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Query: UMLS:C0684275 (haemophilia)
10,958 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Whilst this conference presented no new or exciting breakthroughs in our understanding of the psychosocial or neuropsychological aspects of HIV disease, there were some useful contributions to our knowledge in these areas. Previous work suggesting that HIV seropositive individuals with HIV are no more likely to suffer poor psychosocial adjustment than matched HIV-controls. However, where psychiatric disorders are seen, the most common are adjustment disorders followed by drug and alcohol abuse. Mania, whilst quite rare, may be more commonly seen with HIV than should be expected. People with asymptomatic HIV infection appear to be no more likely to show a cognitive deficit than matched HIV-individuals and this appears to be equally true for gay men, intra-venous drug users and people with haemophilia. However, as the disease progresses, neuropsychological impairment, or even dementia, may be seen and when this occurs the pattern of decline appears to be precipitous rather than insidious.
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PMID:Neuroscience of HIV infection: basic and clinical frontiers. 178 84

Persons with HIV may have previous or concurrent liver impairment as a result of injection drug use, hepatitis, alcohol abuse, and damage from medications. Additional stress is placed on the liver by low-grade opportunistic infections and hemophilia. It is especially important that persons with HIV care for their liver to help this organ remain physiologically normal during chronic and acute management of HIV infection. Although modern pharmaceutical medicine does not provide liver tonics or supportives, herbal medicines have been used to ease liver stress for ages. Readily available liver protectants and their actual mechanism of action, including thioctic acid, glycyrrhizin, and Silybum marianum, are described.
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PMID:Liver function and HIV-1 infection. 1136 18

Hepatitis C is a major cause of morbidity and mortality in haemophiliacs who received clotting factor concentrates before the availability of virus-inactivated factors in the mid-1980s. Early studies gave conflicting indications as to the severity of hepatitis C (originally termed non-A non-B hepatitis), as mild, slowly progressive hepatitis was documented in several infants and young adults with haemophilia who were examined with repeat liver biopsies, whereas more progressive hepatitis and cirrhosis was documented in others. One major point of dispute was whether these discrepancies could in part be accounted for by epidemiological differences among studies, as hepatitis C acquired early in life may initially run a benign course and later worsen owing to spontaneous recrudescence of hepatitis or interference with such comorbidity factors as alcohol abuse or infection with the human immunodeficiency virus (HIV). In the mid 1990s, the latter infection overshadowed hepatitis C as a cause of death in this patient population. Because hepatocellular carcinoma is emerging as an important complication in haemophiliacs with long-standing hepatitis C virus (HCV) infection who survived HIV infection, and because of recent advances in treating HIV, morbidity and mortality associated with chronic hepatitis C have regained emphasis amongst haemophiliacs. The development of newer interferon-based therapies provides an opportunity for modifying the natural history of HCV infection in a substantial number of haemophilic patients.
Haemophilia 2004 Oct
PMID:Hepatitis C in haemophilia: lights and shadows. 1547

Prior to 1990, many patients with inherited bleeding disorders were infected with hepatitis C virus (HCV). This study assessed the risk of end-stage liver disease (ESLD) in patients with hemophilia with chronic hepatitis C. Patients were infected between 1961 and 1990 and were followed up to August 2005. Of 847 anti-HCV(+) patients, 160 (19%) spontaneously cleared HCV and 687 (81%) developed chronic hepatitis C. Coinfection with HIV was present in 210 patients. After 35 years of infection the cumulative incidence of ESLD was 11.5% (95% CI, 8.2%-14.8%) in HIV(-) patients and 35.1% (95% CI, 29.2%-41.0%; P < .001) in patients coinfected with HIV. Independent risk factors of ESLD were HIV coinfection (hazard ratio 13.8; 95% CI, 7.5-25.3), older age at infection (hazard ratio 2.3 per 10 years; 95% CI, 2.0-2.8), alcohol abuse (hazard ratio 4.9; 95% CI, 2.5-9.6), and presence of HCV genotype 1 (hazard ratio 2.2; 95% CI, 1.1-4.2). With longer duration of HCV infection, the risk of developing ESLD is emerging in patients with inherited bleeding disorders. Risk factors for rapid progression to ESLD are alcohol abuse, coinfection with HIV, older age at infection, and presence of HCV genotype 1.
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PMID:Progression to end-stage liver disease in patients with inherited bleeding disorders and hepatitis C: an international, multicenter cohort study. 1721 88