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

A case of idiopathic portal hypertension associated with connective disease resembling systemic lupus erythematosus is described. The patient was a 50-year-old woman with splenomegaly, ascites, esophageal varices, and pancytopenia, but without extrahepatic portal obstruction or cirrhosis of the liver. Electron microscopy of the liver showed perisinusoidal fibrosis. High titers of autoantibodies against proliferating cell nuclear antigen (PCNA) were found in the sera as well as in ascites; anti-DNA antibodies appeared after anti-PCNA antibodies and remained thereafter at a moderate titer. The possibility of an immunological process in the pathogenesis of idiopathic portal hypertension is discussed.
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PMID:[Idiopathic portal hypertension associated with connective tissue disease similar to systemic lupus erythematosus]. 259 83

The late complications in 25 patients more than 3 years after successful Kasai's operation for biliary atresia were presented. Nine (36%) of these 25 patients had episodes of variceal hemorrhage with portal hypertension, and biliary reobstruction in 3 (12%), bleeding from duodenal ulcer in 2, bleeding from gastric erosion in one, and diffuse pulmonary arterio-venous shunt in one were observed. Recently esophageal varices have been well controlled by endoscopic sclerotherapy, and the surgical treatment may be indicated in older children complicated with severe hypersplenism and marked splenomegaly, which are resistant for sclerotherapy. As a surgical procedure, splenectomy with periesophago-gastric devascularization (Hassab's operation) seems to be very useful for portal hypertension by biliary cirrhosis. The reoperation, hepaticoenterostomy, was performed in 3 patients with biliary reobstruction. All three patients are alive and well, and two of them are at 19 years (university student) and 24 years (married life) of age. Hepatic Rehepaticoenterostomy is well indicated rather than liver transplantation, if biliary reobstruction is incurable with conservative therapy. Many of the patients with complications were able to carry on an almost normal life, if suitable treatments for complications were taken.
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PMID:[Late complications after successful Kasai's operation for biliary atresia]. 258 18

To examine the pathogenesis of thrombocytopenia associated with liver cirrhosis, the platelet count, spleen size and serum cholinesterase levels were measured together with plasma concentration of beta-thromboglobulin, fibrinopeptide A and serum albumin in 38 patients with histologically proven, severe but stable liver cirrhosis. The spleen size contributed most significantly to thrombocytopenia in this disorder and the serum cholinesterase level also correlated with the platelet count, both in decompensated and compensated liver cirrhosis. Plasma beta-thromboglobulin, serum fibrinopeptide A levels and serum albumin did not correlate with the platelet count. These findings indicate that disseminated intravascular coagulation is not likely to be the cause of thrombocytopenia in liver cirrhosis. Splenomegaly as well as the diminished protein synthetic activity of the liver participates in the pathogenesis of the thrombocytopenia in this disease.
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PMID:Thrombocytopenia in liver cirrhosis. 261 53

We are reporting on a 62 year old female patient with portal hypertension (splenomegaly, esophageal varicosis) without signs of liver cirrhosis, who was hospitalized for sclerotherapy of her esophageal varices. Physical examination showed up palmar- and plantar hyperkeratosis and Morbus Bowen or basalioma-like skin lesions++. Anamnestic evaluation revealed, that the patient's psoriasis had been treated with arsenic for many years. This kind of treatment may have induced intraluminal proliferation and obliteration of the portal vein's endothelium, thus being the etiologic factor responsible for noncirrhotic portal hypertension in this patient.
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PMID:[Portal hypertension and chronic arsenic exposure. A differential diagnostic challenge]. 262 81

The authors collected 41 cases of cirrhosis in native Tibetans with pathological verification. It is found that 60.98% of the cases were alcoholic cirrhosis, with an incidence significantly higher than that of 7.6% reported in the inland (P less than 0.01). 74.19% and 20% of the cirrhosis in male and female patients respectively were alcoholic, while 50% of the cirrhosis in the female were due to malnutrition. Among the Tibetan cirrhotics 12.2% was due to hepatitis and 4.88% each to hydatid disease and tuberculosis. The average age of these cirrhotics was 47.05. The frequent occurring age was between 41-50 (about 41.46%). The Tibetan cirrhosis in peasants and herdsmen were 51.28%. At the first admission, 84.49% got jaundice and 90.2% got ascites. These percentages were higher respectively than 43.2% and 50.5% from the Lanzou report (P less than 0.01). Ascites was serious, portal vein expanded, but splenomegaly were merely 17.07% (normal were 82.93%). These spleens expanded just a little, macrosplenopathy was not found.
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PMID:[Analysis of the etiology and clinical characteristics of Tibetan cirrhosis in Tibet]. 262 23

Basic data on pathomorphology and symptomatology of the alcohol-induced liver cirrhosis accompanied by portal hypertension are discussed. Respective data were compared with the group of cirrhotic patients not abusing alcohol. A high percentage of encephalopathic disorders and nearly 50% of the patients suffering from the hemorrhage from esophageal varices were the first sign of the cirrhosis in both groups. Despite hemorrhage from esophageal varices a few patients obtained surgical help preventing recurrence of the hemorrhage. Liver functional reserve, incidence of encephalopathies and the degree of liver involvement are in favour for non-alcohol cirrhosis. Inflammatory process in the liver, splenomegaly and hypersplenism were more frequent in the liver cirrhosis of non-alcohol origin.
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PMID:[Alcohol-induced liver cirrhosis as a cause of portal hypertension]. 263 Oct 74

