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  • 1
    Electronic Resource
    Electronic Resource
    Two novel mutations in the MHC class II transactivator CIITA in a second patient from MHC class II deficiency complementation group A (1997)
    Springer
    Human genetics 〈Berlin〉 99 (1997), S. 541-546 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Congenital MHC class II deficiency or bare lymphocyte syndrome (BLS; McKusick 209920) is caused by defects in trans-acting regulatory factors that control MHC class II expression and is therefore a disease of gene regulation. There are at least four complementation groups and the genetic and molecular dissection of this rare disease has contributed considerably to our current understanding of the molecular mechanisms governing MHC class II expression. Identification of the gene that is defective in BLS complementation group A, CIITA (MHC class II transactivator), has led to the discovery that CIITA acts as a master control factor of MHC class II expression. We have identified the CIITA mutations in a second patient from BLS group A. Two novel mutations abolish CIITA function, as shown by transfection experiments. Molecular analysis of these two novel mutations, together with the one described earlier in the first patient, is informative in terms of CIITA structure-function relationships.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004390050403
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Acute-phase-proteins and parameters of humoral immunity in patients with advanced Hodgkin's disease (1985)
    Springer
    Journal of cancer research and clinical oncology 110 (1985), S. 65-70 
    Details
    ISSN: 1432-1335
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Serum concentrations of acute-phase-proteins C-reactive protein (CRP), α1-antitrypsin (AAT), α1-acid glycoprotein (AGP) as well as levels of immunoglobulins G, A and M and of complement components C3 and C4 were evaluated in 15 patients with advanced (stages III and IV) Hodgkin's disease. Of these patients 9 suffered from B symptoms including pruritus, night sweats and fever. While all patients had highly increased concentrations of CRP and AAT and 11 patients also had elevated levels of AGP in their sera, these concentrations were significantly (P〈0.001) reducible by the administration of chemotherapy. Patients with B symptoms also had significantly higher concentrations of CRP (P〈0.02), AAT (P〈0.05) and AGP (P〈0.05) in their sera than patients without. Plasmapheresis which was performed in 3 patients did not achieve a long-lasting reduction of serum concentrations of any acute-phase-protein tested. Complement components C3 and C4 exhibited a similar behaviour as acute-phase-proteins in that they were elevated in patients with B symptoms and reducible by the administration of chemotherapy (P〈0.001 and P〈0.02, respectively). We conclude that serum concentrations of CRP, AAT and AGP can serve as useful markers for the assessment of tumour activity in patients with advanced Hodgkin's disease. Whereas the concentrations of immunoglobulins G and A in patients were comparable to normal controls, IgM was significantly (P〈0.05) reduced in patients who had received chemotherapy, but not in those who were newly diagnosed and hat not received any treatment. Thus, chemotherapy lowered serum concentrations of IgM without influencing levels of IgG and IgA.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00402504
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Prophylaxis of necrotizing enterocolitis by oral IgA-IgG: Review of a clinical study in low birth weight infants and discussion of the pathogenic role of infection (1990)
    Springer
    Journal of clinical immunology 10 (1990), S. 72S 
    Details
    ISSN: 1573-2592
    Keywords: Low birth weight ; necrotizing enterocolitis (NEC) ; oral IgA-IgG
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Necrotizing enterocolitis, a severe gastrointestinal disease in the neonatal period, affects primarily premature infants. Perinatal complications that predispose the neonate to systemic hypoxia are frequent in infants with necrotizing enterocolitis. Ischemia of the intestinal mucosa may facilitate the invasion of enteric microorganisms in stressed low birth weight infants. Geographical and temporal clustering of outbreaks of the disease and the termination of epidemics by standard infection control underline the importance of infectious agents in the development of this disease. Several studies have established the immunoprotective effect of orally administered antibodies against infection of the gastrointestinal mucosa in children and adults. Anecdotal evidence suggested that feeding of human immune globulin might have a positive effect on the incidence of necrotizing enterocolitis in premature infants. This paper reviews a prospective, randomized, controlled trial of the efficacy of an oral immune globulin preparation (published in detail in theNew England Journal of Medicine, Vol. 319, pp. 1–7, 1988) and discusses the pathogenic role of infection in necrotizing enterocolitis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00918694
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Impaired primary antibody responses after vaccination against hepatitis B in patients with breast cancer (1992)
    Springer
    Breast cancer research and treatment 23 (1992), S. 233-240 
    Details
    ISSN: 1573-7217
    Keywords: breast cancer ; hepatitis B vaccination ; immune competence ; impaired immune response ; radiation therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The response to hepatitis B (HBV) vaccination was studied in 32 breast cancer patients who were HBV antigen- and antibody-negative at the time of diagnosis. Ten of the patients included into the study received radical mastectomy without adjuvant therapy, 15 patients underwent breast-conserving surgery with subsequent irradiation with or without tamoxifen treatment, and 7 patients were treated by modified radical mastectomy plus adjuvant chemotherapy. Eight patients received the first dose of vaccine 3 months after irradiation therapy. Seven patients were first vaccinated 6 months following irradiation therapy. Seven healthy age-matched persons were vaccinated as controls. Antibody response in the patients was significantly delayed. Four weeks after the second vaccination 6 out of 7 controls showed a significant (〉10IU/ml) HBs antibody titer, while only 6 out of 32 patients responded. In addition, HBs antibody titers were also significantly lower after the second vaccination even in those patients treated with surgery alone, although they were first vaccinated 3 months after surgery and had no residual tumors. This difference disappeared after the second booster immunization, at which time the frequency of significant antibody titers and the levels of HBs antibody titers were comparable between patients and controls. As the population of patients undergoing treatment for breast cancer is increasing, additional studies are needed to determine optimal immunization regimens in this group of patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01833520
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    Paper (German National Licenses)
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