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)

Hypersplenism was not relieved by distal splenorenal shunting in 46 of 47 Japanese patients with nonalcoholic cirrhosis of the liver. However, the platelet count significantly increased by 40 percent of the preoperative value, whereas the increment in the white blood cell count was nil. Though the platelet count in 47 patients with a patent shunt did not significantly differ from that in another 7 patients with an occluded shunt, the rate of increase was significantly higher in those with patency than in those with early occlusion throughout the postoperative period and in those with late occlusion 6 months after operation. The increased rate of the platelet count can thus serve to screen patients for shunt patency.
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PMID:Increased platelet count as a screening test for distal splenorenal shunt patency. 339 90

Over a 6-year period 26 infants and children with homozygous (2 Z and 6 ZZ) or heterozygous alpha 1-antitrypsin deficiency (12 MZ, 6 MS) were observed prospectively and their families investigated. 7 of 8 homozygous patients had neonatal hepatitis, whereby 3 of these showed maximum transferase activities during the 5th to 9th months of life. At the age of 7 years 2 of these patients were clinically normal, but only one patient had normal transferases. One patient had cirrhosis with portal hypertension at the age of 16 years 6 months; her nephew showed hypersplenism. Family studies revealed a further 5 relatives of phenotype Z, 16 of phenotype MZ, 3 of phenotype SZ and 1 of phenotype MS; 6 of these had slightly elevated serum transferase activities. 6 patients of phenotype MZ and 2 patients of phenotype MS had neonatal hepatitis but generally with a much better prognosis than in homozygous patients. The other heterozygous patients (6 MZ and 4 MS) had a variety of additional factors determining the disease and the prognosis. Family studies showed a further 7 family members of phenotype MZ, and 2 of phenotype MS; 2 of these had slightly elevated transferase activities, 3 parents had hereditary hyperbilirubinaemia.
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PMID:[Liver diseases in children and their relatives with homozygous and heterozygous alpha 1-antitrypsin deficiency]. 349 72

The prevalence of cholelithiasis in liver cirrhosis was analyzed on 1320 consecutive necropsies which included 245 subjects with gallstones and 133 with liver cirrhosis. Gallstones were found in 24.8% of the cirrhotics, a prevalence significantly higher than in noncirrhotic subjects (17.8%) (p less than 0.05), and were more frequent in cirrhosis for all age-groups. The mean age of death was lower in cirrhotic than in noncirrhotic subjects (p less than 0.05). It was also lower, but without statistical significance, in cirrhotics without gallstones than in cirrhotics having gallstones. The ratio between lithiasic women and men was 0.8/1 in liver cirrhosis, as compared to 1.6/1 in noncirrhotic subjects. The proportion of pigment stones was significantly increased in liver cirrhosis (47.5%) (p less than 0.02). Chronic hemolysis secondary to hypersplenism, a know lithogenic factor in liver cirrhosis, might account for the predominance of pigment stones in this disease. Other lithogenic factors could be hyperestrogenism, changes in the proportion of biliary lipids etc. Complications of gallstones occurred less frequently in cirrhotic than in noncirrhotic patients, but complications of cholecystectomy represented the cause of death in 27.2% of cirrhotics as compared to 14.0% of noncirrhotic patients (p less than 0.02). These observations argue for a conservative, non-surgical attitude towards silent or uncomplicated gallstones in cirrhotic patient.
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PMID:A necroptic study of the prevalence of cholelithiasis in liver cirrhosis. 370