For the detection of mild liver disease (acute viral hepatitis, chronic persistent hepatitis, fatty liver) serum bile acids levels have not proved to be superior to transaminases or other common liver tests with almost similar sensitivity and/or specificity. Indeed it has been possible to show in patients with compensated cirrhosis of the liver that the serum bile acids concentration is related to the degree of intrahepatic shunts and that there was a significant relationship between the fasting serum bile acids and the intrinsic clearance of ICG. Measurement of serum bile acids appear to be more sensitive for detection of cirrhosis than commonly used tests. The elevation of bile acids concentration in cirrhotic patients is thought to result from a reduced hepatic clearance and/or from portosystemic shunting. In order to determine the role of serum bile acid estimation in the indirect assessment of portal hypertension, fasting and two-hour postprandial serum bile acids concentration were measured in 36 patients with liver cirrhosis, classified according to Child-Pugh's criteria. Real time ultrasonography, esophagogastroscopy and static liver scintigraphy of the liver were carried out in all patients. The size of esophageal varices, the portal vein and its related structure, the nuclear criteria were graded according to the common methods. Between the clinical findings, splenomegaly, was noted and graded, though the size of spleen does not correlate well with the level of portal pressure. In our patients a good correlation (p less than 0.001) existed between the two hour postprandial bile acids concentration and ultrasonographic findings of portal hypertension. Fasting serum bile acids (SBA) were significantly higher in severe than in mild liver cirrhosis according to Pugh's criteria (p less than 0.001). In conclusion we think that SBA concentrations have a great prognostic value in assessment of major complications (upper gastrointestinal hemorrhage particularly). The reduced liver blood flow, for intra-and extrahepatic porto-systemic shuntings, is probably the main cause of reduced hepatic clearance of bile acids.
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PMID:[Serum concentration of bile acids and portal hypertension in cirrhotic patients. Possible correlations]. 264 42

In a group of 23 patients with advanced liver cirrhosis we have found a statistically significant positive correlation (r = 0.746; p less than 0.0001) between fibronectin and prealbumin levels measured in plasma by immunonephelometric methods and found significantly lower than in healthy controls (p less than 0.001). On the contrary, no correlation of fibronectin neither to albumin nor to the presence of an enlarged spleen was observed. Since the sensitivity of prealbumin as an index of liver function is believed to be higher than that of albumin, our results support the view that the decreased fibronectin in advanced cirrhotics is mainly due to their liver failure, an enlarged spleen playing only a minor role.
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PMID:Fibronectin is related to prealbumin in plasma of decompensated cirrhotics. 268 40

This study was designed to evaluate superparamagnetic iron oxide (AMI-25) as a contrast agent for MR to distinguish normal spleens from those diffusely infiltrated by lymphoma. As diffuse splenic involvement lacks visible tumor-tissue boundaries, signal-intensity measurements of spleens were used as a diagnostic criterion in 33 patients (lymphoma, n = 8; benign splenomegaly, n = 5; normal subjects, n = 20). Unenhanced MR images were insensitive (four of eight patients) and nonspecific (20 of 25 patients) in the diagnosis of lymphoma. After injection of superparamagnetic iron oxide (40 mumol Fe/kg), lymphomatous spleens showed a significantly higher signal intensity (p less than .05) than did normal spleens or spleens enlarged by benign disease (hepatic cirrhosis, n = 4; spherocytosis, n = 1). Changes in splenic MR signal intensity unambiguously identified eight of eight lymphomatous spleens and 25 of 25 normal or enlarged spleens that did not contain lymphoma. Phagocytosis of superparamagnetic iron oxide in lymphomatous spleens is reduced because of diffuse displacement of splenic macrophages by lymphoma cells and/or by immunologic suppression of macrophage activity. Our results suggest that superparamagnetic iron oxide (AMI-25) can improve the accuracy of MR imaging in the diagnosis of splenic lymphoma. With further development, this noninvasive technique may reduce the need for diagnostic splenectomy in lymphoma patients.
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PMID:The diagnosis of splenic lymphoma by MR imaging: value of superparamagnetic iron oxide. 278 73

Of 501 patients with chronic myeloproliferative diseases (c-MPD) 18 developed thrombosis of major abdominal vessels including 6 with hepatic vein thrombosis (Budd-Chiari syndrome). The complication was seen in 14 of 140 (10%) patients with polycythemia vera (PV), 3 of 23 (13%) patients with essential thrombocythemia (ET), 1 of 106 (1%) patients with idiopathic myelofibrosis (IMF), and none of 232 patients with chronic myelogenous leukemia (CML). Leading symptoms and signs were abdominal pain, progressive splenomegaly, widening abdominal girth, ascites, venous collaterals, and nausea and vomiting. The diagnostic modalities with highest specificity were angiography and explorative laparotomy. A causal relationship between the thrombotic event and hematocrit, thrombocyte count, or hemostatic abnormalities at the time of diagnosis could not be established. Detailed laboratory tests of platelet function and coagulation and fibrinolytic parameters of 5 surviving patients did not show any specific defect. Despite medical and surgical intervention, 39% of the patients died within 2 months after diagnosis of the thrombosis. The majority of the survivors developed further complications like liver cirrhosis with portal hypertension and esophageal varices or the short bowel syndrome after extensive bowel resection for mesenterial infarction.
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PMID:Budd-Chiari syndrome and thrombosis of other abdominal vessels in the chronic myeloproliferative diseases. 279 52


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