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  • 1
    ISSN: 1432-1440
    Keywords: Human immunodeficiency virus ; Acquired immunodeficiency syndrome ; Hemophiliacs ; Natural history ; Virus isolation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary At our institution 686 hemophiliacs are being treated. Of them 402 (59%) are anti-HIV-seropositive. The general use of heat-treated clotting factor products was begun in July 1983, and from May 1984 all patients exclusively used heat-treated clotting factors. Thus, one can assume that infection occurred no later than early 1984 in our patients. Since December 1985 HIV-positive hemophiliacs have regularly been clinically and immunologically examined. Most of the 306 patients who could be investigated were clinically symptom-free at the time of their first visit. However, 45 patients have developed AIDS from 1982 through August 1988. The mean survival time of hemophiliacs with AIDS is less than 6 months. In 36% of those 274 patients who have been followed for a mean period of 14 months the clinical stage of the disease worsened by at least one stage according to the classification system proposed by the Centers for Disease Control. We did not find a correlation between clotting-factor consumption during the years 1984–1986 and the actual clinical stage of the patients. Virus isolation from peripheral blood lymphocytes (PBIs) answered the question whether anti-HIV seropositive hemophiliacs are not only immunized but really infected in many more cases than those revealed by detection of p24 antigen or decline of p24 antibody. Positive viral culture correlated strongly with a drop in CD4+ lymphocytes under the level of 400/µl. However, HIV could not be cultured regularly in advanced cases, suggesting that virus replication in PBLs is not necessarely the cause of depletion of T-helper cells. There is no evidence that the natural history of HIV infection in hemophiliacs is different from that in other HIV-infected patients.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0584
    Keywords: Haemophilia ; Heterozygotes ; DNA-Analysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary From 46 families of predominantly German origin, afflicted with haemophilia A, 178 females were tested for carrier status. Two polymorphic restriction endonuclease sites, the extragenic marker locus DXS 52 (St 14 probe) and the intragenic Bcl I RFLP were investigated in these families. In some cases the results were corroborated by identifying (i) deletions within the factor VIII:C gene and (ii) eliminating a restriction endonuclease site. Two new alleles of the DXS 52 marker locus were found. According to this strategy, 27 women were classified as carriers and 74 as non-carriers. Forty-six women were classified as carriers according to pedigree analysis. Twenty-five females of families with sporadic cases and 6 test persons, who had mothers who where homozygous for the marker alleles, were diagnosed by additional use of conventional carrier detection.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Langenbeck's archives of surgery 372 (1987), S. 929-929 
    ISSN: 1435-2451
    Keywords: Hemophilia ; Surgery ; Factor level ; Complications ; Hämophilie ; Operation ; Faktorkonzentration ; Komplikationen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Von 1980-1986 wurden 38 OP bei 32 Patienten mit Hämophilie und v. Willebrand-Jürgens-Syndrom durchgeführt, u. a. I7 Laparotomien (Appendektomie, Splenektomie, Ileus, Anus praeter, SPV, Cholecystektomie); darunter waren 8 Notfälle. Die Gerinnungsfaktorenkonzentration wurde bei kleineren Eingriffen 〉 30% der Normalaktivität und bei Laparotomien 〉 60% substituiert. Postoperative Komplikationen waren Nachblutungen in I3 %, Abscesse in I0,5% sowie Wundheilungsstörungen in 5% der Fälle. 2 Patienten, die gleichzeitig HIV-positiv waren, verstarben (5%) 3 bzw. I3 Tage postoperativ.
    Notes: Summary From I980 to I986 38 operations were performed on 32 patients with hemophilia and von Willebrand's disease, including I7 laparotomies (appendectomy, splenectomy, ileus, anus praeter, SPV, cholecystectomy): 8 of these were emergency operations. Coagulation factor levels were raised to 〉 30% of normal activity for minor operations and 〉 60% for laparotomy. Hemorrhagic complications occurred in 13%, septic complications in I0.5% and poor wound healing in 5%. There were two deaths (5%): patients who were also HIV-positive, 3 and I3 days after surgery.
    Type of Medium: Electronic Resource
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