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  • 1
    ISSN: 1432-1440
    Keywords: AIDS ; Neopterin ; Stimulated monocytes ; Immunodeficiencies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An increase in total urinary neopterin was observed in 12 of 13 patients with acquired immunodeficiency syndrome (AIDS), seven of 13 patients with lymphadenopathy, one of six healthy homosexual males, seven of ten adult patients with staphylococcal pneumonia, 11 of 12 children with viral infections, four of seven children with bacterial infections, and 12 of 13 children with various immune defects. Extremely high values of total urinary neopterin and monapterin were observed in severely ill patients with AIDS and those with familial hemophagocytic lymphohistiocytosis. Neopterin excretion was normal in two AIDS patients with Kaposi's sarcoma, but without opportunistic infections at that time. On reexamination of one of these patients later on, elevated neopterin values were noted. Parallel increases in neopterin and monapterin were found, whereas biopterin was usually normal. The increase in total neopterin was mainly due to 7,8-dihydroneopterin and was accompanied by an increase in 3′-hydroxysepiapterin. Increased neopterin in urine is assumed to reflect the increase in GTP pool and GTP cyclohydrolase I activity as observed in stimulated monocytes. Thus, neopterin, as a measure of the activation of the nonspecific cellular immune system, may be used diagnostically to detect allograft rejection after transplantations and to follow-up HTLV-III positive patients.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Hyperphenylalaninemia ; Muscular hypotonia ; Deficiencies of: GTP cyclohydrolase I, neopterin, tetrahydrobiopterin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 4-year-old patient is described with hyperphenylalaninemia, severe retardation in development, severe muscular hypotonia of the trunk and hypertonia of the extremities, convulsions, and frequent episodes of hyperthermia without infections. Urinary excretion of neopterin, biopterin, pterin, isoxanthopterin, dopamine, and serotonin was very low, although the relative proportions of pterins were normal. In lumbar cerebrospinal fluid, homovanillic acid, 5-hydroxy-indoleacetic acid, neopterin and biopterin were low. Oral administration ofL-erythro tetrahydrobiopterin normalized the elevated serum phenylalanine within 4 h, serum tyrosine was increased briefly and serum alanine and glutamic acid for a longer time. Urinary dopamine and serotonin excretion were also increased. Administration of an equivalent dose ofd-erythro tetrahydroneopterin was ineffective and demonstrated that this compound is not a cofactor in vivo and cannot be transformed into an active cofactor. GTP cyclohydrolase I activity was not detectable in liver biopsies from the patient. The presence of an endogenous inhibitor in the patient's liver was excluded. This is the first case of a new variant of hyperphenylalaninemia in which the formation of dihydroneopterin triphosphate and its pterin metabolites in liver is markedly diminished. Normal activities of xanthine oxidase and sulfite oxidase were apparent since uric acid levels were normal and no increase in hypoxanthine, xanthine, andS-sulfocysteine concentrations could be observed in urine. It is concluded that the molybdenum cofactor of these enzymes may not be derived from dihydroneopterin triphosphate in man. Also, since no gross abnormalities in the patient's immune system could be found, it seems unlikely that dihydroneopterin triphosphate metabolites, such as neopterin, participate actively in immunological processes, as postulated by others. See Note added in proof.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Plasmacytoma ; Monoclonal gammopathy ; Lymph node plasmacytoma ; Surface markers ; Complement receptor subtypes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A ten year-old girl presented with increased susceptibility to infection, massive enlargement of the cervical lymph nodes, a high erythrocyte sedimentation rate and an extremely high serum IgA (peak level 66 g/l). The diagnosis of plasmacytoma was confirmed by the identification of a monoclonal immunoglobulin (IgAk) in the serum and in the plasma cells present in a cervical lymph node biopsy. Her disturbed blood coagulation and thrombelastogram, serum hyperviscosity, cryoglobulinemia, hyponatremia and constantly positive test for rheumatoid factor