Pediatr Hematol Oncol
May 1994
Detection of hemophilia carriers is an important issue and should be addressed with great care. The allelic frequencies of three intragenic probes (Bcl I for probe p114.12, Xba I for probe p482.
View Article and Find Full Text PDFTwenty-seven factor VIII deficient patients who had previously not been treated with blood or blood products were studied after infusion of a total of 24 batches of NHS factor VIII (8Y) concentrate produced by Bio-Products Laboratory, Elstree. Follow-up was carried out according to guidelines laid down by the International Society for Thrombosis and Haemostasis. Serial estimations of amino transferase level carried out over a 26-week period revealed no elevation of these enzymes attributable to hepatitis.
View Article and Find Full Text PDFConcern has been expressed that intermediate purity clotting factor concentrates may cause immunological abnormalities in haemophilic patients, distinct from those related to HIV infection. Early reports of lymphocyte dysfunction in anti-HIV seronegative haemophiliacs pointed to activation of their lymphocytes; a potential cause of CD4+ ve lymphocyte decline in anti-HIV seropositive patients. Recent reports have suggested that the use of high purity FVIII concentrates might retard the rate of decline in CD4+ ve lymphocytes in haemophiliacs infected with the HIV virus.
View Article and Find Full Text PDFAll 14 children who were conceived at the time their 12 haemophilic fathers were anti-HIV positive were shown to be HIV negative and to be healthy physically and mentally. Eleven of the 12 female partners have remained anti-HIV negative and one has seroconverted. Despite counselling it is likely that children will continue to be conceived by anti-HIV positive haemophiliacs.
View Article and Find Full Text PDFWith the advent of standard flow cytometric methods using two-colour fluorescence on samples of whole blood, it is possible to establish the ranges of CD3, CD4 and CD8 T lymphocyte subsets in the routine laboratory, and also to assist the definition of HIV-1-related deviations from these normal values. In 676 HIV-1-seronegative individuals the lymphocyte subset percentages and absolute counts were determined. The samples taken mostly in the morning.
View Article and Find Full Text PDFAfter a maximum of 11 years (median 8.3 years) from the time of HIV seroconversion, 25 out of 59 (42%) of CMV-seropositive haemophiliacs had progressed to AIDS, as opposed to eight out of 50 (16%) CMV seronegatives. The age-adjusted relative risk for AIDS among CMV seropositives was 2.
View Article and Find Full Text PDFFactor XI deficiency is an uncommon bleeding disorder usually manifested by excessive bleeding after surgery or trauma. Until recently the only effective therapy has been fresh-frozen plasma (FFP) infusion. We describe the efficacy and safety of a new factor XI concentrate produced from human donor plasma by a modification of the method used for antithrombin III concentrate.
View Article and Find Full Text PDFThe serological response during acute hepatitis C virus (HCV) infection was examined by enzyme-linked immunosorbent assay (ELISA) in sequential serum samples from 13 haemophiliacs following their first exposure to factor VIII concentrates contaminated with HCV. The commercially available C100-3 peptide and a new 22 kDa recombinant protein (p22) encoded by the nucleocapsid region of the viral genome were used for antibody detection, whilst a nested polymerase chain reaction (PCR) method was used for the detection of viraemia. In addition, eight sporadic cases of acute HCV infection were studied.
View Article and Find Full Text PDFA multicenter prospective study was carried out to evaluate whether a vapor-heated factor VIII concentrate transmitted blood-borne viral infections over a surveillance period of 15 months. Thirty-five patients with hemophilia and von Willebrand disease who had never received any blood components were treated. Twenty-eight were analyzed and found not to have non-A, non-B hepatitis.
View Article and Find Full Text PDFClin Lab Haematol
August 1992
The techniques of Western blotting and the monoclonal antibody specific immobilization of platelet antigen (MAIPA) assay were used to detect antibodies to platelet glycoproteins in 43 samples of serum from 23 anti-HIV positive haemophiliacs (8 with severe thrombocytopenia, 6 with moderate thrombocytopenia, and 9 with a normal platelet count), six anti-HIV negative haemophiliacs and ten controls. Antibodies were present in the majority of anti-HIV positive patients' sera even before the onset of thrombocytopenia. Thrombocytopenia was associated with an increase in the incidence of antibodies to GPIIIa and GPIb, whereas the antigen most frequently recognized in patients without thrombocytopenia was GPIIb.
View Article and Find Full Text PDFJ Acquir Immune Defic Syndr (1988)
February 1992
A method is proposed for assessing the cumulative risk of various AIDS-defining conditions as the CD4 lymphocyte count declines in HIV-infected individuals. The method is analogous to survival analysis but is based on the CD4 lymphocyte count rather than on time. Thus, the level to which the CD4 lymphocyte count has declined, rather than the length of time since seroconversion, is considered as an individual's survival interval.
