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)

Ninety-five patients with Hemophilia A, B and von Willebrand's disease followed over a three year period were evaluated for liver disease. Half of the patients were treated episodically and half received additional prophylactic treatment. Fifty-five per cent have significant transaminitis (elevated SGOT-SGPT), 42 per cent have biochemical evidence of chronic active liver disease and two per cent have subacute and active cirrhosis. There is an annual attack rate of Hepatitis B disease of 3.5 per cent and Hepatitis B antibody titres are present in 84 per cent. This asymptomatic liver disease requires close monitoring for clinical significance.
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PMID:Liver dysfunction in patients with hemophilia A, B, and von Willebrand's disease. 30 96

A questionnaire-based survey involving 11,801 hemophiliacs from 54 hemophilia centers in the USA and Europe documented the occurrence of hepatocellular carcinoma (HCC) in 10 patients. The crude rate of HCC was 3.2/100,000 patients/year, at least 30 times higher than the background incidence of this tumor in the countries of origin of the patients. All patients were Caucasians with hemophilia A, 39 to 74 years of age, and had liver cirrhosis. All had one or more risk factor for cirrhosis and HCC: 5 were positive for serum hepatitis B surface antigen, 4 had the antibody to hepatitis C virus, and 4 had histories of alcohol abuse. Serum alpha-fetoprotein, measured in 6 patients, was significantly elevated in 4 (range: 807-1399 ng/ml), and only moderately elevated in 2 (25 and 171 ng/ml). The onset of HCC was asymptomatic in 5 patients, whereas it was accompanied by jaundice, abdominal pain, or ascites in the remaining patients. Thus, HCC seems to be a more important secondary disease for hemophiliacs than formerly recognized. Since HCC is often asymptomatic, screening hemophiliacs with chronic liver disease with periodic ultrasound scans might increase the changes of detecting HCC at a stage amenable to surgical treatment.
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PMID:Hepatocellular carcinoma in hemophilia. 165 Jan 34

The prevalence of antibodies to hepatitis C virus (anti-HCV) was studied in various population subsets in the Netherlands with anti-HCV C100 enzyme linked immunosorbent assay (ELISA), and confirmed with recombinant immunoblot assay (RIBA). Anti-HCV C100 ELISA positivity and RIBA positivity were found in 39 (0.7%) and 5 (0.1%) of 5,434 blood donors from Amsterdam; 25 (5%) and 2 (0.4%) of 481 blood donors from Surinam (South America); 19 (9%) and 2 (1%) of 213 multitransfused patients; 28 (4%) and 15 (2%) of 633 hemodialysis patients; 179 (80%) and 150 (67%) of 225 hemophilia A and B patients; 8 (80%) and 4 (40%) of 10 intravenous drug abusers; 18 (15%) and 2 (2%) of 119 anti-HIV-positive homosexual men; 2 (2%) and none of 106 anti-HIV-negative homosexual men; 6 (32%) and 3 (16%) of 19 patients with acute hepatitis non-A, non-B (NANBH); 13 (65%) and 8 (40%) of 20 patients with chronic NANBH and/or cryptogenic cirrhosis; and 4 (40%) and 1 (10%) of 10 patients with idiopathic autoimmune chronic hepatitis. Among blood donors, a positive correlation between a history of jaundice after the age of 18 years and the presence of RIBA-confirmed anti-HCV antibodies was found. Among both blood donors and hemodialysis patients, a positive correlation of RIBA-confirmed anti-HCV positivity with elevated alanine aminotransferase levels, but not with the presence of anti-hepatitis B core antibodies was found.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Prevalence of anti-HCV antibodies confirmed by recombinant immunoblot in different population subsets in The Netherlands. 164 6

A 38-year-old man with severe factor IX and mild factor VIII deficiencies complicated by cirrhosis secondary to chronic non-A non-B hepatitis underwent orthotopic liver transplantation as treatment for both the cirrhosis and his congenital coagulopathy. Intraoperative hemostasis was obtained with factor VII-depleted prothrombin complex concentrate and fresh frozen plasma. Factor VIII and factor IX levels were assayed frequently in the perioperative period, and both returned to normal within 24 hr and remained normal postoperatively. Liver transplantation can be considered as definitive therapy for hemophilia A and/or B with transfusion-related liver disease.
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PMID:Orthotopic liver transplantation in a patient with combined hemophilia A and B. 210 34