without evidence of rheumatic disease were attributed to the effects of the myeloma proteins. Other signs often associated with plasmacytoma (bone pain, osteolytic lesions, anemia, bone marrow failure or infiltration) were absent indicating that the plasmacytoma originated in a lymph node, which is unusual. This case is the second well-documented childhood plasmacytoma to be reported. With combined melphalan and prednisone therapy, the greatly enlarged lymph nodes regressed in size and the plasma IgA concentration fell to normal. Immunological investigations revealed a considerable reduction of blood T cells, and abnormal skin tests at the time of diagnosis indicated a disturbance of cellular immunity. There were also abnormalities in the B-cell system. The blood B lymphocytes were found to carry only complement receptors for C3b and mainly for IgA, whereas in normal individuals blood B cells bear complement receptors for both C3b and C3d and mainly surface Ig of the IgM class. In the lymph node biopsy, there were many primary follicles expressing complement receptors for both C3b and C3d among the dense plasma cell infiltrates, but germinal centers, which mainly serve to renew B cells, were completely absent. In conjunction with data from the literature, we concluded 1) that the production of normal B lymphocytes is blocked, probably due to a secondary T cell deficiency, and 2) that the majority of the residual blood and bone marrow B cells and the plasmacytoma cells are parts of the same neoplastic cell clone.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 139 (1982), S. 176-180 
    ISSN: 1432-1076
    Keywords: Chronic mucocutaneous candidiasis ; Ketoconazole ; Intermittent therapy ; Mannan ; Lymphocyte transformation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report the clinical and laboratory findings in two children with chronic mucocutaneous condidiasis (CMC) treated successfully with intermittent long-term ketoconazole therapy. Both had chronic infection of the nails, skin and mucous membranes with positive cultures for candida. Both were resistant to multiple local and systemic antifungal agents. After institution of ketoconazole therapy there was a dramatic improvement with clearing of the oral (one week), skin (two months) and nail lesions (5 months). After 8 months the drug was stopped and clinical remission persisted for 10 and 7 months respectively. Relapse of oral candidiasis was treated with a short course of ketoconazole (4–16 weeks) leading to complete healing of the lesions. Clinical improvement was not related to an amelioration in lymphocyte transformation. There was no change in the progressive deterioration of the lymphocyte responses to candida antigen which was probably due to persisting candida cell wall components (e.g. mannan).
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 0009-8981
    Keywords: GTP cyclohydrolase I ; Hyperphenylalaninemia ; Monocytes stimulation ; Neopterin triphosphate
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 42 (1995), S. 0 
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The ability of inactivated viruses, bacteria, protozoa and fungi to modulate the expression of CD14, CD49d, CD49f, CD11a (LFA-1), CD61, and CD54 (ICAM-1) molecules in unprimed bone marrow-derived mononuclear phagocytes (BMMø) was investigated by means of flow cytometry. Incubation with bacterial agents resulted in the large majority of experimental situations in enhanced expression of these macrophage surface molecules. In contrast, viruses and fungi down-regulated the expression of several adhesion receptors, especially integrins. Amplification of MHC class II expression triggered in macrophages by interferon γ was clearly inhibited by viruses, bacteria, protozoa and fungi. The findings explain earlier results showing that, under the same experimental conditions, bacterial agents are, for the most part, potent stimulators of secretory and cell-mediated macrophage activities while viruses, protozoa and fungi are poor in this respect.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 28 (1988), S. 0 
