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  • 1
    ISSN: 1432-1076
    Keywords: Key words     X-linked lymphoproliferative disease ; Inherited immunodeficiency ; Epstein-Barr virus infection ; Carrier status ; Haplotype analysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract      Three families with X-linked lymphoproliferative disease were studied. Affected males clinically presented with severe or fatal infectious mononucleosis, acquired hypogammaglobulinaemia, hypergammaglobulinaemia M, and malignant lymphoma including Hodgkin disease. Haplotype analysis using various DNA markers from Xq25-q27 allowed the prediction of the carrier status in females and identification of the XLP status in asymptomatic males.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: X-linked lymphoproliferative disease ; Inherited immunodeficiency ; Epstein-Barr virus infection ; Carrier status ; Haplotype analysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three families with X-linked lymphoproliferative disease were studied. Affected males clinically presented with severe or fatal infectious mononucleosis, acquired hypogammaglobulinaemia, hypergammaglobulinaemia M, and malignant lymphoma including Hodgkin disease. Haplotype analysis using various DNA markers from Xq25-q27 allowed the prediction of the carrier status in females and identification of the XLP status in asymptomatic males.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Histopathology 45 (2004), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Aims : To determine whether chorioamnionitis has an impact on the extent of apoptosis and proliferation in fetal lungs. Fetuses exposed to chorioamnionitis have an increased risk of aquiring lung tissue damage in utero.Methods and results : Lung tissue sections from 35 stillborn fetuses were used in this study. Chorioamnionitis-exposed fetuses were subdivided depending on whether pneumonia was diagnosed (n = 13) or not (n = 10); 12 unaffected fetuses served as controls. Apoptotic and proliferating cells were determined by in-situ terminal deoxytransferase-mediated dUTP nick end labelling (TUNEL) assay and by anti-Ki67 immunohistochemistry, and quantified. The median apoptotic index in lungs of chorioamnionitis-exposed fetuses increased 2.4-fold compared with chorioamnionitis-negative stillborn controls (P = 0.043) and rose 21.6-fold when chorioamnionitis-exposed fetuses additionally developed pneumonia (P 〈 0.001). Compared with the proliferation index of the control group (PI =2.3), the median percentage of proliferating cells in the lungs of chorioamnionitis-exposed fetuses decreased (PI = 1.4) (P = 0.036), but increased 1.8-fold (P = 0.036) in fetal lungs of the chorioamnionitis/pneumonia group. By double labellings combining the TUNEL assay or the Ki67 antigen with cell marker proteins, we identified distal airway epithelial cells as the cell type undergoing apoptosis in chorioamnionitis-exposed fetal lungs, while epithelial, endothelial and smooth muscle cells proliferated. Immunolabellings of cleaved caspases -8 and -9 revealed that apoptosis is mediated via initiator caspase-8.Conclusion : Chorioamnionitis induces apoptosis of distal airway epithelial cells via the caspase-8 pathway and interferes with the normal proliferative activity of epithelial, endothelial, and smooth muscle cells in fetal lungs. Thus, apoptosis and proliferation are an important feature of chorioamnionitis-associated lung injury in utero.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1439-0973
    Keywords: Key Words EBV-EBNA-1 variants ; Infectious mononucleosis ; Lymphoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Background: The Epstein-Barr virus (EBV) nuclear antigen 1 (EBNA-1) is essential for replication and maintenance of circular EBV genomes in latently infected B lymphocytes and is the only EBV protein expressed in nearly all cells carrying the virus. EBNA-1 is suggested to be oncogenic in vivo since its expression induces B-cell neoplasia in transgenic mice. Patients, Materials and Methods: EBV wild-type isolates from ten malignant tumors and from 15 children with various benign EBV-associated disorders were examined for the presence of EBNA-1 variant strains by PCR and sequencing. Results: One isolate harbored both the B95-8-like and a variant sequence within the C-terminus of the EBNA-1 gene. All other isolates (n = 24) revealed clustered nucleic acid sequence alterations within the EBNA-1 gene, which led to amino acid exchanges at positions 524, 563, 574, 585, 594 and 595. Few isolates exhibited additional amino acid exchanges at positions 564, 571 or 588. Conclusions: The observed EBNA-1 sequence variation pattern seems not to be restricted to a certain EBV-associated disease or tumor type. The EBNA-1 variant strains reported here may reflect the most prevalent EBV strains in the exposed population. In none of all the cases studied so far did the sequence alteration affect any known functionally crucial amino acids in the core domain of EBNA-1. This suggests that strict conservation of most of the C-terminal portion of EBNA-1 sequence may be essential for survival of EBV in the infect host.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Monatsschrift Kinderheilkunde 147 (1999), S. 917-920 
