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  • 1
    Electronic Resource
    Electronic Resource
    IgG Subclass Deficiency in Patients with Down's Syndrome and Aberrant Hepatitis B Vaccine Response (1988)
    Oxford, UK : Blackwell Publishing Ltd
    Scandinavian journal of immunology 28 (1988), S. 0 
    Details
    ISSN: 1365-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Seventeen adult patients with Down's syndrome (DS) and 19 adult healthy references were vaccinated with a hepatitis B vaccine in order to study the IgG subclass response. An enzyme-linked immunosorbent assay (ELISA) using monoclonal antibodies specific for IgG subclasses was employed. In spite of normal levels of total IgG1 and normal or even high levels of IgG3 in the DS patients, a significantly lower IgG1 response to the vaccine was observed in trisomic patients than in the references.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-3083.1988.tb01477.x
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  • 2
    Electronic Resource
    Electronic Resource
    Drugs and depression of lymphocyte blastogenesis in children (1973)
    Springer
    European journal of pediatrics 115 (1973), S. 131-139 
    Details
    ISSN: 1432-1076
    Keywords: Blastogenesis-inhibition ; Drug induced blastogenesis depression ; Non antiblastic drugs and lymphocyte blastogenesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Zweck dieser Arbeit war, die Einwirkung einiger grundsätzlich verschiedener Arzneimittel (Phenilbutazon: Ph, Thiamphenicol: Th, Sulfamethopyrazine: Sl, Diazepam: Dz und des Antiblasticum Cis-Platinum II diammino dichloride: PDD) auf die Blastogenese der Lymphocyten zu prüfen. Es wurde a) der Effekt der Zugabe der einzelnen Mittel zur PHA-Kultur von Lymphocyten normaler Kinder ausgewertet und b) die PHA-Blastogenese der Lymphocyten von Kindern, die sich in Behandlung mit den verschiedenen Arzneien (mit der Ausnahme vom PDD) betrafen, erforscht. Ph, Th, Sl und PDD (dieses in den in vitro-Erfahrungen) haben eine deutliche Hemmung auf die Blastogenese ausgeübt. Dz übt keine (oder nur eine sehr spärliche) Blastogenese-Depression aus. c) Wenn man dieselben Arzneimittel dem MLC-System zufügt, kommen ähnliche Ergebnisse zum Vorschein. Bei der Anwendung im Tripan-Blau-Test von Arzneimengen, die fähig sind, die PHA-Blastogenesewerte auf 50% der Kontrollwerte herabzusetzen, haben weder das Ph, das Th und das Sl noch das PDD eine cytotoxische Wirkung erwiesen.
    Notes: Abstract Data are presented which demonstrate an inhibitory effect of non anti-blastic drugs with different therapeutical indications: Phenilbutazone (Ph), Thiamphenicol (Th) and Sulfamethopyrazine (Sl) on PHA and mixed stimulation of normal children peripheral blood lymphocytes, when added in vitro. Similarly a depressed response to PHA was observed for the lymphocytes of patients treated with the same drugs. When tested on the same experimental system, Diazepam (Dz) failed to show a significant inhibition. A first trial with a new antiblastic (cis-platinum II diammino dichloride) has also been performed. The “in vitro” addition of this antiblastic strongly depressed lymphocyte blastogenesis. The viability Trypan-Blue Exclusion Test was performed for all the drugs with inhibiting properties. No citotoxic effect could be seen when the 50% PHA-blastogenesis inhibiting dose was employed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00440538
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  • 3
    Electronic Resource
    Electronic Resource
    Selective IgA deficiency: Clinical and immunological evaluation of 50 pediatric patients (1980)
    Springer
    European journal of pediatrics 133 (1980), S. 101-106 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00441577
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  • 4
    Electronic Resource
    Electronic Resource
    The thalidomide disaster briefly revisited (1981)
    Springer
    European journal of pediatrics 136 (1981), S. 229-230 
    Details
    ISSN: 1432-1076
    Keywords: Contergan ; Embryopathy ; Limb defects ; Malformations ; Thalidomide
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442988
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  • 5
    Electronic Resource
    Electronic Resource
    The proteus syndrome (1983)
    Springer
    European journal of pediatrics 140 (1983), S. 5-12 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00661895
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  • 6
    Electronic Resource
    Electronic Resource
    Errors of morphogenesis and inborn errors of immunity 20 years after the discovery of DiGeorge anomaly (1985)
    Springer
    European journal of pediatrics 144 (1985), S. 9-12 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00491916
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  • 7
    Electronic Resource
    Electronic Resource
    VMA-negative, MIBG scan-positive neuroblastoma in children (1986)
    Springer
    European journal of pediatrics 145 (1986), S. 325-325 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00439418
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  • 8
    Electronic Resource
    Electronic Resource
    Atopy as a minimal immunodeficiency? (1978)
    Springer
    European journal of pediatrics 129 (1978), S. 221-229 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00441353
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  • 9
    Electronic Resource
    Electronic Resource
    Further and new details on the Proteus syndrome (1984)
    Springer
    European journal of pediatrics 143 (1984), S. 71-73 
    Details
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442754
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  • 10
    Electronic Resource
    Electronic Resource
    Cell-mediated cytotoxicity in down syndrome: impairment of allogeneic mixed lymphocyte reaction, NK and NK-like activities (1988)
    Springer
    European journal of pediatrics 148 (1988), S. 53-57 
    Details
    ISSN: 1432-1076
    Keywords: Down syndrome ; Immunodeficiency ; Mixed lymphocyte reaction ; NK activity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Peripheral blood mononuclear cells (PBMC) from 16 non-institutionalized patients with Down syndrome (DS) were studied with various monoclonal antibodies and analysed for natural killer (NK), and NK-like activity. Lymphocyte proliferation and cytotoxic T-lymphocyte (CTL) cytotoxicity generated in mixed lymphocyte culture (MLC) were also evaluated in 11 DS patients. Phenotypic characterization of PBMC from DS subjects confirms our previous findings of high numbers of CD8+ lymphocytes and HNK-1+, and CD16+ cells. Lymphocyte proliferation and CTL cytotoxicity generated in MLC were low or absent in most patients. NK activity was low in almost all DS patients, while NK-like cytotoxicity generated in MLC was normal in the majority and did not correlate with NK activity from unstimulated PBMC.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441815
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