We tested an in vitro system simulating bleeding time reported by Kratzer et al. Primary hemostasis was studied perfusing an artificial vessel with citrated blood under a constant pressure of 40 mmHg, measuring the blood volume perfused (bleeding volume) and the time until blood flow stopped (bleeding time). The artificial vessel consists of a glass capillary simulating arteriole and a filter covered with collagen type I to provide a surface for the adhesion of platelets. The bleeding volume (mean +/- SD microliters) was 317.7 +/- 93.8 in controls (n = 19), 487.3 +/- 242.1 in idiopathic thrombocytopenic purpura (n = 9), 666.8 +/- 224.1 in aplastic anemia and paroxysmal nocturnal hemoglobinuria (n = 4), greater than 820 in von Willebrand's disease (n = 3), 231.0 +/- 74.5 in hemophilia A (n = 3), 499.0 +/- 269.4 in liver cirrhosis (n = 6), and 457.7 +/- 229.0 in myeloproliferative disorders (n = 11). When citrated blood was applied to this system after incubation with monoclonal antibodies (MoAb) to von Willebrand factor or platelet membrane glycoprotein Ib (GPIb), bleeding volume was significantly increased while no effects were observed after incubation with MoAb to GPIIb/IIIa, factor VIII: CAg and factor XIIIa. These data suggest that in vitro model of primary hemostasis could be used for not only diagnosing bleeding disorders although 'time' is not reliable, but also investigating the mechanisms of hemostasis.
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PMID:[Bleeding time and volume in vitro by THROMBOSTAT]. 231 3

Chronic liver disease has become a significant complication of the therapy of hemophilia disorders. We describe two patients with hemophilia A and hepatitis B virus hepatitis who progressed to cirrhosis with bleeding esophageal varices. Each underwent distal splenorenal shunt under plasma concentrate therapy without difficulty. One patient died 19 months after operation and unsuspected hepatocellular carcinoma was found at autopsy. These cases illustrate the potential severity of liver disease in hemophilia and the ability to safely perform surgery for portal hypertension if required.
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PMID:Cirrhosis, variceal bleeding, and distal splenorenal shunt in hemophilia A. 712 25

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

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

Eleven patients with chronic subdural hematoma (CSDH) with bleeding tendency (BT) were surgically treated in the last 12 years. To study the clinical problem of CSDH with BT, 11 surgical cases were divided into 3 groups, Group A; CSDH with primary BT (1 case of hemophilia A), Group B; CSDH with BT secondary to a basic disease (3 cases of leukemia, 2 cases of malignant tumor with DIC, 1 case of chronic renal failure and 1 case of liver cirrhosis), Group C; CSDH with BT by anticoagulants (warfarin) (2 cases of replacement of mitral valve, 1 case of A-C bypass). Evacuation of the hematoma was performed by means of one or two burr holes with irrigation of the hematoma cavity and a drainage tube was placed in the subdural space. The outcome was excellent in Groups A and C. In Group B, two patients with DIC due to gastric cancer and prostatic cancer died, and 3 patients with leukemia recovered dramatically from CSDH, but the poor course of the disease itself resulted in death. The outcome of other patients in Group B was excellent. Since CSDH with BT is often fatal, those patient had usually been treated conservatively. However, from this analysis, we stress that CSDH with BT should be surgically treated after checking the blood conditions carefully to determine whether or not the patient has need for surgery.
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PMID:[Chronic subdural hematoma with bleeding tendency; clinical analysis of 11 surgical cases]. 784 20

We observed six cases of haemophiliacs with HIV-induced immunodeficiency who died from fatal liver failure despite the absence of evident cirrhosis. They all had the infection with hepatitis viruses (two patients with hepatitis B and D viruses and four patients with hepatitis C virus) and their CD4 counts were severely decreased. They were much younger than cirrhotic haemophiliacs without HIV. Their serum levels of hyaluronic acid and type IV collagen were lower than those in haemophiliacs with cirrhosis, and were normal. No patients had experienced symptoms or concomitant diseases characteristic of cirrhosis, such as ascites, jaundice, oesophageal/gastric varices or hepatocellular carcinoma, except for one case who had a history of mild ascites. The characteristics of this liver failure were different from liver failure resulting from cirrhosis caused by chronic hepatitis, which suggests liver failure that is specific to patients with immunodeficiency. This kind of liver failure can be a factor threatening survival in patients with HIV infection and with hepatitis virus co-infection in an immunodeficient state.
Haemophilia 1999 Mar
PMID:Fatal liver failure in haemophiliacs with HIV-induced immunodeficiency: observation of six patients. 1021 59


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