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Erschienen in:

01.05.2014 | Key Review Article

ICON: The Early Diagnosis of Congenital Immunodeficiencies

verfasst von: John Routes, Mario Abinun, Waleed Al-Herz, Jacinta Bustamante, Antonio Condino-Neto, Maria Teresa De La Morena, Amos Etzioni, Eleonora Gambineri, Elie Haddad, Lisa Kobrynski, Francoise Le Deist, Shigeaki Nonoyama, Joao Bosco Oliveira, Elena Perez, Capucine Picard, Nima Rezaei, John Sleasman, Kathleen E. Sullivan, Troy Torgerson

Erschienen in: Journal of Clinical Immunology | Ausgabe 4/2014

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Abstract

Primary immunodeficiencies are intrinsic defects in the immune system that result in a predisposition to infection and are frequently accompanied by a propensity to autoimmunity and/or immunedysregulation. Primary immunodeficiencies can be divided into innate immunodeficiencies, phagocytic deficiencies, complement deficiencies, disorders of T cells and B cells (combined immunodeficiencies), antibody deficiencies and immunodeficiencies associated with syndromes. Diseases of immune dysregulation and autoinflammatory disorder are many times also included although the immunodeficiency in these disorders are often secondary to the autoimmunity or immune dysregulation and/or secondary immunosuppression used to control these disorders. Congenital primary immunodeficiencies typically manifest early in life although delayed onset are increasingly recognized. The early diagnosis of congenital immunodeficiencies is essential for optimal management and improved outcomes. In this International Consensus (ICON) document, we provide the salient features of the most common congenital immunodeficiencies.

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Titel: ICON: The Early Diagnosis of Congenital Immunodeficiencies
verfasst von: John Routes
Mario Abinun
Waleed Al-Herz
Jacinta Bustamante
Antonio Condino-Neto
Maria Teresa De La Morena
Amos Etzioni
Eleonora Gambineri
Elie Haddad
Lisa Kobrynski
Francoise Le Deist
Shigeaki Nonoyama
Joao Bosco Oliveira
Elena Perez
Capucine Picard
Nima Rezaei
John Sleasman
Kathleen E. Sullivan
Troy Torgerson
Publikationsdatum: 01.05.2014
Verlag: Springer US
Erschienen in: Journal of Clinical Immunology / Ausgabe 4/2014
Print ISSN: 0271-9142
Elektronische ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-014-0003-x

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ICON: The Early Diagnosis of Congenital Immunodeficiencies

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