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)

In the present study fibrinogen was assayed by the immunonephelometric method in 19 patients afflicted with hepatocarcinoma and 24 patients afflicted with cirrhosis. The two groups were similar in age, sex and presence of HbsAg. The incidence of values above the norm was significantly greater in patients with hepatocarcinoma (p less than 0.05). Then, the concentration of fibrinogen was measured in all using two other immunological methods (Laurell and Mancini) and two coagulative methods (Clauss and Ratnoff). A dysfibrinogenemia (an excess of fibrinogen assayed by immunological methods to be greater than 100 mg/dl with respect to biological methods). is more frequent using the Nephelometer-Clauss (p less than 0.01) and Mancini-Clauss (p less than 0.01) methods in patients with hepatocarcinoma with respect to those with cirrhosis. The study of the kinetics of antifibrinogen antigen-antibody reaction failed to show differences between patients with hepatocarcinoma or cirrhosis and normal subjects.
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PMID:The use of laser-nephelometry in evaluating fibrinogenaemia in patients with hepatocarcinoma and cirrhosis. 241 22

Patients with liver disease have a wide range of coagulation problems. Patients with parenchymal liver disease are likely to have reduced synthesis of proteins and inhibitors (Table 1), whereas dysfibrinogenemia and hyperplenism develop in cirrhosis. Patients with cholestasis without cirrhosis often have elevated levels of fibrinogen, Factors V and VIII, probably due to reduced clearance of proteins (Table 1). The hemostatic defects in liver disease are complex and multifactorial. They are often unpredictable and the mechanisms elusive. Developments in molecular genetics and immunology have shown that both qualitative and quantitative abnormalities of coagulation proteins and protein inhibitors contribute to the abnormalities in liver disease. Future research will certainly result in a better understanding of these defects and more effective therapy.
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PMID:Hemostasis in liver disease. 331 57

The possible existence of acquired dysfibrinogenemia was investigated in blood samples from 30 patients with liver cirrhosis, 15 newborns and 30 healthy control subjects. Alterations of thrombin time were found in newborns and in 14 cirrhotic patients; glucide fraction levels were measured in these subjects and an increase in sialic acid content was observed. Its functional role was studied by comparing thrombin time and electrophoretic mobility of purified and desialylated forms of fibrinogen. We observed a thrombin time normalization, which was initially prolonged upon the removal of the sialic acid. The anodic electrophoretic mobility underwent changes due to the removal of sialic acid.
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PMID:Role of sialic acid in acquired dysfibrinogenemia associated with liver cirrhosis. 357 54

Acquired dysfibrinogenemia was documented in a 4-year-old child with obstructive jaundice of 1-month duration, secondary to a choledochal cyst involving the distal common bile duct. It was characterized by decreased thrombin coagulable protein with elevated immunoassayable fibrinogen resulting in abnormal thrombin and reptilase times. The liver morphology was compatible with extrahepatic obstruction, without evidence of cirrhosis or hepatocyte abnormality. All the coagulation abnormalities promptly resolved after surgical correction of the obstruction. Dysfibrinogenemia has been associated with serious liver disease in adults, including tumors, chronic active hepatitis, and cirrhosis, but never with isolated obstructive jaundice. This report documents a case of acquired dysfibrinogenemia due to extra-hepatic biliary obstruction and also emphasizes the importance of the consideration of this disorder in coagulation abnormalities associated with hepatobiliary disease.
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PMID:Dysfibrinogenemia in obstructive liver disease. 368 83

Dysfibrinogenemia in 36 patients with primary hepatocarcinoma and in 25 patients with cirrhosis of the liver was studied by means of reptilase time, thrombin coagulase time, fibrin polymerization and fibrinogen assays. Both groups of patients were similar in age, sex and incidence of HBs Ag. No electrolyte or fluid imbalances were present. Prolonged reptilase time and prolonged polymerization time were found in both groups; however, thrombin coagulase time was prolonged in 80% of the hepatocarcinoma group, but was normal in almost all patients with cirrhosis (p less than 0.001). In the hepatocarcinoma group, a difference of more than 100 mg per 100 ml was present between the immunologic and coagulative methods of fibrinogen determination in 36.1% of the cases, but in the cirrhotic group this difference was not present in any of the patients (p less than 0.01). We also found that by simply measuring fibrinogen levels by the Mancini method, we could distinguish hepatocarcinoma from cirrhosis in most cases.
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PMID:The importance of simple coagulation tests (fibrinogen assays and thrombin coagulase clotting time) in the diagnosis of liver cancer. 609 71

Using a new and sensitive screening method, dysfibrinogenaemia (DF) was detected in 76% of patients with cirrhosis, 78% with chronic active liver disease and 86% with acute liver failure. The incidence was much lower in obstructive jaundice (8%) and miscellaneous liver disorders (4%). It is concluded that the fibrin monomer polymerisation (FMP) ratio test is a simple and sensitive test for the detection of DF, and is useful in the differential diagnosis of hepatocellular and obstructive jaundice. Hyperfibrinogenaemia, particularly in patients with obstructive jaundice, may explain the high incidence of abnormal thrombin and Reptilase clotting times despite normal FMP ratios. Dysfibrinogenaemia dose not appear to be related to the degree of liver function impairment, but may be associated with regeneration of hepatic tissue.
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PMID:Acquired dysfibrinogenaemia in liver disease. 708 17

