UMLS:C0019163 - FACTA Search

Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019163 (hepatitis B)
38,309 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Hemophilia A and B patients seen at nine US regional treatment centers were tested for serologic markers of hepatitis B virus (HBV), hepatitis C virus (HCV), and hepatitis delta virus (HDV) during 1987 and 1988. Because human immunodeficiency virus (HIV) infection, a potentially confounding variable, was present in 53% of the group, the population was divided by HIV status for analysis purposes. In the HIV-positive group (N = 382), less than 1% had not been infected with HBV, HCV, or HDV, whereas 75% had evidence of infection with HBV and 98% with HCV. HBsAg, a marker of active HBV infection, was present in 12% of subjects; 96% of these were HCV positive. Anti-HDV was detected in 35 subjects (9.1%); all were anti-HBc positive. Ten of the 35 (29%) also were positive for IgM anti-HDV, indicating current infection. All 10 were HBsAg positive and 7 of the 9 tested were HDV RNA positive. Severe/moderate hemophilia B patients were more likely to have experienced an HBV infection and to be anti-HDV positive than were similar hemophilia A patients (22% v 8%, P < .05). In the HIV-negative group (N = 345), the subjects were younger and had less severe hemophilia than the HIV-positive patients. No evidence of HBV, HCV, or HDV infection was found in 18%, whereas 33% had experienced HBV infection and 79% were anti-HCV positive. Within this group, 4% were HBsAg positive. All 13 subjects with anti-HDV (4% of the HIV-negative group) also possessed anti-HBc. One (7.7%) was IgM anti-HDV positive and the serum from another contained HDV RNA. Both of these individuals were HBsAg positive. As in the HIV-positive group, severe/moderate hemophilia B patients were more likely to be HBV and HDV positive than were hemophilia A patients (9% v 3%, P < .05). A prevalence study of viral hepatitis in a large US hemophilic population showed that active infection with HCV is common, occurring in 89% of all study patients regardless of HIV status. Evidence of active HBV infection was found in 8%; 19% of these were actively infected with HDV. HDV was more common in hemophilia B patients after controlling for disease severity.
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PMID:A multicenter study of viral hepatitis in a United States hemophilic population. 767 17

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

In the last few years, plasma fractionation has been subjected to major technological changes which have contributed to improve the viral safety and overall purity of plasma derivatives. New viral inactivation treatments, primarily solvent-detergent and pasteurization, have been introduced in the manufacturing processes of plasma derivatives to ensure the inactivation of major plasma-borne viruses, including HIV and hepatitis B and C viruses. Concurrently, new highly purified products obtained by chromatographic methods (mainly ion exchange and/or immunopurification) have been developed in the last five years and have replaced former preparations, providing a significantly higher safety level in terms of purity and viral risks. For an example, the new generation of Factor VIII and Factor IX concentrates (to treat hemophilia A and hemophilia B, respectively), which have been introduced in the last five years, are purified over 10,000- to 20,000-fold from plasma, as compared to only 50- to 100-fold for the former products. Similarly, new, standardized, clotting factor or protease inhibitor concentrates have been made available, thus permitting to carry out selective hemotherapy of specific diseases. Examples include the development of von Willebrand factor, factor XI, protein C, or alpha 1-antitrypsin concentrates for the substitutive therapy of congenital or acquired deficiencies. In addition, the concept of good manufacturing practices has been implemented, whereas carefully controlled, validated processes are contributing to the consistency in the quality of those products. Current major problems in plasma fractionation relate to the potential occurrence of new pathogenic agents that could resist present viral inactivation treatments and to the potential effect of given purification technologies on the development of immunogenic properties of proteins. Current trends indicate that significant progress in viral safety of plasma derivatives (for example through the introduction of new concept such as viral filtration) are to be expected very soon. Further research in this very important field is mandatory as plasma should remain the starting material of important therapeutic products in the coming years.
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PMID:[Plasma fractionation. Progress, problems and perspectives]. 799 59

