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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 upper gastrointestinal tract hemorrhage secondary to esophageal varices in a patient with Felty's syndrome prompted a review of the pathogenesis and treatment of this condition. Six previously reported cases of this association were found. The clinical picture is that of long-standing rheumatoid arthritis with severe articular and extraarticular manifestations including splenomegaly, depression of the blood elements, mild liver function abnormalities, portal hypertension without cirrhosis or portal vein obstruction, an elevated splenic blood flow, and a reduction in portal hypertension by simple splenectomy. The presence of portal hypertension with varices may be another indication of splenectomy in patients with Felty's syndrome.
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PMID:Esophageal varices in Felty's syndrome: A case report and review of the literature. 108 37

This report describes the clinical, biochemical, and hepatic morphologic findings in ten children with severe serum alpha1-antitrypsin deficiency. Genetic protease inhibitor (Pi) phenotyping, using acid-starch gel and crossed antigen-antibody electrophoresis, demonstrated Pi phenotype ZZ in all our cases. In eight patients, manifestations of liver disease appeared during the first year of life. The case reports show that alpha1-antitrypsin deficiency should be suspected in any child with neonatal hepatitis, unexplained hepatomegaly or splenomegaly, or cirrhosis. In our report, one infant is normal at age 6 months, and one infant had progressive hepatic damage that culminated in liver failure and death at age 6 months. The variable clinical course and prognosis for infants with severe alpha1-antitrypsin deficiency is well illustrated by these two infants.
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PMID:alpha1-Antitrypsin deficiency and liver disease in children. 108 88

Idiopathic portal hypertension is reported in five cases including one case of chronic arsenical intake and one case of chronic industrial vinyl chloride exposure. In all five cases the patients presented with gastrointestinal bleeding as the chief complaint. Physical examination was within normal limits except for splenomegaly in all. Results of liver function tests were normal, except for the relative clearance of sulfobromophtalein. A surgical liver biopsy specimen was obtained in all cases and showed moderate degrees of portal fibrosis, but no cirrhosis. Combined umbilicoportal, hepatic vein and superior mesenteric artery catheterization was performed in all cases. Hepatoportographies showed distortion of the intrahepatic portal venous system and cut-off of small portal venules. Porto-hepatic gradients ranged from 14.0 to 20.5 mm Hg. The portal hypertension was both sinusoidal and presinusoidal in nature but mainly presinusoidal. Hepatic extraction of indocyanine green and of albumin microaggregates was normal, thereby suggesting normal functional portal blood supply to the liver. The patients with arsenical or vinyl chloride exposure could not be differentiated from the other three patients with idiopathic portal hypertension. These results suggest that idiopathic portal hypertension may be related to domestic or industrial exposure to other hepatotoxins.
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PMID:Idiopathic portal hypertension. 108 57

A 39-year-old male with bleeding esophageal varices due to portal hypertension was observed. The patient had taken an arsenical preparation during a period of 12 yr because of psoriasis and subsequently developed keratotic changes of the palms and soles of his feet and an epithelioma of the scrotum. Physical examination was unremarkable except for splenomegaly and skin lesions. Liver function tests were normal; a needle biopsy of the liver (right lobe) showed nonspecific changes. Combined hepatic and umbilicoportal catheterization revealed, on splenography and portography, huge esophageal varices and patent portal vein; dilation, distortion, and cut-off of many intrahepatic portal branches were found. A marked gradient existed between the free portal venous pressure (25 mm Hg) and the wedged hepatic venous pressure (9.5 mm Hg). Hepatic blood flow, portal PO2, cardiac output, cardiac index, and blOOD volume were within normal range. Arteriographies did not reveal arteriovenous shunts in the splanchnic or splenic vessels. A splenorenal shunt were performed and a wedged biopsy of the liver (left lobe) revealed nonspecific changes. Three years later the patient had not experienced any episode of hemorrhage or hepatic encephalopathy but developed an epithelioma of the tongue. No known cause could be incriminated in the pathogenesis of the portal hypertension. However, there was unequivocal chronic arsenic intoxication. Toxic hepatitis, cirrhosis, noncirrhotic portal hypertension, and hemangiosarcoma of the liver have been reported with the intake of arsenicals. Thus, it is suggested that in this patient, presinusoidal portal hypertension was secondary to chronic arsenical intake associated with marked intrahepatic vascular changes seen on portography.
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PMID:Noncirrhotic presinusoidal portal hypertension associated with chronic arsenical intoxication. 112 3

Exploratory laparotomy, splenectomy and liver biopsy were carried out as a pre-treatment staging procedure in 32 cases of pathologically proved malignant lymphoma with clinically palpable spleen. The spleen was found to be negative for lymphoma in 17 cases, of which liver pathology showed bilharzial hepatic fibrosis in eight cases, nutritional cirrhosis in two cases and non-caseating granuloma in three cases. The liver was clinically positive in 13 cases. After laparotomy, three cases showed bilharzial pathology only. Bilharziasis has accounted for about one half of the false positive hepato-splenic involvement. These findings exemplify the usefulness of laparotomy as a staging procedure in regions endemic for hepato-splenomegaly.
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PMID:Clinical staging of malignant lymphoma in patients suspected to have hepato-splenic schistosomiasis. 114 72

