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  • 1
    ISSN: 1432-1076
    Keywords: Granular lymphocyte Lymphoproliferative disease of granular lymphocytes ; Epstein-Barr virus (EBV) genome ; Severe chronic active EBV infection syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We describe an 11-year-old girl who initially had mild hepatosplenomegaly and then presented with abnormal expansion of CD3-negative granular lymphocytes in peripheral blood and Epstein-Barr virus (EBV) genome in the DNA obtained from the peripheral blood mononuclear cells (PBMNC). After approximately 3 years, she developed oedema, ascites, marked hepatosplenomegaly, and pancytopenia, and showed both a profile of anti-EBV antibodies of reactivated infection and a high titre of anti-cytomegalovirus antibody. Although she was treated with antibiotics, ganciclovir, and prednisolone, she died of hepatic failure. Conclusion Careful clinical observation, periodic examination of anti-EBV antibodies, and the analysis of EBV genome from PBMNC are needed in young patients with CD3-negative lymphoproliferative disease of granular lymphocytes.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Key words     Granular lymphocyte ; Lymphoproliferative disease of granular lymphocytes ; Epstein-Barr virus (EBV) genome ; Severe chronic active EBV infection syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract      We describe an 11-year-old girl who initially had mild hepatosplenomegaly and then presented with abnormal expansion of CD3-negative granular lymphocytes in peripheral blood and Epstein-Barr virus (EBV) genome in the DNA obtained from the peripheral blood mononuclear cells (PBMNC). After approximately 3 years, she developed oedema, ascites, marked hepatosplenomegaly, and pancytopenia, and showed both a profile of anti-EBV antibodies of reactivated infection and a high titre of anti-cytomegalovirus antibody. Although she was treated with antibiotics, ganciclovir, and prednisolone, she died of hepatic failure. Conclusion     Careful clinical observation, periodic examination of anti-EBV antibodies, and the analysis of EBV genome from PBMNC are needed in young patients with CD3-negative lymphoproliferative disease of granular lymphocytes.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1912
    Keywords: Adenosine ; Phenylisopropyladenosine ; Adenosine receptors ; Negative inotropic effect ; G proteins ; Ferret ventricular myocardium
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An adenosine A1 receptor agonist R-N6-phenylisopropyladenosine (R-PIA) elicited a pronounced negative inotropic effect with the EC50 value of 0.69 μmol/1 in the presence of a β-adrenoceptor blocking agent bupranolol (0.3 μmol/1) in the isolated ferret papillary muscle. The negative inotropic effect of R-PIA was not associated with changes in cyclic AMP level. Adenosine and other A1 receptor agonists also elicited a negative inotropic effect. DPCPX (1,3-dipropyl-8-cyclopentyl xanthine) antagonized the negative inotropic effect of R-PIA in a competitive manner (pA2 value = 8.4). The inhibitory action of R-PIA was markedly attenuated in the ventricular muscle preparation isolated from ferrets pretreated with pertussis toxin that caused ADP-ribosylation of 39 kDa proteins in the membrane fraction. In the membrane fraction derived from the ferret ventricle, [3H]-DPCPX bound to a single binding site in a saturable and reversible manner with high affinity (Kd value = 1.21±0.41 nmol/l; B max = 12.8±3.02 fmol/mg protein; n = 7). The binding characteristics of [3H]-DPCPX in the rat ventricle (Kd value = 1.51 ±0.09 nmol/l; B max = 12.7±1.47 fmol/mg protein; n = 5) were similar to those in the ferret. On the other hand, the content of Go, a major pertussis toxin-sensitive G protein in the ferret heart, was much higher in the ferret than in the rat ventricle. The present results indicate that adenosine receptors may play an important role in the inhibitory regulation of ventricular contractility in the ferret in contrast to other mammalian species. The signal transduction process subsequent to agonist binding to A1 receptors including the pertussis toxin-sensitive G protein and ion channels may be responsible for the unique inhibitory action of adenosine in this species.
    Type of Medium: Electronic Resource
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