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)

Investigated were the haemostasis of 45 patients with histologically confirmed liver cirrhosis. The patients were subdivided into three groups according to the extent of their porto caval collateral circulation as proved by laparoscopy, gastroscopy and radiology: I = no porto caval shunts (n = 10); II = moderate porto caval shunts (n = 13); III = distinct porto caval shunts (n = 14). A 4th group consisted of 8 patients with bleeding from oesophageal varices. The results indicated a significant decrease in the stages I-III of the coagulation factors produced in the liver (incl. factor XIII and AT III) and the thrombocytes. Unchanged remained the concentration of factor VIII, whereas the factor VIII associated antigen showed an increased activity depending on the severity of the disease (stages I-III). In patients with bleeding from oesophageal varices, values of about 300% of normal could be demonstrated. Depending on the stage of the porto caval collateral circulation, the concentration of fibrin(ogen) split products were also increased. For comparison, patients with pre- and posthepatic blockage were investigated, whose portal hypertension was not caused by liver cirrhosis. Besides a mild thrombopenia they only showed a secondary hyperfibrinolysis. The results, above all in the cases of liver cirrhosis, can be explained by pathophysiological mechanism: a decreased synthesis of clotting factors-a disturbed portal microcirculation with fibrin deposition-an impaired function of the liver RES.
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PMID:[Blood coagulation disorders in liver cirrhosis in relation to the degree of portal hypertension]. 697 Jan 56

Liver biopsies were performed in 5 boys aged between 2 and 9 years with severe classical haemophilia who had persistently abnormal liver function tests. Abnormal histology was present in all; 4 had chronic persistent hepatitis and the fifth chronic aggressive hepatitis with early cirrhosis. Evidence of previous hepatitis B infection was present in one patient, 3 had antibodies to hepatitis, A, and 2 had subnormal levels of alpha-1-antitrypsin. Haemobilia occurred as a late complication of biopsy in one. The significance of these findings in young boys is discussed, as is the role of exposure to factor VIII containing blood products. It is concluded that cryoprecipitate should be used in preference to large pool factor VIII concentrates in children with haemophilia.
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PMID:Liver disease complicating severe haemophilia in childhood. 743 4

The aim of this double-blind, placebo-controlled crossover study was to investigate the effect of 1-deamino-8-D-arginine vasopressin (dDAVP) on hemostasis in patients with chronic liver disease. Nine consecutive patients with biopsy-proven liver cirrhosis and related coagulation abnormalities received in a random order dDAVP, 0.5 microgram/kg, or saline intravenously. Blood samples were taken before dDAVP infusion and 30, 60 and 180 min after its end. dDAVP infusion induced a statistically significant shortening of the bleeding time from 9 min (range 6.5-15.5) to 6 min (range 4.5-9.5) at 1 h after the infusion. The activated partial thromboplastin time was significantly shortened at 30 and 60 min after dDAVP infusion. Plasma levels of factor VIII, XI and XII coagulant activities were significantly increased at all sampling times after dDAVP infusion. The maximum increase over basal values in plasma levels of factor VIII, XI and XII was 63, 22 and 40%, respectively. dDAVP did not induce any significant changes of prothrombin time, thrombin clotting time, fibrinogen, plasma levels of factor II, V, VII, IX, X, factor XII antigen, protein C (activity and antigen), antithrombin III, plasminogen and alpha 2-antiplasmin. Placebo infusion did not produce any significant changes in the evaluated parameters. We conclude that dDAVP can positively influence the hemostatic system in patients with liver cirrhosis. The clinical relevance of this hemostatic improvement deserves further evaluation.
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PMID:Effects of desmopressin on hemostasis in patients with liver cirrhosis. 748 63

Eighty percent of hemophiliacs exposed to plasma products are seropositive to hepatitis B and an even higher percentage are seropositive to hepatitis C. Post-transfusion hepatitis is followed by cirrhosis in up to 25% of the cases. In the wake of portal hypertension, the development of oesophageal varices entails the risk of life-threatening hemorrhage. We report on a patient with moderate hemophilia A (factor VIII:C 4-11%) who suffered from massive hematemesis, melaena and evolving shock after excessive alcohol ingestion. The diagnosis of Mallory-Weiss syndrome and the differential diagnosis of bleeding oesophageal varices as well as prognostic consequences are discussed.
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PMID:Mallory-Weiss syndrome in a patient with hemophilia A and chronic liver disease. 757 95

Over the past decade, with the use of plasma-derived factor VIII and factor IX, treated with virucidal methods, as well as with recombinant factor VIII, the replacement therapy of hemophilia has been intensified. In developed countries, a majority of patients are being treated at home, and large groups of children benefit from primary prophylaxis. A serious task in these countries for the coming years is the management of patients infected with HIV. In Poland and less-developed countries, the supply of antihemophilic factor concentrates is inadequate. Patients with inhibitor antibodies should be included in programmes of immune tolerance inducement. Many patients who had been multitransfused with cryoprecipate or received lyophilized concentrates before 1985, have developed chronic hepatitis associated with viral infections. About 15-30% show evidence of cirrhosis. Recombinant technologies should be improved and become more accessible in order to provide patients with safe and cheap antihemophilic factor concentrates. A true break-through in the hemophilia treatment would be a repair of the inherited clotting defect with gene therapy.
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PMID:[Current status and future prospects of hemophilia treatment]. 765 34

