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  • 1
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: HIV ; Children ; Intravenous immunoglobulins ; Zidovudine ; CMV
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract From 1988 to 1991 the long-term efficacy of a combined therapy with a polyvalent immunoglobulin/cytomegalovirus (CMV) hyperimmunoglobulin, oral low dose zidovudine, oral cotrimoxazole or inhaled pentamidine was investigated in three groups of human immunodeficiency virus (HIV)-infected children. Group 1A consisted of three perinatally infected children with a CD4 cell decrease of 〉400, cells/μl per year. Group 1B were 17 perinatally infected children with a CD4 cell decrease of 〈400 cells/μl per year. Group 2 comprised eight haemophilic children infected by clotting factors. Despite combined therapy none of group 1A survived longer than 12 months showing a rapid loss of CD4 cell counts, progressive encephalopathy, wasting syndrome and severe bacterial, fungal and CMV reactivation. Under pure intravenous immunoglobulin (IVIG) therapy severe bacterial infections were seen in 1 of 12 children in group 1B. The majority of these patients showed increases or stabilisation of length and weight percentiles. In this group low dose zidovudine therapy was of benefit in HIV-associated neurological symptoms. Nevertheless combined therapy could not prevent further deterioration of CD4 cell counts. In group 2 severe bacterial infections were not seen under IVIG therapy. In this group a temporary increase (6 months) of CD4 cell counts under IVIG/zidovudine combined therapy occurred.Pneumocystis carinii pneumonia (PCP) prophylaxis with oral cotrimoxazole or inhaled pentamidine successfully prevented PCP in all three groups. Under CMV hyperimmunoglobulins (n=22), ten out of ten patients did not acquire primary CMV infection, whereas CMV reactivations mainly located in the CNS could not be prevented in 5 of 12 patients. Our findings indicate that this combined therapy showed remarkable differences in therapeutic efficacy in children with different modes of HIV progression. These modes must be considered for correct timing, dosage and evaluation of therapeutic measures.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Key words Shwachman-Diamond syndrome ; Bone marrow transplantation ; Leukaemia ; Pancytopenia ; AbbreviationsBMT bone marrow transplantation ; GvHD graft-versus-host disease ; SDS Shwachman-Diamond syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Shwachman-Diamond syndrome (SDS) is an autosomal recessive disorder characterised by exocrine pancreas insufficiency, metaphyseal dysostosis and bone marrow dysfunction. Recurrent severe bacterial infections and susceptibility to leukaemia are the major causes of morbidity and mortality occurring preferentially in patients with pancytopenia and features of myelodysplasia. Here we report a patient with SDS leading to recurrent bacterial infections and a deteriorating condition since early infancy. Extensive investigations disclosed severe pancytopenia, myelodysplasia and a clonal cytogenetic abnormality, inv(14)(q11q32), as risk factors of leukaemic transformation. He therefore underwent allogeneic geno-identical bone marrow transplantation which resulted in correction of all haematological and immunological abnormalities within an 18-month follow up period. Conclusion Bone marrow transplantation may be considered early as a valuable treatment option especially in high risk Schwachman-Diamond syndrome patients anticipating malignant transformation, life-threatening severe infections or further organ damage.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 154 (1995), S. 865-865 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1525-1314
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Geosciences
    Notes: Abstract P–T conditions, mineral isograds, the relation of the latter to foliation planes and kinematic indicators are used to elucidate the tectonic nature and evolution of a shear zone in an orogen exhumed from mid-crustal depths in western Turkey. Furthermore, we discuss whether simple monometamorphic fabrics of rock units from different nappes result from one single orogeny or are related to different orogenies. Metasedimentary rocks from the Çine and Selimiye nappes at the southern rim of the Anatolide belt of western Turkey record different metamorphic evolutions. The Eocene Selimiye shear zone separates both nappes. Metasedimentary rocks from the Çine nappe underneath the Selimiye shear zone record maximum P–T conditions of about 7 kbar and 〉550 °C. Metasedimentary rocks from the overlying Selimiye nappe have maximum P–T conditions of 4 kbar and c. 525 °C near the base of the nappe. Kinematic indicators in both nappes are related to movement on the Selimiye shear zone and consistently show a top-S shear sense. Metamorphic grade in the Selimiye nappe decreases structurally upwards as indicated by mineral isograds defining the garnet-chlorite zone at the base, the chloritoid-biotite zone and the biotite-chlorite zone at the top of the nappe. The mineral isograds in the Selimiye nappe run parallel to the regional SR foliation, parallel the Selimiye shear zone and indicate that the Selimiye shear zone formed during this prograde greenschist to lower amphibolite facies metamorphic event but remained active after the peak of metamorphism. 40Ar/39Ar mica ages and the tectonometamorphic relationship with the Eocene Cyclades–Menderes thrust, which occurs above the Selimiye nappe in the study area, suggests an Eocene age of metamorphism in the Selimiye nappe.Metasedimentary rocks of the Çine nappe 20–30 km north of the Selimiye shear zone record maximum P–T conditions of 8–11 kbar and 600–650  °C. An age of about 550 Ma is indicated for amphibolite facies metamorphism and associated top-N shear in the orthogneiss of the Çine nappe. Our study shows that simple monophase tectonometamorphic fabrics do not always indicate a simple orogenic development of a nappe stack. Preservation in some areas and complete overprinting of those fabrics in other areas apparently occur very heterogeneously.
    Type of Medium: Electronic Resource
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