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Dem menschlichen Abwehrsystem können unterschiedliche Aufgaben zugeordnet werden, z.B.:

  1. a)

    Abwehr von Infektionserregern (Bakterien, Viren, Pilze, Parasiten),

  2. b)

    Toleranz gegenüber körpereigenen Strukturen,

  3. c)

    Regulation von pathologischen Immunreaktionen gegen Allergene,

  4. d)

    Elimination von Tumorzellen.

Im Normalfall können all diese Funktionen in vollem Umfang wahrgenommen werden. Aus einem Verlust der unter a) erwähnten Funktion resultiert eine abnorme Anfälligkeit gegenüber Infektionen, beim Verlust der unter b) genannten Funktion kommt es zu Autoimmunerkrankungen. Werden Immunreaktionen, insbesondere IgE-vermittelte, gegenüber Allergenen der Umwelt (c) nicht kontrolliert, können sich allergische Erkrankungen entwickeln. Das Fehlen von d) trägt mit einer Reihe anderer Faktoren zur Entstehung von Malignomen bei.

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Literatur

  • Buckley RH (2004) Molecular defects in human severe combined immunodeficiency and approaches to immune reconstitution. Annu Rev Immunol 22: 625–655

    Article  PubMed  CAS  Google Scholar 

  • Burns S, Cory GO, Vainchencker W, Thrasher AJ (2004) Mechanisms of WASp-mediated hematologic and immunologic disease. Blood 104: 3454–3462

    Article  PubMed  CAS  Google Scholar 

  • Bux J, Behrens G, Jaeger G, Welte K (1998) Diagnosis and clinical course of autoimmune neutropenia in infancy: analysis of 240 cases. Blood 91: 181–186

    PubMed  CAS  Google Scholar 

  • Bux J, Behrens G, Jaeger G, Welte K (1998) Diagnosis and clinical course of autoimmune neutropenia om omfamcy: analysis of 240 cases. Blood 91: 181–186

    PubMed  CAS  Google Scholar 

  • Casanova JL, Abel L (2004) The human model: A genetic dissection of immunity to infection in natural conditions. Nat Rev Immunol 4: 55–66

    Article  PubMed  CAS  Google Scholar 

  • Chan B, Wara D, Bastian J et al. (2005) Long-term efficacy of enzyme replacement therapy for adenosine deaminase (ADA)-deficient severe combined immunodeficiency (CSCID). Clin Immunol 117: 133–143

    Article  PubMed  CAS  Google Scholar 

  • Chinen J, Puck LM (2004) Successes and risks of gene therapy in primary immunodeficiencies. J Allergy Clin Immunol 113: 595–603

    Article  PubMed  CAS  Google Scholar 

  • Conley ME, Saragoussi D, Notarangelo L et al. (2003) An international study examining therapeutic options used in treatment of Wiskott-Aldrich syndrome. Clin Immunol 109: 272–277

    Article  PubMed  CAS  Google Scholar 

  • Cunningham-Rundles C, Bodian C, Ochs HD, Martin S, Reiter-Wong M, Zhuo Z (2001) Long-term low-dose IL-2 enhances immune function in common variable immunodeficiency. Clin Immunol 100: 181–190

    Article  PubMed  CAS  Google Scholar 

  • Cunningham-Rundles C, Bodian C, Ochs HD, Martin S, Reiter-Wong M, Zhuo Z (2001) Long-term low-dose IL-2 enhances immune function in common variable immunodeficiency. Clin Immunol 100: 181–190

    Article  PubMed  CAS  Google Scholar 

  • Donadieu J, Michel G, Merlin E et al. (2005) Hematopoetic stem cell transplantation for Shwachman-Diamond syndrom: experience of the French neutropenia registry. Bone Marrow Transpl 36: 787–792

    Article  CAS  Google Scholar 

  • Farkas H, Harmat G, Fust G, Vaga L, Visy B (2002) Clinical management of hereditary angio-oedema in children. Pediatr Allergy Immunol 13: 153–161

    Article  PubMed  Google Scholar 

  • Fijen CA, Kuijper EJ, Drogari-Apiranthitou M, Leeuwen Y van, Daha MR, Dankert J (1998) Protection against meningococcal serogroup ACYW disease in complement-deficient individuals vaccinated with the tetravalent meningococcal capsular polysaccharide vaccine. Clin Exp Immunol 114: 362–369