Clinical cure of hemophilia A by orthotopic liver transplantation has been reported in 11 cases. We describe the first successful Italian case. A 27-year-old man had cirrhosis caused by previous infections with the hepatitis B, C and D viruses following life-long treatment with factor VIII concentrates made from large plasma pools. He was, however, seronegative for the human immunodeficiency virus. In the year before transplantation, life-threatening gastrointestinal bleeding due to severe esophageal varices required a large transfusion regimen (on average, 13 bags of red cell concentrates and 35,000 U of factor VIII/week). To perform orthotopic liver transplantation 8,000 U of factor VIII were given during surgery together with 10 bags of red cells and 11 of fresh-frozen plasma. Intraoperative bleeding was not different from that of non-hemophilic patients undergoing orthotopic liver transplantation. No additional factor VIII was used after transplantation and factor VIII levels in plasma were always above 50 U/dl, reaching the highest value of 184 U/dl on day 4 post transplantation. He was discharged from hospital 10 weeks after transplantation with factor VIII levels of 68 U/dl. All virological markers are currently negative, except anti-hepatitis C virus antibodies. In this patient orthotopic liver transplantation was a life-saving treatment for end-stage cirrhosis and a cure for hemophilia A.
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PMID:Orthotopic liver transplantation in a patient with severe hemophilia A: a life-saving treatment for the first Italian case. 778 10

Six cases of hepatic sarcoma are reported: leiomyosarcoma in two, malignant fibrous histiocytoma in two malignant hemagiopericytoma in one and fibrosarcoma in one. In addition to the routine paraffin section and HE stain, immuno-histochemical studies with antibodies against vimentin, EMA, CK, S100, ACT, AAT, desmin, AFP, lysozyme and factor VIII and Masson trichrome staining and argyrophilia staining were done. AFP was negative in all 6 patients and the primary sarcoma was characterized by the absence of accompanying liver cirrhosis. The diagnosis, histogenesis and prognosis of primary liver sarcoma are discussed.
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PMID:[Primary sarcoma of the liver]. 795 5

The peribiliary vascular plexus (PVP) plays an important role in the pathophysiology of the biliary tree. We histologically examined vascular endothelial cells of the intrahepatic PVP in various hepatobiliary diseases by immunohistochemistry and lectin histochemistry with antibodies to factor VIII-related antigens (F-VIII-R-Ag) and Ulex europaeus agglutinin I (UEA-I). The PVP around the intrahepatic large bile ducts (LBDs) and septal bile ducts (SBDs) in normal livers consists of three layers: inner layer vessels immediately adjacent to the epithelium, intermediate layer vessels within the ductal wall, and outer layer vessels outside the ductal wall. In some bile ducts that show active inflammation in hepatolithiasis, primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and extrahepatic biliary obstruction (EBO), vessels in the intermediate layer and, to a lesser degree, in the inner layer, are increased in number. In sclerotic bile ducts of PSC, EBO, and hepatolithiasis, the number of inner and intermediate layer vessels are markedly and variably reduced, respectively. In liver cirrhosis or chronic advanced liver diseases, the vessels in all three layers, particularly those in the outer layer, are increased in number and dilated, probably reflecting intrahepatic microcirculatory disturbance. The PVP showed several types of numerical and luminal changes, each of which may be related to disease processes in the intrahepatic biliary tree as well as to intrahepatic microcirculatory disturbance.
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PMID:Intrahepatic peribiliary vascular plexus in various hepatobiliary diseases: a histological survey. 808 71

We characterized the structural and immunohistological changes of sinusoidal endothelial cells that occur during cirrhosis in rats made cirrhotic with thioacetamide. Thioacetamide (200 mg/kg body wt) was injected intraperitoneally three times a week into male Wistar rats. Two, 4, 6 and 12 wk later, rat livers were observed under transmission and scanning electron microscopy and regular microscopy and immunostained with laminin and von Willebrand factor (factor VIII-related antigen) antibodies. The diameters and numbers of sinusoidal endothelial fenestrations did not change significantly after 2 wk in the thioacetamide-treated rats; however, they decreased within 4 wk after thioacetamide treatment. A basement membranelike structure in Disse's space was noted 6 wk after thioacetamide treatment. Laminin was detected in Disse's space after 4 wk. In vitro, in cultured sinusoidal endothelial cells, the diameter of sinusoidal endothelial fenestrations was significantly lower at 6 wk in thioacetamide-treated rats. von Willebrand factor was detected in the cytoplasm as granular fluorescence after 6 wk of thioacetamide treatment. These results suggest that as fibrosis develops in cirrhosis, the structural and immunohistochemical characteristics of sinusoidal endothelial cells change.
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PMID:Defenestration of the sinusoidal endothelial cell in a rat model of cirrhosis. 824 83

In seven dogs with histologically proven liver cirrhosis the activity of the single coagulation factors with the exception of factor VIII:C, of the inhibitors antithrombin III and protein C as well as plasminogen and alpha 2-antiplasmin was distinctly lower than in the control group (p < 0.0001). The changes of the factors VII [median (x0.50) = 17 %] and X (x0.50 = 18 %) as well as of protein C (x0.50 = 15 %) were particularly pronounced. Diminution of activity certainly exceeded also in nearly all of the remaining haemostatic proteins the decrease of albumin concentration. Besides the shorter half life time, this reflected an increased consumption in consequence of intravascular coagulations and fibrinolysis. The latter could also be seen from the significantly increased concentrations of soluble fibrin and fibrin(ogen) degradation products. Therefore, the alterations of the haemostatic system measured in dogs in many details were in accordance with findings in human beings suffering from liver cirrhosis.
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PMID:[Alterations of hemostasis in liver cirrhosis of the dog]. 952 5


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