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)

Hepatic involvement in hereditary hemorrhagic telangiectasia (HHT) consists of vascular malformations associated with arteriovenous (AV), arterioportal, and/or portovenous shunting. Most patients with HHT have liver involvement. Symptoms, although rare, consist of cardiac failure, pulmonary hypertension, portal hypertension, portosystemic encephalopathy, cholangitis, and atypical cirrhosis. Reported treatments for symptomatic AV malformations have been associated with substantial morbidity and mortality. This report describes a case of hepatic HHT that required liver transplantation after hepatic artery embolization. Recurrent vascular malformations developed in the transplant, resulting in portal hypertension and life-threatening variceal hemorrhage that was controlled with transjugular intrahepatic portosystemic shunt creation.
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PMID:Transjugular intrahepatic portosystemic shunt for variceal hemorrhage due to recurrent of hereditary hemorrhagic telangiectasia in a liver transplant. 1992 94

The association of liver abnormalities with carcass grading performance, value, and dressed yield was evaluated on data from 76,191 carcasses housed in 2 databases. Liver abnormalities were assigned as follows: normal = edible liver; A- = 1 to 2 small abscesses or inactive scars; A = 1 to 2 large abscesses or multiple small abscesses; A+ = multiple large abscesses; A+AD = liver adhered to gastrointestinal tract or diaphragm or both; A+OP = open liver abscess; cirrhosis; distoma; and telangiectasis. Overall liver abnormality rates among both databases were A- = 5.0%, A = 2.6%, A+ = 2.0%, A+AD = 2.7%, A+OP = 1.4%, cirrhosis = 0.1%, distoma = 5.4%, and telangiectasis = 0.3%, with 80.5% of livers being normal. In database 1 (n = 3,936), BW was 33.3 kg less (P < 0.01) for carcasses with cirrhotic livers compared with carcasses with normal livers. Dressed yields for carcasses with A-, A+, A+AD, A+OP, and cirrhosis liver abnormalities were 0.28 to 0.89 percentage points less (P < 0.05) than carcasses with normal livers. In database 2 (n = 72,255), carcasses with A-, A+, A+AD, A+OP, cirrhosis, distoma, and telangiectasis liver abnormalities had reduced (P < 0.05) HCW and reduced (P < 0.05) LM area as compared with carcasses with a normal liver. Less (P < 0.05) 12th-rib subcutaneous fat was observed for carcasses with A-, A, A+, A+AD, A+OP, cirrhosis, and distoma abnormalities compared with carcasses with normal livers. Estimated KPH was less (P < 0.05) for carcasses with A-, A+, A+AD, A+OP, cirrhosis, and distoma abnormalities. Calculated yield grade was less (P < 0.05) for carcasses with A+AD, A+OP, cirrhosis, and distoma abnormalities compared with carcasses with normal livers. Marbling score of carcasses that had livers with A+AD or A+OP abscess scores or distoma was less (P < 0.05) than that of carcasses with normal livers. In database 2, market price per 45.35 kg did not differ (P = 0.32) among liver classification. Gross carcass value analyses indicated carcasses with A-, A, A+, A+AD, A+OP, cirrhosis, and distoma liver abnormalities were less valuable (P < 0.05) than carcasses with normal livers. These data delineate the association of liver abnormalities present at harvest with subsequent changes in carcass characteristics and ultimately lost carcass value.
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PMID:Association of liver abnormalities with carcass grading performance and value. 2081 55

Acute hepatic vascular complications are rare. Acute portal vein thrombosis (PVT) and the Budd-Chiari syndrome (BSC) are the leading causes. Coagulopathy and local factors are present in up to 80% of cases. Diagnosis is established by colour-coded Doppler sonography, contrast-enhanced computed tomography or magnetic resonance imaging. Patients with acute PVT present with abdominal pain and disturbed intestinal motility. In the absence of cirrhosis anticoagulation with heparin is established followed by oral anticoagulation. In severe cases, surgical thrombectomy or transjugular thrombolysis with stent shunt may be necessary. Acute or fulminant BCS may require emergency liver transplantation or a transjugular intrahepatic portosystemic stent shunt, if patients present with acute liver failure. Milder cases receive anticoagulation for thrombolysis of occluded hepatic veins. Sinusoidal obstruction syndrome (SOS) is diagnosed after total body irradiation or chemotherapy, the term SOS replacing the former veno-occlusive disease. The treatment of congenital vascular malformations, complications in the setting of OLTX as well as patients with hepatic involvement of hereditary hemorrhagic telangiectasia requires significant expertise in a multidisciplinary approach.
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PMID:[Acute hepatic vascular complications]. 2166

Gastric antral vascular ectasia (GAVE) continues to be a challenge in both diagnosis and treatment. GAVE has a diverse group of associations and presumed causes, including cirrhosis, chronic renal failure and autoimmune connective tissue diseases. However, in most occasions, the management plan of GAVE itself is the same whatever the underlying disease by using Argon plasma coagulation (APC). Herein, we will discuss three cases of systemic sclerosis-associated GAVE presenting with either acute or chronic gastrointestinal bleeding showing variable responses to APC. Anemia and telangiectasia may be the first striking presentation of systemic sclerosis (SSc). Renal artery stenosis, aortic stenosis, widespread cutaneous and mucosal telangiectasia and hypertension seem to be associated with poor prognosis and should prompt rapid intervention and careful follow-up. Moreover, the hunt for molecular underpinnings of the broad array of vascular lesions in SSc has to include von Willebrand factor and endoglin. Eventually, we will review the recent alternatives that can be effective in SSc-GAVE, such as band ligation, hematopoietic stem cells transplantation and immunotherapy.
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PMID:Gastric antral vascular ectasia in systemic sclerosis: Where do we stand? 2821 87