The fibrinogen molecule is becoming increasingly understood. Amino acid sequencing has been undertaken and studies of abnormal fibrinogens are leading to a more functional concept of its structure. Acquired dysfibrinogenemia appears to be a more common problem than previously thought, and may be found in patients with liver disease, cancer, fibrinolysis, and disseminated intravascular coagulation (DIC). While previous evidence suggested that anti-thrombin activity and fibrin polymerization inhibitors were formed in these conditions, recent studies suggest that slow fibrin formation occurs as a result of structural changes induced in the fibrinogen molecule itself. These relatively minor, alterations in structure cause a functional dysfibrinogenemia simulating abnormalities seen in some congenital fibrinogenopathies. A case is presented illustrating such a dysfibrinogenemia in a patient with cirrhosis of the liver and evidence for DIC.
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PMID:Fibrinogen and dysfibrinogenemia. 744 88

The proposita suffered from liver cirrhosis and biopsy showed type 1 membrane-bound fiberglass inclusions. The hepatic inclusion bodies were weakly periodic acid-Schiff diastase-positive, and on immunoperoxidase staining reacted specifically with anti-fibrinogen antisera. Coagulation investigations revealed low functional and antigenic fibrinogen together with a prolonged thrombin time of 37 seconds (normal, 17 to 22 seconds) suggestive of a hypodysfibrinogenemia. DNA sequencing of all three fibrinogen genes showed a single heterozygous mutation of GGG (Gly)-->CGG (Arg) at codon 284 of the gamma-chain gene. However, examination of purified fibrinogen chains by sodium dodecyl sulfate-polyacrylamide gel electrophoresis, reverse-phase high-performance liquid chromatography, ion-exchange high-performance liquid chromatography, and isoelectric focusing, failed to show any evidence of the mutant gamma(Br) chain in plasma fibrinogen. This finding was substantiated by electrospray ionization mass spectrometry, which showed only a normal gamma (and Bbeta) chain mass, but a large increase in the portion of their disialo isoforms. We speculate that misfolding of the variant protein causes hepatic retention and the subsequent hypofibrinogenemia, and that the functional defect (dysfibrinogenemia) results from hypersialylation of otherwise normal Bbeta and gamma chains consequent to the liver cirrhosis. These conclusions were supported by studies on six other family members with hypofibrinogenemia, and essentially normal clotting times, who were heterozygous for the gamma284 Gly-->Arg mutation.
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PMID:Fibrinogen brescia: hepatic endoplasmic reticulum storage and hypofibrinogenemia because of a gamma284 Gly-->Arg mutation. 1088 Mar 89

Before elective surgery, it is mandatory that a precise history be taken to detect increased hemorrhagic diathesis and that thrombocytes, Quick/INR, and aPTT be determined. If pathological levels are found, further laboratory tests are necessary after frequent causes (e.g., liver cirrhosis) have been excluded. Single-factor analysis for the von Willebrand's factor antigen and if necessary further tests to check for von Willebrand's syndrome (multimeric analysis) as well as platelet function tests should be performed.Dysfibrinogenemia is a rare coagulation disorder, which causes elevated INR. It shows a wide spectrum of clinical manifestations including thrombophilia, excessive bleeding, and even asymptomatic cases. We present a 72-year-old patient with asymptomatic dysfibrinogenemia who needed hip replacement due to arthrosis. Lowered fibrinogen levels were substituted prior to operation and the clinical course afterwards was uneventful under additional prophylactic anticoagulation in order to prevent thrombosis. The case report illustrates the interdisciplinary teamwork which is very important in the management of patients with coagulation disorders.
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PMID:[A rare coagulation disorder. Diagnostics and management in cases of hereditary dysfibrinogenemia]. 1904 87

Liver cirrhosis is associated with number of hematological complications and coagulation disturbances. In view of various haemostatic abnormalities it is surprising that many patients do not bleed spontaneously. Severe coagulopathy of liver disease is more frequently seen in acute liver failure, but still remains important complication of liver cirrhosis and chronic liver failure. Decreased production of blood coagulation factors by the liver plays a key role in altered haemostasis in liver diseases. Altered fragile balance of blood coagulation proteins and infection are associated with both worsening coagulopathy and bleeding risk. Additional haemostatic abnormalities in patients with severe liver diseases are thrombocytopenia, chronic disseminated intravascular coagulation, accelerated fibrinolysis, hypofibrinogenemia and dysfibrinogenemia. In this review we discuss a complicated issue of multiple coagulopathies in patients with advanced liver dysfunction.
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PMID:Coagulopathy in liver diseases. 2051 45


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