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)

The final value of portal blood flow pressure depends on the degree of vascular obstruction, then on the resistance in collateral vessels and, last, on splanchnic blood flow. The iniciating cause of portal hypertension most often lies in advancing anatomical damage leading to increased resistance and, consequently, to a reduction of portal blood flow, and simultaneous reciprocal development of extrahepatic collaterals. The determination of a true portal flow is a necessity particularly when deciding about a shunt surgery and its type, but it also supplies valuable information on the degree of portal flow restriction and, in this way, on the progress of pathophysiological changes, their extent and advance. The technique of radionuclide angiography and determination of the hepatic perfusion index (HPI) proposed by Sarper appears to be a profitable noninvasive method supplying well reproducible information on portal blood flow. Sarper proved it to be correlated with the degree of portal hypertension established by angiography. Ultrasonographic criteria of portal hypertension include dilatation of the portal vein in the region of the hilus hepatis exceeding 15 mm, and a more than 10 mm dilatation of the splenic vein above the spine. The mean HPI value obtained from the examination of 19 subjects without liver involvement was 0.6956 +/- 0.0583. The group of chronic hepatopathies included 19 patients with bioptically verified chronic hepatitis without reconstruction and/or steatosis, and 32 patients with liver cirrhosis likewise confirmed by biopsy: portosystemic shunts could be demonstrated in 14 of the latter. (ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Radionuclide hepatic perfusion index and ultrasonography: assessment of portal hypertension in clinical practice. 253 Aug 18

Patients with portal hypertension of varying etiology may develop pulmonary artery hypertension. In the present autopsy study, pulmonary and hepatic tissue was studied in 12 patients in whom pulmonary and portal hypertension coexisted. Plexogenic pulmonary arteriopathy was present in 10 patients, 7 of whom had coexistent thromboembolic lesions. One patient had isolated medial hypertrophy, which may be an early stage in the plexogenic category, whereas isolated thromboembolic pulmonary vascular disease was observed in one subject. Hepatic disease was consistent with alcoholic cirrhosis in seven patients, cryptogenic cirrhosis in four and extrahepatic portal hypertension without cirrhosis in one. Thrombocytopenia was present in all 10 patients whose platelet count was determined. This study suggests that pulmonary hypertension associated with portal hypertension commonly has a plexogenic appearance on histologic examination. However, thrombosis (whether embolic or in situ) may also contribute to vascular obstruction.
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PMID:Coexistent pulmonary and portal hypertension: morphologic and clinical features. 368 Jul 90

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

In clinical studies, frequent hepatic dysfunction associated with crises in sickle cell disease has been noted, but whether irreversible morphologic changes arise from these transient episodes is uncertain. We studied 70 patients with sickle cell disease (57 SS, 12 SC and one S-thalassemia (S-thal) hemoglobin) autopsied at The Johns Hopkins Hospital. They ranged in age from five months to 75 years (average 21 years) and 35 (50 percent) were female, In 64 patients (91 percent), livers were enlarged and had distention of Kupffer cells with phagocytized sickled red cells; this was massive in 10. In 19 patients (27 percent) the sinusoids were markedly distended with sickled red cells and appeared obstructed. Focal parenchymal necroses were present in 24 patients (34 percent) and were explained in 12, eight by cardiac dysfunction and four by sepsis. Reparative changes, portal fibrosis and regenerative nodules were each found in 14 patients (20 percent), only one of whom had a known history of viral hepatitis despite the frequency of transfusions. Cirrhosis of unknown cause was present in seven patients and cardiac cirrhosis in one. Cirrhosis with hemochromatosis was present in three patients and 30 others had parenchymal iron accumulation. Thus, unexplained hepatic necroses, portal fibrosis, regenerative nodules and cirrhosis were frequently encountered in these patients. This spectrum of liver disease appears to be best understood as a consequence of recurrent vascular obstruction, necrosis and repair arising as a component of sickle cell disease.
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PMID:The liver in sickle cell disease. A clinicopathologic study of 70 patients. 744 49

