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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Annals of hematology 21 (1970), S. 273-282 
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Die Wirkung von Asparaginase auf die zelluläre Immunität wurde an menschlichen Blutlymphozyten untersucht, die in dem Kultur-System inkubiert wurden. Die Ergebnisse zeigten, daß Asparaginase die Blastogenese der Lymphozyten inhibiert, sowohl bei PHA- oder PKW-Stimulation, als auch in der gemischten Lymphozytenkultur. Dies geschah sowohl bei Zufügen des Enzyms direkt zum Kulturmedium, als auch bei i.v.-Verabreichung des Enzyms beim Menschen. In letzterem Fall wurden die Lymphozyten isoliert und die Kultur-Medien hinzugefügt. Gekreuzte Experimente zeigten, daß a) Lymphozyten von Asparaginase-injizierten Personen, die in ein PHA-Medium gebracht wurden, welchem das Plasma (20%) von nicht-injizierten Personen zugefügt worden war, eine normale Blastogenese durchmachten; und b) Lymphozyten von nicht-injizierten Personen, die einem PHA-Kulturmedium mit Plasma (20%) von Asparaginase-injizierten Personen zugesetzt wurden, eine Inhibition in ihrer blastogenetischen Transformation zeigen. Die Wirkung der Asparaginase auf die Zell-Vorläufer der humoralen Immunität wurde an den lymphatischen Follikeln der Milz von ausgewachsenen Kaninchen, denen diese Enzym-Präparation i.v. verabreicht worden war, untersucht. Es wurde gezeigt, daß die Asparaginase einerseits eine Abnahme der Zelldichte und-größe bis zum vollkommenen Verschwinden des lymphatischen Ringwalls verursachte, andererseits aber eine Zunahme des Zellwachstums und Vergrößerung der Keimzentren zusammen mit einer Vermehrung der Plasmazellen auslöst. Diese Ergebnisse zusammengenommen zeigen, daß Asparaginase eine depressorische Wirkung auf die lymphozyten-abhängige Immunität ausübt, nicht aber auf die Plasmazelluläre, d. h. humorale Immunität.
    Notes: Summary The effect of asparaginase on cellular immunity was investigated on human peripheral blood lymphocytes incubated in the culture system. Results showed that asparaginase inhibits lymphocyte blastogenesis, both when stimulated with PHA or PKW, and in the mixed lymphocyte cultures. This happened when the enzyme was added directly to the culture medium, as well as when the enzyme was administered i.v. in humans, and then the blood lymphocytes were removed and put into the PHA-culture medium added to the lymphocyte donor's plasma. Crossed experiments showed that: a) lymphocytes from asparaginase injected subjects which were set up in a PHA-culture-medium to which plasma (20%) from non-injected subjects had been added, undergo a normal amount of blastogenesis; and b) lymphocytes from non-injected subjects, set up in a PHA-culture-medium with plasma (20%) from asparaginase injected subjects show an inhibition in their blastic transformation. The effect of asparaginase on the cell precursors of humoral immunity was investigated on the spleen lymphatic follicles of adult rabbits i.v. injected with that enzyme preparation. It appeared that asparaginase causes, on the one hand, a decrease in cellular density and size until there is a complete disappearance of the lymphatic collars, and, on the other hand, an increase in the cellular growth and size of the germinal centers, accompanied by an increase of the plasma-cell frequency. These results, taken as a whole, show that asparaginase is a depressing agent of the lymphocyte-depending immunity, but not of the plasma cell-system, i.e., the system which provides the humoral immunity.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 133 (1980), S. 101-106 
    ISSN: 1432-1076
    Keywords: IgA deficiency ; Atopy ; Infection ; Chromosome defects
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Fifty children with IgA deficiency were followed for 1 to 4 years from 1975 to 1978. Thirty-five had complete deficiency of serum IgA (〈2.5 IU/ml) and 15 partial deficiency (serum IgA below the 10th centile for age). Patients with another associated immunodeficiency, such as ataxia-telangiectasia, were not included. Most children with complete deficiency of IgA had recurrent respiratory and/or gastrointestinal infections, about half with onset in the first year of life, while partial deficiency of IgA has probably little if any importance for anti-infectious immunity but is important in the pathogenesis of atopy. Atopic diseases were frequent in both groups. Chromosomal abnormalities were found in 2 patients: trisomy 21 in one and in the other a ring chromosome 18. No important defects in cellular immunity were detected but some isolated, borderline abnormalities were often present.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 129 (1978), S. 221-229 
    ISSN: 1432-1076
    Keywords: Atopy ; Immunodeficiency ; Immunoglobulin E ; IgA deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Despite impressive recent advances in the understanding of the chemical and cellular bases of the reaginic response, the pathogenesis of atopic diseases still remains a matter of speculation. The frequent finding of atopic diseases in some primary immunodeficiencies such as selective IgA deficiency and the Wiskott-Aldrich syndrome offers a unique opportunity for studying the immune mechanisms underlying the genesis of atopy. Recent studies in subjects with selective IgA deficiency have challenged the well known hypothesis that atopy is the result of defective “immune exclusion” by the secretory immune system. A number of immunological features found in the primary immunodeficiencies associated with atopic disorders suggest that defective homeostatic mechanisms regulating reaginic responses may play a major role in the pathogenesis of atopy. A thorough analysis of these disease combinations may help to generate new working hypotheses concerning the immune pathogenesis of atopic diseases.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 136 (1981), S. 229-230 