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Infektiöse Mononukleose ; Epstein-Barr-Virus ; Virale Genexpression ; Key words Infectious mononucleosis ; Epstein-Barr virus ; Viral gene expression
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background: Acute infectious mononucleosis (IM) is a selflimiting lymphoproliferative disease of EBV-infected (immortalised) B cells and poly-/oligoclonal cytotoxic T cells which are to a high percentage EBV-specific. In B cells EBV infection mainly leads to a latent infection with expression of EBV nuclear antigens 1–6 (EBNA1–6) and membrane antigens 1, 2A and 2B (LMP1, LMP2A and LMP2B). Expression of these EBV latent antigens leads to transformation and immortalisation of infected B cells. Here we examined if also lytic EBV genes (i.e. BZLF1), which are associated with a productive EBV infection, are expressed during IM. Methods: RNA expression of EBV latent genes EBNA1, LMP1 and LMP2A and EBV lytic gene BZLF1 in peripheral blood mononuclear cells (PBMC) of 12 patients with IM and 1 patient with acute T cell leukemia (HTLVI positive) was studied by nested RT-PCR and subsequent hybridisation with an EBV specific oligonucleotide. Results: Expression of latent EBV genes, EBNA1, LMP1 and LMP2A, was found in 50%, 83% and 92%, respectively, of 12 patients with IM. Expression of lytic EBV gene BZLF1 was detected in 58%. One patient with T cell leukemia exhibited expression of all latent EBV genes and of lytic EBV gene BZLF1. Conclusion: EBV lytic gene BZLF1 is expressed during IM in a high percentage. Certain nucleosidanaloga (i.e. aciclovir and others), which inhibit only lytic but not latent EBV infection, may eventually be useful in complicated and chronic EBV infections, when EBV lytic infection is present.
    Notes: Zusammenfassung Fragestellung: Die akute infektiöse Mononukleose ist eine selbstlimitierende lymphoproliferative Erkrankung, hervorgerufen durch proliferierende Epstein-Barr-Virus (EBV)-infizierte (immortalisierte) B-Zellen und durch eine poly-oligoklonalen Expansion von zytotoxischen T-Zellen, die zum großen Teil EBV-spezifisch sind. Es ist seit längerem bekannt, daß in den EBV-infizierten B-Zellen eine sog. latente EBV-Infektion vorliegt. Dies bedeutet, daß nur wenige EBV-Gene [EBV-Kernantigene 1–6 (EBNA1–6), latente EBV-Membranproteine 1, 2A und 2B (LMP1, LMP2A und LMP2B)], die v.a. für die Transformation und Immortalisation dieser Zellen verantwortlich sind, exprimiert werden. Wir untersuchten in dieser Studie, ob bei einer Mononukleose auch ein EBV-Gen (BZLF1), welches im Rahmen einer lytischen EBV-Infektion mit Produktion von infektiösem Virus eine wichtige Rolle spielt, exprimiert wird. Methode: Bei 12 Patienten mit florider akuter infektiöser Mononukleose sowie 1 Patienten mit T-Zell-Leukämie wurde in peripheren mononukleäre Zellen die RNA-Expression der latenten EBV-Gene EBNA1, LMP1 und LMP2A sowie des lytischen EBV-Gens BZLF1 mittels reverser Transkription, 2maliger PCR („nested PCR”) und anschließender Hybridisierung mit einer EBV-spezifischen Gensonde untersucht. Ergebnisse: Eine Expression der latenten EBV-Gene EBNA1, LMP1 und LMP2A fand sich in 50%, 83% und 92% der 12 untersuchten Patienten mit infektiöser Mononukleose. BZLF1 wurde in 58% der Fälle exprimiert. Bei 1 Patienten mit akuter T-Zell-Leukämie und chronischer EBV-Infektion fand sich in CD8+-T-Zellen eine Expression aller untersuchten latenten EBV-Gene sowie des lytischen EBV-Gens BZLF1. Schlußfolgerungen: Unsere Untersuchung zeigt, daß es im Rahmen einer akuten infektiösen Mononukleose häufig – neben einer latenten Infektion – auch zu einer lytischen EBV-Infektion kommt. Es ist bekannt, daß verschiedene Nukleosidanaloga (u.a. Aciclovir) nur die lytische, nicht aber die latente EBV-Infektion inhibieren. Vielleicht zeigen diese Substanzen bei chronischen und komplizierten EBV-Infektionen, bei denen v.a. lytische EBV-Gene exprimiert werden, eine therapeutische Wirkung.
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