A two-stage radioactive antiglobulin test--using unlabelled antisera specific for IgG, IgA, IgM and C3 followed by binding of 125I-staphylococcal protein A--was applied to determine platelet-associated immunoglobulins (PAIg) and complement (PAC3) in thrombocytopenias of various etiologies. One hundred and one patients with immune thrombocytopenia (chronic autoimmune, 48; acute autoimmune, 37; Evans syndrome, nine; connective tissue diseases, seven) and 20 patients with presumed nonimmune thrombocytopenia (bone marrow aplasia or malignancy, six; septicemia, five; hypersplenism, five; cirrhosis of liver, three; others, one) were studied. Increased levels of PAIg/C3 were found in 76% of patients with immune thrombocytopenia. PAIgG was raised in 66%, PAIgM in 57%, PAIgA in 44%, and PAC3 in 29%. Isolated elevation of PAIgG and of PAIgM was found in four and three cases, respectively; PAIgA and PAC3 were elevated in one case each. PAIgG was associated with PAIgM in 56%, with PAIgA in 34%, and with PAC3 in 27%. Both patients with Evans' syndrome and patients with connective tissue diseases had significantly higher PAIgM levels than the other patients with immune thrombocytopenia. In patients with nonimmune thrombocytopenia, increased rates of PAIg/C3 were also encountered. Positive test results were found in 88% (PAIgG 88%, PAIgM 47%, PAIgA 35%, and PAC3 24%). In immune-mediated thrombocytopenia, we observed a significant inverse correlation between platelet counts and PAIgG, PAIgA, and PAC3, but not with PAIgM. In contrast, no such correlation was found in patients with nonimmune thrombocytopenia. Our data indicate that the evaluation of neither parameter alone nor the combination of PAIg/C3 will discriminate between immune and nonimmune thrombocytopenia. Preferential coating with certain immunoglobulins, however, may be present in some subgroups of immune thrombocytopenias.
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PMID:Platelet-associated immunoglobulins IgG, IgM, IgA and complement C3 in immune and nonimmune thrombocytopenic disorders. 375 69

Results of subacute embolization of the splenic artery with a metallic spiral obtained in 22 patients with cirrhosis of the liver with splenomegaly were studied at the stage of sub- and decompensation of the portal blood circulation. Results of the study and their clinical evaluation suggest that the subacute embolization of the splenic artery is a relatively safe, atraumatic and effective method of surgical treatment of splenomegaly, hypersplenism resulting from liver cirrhosis with portal hypertension. The method allows to decrease hypersplenism, splenomegaly, portal hypertension, to eliminate the pain syndrome due to splenomegaly.
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PMID:[Embolization of the splenic artery in liver cirrhosis with portal hypertension and splenomegaly]. 376 85

An unusual hepatic disease developed in 3 patients with a well-functioning kidney graft 16-24 months after transplantation. Vague abdominal pain, increased bleeding tendency and edema were initial complaints, and hepato- or splenomegaly and ascites were found as well. Liver function tests were not or only mildly disturbed; hemolysis and pancytopenia were always present. Colloid uptake was absent at liver scintigraphy and the hepatic venous wedge pressure was increased. Esophageal varices were demonstrated. Liver biopsy showed extensive midzonal and pericentral sinusoidal dilatation. After discontinuation of azathioprine the symptoms and the extent of sinusoidal dilatation disappeared gradually, but after 1-3 years fibrosis or micronodular cirrhosis had developed and splenomegaly with hypersplenism remained. These observations strongly suggest an association between chronic use of azathioprine and the development of venous congestion of the liver with sinusoidal dilatation, eventually resulting in chronic liver disease.
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PMID:Hepatic sinusoidal dilatation with portal hypertension during azathioprine treatment after kidney transplantation. 390 52