View Article and Find Full Text PDFHighly purified porcine factor VIII:C (FVIII:C) concentrate prepared by polyelectrolyte fractionation has been available for therapeutic use since 1980. Over the last decade substantial international experience has confirmed the value of porcine FVIII:C in management of hemophilia with inhibitors, and recent studies have underlined its particular effectiveness in treating patients with the acquired form of the disease. The rationale for use of porcine FVIII:C is based on a twofold premise.
View Article and Find Full Text PDFObjective: To describe the progression of HIV disease in a haemophilic cohort and to show the influence of treatment.
Design: 11 year longitudinal clinical and laboratory study.
Setting: A haemophilia centre.
A cohort of 111 HIV-infected haemophiliacs has been followed for up to 11 years, during which time 33 patients have been diagnosed with AIDS. Twenty-seven of the cohort developed detectable p24 antigenaemia while remaining free of AIDS. These patients experienced an increased risk of progression to AIDS compared with those patients who were persistently p24-negative (relative risk 7.
View Article and Find Full Text PDFStored sera from 28 patients with inherited coagulation disorders who had developed non-A non-B hepatitis (NANBH) following a first exposure to clotting factor concentrates and 15 similar, but unmatched, patients who had received blood products but had normal transaminases on sequential testing were tested using the Ortho enzyme-linked immunosorbent assay (ELISA) anti-HCV assay. Twenty-seven of the 28 patients with NANBH were anti-HCV positive after exposure. In 10 of those in whom dates of first exposure and seroconversion were well-defined, the median time interval to NANBH was 4 weeks (range 1-7) and to anti-HCV seroconversion was 11 weeks (range 7.
View Article and Find Full Text PDFFactor XI deficiency is a rare bleeding diathesis found predominantly in Ashkenazi Jewish kindreds. A recent study of six Jewish patients identified three distinct mutations (Types I, II, and III) in the factor XI gene that were sufficient to fully define the genotypes of the patients. We have investigated 63 patients with factor XI deficiency and find overall allele frequencies of 44% for the type II mutation, 31% for the type III mutation, and 0% for the type I mutation.
View Article and Find Full Text PDFBlood Coagul Fibrinolysis
April 1991
Qualitative abnormalities in von Willebrand Factor (vWF) in patients with cirrhosis have been little studied with contrasting results. We used crossed immunoelectrophoresis (2-DIE) and multimeric analysis of vWF in eight patients with stable hepatic cirrhosis to evaluate abnormalities in vWF before and 1 h following intravenous administration of three doses of desmopressin acetate (0.3 micrograms/kg) given at baseline, 4 and 24 h.
View Article and Find Full Text PDFAliment Pharmacol Ther
April 1991
Candidiasis of the oropharynx and oesophagus is one of the most common problems encountered in patients with HIV disease. Fluconazole is a bis-triazole antifungal agent with a long serum half-life. Sixteen anti-HIV positive patients (15 haemophiliacs and one blood transfusion recipient) with a clinical diagnosis of oropharyngeal candidiasis were treated with 50 mg fluconazole daily for 14-28 days and then either 150 mg fluconazole or placebo weekly for 6 months in a prophylactic phase.
View Article and Find Full Text PDFLow CD4 lymphocyte counts are associated with increased risk of progression to AIDS in human immunodeficiency virus (HIV) infection. We investigated the extent to which the timing of progression to AIDS can be explained solely in terms of decline of the CD4 lymphocyte count in 111 haemophiliacs followed for up to 11 years since infection with HIV. A median of 10 CD4 lymphocyte counts were made per patient.
View Article and Find Full Text PDFHaemophilic patients are at increased risk from hepatitis B virus infection because of their need for blood product therapy. They are potentially poor responders to hepatitis B vaccine due to immunological abnormalities resulting from two causes: infection with the human immunodeficiency virus and treatment with clotting factor concentrates. The protective antibody response to hepatitis B virus in vaccinated haemophiliacs was investigated using a competitive enzyme-linked immunosorbent assay which employs a monoclonal antibody, RF-HBs-1, that recognises a virus-neutralising epitope on HBsAg.
View Article and Find Full Text PDFJ Acquir Immune Defic Syndr (1988)
October 1991
The tendency for older people with HIV infection to progress more rapidly to AIDS than younger people was studied in a group of 111 anti-HIV-positive haemophiliacs followed for up to 10 years from seroconversion. After 7 years of seropositivity, those aged over 30 years at the time of the first positive anti-HIV test had a cumulative progression rate to AIDS of 50%, compared with only 12% for those aged 10-19 years (Kaplan-Meier estimates). Overall, the relative risk of developing AIDS by any given time after seroconversion was 1.
View Article and Find Full Text PDFHome therapy with porcine factor VIIIC was safe and effective when administered to five hemophilic patients over periods of 8 1/2, 6, 4, 3 1/2, and 2 years. No significant transfusion reactions occurred. Before treatment with porcine factor VIIIC, all five had high-level, high-responding anti-human VIIIC inhibitors initially lacking anti-porcine factor VIIIC activity.
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