Hepatopulmonary syndrome (HPS) is a severe complication of advanced liver disease associated with an extremely poor prognosis. HPS is diagnosed in 4-47% of patients with cirrhosis and in 15-20% of candidates for liver transplantation. In addition, severe hypoxia is associated with a high risk of complications of liver transplantation (a 30% chance during the first 90 days) and increases the gap between transplantation and improving arterial oxygenation. The pathogenesis of HPS is not fully understood, and no effective pharmacological treatment has been developed yet. Currently, the treatment of choice for HPS is orthotopic liver transplantation. Non-specific clinical criteria and the lack of standardized diagnostic criteria for determining HPS can lead to diagnostic errors. Portopulmonary hypertension and hereditary hemorrhagic telangiectasia, also known as Osler-Weber-Rendu syndrome, are pulmonary complications of liver disease which should be differentially diagnosed from HPS.
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PMID:Differential diagnosis of hepatopulmonary syndrome (HPS): Portopulmonary hypertension (PPH) and hereditary hemorrhagic telangiectasia (HHT). 2875 37

Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of cutaneous mastocytosis. While most cutaneous mastocytoses occur in children and are asymptomatic, TMEP occurs predominantly in adults and is associated with systemic manifestations, requiring medical management. TMEP is typically characterised by scattered red-brown macules on the trunk and extremities, but must be differentiated from other telangiectatic conditions such as scleroderma, hereditary haemorrhagic telangiectasia and telangiectasias secondary to cirrhosis. Practitioners must be aware that variants to the classic presentation of TMEP exist, such as the ringed telangiectasias we describe. Diagnostic workup including tissue biopsy must be considered in such patients after a thorough history and physical have been performed and other telangiectatic processes have been ruled out. The treatment of cutaneous mastocytosis aims at controlling symptoms and preventing mast cell degranulation. Cosmetic treatment includes the use ofPsoralen and ultraviolet A (PUVA) therapy, total skin electron beam radiation and flashlamp pulsed-dye laser treatment.
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PMID:Ringed telangiectasias: an unusual presentation of telangiectasia macularis eruptiva perstans. 2919 83

Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu syndrome is a rare autosomal dominant vascular disorder characterized by epistaxis, mucocutaneous telangiectasias, and arteriovenous malformations affecting various organs and systems. The liver is a commonly involved organ (74% of patients with hereditary hemorrhagic telangiectasia), although symptomatic liver disease is quite infrequent. In symptomatic cases, clinical manifestations relate most commonly to the predominant type of vascular shunting present (arteriovenous, arterioportal, or portovenous). Clinically, liver disease can manifest as a high-output cardiac failure, portal hypertension, or biliary disease. Imaging plays an important role in diagnosis, characterization, and follow-up of liver involvement, with ultrasound, computed tomography, magnetic resonance imaging, and angiography being useful in this context. We present a case of congestive cirrhosis with florid liver findings in a patient with Osler-Weber-Rendu syndrome. Imaging findings that clinched the diagnosis are reviewed. A brief literature review is also provided.
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PMID:Congestive cirrhosis in Osler-Weber-Rendu syndrome: A rare case report. 2955 42

A 76-year-old woman with hereditary hemorrhagic telangiectasia (HHT) showed elevated serum hepatobiliary enzyme levels, and abdominal imaging studies revealed a hepatic tumor. Her serum alpha-fetoprotein level was 759.5 ng/mL. A pathological examination after hepatectomy confirmed a diagnosis of hepatocellular carcinoma (HCC). An examination of the surrounding liver revealed dilated vessels and thickened endothelial cells without inflammations. HHT patients without other risk factors (like this patient) reportedly have a lower incidence of common cancers, including HCC, in comparison to the unaffected population. One intriguing hypothesis that might explain the hepatocarcinogenesis in this situation is the ischemic liver cirrhosis theory, which suggests that chronic ischemia may cause parenchymal strain and promote inappropriate hepatocyte proliferation.
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PMID:Hepatocellular Carcinoma in a Patient with Hereditary Hemorrhagic Telangiectasia. 3014 56

Hereditary hemorrhagic teleangectasia (HHT, or Rendu-Osler-Weber disease) is a rare inherited syndrome, characterized by arterio-venous malformations (AVMs or Telangiectasia). The most important and common manifestation is nose bleeds (epistaxis). The telangiectasias (small AVMs) are most evident on the lips, tongue, buccal mucosa, face, chest, and fingers, however; large arterio-venous malformations can also occur in the lungs, liver, pancreas, or brain. Telangiectasias in the upper gastrointestinal tract are known to occur, however data regarding possible small-bowel involvement is limited due to technical difficulties in visualizing the entire gastrointestinal tract. The occurrence of AVMs in the stomach and small bowel can result in chronic bleeding and anaemia. Less frequently, this may occur due to bleeding from oesophageal varices, as patients with HHT can develop hepatic parenchymal AVMs or vascular shunts which cause hepatic cirrhosis and portal hypertension. Gastroenterologists have a crucial role in the management of these patients, however difficulties remain in the detection and management of complications of HHT in the gastrointestinal tract.
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PMID:Rendu-Osler-Weber disease: a gastroenterologist's perspective. 3117 68


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