We analyzed the clinical and bacteriologic features of 12 episodes of spontaneous bacterial peritonitis (SBP) in 11 children (four boys, median age 5.5 years) with chronic liver disease. All patients had cirrhosis and ascites; four had hypersplenism, and one was asplenic. Symptoms included increasing abdominal distention, pyrexia, abdominal pain, gastrointestinal disturbance, and encephalopathy. Nine had rebound tenderness on abdominal palpation, and 12 had reduced bowel sounds. The most frequent organisms isolated from culture of ascitic fluid were Streptococcus pneumoniae (nine). Klebsiella (two), and Haemophilus influenzae (one); blood cultures grew identical organisms in nine. Seven patients died despite intensive antibiotic therapy. In the 3 months prior to onset of SBP, defective yeast opsonization and reduced serum concentration of C4 were found in all nine children tested; eight had reduced concentration of C3. Functional deficiency of all complement components was present in four tested within 1 to 5 months of the onset. In contrast, only eight of 59 cirrhotic children without SBP had low C3, and eight had defective yeast opsonization, although 35 had low C4 values. Four of the patients with SBP and low C3 and C4 concentrations had normal concentrations at the time of diagnosis of liver disease 2 to 5 years previously. Opsonization of type III pneumococci was reduced in sera from three patients who subsequently developed pneumococcal peritonitis. The incidence of SBP in children with chronic liver disease is similar to that in adults, as are the clinical features. Our observations suggest that complement deficiency induced by chronic liver disease may be important in the pathogenesis of SBP.
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PMID:Spontaneous bacterial peritonitis in children with chronic liver disease: clinical features and etiologic factors. 399 46

Transcatheter variceal embolization (PTO, TIO) has been performed in 71 cases and splenic artery embolization (SAE) in 22 cases (15 PTO-SAE combination and 7 SAE alone). Results for varices of PTO combined with SAE were better than with PTO alone. Furthermore PTO combined with SAE was found to be reliable even for long term control of bleeding, the longest follow up being almost over 3 years during which time we have had no case of rebleeding. Improvement of Child's criteria was seen to be better in SAE cases (52.4%) than in splenectomized cases (12.5%) and PTO alone (17.4%). Hepaplastin test and level of cholinesterase were used to assess liver function, before and after treatment. It was found that SAE cases improved considerably, in contrast to the splenectomized and control cases which showed little or no improvement. Thus to increase durability for long term control of bleeding and general condition, PTO should be combined with SAE. Furthermore, it is suggested that this combined embolization therapy should be used for nonsurgical treatment of esophageal varices and hypersplenism with liver cirrhosis.
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PMID:[Transcatheter embolization therapy of esophageal varices and hypersplenism with liver cirrhosis]. 408 44

Thirteen patients with Wilson's disease were compared with seven cirrhotic and 13 normal controls to define better the haematological abnormalities in this condition. Hypersplenism (anaemia, leukopenia, thrombocytopenia, and reduced red cell survival) commonly occurred in patients with both Wilson's disease and cirrhosis. These abnormalities correlated with splenic enlargement. Despite reduced haematocrits, red cell mass was greater in these two groups than in normal controls. Plasma volume and the body haematocrit/peripheral haematocrit ratios were also greater in patients with Wilson's disease and cirrhosis. Increased splenic sequestration of (51)Cr-tagged red blood cells was not demonstrated in any subjects. The hypersplenism in patients with Wilson's disease is similar to that found in patients with cirrhosis from other causes.
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PMID:Hypersplenism in Wilson's disease. 502 27

74 patients with cystic fibrosis aged 1-19 years were assessed prospectively for 1-7 years for evidence of liver involvement. 20 of these patients were referred primarily because of hepatic problems. 3 of 4 with neonatal hepatitis recovered. Chronic active hepatitis developed in a further child but resolved spontaneously. 6 patients had abnormal liver-function tests without clinical evidence of liver disease. In 18 cirrhosis was detected at age 4-13 years. Liver disease was stable in these except terminally in 3 with cor pulmonale. The principal hepatic problem was variceal bleeding, which occurred in 6 patients. 50% of bleeds followed aspirin ingestion. This drug therefore should be avoided in such patients. 13 had hypersplenism. 2 had severe splenic pain necessitating splenectomy with lienorenal shunt, which was performed also in 2 patients who had bled. 3 remain well up to 5 years later. In 3 patients seen in the past 3 years injection sclerotherapy has controlled bleeding. This technique was well tolerated without the pain associated with, or intensive physiotherapy necessary after, shunt surgery; and this may be the method of choice for controlling variceal bleeding in cystic fibrosis.
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PMID:Hepatic complications of cystic fibrosis. 611 50


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