    Article  PubMed  CAS  Google Scholar 

  • Fischer A (2004) Human primary immunodeficiency diseases: an perspective. Nat Immunol 5: 23–30

    Article  PubMed  CAS  Google Scholar 

  • Hong R (2001) The DiGeorge anomaly. Crit Rev Allergy Immunol 20: 43–60

    Article  CAS  Google Scholar 

  • Horwitz ME (2000) Stem-cell transplantation for inherited immunodeficiency disorders. Pediatr Clin North Am 47: 1371–1387

    Article  PubMed  CAS  Google Scholar 

  • Jawad AF, McDonal-McGinn DM, Zackai E, Sullivan KE (2001) Immunologic features of chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome). J Pediatr 139: 715–725

    Article  PubMed  CAS  Google Scholar 

  • Knutsen AP, Wall DA (2000) Umbilical cord blood transplantation in severe T-cell immunodeficiency disorders: two-year experience. J Clin Immunol 20: 466–476

    Article  PubMed  CAS  Google Scholar 

  • Lekstrom-Himes JA, Gallin JI (2000) Immunodeficiency diseases caused by defects in phagocytes. N Engl J Med 343: 1703–1714

    Article  PubMed  CAS  Google Scholar 

  • Markert ML, Alexieff MJ, Li J et al. (2004) Postnatal thymus transplantation with immunosuppression as treatment for DiGeorge syndrome. Blood 104: 2574–2581

    Article  PubMed  CAS  Google Scholar 

  • Notarangelo L, Casanova JL, Conley ME et al. for the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee (2006) Classification Committee Meeting in Budapest, 2005. J Allergy Clin Immunol 117: 677–687

    Google Scholar 

  • O’Neil KM (2000) Complement deficiency. Clin Rev Allergy Immunol 19: 83–108

    Article  PubMed  CAS  Google Scholar 

  • Requeiro JR, Porras O, Lavin M, Gatti RA (2000) Ataxia Teleangiectasia — a primary immunodeficiency revisited. Immunol Allergy Clin N Am 20: 177–205

    Article  Google Scholar 

  • Renner Ed, Belohradsky BH, Grimbacher B (2002) Hyper-IgE-Syndrom. Monatsschr Kinderheilkd 150: 1168–1179

    Article  Google Scholar 

  • Rocha V, Wagner JE Jr, Sobocinski KA et al. (2000) Graft-versus-host disease in children who have received a cord-blood or bone marrow transplant from an HLA-identical sibling. Eurocord and International Bone Marrow Transplant Registry Working Committee on Alternative Donor and Stem Cell Sources. N Engl J Med 342: 1846–1854

    Article  PubMed  CAS  Google Scholar 

  • Rosenzweig SD, Holland SM (2004) Phagocyte immunodeficiences and their infections. J Allergy Clin Immunol 113: 620–626

    Article  PubMed  CAS  Google Scholar 

  • Segal BH, Holland SM (2000) Primary phagocytic disorders of childhood. Pediatr Clin North Am 2000 47: 1311–1338

    Article  CAS  Google Scholar 

  • Wahn U, Seger R, Wahn V, Holländer G (Hrsg) (2005) Pädiatrische Allergologie und Immunologie, 4. Aufl. Elsevier, München

    Google Scholar 

  • Wahn V, Belohradsky BH, Borte M et al. (im Druck) Früherkennung von Patienten mit primären Immundefekten, Behandlung von Patienten mit Antikörper-Mangel — Ergebnisse einer interdisziplinären Konferenz. pädiatr prax

    Google Scholar 

  • Zeidler C, Welte K (2005) Kongenitale Neutropenien. In: Wahn U, Seger R, Wahn V, Holländer G (Hrsg) Pädiatrische Allergologie und Immunologie, 4. Aufl. Elsevier, München

    Google Scholar 

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Wahn, V. (2007). Immundefekte. In: Reinhardt, D. (eds) Therapie der Krankheiten im Kindes- und Jugendalter. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-71899-4_42

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  • DOI: https://doi.org/10.1007/978-3-540-71899-4_42

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