Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0684275 (haemophilia)
 10,958 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This overview provides a discussion of the special concerns of sexually transmitted diseases (STDs) for women, particularly because of its asymptomatic character; screening; primary prevention; e.g., abstinence, selection of sexual partners restriction of sexual activities, use of barriers (condoms, vaginal spermicides, diaphragm in conjunction with spermicides), and vaccines; and the role of the gynecologist in StD prevention. Gonorrhea and chlamydial infection are usually asymptomatic STD infections in women; long term sequelae are pelvic inflammatory disease (PID), infertility, and pregnancy complications. There is an increased risk of cervical cancer. Infection is lifelong for herpes simplex virus (HSV) and HIV and malingering for chronic hepatitis B (HPB). Genital human papillomavirus (HPV) and HSV infections cannot be identified serologically. The fetus can be fatally or severely affected by STDs. Abstinence is the only effective prevention for STDs. Likelihood of infection may be reduced by limiting partners, but how partners are chosen and knowledge of infection is a more important determinant. Partners need to be asked about current symptoms, history of STDs, multiple partners, and history of known STD partners, as well as past history of homosexual activity, intravenous drug use, hemophilia, and previous exposure to high-risk persons for STDs. Visible genital warts or lesions, wartlike growths, ulcers, or rash need explanations. Avoidance of oral anal and digital anal activity reduces transmission of hepatitis A, giardiasis, amebiasis, and shigellosis. Any mechanical barrier that remains intact should reduce the risk of STD; barriers specifically covering the cervix are excellent. Condom use is effective when used as follows: 1) at the onset of sexual activity, 2) without petroleum jelly or baby oil on latex, 3) with care of fingernails which may tear holes, 4) with complete withdrawal of the penis before complete detumescence, and 5) with a withdrawal hold at the base of the penis. Spermicides, such as nonoxynol 9, are effective against STDs. Diaphragm use with spermicide may be effective because of the spermicide. There is a reduced risk of transmission of HSV or HPV to a partner. Vaccines are only available for hepatitis B. Obstetrics and gynecology residency training in STDs in unavailable in 4 out of 5 medical schools, and gynecologists are ethically obligated to accurately inform about STD diagnosis, treatment, and diagnosis.
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PMID:Avoiding sexually transmitted diseases. 209 42

Early reports suggested that hemophiliacs with factor IX deficiency (Christmas Disease) may be at less risk for developing the acquired immunodeficiency syndrome (AIDS) than patients with classic hemophilia. We evaluated 12 factor IX deficient patients for clinical and immunologic abnormalities related to infection with the human immunodeficiency virus (HIV). Antibody to HIV was not detected in these patients prior to 1982. By 1985, 66 percent (eight of 12) patients were seropositive. All three concentrates available commercially before 1985 were associated with seropositivity. Furthermore, seropositive hemophiliacs had received on average significantly more factor IX concentrate than seronegative hemophiliacs (27,825 +/- 17,976 (S.D.) versus 1,250 +/- 1,500 factor units/year, (p less than 0.02). Half of the seropositive individuals had generalized lymphadenopathy with splenomegaly. Two seropositive patients have developed AIDS, one with cryptococcal meningitis and another with a large cell immunoblastic lymphoma. Infection with HIV has occurred with high frequency in hemophiliacs who received unmodified factor IX concentrates.
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PMID:The spectrum of human immunodeficiency virus infection in patients with factor IX deficiency (Christmas disease) 303 83

Hematologic abnormalities, including thrombocytopenia, have been reported in persons with acquired immune deficiency syndrome and in hemophiliacs. Seven of 92 adult hemophiliacs followed in a comprehensive hemophilia center developed mild to moderately severe thrombocytopenia in 1983. All had benign courses. None required therapy for thrombocytopenia, although several had increased cutaneous bleeding. Infection with human immunodeficiency virus may be important in the pathogenesis of these patients' thrombocytopenia as it has been in that of homosexual men.
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PMID:Successful conservative management of thrombocytopenia in adult hemophiliacs. 334 Oct 71

Immunoglobulins and aminotransferases were followed in 66 haemophilia patients and 13 von Willebrand patients over a six-year period. The results were correlated to HIV serology and lymphocyte subsets. Elevated IgG levels were found in 29/53 patients with haemophilia A, 2/13 with haemophilia B and in 0/13 with von Willebrand's disease. Elevated IgA and IgM levels were seen in 20% and 27% of the patients respectively, with a distribution similar to the elevated IgG levels, except that elevated IgA and IgM levels were also seen in 4/13 patients with von Willebrand's disease. Patients with HIV antibodies had significantly higher immunoglobulin levels than seronegative patients, and this elevation occurred in connection with seroconversion in the majority of the former. The IgG levels could not be correlated to the T4 cell count, but there has been a trend to less clinical symptoms related to HIV infection among those with stable IgG levels during the past few years. No correlation was found between elevated IgG levels and the aminotransferase levels, nor was any correlation found with the amount of blood coagulation factor concentrate given to the patients. The elevation of immunoglobulins observed in our haemophiliacs is multifactorial, but HIV infection is maybe the most important mechanism. The longitudinal IgG pattern may contribute to the prediction of the clinical outcome of this infection.
Infection
PMID:Immunoglobulin levels in haemophiliacs at HIV seroconversion and during follow up. 349 99

