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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

There is no doubt that patients with liver cirrhosis, large esophageal varices and extremely elevated intraesophageal variceal pressure have a high risk of variceal bleeding. Telangiectasias on the top of large varices, extremely elevated wedged hepatic venous pressure and a severe reduction of clotting factors are probable risk factors for a variceal hemorrhage, too. Several of these risk factors were analysed in a prospective controlled randomized trial. Endoscopic sclerosis was not only able to reduce the frequency of variceal bleeding but also to prolong life significantly. Thus the value of such risk factors for variceal hemorrhage in patients with liver cirrhosis is well established.
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PMID:[Indications for preventive sclerosing treatment of esophageal varices]. 354 79

A case of hereditary hemorrhagic telangiectasia with prominent nodular transformation of the liver is described. The presence of enlarged arteries was documented morphometrically. Artery-to-portal vein shunts were also found. The association of abnormal vessels with hepatic nodules supports the hypothesis that abnormalities of blood flow cause nodular transformation. Nodular transformation may be the lesion that has heretofore been termed cirrhosis hepatis telangiectasia or atypical cirrhosis.
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PMID:Nodular transformation of the liver in hereditary hemorrhagic telangiectasia. 375 22

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

We present four observations of unilateral nevoid telangiectasia syndrome, pointing out its common cervicothoracic distribution and its relation with hyper-estrogenic conditions, both physiological (puberty, pregnancy, use of contraceptive pills), and pathologic (post-alcoholic hepatic cirrhosis). We also comment on its differential diagnosis with similar diseases. A study of estrogenic receptors in the affected skin was carried out, thus supplying further data for the ethiopathogenesis of the process.
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PMID:[Unilateral nevoid telangiectasia syndrome. Study of cutaneous estrogen receptors]. 639 68

The finding of a rise in VD/VT or failure of VD/VT to fall during exercise has been proposed as a useful noninvasive indicator of pulmonary vaso-occlusive disease in patients with unexplained dyspnea. However, we previously reported a normal fall in VD/VT during exercise in patients with pulmonary hypertension at rest and/or during exercise due to collagen vascular disease. To investigate further the relationship between pulmonary vascular abnormalities and VD/VT responses to exercise, we studied 4 subjects with severe hypoxemia due to cirrhosis or diffuse telangiectasia. We found an abnormal VD/VT response to exercise in these subjects despite normal pulmonary hemodynamics which effectively excluded hemodynamically significant pulmonary vascular obstruction. These findings provide further support for the lack of utility of the VD/VT measurement at rest and during exercise as a screening method for detecting pulmonary vaso-occlusive disease, since the VD/VT cannot only fall appropriately during exercise in patients with pulmonary hypertension but can also remain unchanged or rise during exercise in patients with normal pulmonary hemodynamics.
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PMID:Abnormal responses of wasted ventilation fraction (VD/VT) during exercise in patients with pulmonary vascular abnormalities. 682 5

Unilateral nevoid telangiectasia was observed on the back of the left hand and wrist in a 19-year old man with chronic liver disease and portal hypertension. Moreover, abnormalities of the elastic fibers of the skin were found. Examination of the liver biopsy showed extensive portal fibrosis with many dilated blood vessels. No usual cause of juvenile cirrhosis was detected. In the comment, the authors suggest that unilateral nevoid telangiectasia and liver disease could be manifestations of a disease involving skin and liver vessels, as in hereditary hemorrhagic telangiectasia.
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PMID:Unilateral nevoid telangiectasia and chronic liver disease. Report of a case and review of the literature. 730 42

Symptomatic hepatic involvement is a rare complication of hereditary hemorrhagic telangiectasia and most commonly consists of fibrosis or cirrhosis. We describe a 33-year-old woman in whom multiple hepatic arteriovenous malformations led to high output cardiac failure and liver failure due to biliary necrosis with refractory biliary sepsis, requiring orthotopic liver transplantation. Hepatic arteriovenous malformations were the first manifestation of the disease and a similar asymptomatic hepatic tumour was subsequently detected in her 60-year-old father who also had the classical cutaneous stigmata of the syndrome. Unrecognised genetic factors may determine the clinical spectrum of hereditary haemorrhagic telangiectasia including the hepatic manifestations.
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PMID:Liver transplantation for hepatic arteriovenous malformation in hereditary haemorrhagic telangiectasia. 765 Mar 40

Forty one patients with bleeding vascular ectasias of the upper gastrointestinal tract who required blood transfusion were treated with endoscopic Nd:YAG laser photocoagulation and followed for 34 months (median). Four distinct groups of patients were identified. There was a sustained reduction in transfusion requirements after laser treatment in all those with single (nine patients) and multiple (seven patients) angiodysplasia, in 12 of 16 (75%) patients with watermelon stomachs, and in six of nine (66%) patients with hereditary haemorrhagic telangiectasia. Overall, 25 patients (61%) required minimal or no transfusion after treatment and nine (22%) whose bleeding was controlled initially, later developed recurrent bleeding which was controlled with further laser (total 34 of 41, 83%). Surgery succeeded in a further three patients (7%) in whom laser had failed (in one case possibly because of laser induced haemorrhage). Five more cases of possible laser induced haemorrhage resolved with conservative treatment. One patient sustained a treatment related perforation and died: one patient with cirrhosis died of encephalopathy within one month of starting laser treatment. In two patients transfusion requirements were unchanged despite laser. Nd:YAG laser is a safe and effective treatment for most patients with upper gastrointestinal angiodysplasia.
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PMID:Laser ablation of upper gastrointestinal vascular ectasias: long term results. 849 92


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