Forty-one patients with hemophilia A were studied for the prevalence of serological markers for hepatitis A, hepatitis B, hepatitis C (non-A and non-B hepatitis), and delta hepatitis (hepatitis D). Ten of 41 (24.4%) patients demonstrated hepatitis A antibody and 31 of 41 (75.6%) patients had a serologic marker for previous hepatitis B infection; four of these 31 patients (13%) also demonstrated antibody to delta agent (hepatitis D). Thirty-seven of 41 (90.2%) patients demonstrated antibody for hepatitis C. Nine of 31 (29%) patients with a hepatitis B marker (no hepatitis B vaccinees) were negative for anti-HBc but positive for anti-HBs; all of these nine patients were HIV antibody positive, although they had no overt immunodeficiency. Twenty-six of 41 (63.5%) patients were HIV antibody positive. Of HIV antibody positive patients, 27%, 88%, and 100% demonstrated evidence of a previous hepatitis A, hepatitis B, or hepatitis C, respectively. Of HIV antibody negative patients; 20%, 53%, and 73% of the patients demonstrated evidence of a previous hepatitis A, hepatitis B, or hepatitis C infections, respectively. The difference between HIV antibody positive and HIV antibody negative groups was not significant for hepatitis A but was significant for hepatitis B (P < 0.001) and hepatitis C (P < .001). Of the 31 patients with a hepatitis B serologic marker, all had antibody to hepatitis C. Of 10 patients, without a hepatitis B serologic marker, only 6 (60%) had antibody to hepatitis C.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Serologic markers of viral hepatitis A, B, C, and D in patients with hemophilia. 826 2

Virucidal methods to inactivate infectious agents are based on various methods of heating or chemically treating plasma concentrates of coagulation factors VIII and IX used in the treatment of hemophilia A and B. This clinical evaluation of the viral safety of such 'treated' concentrates is mainly based on the prospective study of previously untreated hemophiliacs by means of clinical and serological markers of viral infection. Although there have been a few focal episodes of human immunodeficiency virus (HIV) transmission by clotting factors, these have been traced to ineffective virucidal methods that are no longer used or to clerical errors during the manufacturing process. Viral inactivation by pasteurization, vapor heating, heating in the lyophilized state at 80 degrees C and addition of solvent/detergent definitely decreases the risk of infection with hepatitis B and C. The current screening of plasma units for antibody to hepatitis C virus prior to inclusion in pools for concentrate production should further decrease the risk of hepatitis C infection. Other viruses, such as parvovirus and the hepatitis A virus, may still cause infections because they are quite resistant to virucidal methods. On the whole, virucidal methods have greatly reduced the risk of new HIV infections and, to a lesser degree, hepatitis.
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PMID:Clinical evaluation of viral safety of coagulation factor VIII and IX concentrates. 803 99

The association between heterozygous factor VII deficiency and severe hemophilia A in the same family is described. The 18-year-old proposita had a negative personal history for bleeding events and underwent clotting evaluation after a fortuitous finding of a prolonged PT. Decreased levels of both factor VII activity and antigen to about 50% of normal, compatible with heterozygous deficiency, were found in her and in three other asymptomatic siblings. Two uncles on the paternal side had severe hemophilia A and also showed low factor VII levels. However, in these two patients factor VII deficiency was secondary to chronic liver disease due to hepatitis B and C virus infections. Combined clotting defects in the same kindred may be due to an independent segregation of two separate defects or to common gene(s) malfunction. Factor VII deficiency is frequent and probably underestimated in the general population. For these reasons factor VII deficiency can easily segregate with other clotting defects.
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PMID:Heterozygous factor VII deficiency and severe hemophilia A in the same kindred. 856 40