Infections with the hepatotropic viruses non-A, non-B (NANB), hepatitis B (HBV) and delta agent (HDV) are described in two patients with haemophilia. The first patient illustrates the phenomenon of interference following a simultaneous exposure to NANB and HBV. The second patient, a carrier of hepatitis B surface antigen (HBsAg), acquired superinfection with HDV which resulted in acute hepatitis progressing to chronic hepatitis. Liver disease seen in multitransfused haemophiliacs may be significantly different to that seen in other patients. As a consequence of the infusion of blood clotting factor concentrates, these patients become the site of complex viral interactions.
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PMID:Interactions between hepatotropic viruses in patients with haemophilia. 393 10

The prevalence of overt AIDS in haemophilia A in the USA is about 3% treated patients and about 2% in UK. Nevertheless up to 90% of patients with haemophilia A treated with commercial concentrates of factor VIII prepared from the blood of paid donors are seropositive to the AIDS-associated virus LAV/HTLV III. Infection seems to have commenced in 1978 in USA and in 1980 in Europe. The accession rate to the symptomatic group is not known. Seropositivity is much lower in patients treated with blood products derived from voluntarily donated blood but such patients, even when seronegative, often show cellular immune defects. An additional immunosuppressive agent in factor VIII concentrates may be responsible for this and could predispose to LAV infection. AIDS, immune defects and seropositivity are less common in patients with haemophilia B even when treated with factor IX concentrate derived from paid donors. The reasons for this anomaly are not known. The diagnosis, social, family and counselling problems of infected patients are surveyed and the prospects for effective anti-viral or immunotherapy assessed. Treatment policies using heat-treated factor concentrates and the impact of donor screening are reviewed. It is concluded that the outlook for uninfected haemophiliacs with regard to AIDS is now good.
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PMID:AIDS and haemophilia. 393 97

During each of the four-year periods 1971-1975 and 1975-1979, the annual incidence of hepatitis B infection has been assessed in 56 patients with haemophilia A by measuring plasma HBsAg, anti-HBs and anti-HBc levels. Infection rates of 7% and 9.5% per annum respectively were observed for each four-year period despite the screening of individual blood donations for HBsAg by techniques up to the sensitivity of reversed passive haemagglutination. The highest incidence of seroconversion was amongst severe haemophiliacs many of whom had received treatment predominantly with cryoprecipitate. Of the 16 patients in whom serological evidence of hepatitis B infection was obtained only one had an accompanying clinical episode of hepatitis. We conclude that haemophiliacs are still at high risk of infection by hepatitis B virus despite the screening of individual blood donors for HBsAg.
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PMID:Incidence of infection with hepatitis B virus in 56 patients with haemophilia A 1971-1979. 640 50

Virus load was tested by means of PCR calibrated with standards and HCV genotypes were determined by the LIPA-technique using sera from 123 HCV patients. Of these 39 were on renal hemodialysis treatment, 19 suffered from hemophilia, 13 were i.v. drug users and the remaining 52 had none of these risk factors (chronic hepatitis group). The most prevalent subtype in Austria was 1b followed by 3a and 1a. However, genotype 1b infections were found relatively less often in hemophilia patients and drug users than in the other groups, indicating that hemophiliacs probably had been infected by an antihemophilic plasma coming from South American or Asian donors. The highest amounts of virus were found in patients infected with genotype 3a. Determination of the patient's virus load and of the infecting subtype of HCV may be helpful in planning interferon alpha therapy.
Infection
PMID:Genotypes and virus load in patients with hepatitis C infection. 749

We studied human immunodeficiency virus type 1 (HIV-1) infection incidence over time in a 16-center cohort of hemophiliacs in the United States and Europe and estimated the most likely date of seroconversion for all seropositive subjects. Five U.S. centers enrolled subjects independent of HIV-1 status, whereas 11 centers preferentially included seropositive subjects. We obtained unbiased estimates of HIV-1 infection incidence rates from the five centers and estimated dates of seroconversion from the distribution seen among seropositives from all centers. In the five-center cohort, infection incidence began in 1978, peaked in October 1982 at 22 infections per 100 person-years at risk, and declined to 4 per 100 person-years by July 1984. Few infections occurred after 1987, and by that time, 50% of the cohort had become infected. Median seroconversion dates for subgroups of all seropositives ranged from July 1980 to December 1983, depending on the dose and type of factor concentrate. Median dates in Europe ranged from September 1981 to March 1983 and reflected the use of products manufactured from American plasma. Infection incidence apparently peaked about the same time that public health interventions were introduced to reduce transmission. These interventions, including heat treatment of factor concentrates and deferral of high-risk donors, have prevented HIV-1 infection from becoming endemic among younger birth cohorts of persons with hemophilia.
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PMID:HIV-1 infection incidence among persons with hemophilia in the United States and western Europe, 1978-1990. Multicenter Hemophilia Cohort Study. 810 67

We report a case of progressive encephalitis caused by varicella-zoster virus (VZV) in an adolescent with hemophilia and acquired immunodeficiency syndrome but without cutaneous signs of VZV infection. Magnetic resonance imaging of the brain demonstrated an abnormally increased periventricular signal in T2-weighted images. Infection with VZV was proved by in situ hybridization and immunofluorescence staining of brain tissue, which showed histologic evidence of herpesvirus infection. Encephalitis caused by infection with VZV is a potentially treatable complication of acquired immunodeficiency syndrome and requires a high index of suspicion for diagnosis.
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PMID:Unsuspected varicella-zoster virus encephalitis in a child with acquired immunodeficiency syndrome. 839 1


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