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: In a pure population of rat bone marrow-derived mononuclear phagocytes (BMMø), the expression of major histocompatibility complex (MHC) molecules and ability to manifest tumoricidal activity were simultaneously studied. Resting BMMø, which express low levels of MHC class II molecules and do not manifest tumoricidal activity, become strongly MHC class II-positive, and evolve tumoricidal activity within 24 h when incubated with macrophage-activating lymphokines (MAF) or gamma interferon (IFN-γ). In contrast, BMMø which were interacted for 24 h with heat-killed microbial agents (Corynebacterium parvum, Listeria) evolve tumoricidal activity without parallel enhancement of MHC class II expression. IFN-α,β neither induced tumoricidal activity nor enhanced MHC class II expression. Further experiments have shown that (a) the kinetics of MAF- and/or IFN-γ-induced amplification of MHC class II expression and of tumoricidal activity are different; (b) enhancement of MHC class II expression by rIFN-γ is not invariably paralleled by induction of tumoricidal activity; and (c) inhibitors of macrophage tumoricidal activity differ in their ability to affect MHC class II expression. It is concluded from these findings that in a population of pure BMMø, i.e. in the complete absence of lymphocytes, the expression of MHC molecules and induction of tumoricidal activity are independently regulated phenomena; in particular, the enhanced expression of MHC class II molecules is not a prerequisite for induction and/or manifestation of tumoricidal activity by mononuclear phagocytes.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Research in experimental medicine 176 (1979), S. 81-86 
    ISSN: 1433-8580
    Keywords: Enzyme activity ; Adenosine deaminase ; Enzymes ; Immunity ; Leukemia ; Leukemia in children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Ein Zusammenhang zwischen dem Enzym Adenosindeaminase (ADA) und Immunfunktionen ist gesichert: Patienten mit kongenitalem Mangel der ADA weisen einen schweren kombinierten Immundefekt auf. Bei Patienten, die mit immunsuppressiven Mitteln behandelt wurden, fanden wir im Gegenteil erhöhte ADA-Enzymaktivität. Zu den bisherigen kontroversen Befunden über ADA bei akuten Leukämien trägt unser Material folgendes bei: Kinder mit akuter lymphatischer Leukämie (ALL) und akuter myeloischer Leukämie (AML) in Remission weisen normale bis erhöhte ADA-Aktivität auf, solange sie unter zytostatischer Therapie stehen. Nach Heilung der Leukämie (d. h. in Dauerremission ohne Therapie) normalisieren sich die ADA-Aktivitäten. Eine Heterogeneität innerhalb der Leukämie-Gruppen ist aber nicht ausgeschlossen. — Die zelluläre Immundefizienz bei akuter Leukämie erklärt sich aus diesen Befunden nicht.
    Notes: Summary A causal relation between the enzyme, adenosine deaminase (ADA), and immune dysfunction is well known: patients with congenital inactivity of ADA invariably suffer of severe combined immunodeficiency. In contrast, we found in patients treated with immunosuppressive drugs increased ADA enzyme activity. Previous findings on ADA activity in acute leukemias are until now controversial. We found normal to increased ADA activity in children with acute lymphatic leukemia (ALL) and acute myeloid leukemia (AML) in remission as long as they were treated with cytostatic drugs. In the group of cured leukemics (in continuous remission after suspension of the therapeutic regimen) the ADA activities were normal. These findings do not exclude a heterogeneity within the leukemia group. They do not explain the signs of cellular immunodeficiencies well known in patients with acute leukemias.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Annals of hematology 31 (1975), S. 325-329 
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-0584
    Keywords: Acute idiopathic thrombocytopenic purpura ; Chronic idiopathic thrombocytopenic purpura ; Corticosteroid therapy of ITP ; Immunoglobulin therapy of ITP
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Since in a pilot study i.v. IgG was shown to induce a rapid rise of thrombocytes in children with ITP two prospective multicenter ITP studies were started: one comparing i.v. IgG with oral corticosteroids in previously untreated acute ITP, the other investigating the response to i.v. IgG in pretreated acute or chronic ITP in childhood. In this report preliminary results of both studies are summarized. i.v. IgG treatment of acute and chronic ITP is at least as effective as corticosteroid therapy but is not associated with significant side effects. At least some patients with chronic ITP may benefit from i.v. IgG. Longer observation periods are required for further analysis.
    Type of Medium: Electronic Resource
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