Prophylaxis has been practiced for many years in Europe and is gaining acceptance worldwide with current viral inactivation procedures. Unfortunately, the high cost of prophylaxis is currently the major obstacle to its implementation in developing countries such as Turkey. The aim of this controlled preliminary study is to evaluate the efficacy, safety, and feasibility of prophylaxis. Seven boys aged 1.5-7 years (5.0 +/- 1.8), who had severe hemophilia (six A, one B) received 20-50 IU/kg factor twice weekly and were followed up for 6-24 months (14.5 +/- 6.6). Intermediate concentrates have been used in hemophilia A and ultrapure product for hemophilia B. The data obtained for the same group of patients before prophylaxis were used as a control group. Another control group was selected in another group of 10 hemophiliacs, mean age 12.5, and received treatment on demand. During prophylactic treatment, the episodes of bleeding were decreased (from 10.5 +/- 3.2 to 4.5 +/- 3.6). Orthopedic and radiologic joint scores were stable (from 0 to 1 and from 1.1 +/- 1.2 to 1.0 +/- 1.5). The patients spent significantly fewer days in the hospital (from 18 +/- 12 to 0.7 +/- 0.6). None of the patients was infected with hepatitis A, hepatitis B, or human immunodeficiency virus. One patient was seroconverted with anti-hepatitis C virus in the third month of prophylaxis. Mean consumption of concentrates for prophylaxis was 3489 +/- 960 IU/kg per year compared with 2073 +/- 1302 in conventional therapy. Prophylaxis was superior to treatment on demand even when given in a twice-weekly period with intermediate concentrates. In Third World countries, prophylaxis should be tried at least in selected severely hemophilic children in order to prevent disabilities.
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PMID:Prophylactic therapy for hemophilia in a developing country, Turkey. 908 43

This analysis evaluated the extent to which infections with selected blood-borne viruses, specifically infection with hepatitis B virus, hepatitis C virus, and/or the human immunodeficiency virus (HIV), continue to contribute to the morbidity of persons with hemophilia. The Georgia Hemophilia Surveillance System collected information on 336 state residents with hemophilia A or B who were followed by a physician in 1994. Data abstracted from medical records included information on demographics, sources of hemophilia care, clinical characteristics, joint range of motion measurements, hospitalization, and results of laboratory testing for hepatitis B, hepatitis C, and the human immunodeficiency virus. Prevalence of infection with one or more of these viruses was determined, and relationships with disease severity, bleeding frequency, and amount of clotting factor prescribed were explored. No child under the age of ten was positive for the human immunodeficiency virus; hepatitis infection was also uncommon in this age group, in contrast to the very high frequency of such infections among older subjects. There was a strong association between HIV positive status and infection with one of the hepatitis viruses. The likelihood of all types of viral infection increased with frequency of bleeding and with amount of clotting factor received. Efforts to prevent transmission of lipid-enveloped viruses via clotting factor have been extremely effective. However, currently infected hemophilia patients will likely experience significant morbidity and mortality due to chronic liver disease and AIDS-related complications.
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PMID:Viral infections among patients with hemophilia in the state of Georgia. 972 74

The evolution of transfusion or infusion therapies for diseases requiring specific protein replacements (e.g., hemophilia A and B and severe combined immunodeficiency syndrome) was dramatic over the second half of the 20th century. Unfortunately, it was accompanied by extreme manifestations of transfusion-transmitted diseases, such as human immunodeficiency virus (HIV), hepatitis B, and hepatitis C. The milestones of both the replacement therapies and the associated diseases are discussed in this presentation, which focuses on the technologic advances that resulted in even more "pure" replacement therapies for plasma-protein diseases. From donor screening to the development of viral attenuation techniques, every facet of production for these products was impacted by the exigent push for viral safety created by HIV and hepatitis. Almost invariably, this negatively affects total product yield. At the beginning of the 21st century, success in making plasma products safe from recognized and potential pathogens has dramatically increased societal pressures to produce a zero-risk, plasma-derived protein therapy. However, past improvements and low theoretic risks for future pathogen contamination have increased product cost. This is associated with a possible decrease in the overall supply of these plasma proteins because of the reduced numbers of acceptable donors and the loss of protein from expanded attenuation technology. These impacts and the role of dynamic societal and scientific pressures on these decision processes are discussed.
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PMID:History of plasma-product safety. 1144 15

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