin time) were analyzed. In all patients a percutaneous liver biopsy was performed and tissue stained by hematoxylin-eosin, wilder reticulin and Mallory trichromic. 9 Histologic criteria were analyzed. 4 groups according to the histology were identified. Group 1 (5 patients) hepatic fibrosis and/or fatty liver. Group 2 (5 patients) alcoholic hepatitis. Group 3 (10 patients) cirrhosis. Group 4 (4 patients) normal. 20% of patients with fatty liver, 80% of alcoholic hepatitis and 100% of cirrhotics referred ingestion or more than 160 g of ethanol and important correlation between liver histological damage and alcohol ingestion. Telangiectasia was the most common clinical finding and present in all hepatitis, 70% of cirrhotics and only 20% of fatty livers. Hemosiderosis was found in 60% of cirrhotics and in alcoholic hepatitis. Only 40% of patients with fatty liver and inflammatory cells while this was evident in all patients with alcoholic hepatitis and those with cirrhosis. Mallory bodies were identified in only 20% of cirrhotics and in none of the alcoholic hepatitis. The results suggest that there are significant differences from a histological and clinical point of view that distinguish alcoholic liver disease as seen in Venezuela from that reported in other countries.
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PMID:[Alcoholic liver disease in Venezuela. Clinical hepato-functional and histopathologic course]. 215 50

Nodular regenerative hyperplasia is defined by hepatocellular nodules distributed throughout the liver in the absence of fibrous septa between the nodules. Most reports have been single cases so that the prevalence and clinical significance of nodular regenerative hyperplasia is uncertain. In this study, the hepatic histology of 2,500 consecutive autopsies was reviewed. A spectrum of nodular transformation was found with nodular regenerative hyperplasia present in 2.6% of autopsy livers and qualitatively similar but lesser degrees of nodular transformation in a further 10.2%. Nodular transformation was also seen in 47% of livers with cirrhosis and 69% with incomplete cirrhosis. Obliteration of many small portal veins was seen in all cases with nodular regenerative hyperplasia, but only 4.7% of these had evidence of portal hypertension. The prevalence of various clinical states was compared in nodular regenerative hyperplasia and in controls. The results confirm, extend and quantify the spectrum of associated diseases. Nodular regenerative hyperplasia occurs in 5.6% of individuals over age 80 and with increased frequency in patients with systemic arteritis, polymyalgia rheumatica, massive tumor infiltration and mineral oil deposition. Nodular regenerative hyperplasia appears to be the hepatic analogue of arterial and arteriolar nephrosclerosis. A new classification of nodular transformation is proposed that encompasses the spectrum of lesions described here and the previously defined entities of focal nodular hyperplasia, partial nodular transformation and "cirrhosis telangiectasia hepatis." The major conclusion is that nodular regenerative hyperplasia is a secondary and nonspecific tissue adaptation to heterogeneous distribution of blood flow and does not represent a specific entity.
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PMID:Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2,500 autopsies and a new classification of benign hepatocellular nodules. 218 21

Pulmonary perfusion imaging with Tc-99m MAA revealed significant uptake in the lungs, brain, spleen, and both kidneys of a 48-year-old woman with liver cirrhosis and pulmonary telangiectasia associated with marked hypoxemia and cyanosis. Dynamic pulmonary perfusion imaging revealed a gradual reduction after peak uptake in both lungs. Several weeks after albumin replacement, the hypoxia and dyspnea disappeared with no change in hepatocellular function. At that time, dynamic pulmonary perfusion imaging revealed a plateau-like time-activity curve of uptake in the lungs, as compared with the findings obtained during the state of severe hypoxemia. These observations suggest that pulmonary telangiectasia in a patient with liver cirrhosis may be due to functional vasodilatation. Serial dynamic pulmonary perfusion imaging indicates the passage of the MAA particles through the widened lumen of the pulmonary alveolar capillaries.
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PMID:Reversibility of pulmonary telangiectasia in liver cirrhosis evidenced by serial dynamic pulmonary perfusion imaging. 260 48

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

Hepatic in involvement in hereditary haemorrhagic telangiectasia can lead to cirrhosis and occasionally to portal hypertension and variceal haemorrhage. The ultrasonographic, arteriographic and histological findings are described in a patient with this complication. Hepatic artery embolisation proved unsuccessful in arresting repeated haemorrhage which was eventually controlled by hepatic artery ligation. Porto-systemic venous shunting, an apparently logical approach to management, would probably have aggravated the problem.
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PMID:Variceal haemorrhage in hereditary haemorrhagic telangiectasia. 236 Sep 29

The cases of two brothers with intrahepatic arterio-venous shunt causing hyperdynamic circulation in hereditary hemorrhagic telangiectasia are presented. There was a strong family history of fatal liver involvement in all members of the previous generation. The first patient had embolization of the right hepatic artery with symptomatic relief, but developed cirrhosis with portal hypertension seven years later. The second patient developed cirrhosis with portal hypertension, and died following rupture of esophageal varices, within a year. It is suggested not only that hereditary hemorrhagic telangiectasia is transmitted as a dominant trait, but that there is also a hereditary transmission of hepatic involvement in this disease.
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PMID:Familial occurrence of hyperdynamic circulation status due to intrahepatic fistulae in hereditary hemorrhagic telangiectasia. 318 61


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