    ISSN: 1432-1076
    Keywords: Contergan ; Embryopathy ; Limb defects ; Malformations ; Thalidomide
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 140 (1983), S. 5-12 
    ISSN: 1432-1076
    Keywords: Proteus syndrome ; Macrodactyly ; Hemihypertrophy ; Pigmented nevi ; Skull anomalies ; Lipomas
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Four boys are described with partial gigantism of the hands and/or feet, pigmented nevi, hemihypertrophy, subcutaneous hamartomatous tumors and macrocephaly, and/or other skull anomalies. Three of these patients showed an accelerated growth in their first years of life. Two suffered from cystiform pulmonary abnormalities. The children showed normal mental development with the exception of one with traumatic brain damage. Parental consanguinity was not disclosed. As a result of a review of the literature, we can say that these cases do not conform to any well defined entity and would appear to represent a ‘new’ syndrome to be categorized under congenital hamartomatous disorders. The mode of inheritance of the undoubtedly genetically determined syndrome is yet not clearly understood. We propose the term Proteus syndrome for this ‘new’ syndrome.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 149 (1989), S. 9-10 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Down's syndrome ; Immunodeficiency ; HBsAg ; Thyroglobulin antibodies ; Autoimmunity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The relationship between the presence of hepatitis B surface antigen (HBsAg) and antibodies to human thyroglobulin (HTgAb) has been studied in 110 subjects with Down's syndrome (DS) from 4 months to 50 years of age and in 122 controls carefully matched for sex, age and socio-environmental conditions. The overall percentage of HBsAg carriers was 22.7 in DS and 6.6 in controls and that of HTgAb-positive subjects was 41.8 in DS and 19.7 in controls. In DS the frequency of HTgAb-positive subjects was very high, even in the youngest age groups in which the percentage of HBsAg carriers was relatively low; the latter thereafter showed a marked increase with age. A positive association between the presence of HBsAg and HTgAb was found only in the oldest age group of DS subjects. It is thus concluded that in DS the high frequency of HTgAb cannot be attributed to chronic hepatitis B virus infection. On the contrary, the presence of HTgAb might well represent an early “marker” of immunodeficiency and increased susceptibility to infection with hepatitis B virus.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 138 (1982), S. 288-292 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Immunodeficiency disorders can be classified on clinical grounds into two broad groups according to whether all features are the result of the immune defect (immunodeficiency syndromes) or whether many, even prominent, features cannot be explained by the immune defect (syndromes with immunodeficiency). X-linked agammaglobulinemia and X-linked chronic granulomatous disease are paradigmatic examples of immunodeficiency syndromes. Despite some overlap (for instance extra-immune symptoms, although minor, are present in several variants of severe combined immunodeficiency and chronic granulomatous disease) immunodeficiency syndromes and syndromes with immunodeficiency are easily distinguishable. Together with the pathogenetic classification of the WHO, the present approach to a clinical classification amplifies the operational concept of immunodeficiency also from a therapeutic point of view.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 144 (1985), S. 9-12 
    ISSN: 1432-1076
    Keywords: DiGeorge anomaly ; Immunodeficiency ; Errors of morphogenesis ; Malformation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The heuristic concept of “inborn errors of metabolism” was introduced more then 70 years ago and by analogy has prompted the more recent introduction of the term “inborn errors of immunity”. It is now well recognized that many inborn errors of immunity can be considered inborn errors of metabolism. Typically, many forms of severe combined immunodeficiency result from adenosine deaminase deficiency, i.e., an inborn error of purine metabolism. On the other hand, errors of immunity are often associated with “errors of morphogenesis”, resulting from an intrinsically abnormal developmental process (malformation), a secondary or extrinsic interference with originally normal development (disruption), or an abnormal organization of cells into tissues (dysplasia). Twenty years after the original description, the DiGeorge anomaly should be considered an inborn error of morphogenesis and immunity due either to disruption or less frequently to malformation. In other immunodeficiencies, such as ataxia telangiectasia, the morphologic and immunologic errors result from a dyshistogenesis, i.e, dysplasia. Also, true malformation syndromes, such as Down's syndrome, are consistently associated with immunodeficiency.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 145 (1986), S. 325-325 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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