Ischemia is known to be a cause of hepatocellular apoptosis and atrophy in experimental animals, but the effect of vascular obstruction on such lesions in the normal or cirrhotic human liver has not been studied. The purpose of this study was to investigate the role of ischemia in the development of apoptosis, atrophy, and nodular hyperplasia in cirrhotic and noncirrhotic human livers. Apoptosis, focal atrophy, and nodular hyperplasia were semiquantitated in 203 liver specimens obtained at transplantation, segmental resection, or autopsy. These parameters were correlated with etiology, stage, activity, and acute and healed portal vein thrombosis (PVT). Large numbers of apoptotic cells were found in livers with acute PVT (17.2/medium-power field [MPF]) and in infarcts of Zahn caused by obstruction of portal veins (PVs) by tumor (16.4/MPF). Smaller numbers of apoptotic cells were found in cirrhosis of various etiologies (3.8-10.0/MPF) and rarely in normal livers (0.16/MPF). Evidence of healed PVT was found in 47% of cirrhotic livers and was associated with nodular hyperplasia (58% vs. 32%, P < .01) and focal atrophy (79% vs. 49%, P < .002). Apoptotic cells were found equally in those with and without healed PVT (40% vs. 38%, not significant). These observations suggest that apoptosis is a transient response to acute ischemia and that atrophy and nodular hyperplasia are chronic responses to ischemia. Vascular obstruction may be an important cause of the apoptosis and atrophy, which are found in nodular regenerative hyperplasia (NRH), infarct of Zahn, chronic hepatitis, and cirrhosis.
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PMID:Role of ischemia in causing apoptosis, atrophy, and nodular hyperplasia in human liver. 925 44

Hepatic vein (HV) thrombosis causes ascites, hepatomegaly, and severe congestion of the liver (Budd-Chiari syndrome [BCS]). Severe hepatic fibrosis develops in this syndrome with a variety of histological patterns. Some livers have a pattern of cirrhosis in which there is fibrous bridging between HVs and portal tracts (veno-portal cirrhosis). Other livers have a pattern of "reversed-lobulation cirrhosis" (veno-centric cirrhosis), in which fibrous bridging between HVs and portal tracts is minimal. The prevalence and pathogenesis of these forms of cirrhosis and the effect of portal vein (PV) thrombosis in this disease have not been studied. We examined 15 resected livers from patients with BCS to determine the distribution of vascular obstruction and the character of the parenchymal response. Six livers had veno-portal cirrhosis, and all of these had severe PV obliteration caused by thrombosis. Three livers had veno-centric cirrhosis and had normal medium and large PVs. The remaining six livers had mixed veno-centric/veno-portal cirrhosis and had moderate PV obliteration. The nodules in veno-centric cirrhosis had evidence of an unusual circulation with small arteries supplying a midzonal venous plexus that appeared to drain retrogradely into patent small PVs. Nine livers had large regenerative nodules histologically similar to focal nodular hyperplasia. PV thrombosis is a frequent occurrence in BCS. The correlation between PV thrombosis and the pattern of cirrhosis suggests a role for PV obliteration in the genesis of veno-portal bridging fibrosis in this disease and possibly in other diseases leading to cirrhosis.
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PMID:Pathology of the liver in Budd-Chiari syndrome: portal vein thrombosis and the histogenesis of veno-centric cirrhosis, veno-portal cirrhosis, and large regenerative nodules. 946 48

Tracheal varices and bronchial varices are infrequently reported in adults as a complication of an underlying vascular obstruction, including portal hypertension, pulmonary arterial hypertension, or pulmonary venous hypertension. Tracheal varices and bronchial varices have been reported in adults with failing Fontan physiology, but this occurrence is rare in children. We report the unusual presentation of tracheal-bronchial varices due to veno-venous collaterals in an adolescent patient with Glenn physiology for double-inlet left ventricle and portal hypertension secondary to cardiac cirrhosis. We document complete resolution of these varices after heart and liver transplantation.
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PMID:A case of tracheal varices in an adolescent patient with cyanotic heart disease. 2350 29