To clarify the effect of splenomegaly on portal hemodynamics in patients with portal hypertension and esophageal varices, manometric studies were carried out before and after splenectomy during an operation for esophageal varices. The 118 patients evaluated retrospectively had underlying liver cirrhosis (LC) (62), idiopathic portal hypertension (IPH) (42), and extrahepatic portal occlusion (EHO) (14). The weight of the spleen did not differ significantly among the three diagnostic groups: 640 +/- 473.5 g for LC, 780 +/- 414.6 g for IPH, and 683 +/- 457.2 g for EHO. Before splenectomy, portal pressure was significantly elevated in the patients with EHO (410 +/- 85.2 mm H2O) as compared to either the LC or IPH groups (348 +/- 64.1 and 348 +/- 73.5 mm H2O). Following splenectomy the reduction of portal pressure was significantly greater in the EHO group (29 +/- 15.5%) than in either the LC (18 +/- 17.4%) or IPH (19 +/- 17.0%) groups. Each group was subdivided according to severity of splenomegaly: marked (spleen weight > or = 500 g) or slight (spleen weight < 500 g). Patients with LC and marked splenomegaly showed a reduction in liver function parameters as shown by the prolongation of indocyanine retention rate at 15 min as compared to those with slight splenomegaly. Though it is not statistically significant, the average portal pressure tended to be higher among those with marked splenomegaly.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Portal venous pressure following splenectomy in patients with portal hypertension of differing etiology. 129 40

Splenic trauma with hematoma following extracorporeal shock wave lithotripsy (ESWL) is very rare. We reported a case of subcapsular hematoma of spleen with impending rupture following ESWL for ureteral calculus. This case was noted to have liver cirrhosis and splenomegaly, and received a total of 2000 shock waves under 18 kilovoltage. The subcapsular hematoma occurred 2 months later. Splenectomy was undertaken for a symptomatic huge subcapsular hematoma and thrombocytopenia. We reviewed the literature and concluded that portal hypertension with severe coagulopathy are contraindications for ESWL, even in case with ureteral calculus.
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PMID:Subcapsular hematoma of spleen--a complication following extracorporeal shock wave lithotripsy for ureteral calculus. 129 57

Between July 1986 and April 1989, 334 hospitalized adult Ethiopian patients with chronic liver disease were studied according to a protocol to define their clinical features and to identify risk factors with the aim of preventive intervention. Of these, 14 had chronic hepatitis, 208 cirrhosis and 112 hepatocellular carcinoma (HCC). Both clinical and histological diagnostic criteria were employed. A detailed questionnaire was used to document demographic and clinical data. A common clinical presentation among patients with chronic hepatitis was darkening of the face and hands with or without hypertrichosis of the face and blisters over the dorsi of the hands. This overt or latent form of porphyrea cutanea tarda (PCT) responds to chloroquine. Patients with cirrhosis of the liver commonly present for the first time with ascites, splenomegaly, haematemesis and/or melena from oesophageal varices, and mental changes due to hepatic encephalopathy. Overt or latent forms of PCT are also common features. Peculiar to these cirrhotics is the rarity of spider naevi, gynaecomastia, testicular atrophy, Dupuytren's contracture, parotid gland enlargement and clubbing of the fingers. Exhaustion, loss of appetite, rapid loss of weight, right upper quadrant and/or epigastric pain (all often of less than 6 months' duration, a big, hard, tender and grossly nodular liver with bruit, signs of portal hypertension, and/or hepatic encephalopathy, in a young male with a rapid down hill course characterize the Ethiopian patient with HCC. Serum anti-nuclear factor, anti-mitochondrial anti-bodies and anti-smooth muscle anti-bodies were absent in those with chronic hepatitis and were uncommon in the cirrhotics and HCC cases. One or more hepatitis B virus markers were found in 86% of chronic hepatitis, 88% cirrhosis and 78% HCC and the HBsAg carrier state was found in 36%, 29% and 23%, respectively. Among the HBsAg carriers, HBeAg positivity was less common than anti-HBe but anti-HDV was significantly higher than in the healthy general population. Alphafetoprotein (AFP) levels greater than 500 mg/ml were present in 16 (8%) cirrhotics and 58 (52%) patients with HCC. Histologically, 3 of the chronic hepatitis patients had progressed to cirrhosis, 8 of the cirrhotic patients had chronic active hepatitis and 85% of HCC cases occurred in a background of macronodular cirrhosis. Three cirrhotics developed HCC during follow-up.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Chronic liver disease in Ethiopia: a clinical study with emphasis on identifying common causes. 131

Clinically, idiopathic portal hypertension (IPH) is characterized by overt splenomegaly with pancytopenia, portal hypertension and relatively mild abnormalities in liver function tests. Although its etiology is still undetermined, the liver pathology is characterized by occlusive changes of the intrahepatic portal radicles, portal and periportal fibrosis, irregularly distributed parenchyma atrophies and absent of regeneration nodules. The disease is relatively benign and does not progress to cirrhosis. Differential diagnosis between IPH and liver cirrhosis is mandatory. We now report a case with histologically proven IPH, including clinical course, laboratory data, roentgenographic findings of hepatic venogram and celiac angiogram, hepatic hemodynamic features and intravariceal pressure of esophageal varix which has never been reported in Taiwan.
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PMID:Idiopathic portal hypertension: a case report. 132 89

Fifty-five patients with cytomegalovirus (CMV)-associated neonatal hepatitis (NH) were followed for 12 to 90 months. Six patients (10.9%) died from either a fulminant course or a chronic liver disease. Among the remaining 49 patients, whose liver function was completely recovered, there were eight with retardation of developmental or growth status, and two with hearing impairment. Overall, 20.4% of the survivors suffered from a long-term impact. The unfavorable outcome was related to several clinical and pathological parameters. These included persistence of clay-colored stool, presence of splenomegaly, ascites or anemia, high peak total and direct bilirubin, low nadir albumin levels, diffuse giant cell transformation and cirrhosis of the liver. The seropositivity of CMV infection did not significantly correlate with the outcome.
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PMID:Cytomegalovirus-associated neonatal hepatitis. 133 53


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