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ICON: the early diagnosis of congenital immunodeficiencies.

Publication ,  Journal Article
Routes, J; Abinun, M; Al-Herz, W; Bustamante, J; Condino-Neto, A; De La Morena, MT; Etzioni, A; Gambineri, E; Haddad, E; Kobrynski, L; Perez, E ...
Published in: J Clin Immunol
May 2014

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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Published In

J Clin Immunol

DOI

EISSN

1573-2592

Publication Date

May 2014

Volume

34

Issue

4

Start / End Page

398 / 424

Location

Netherlands

Related Subject Headings

  • Tumor Necrosis Factor Receptor-Associated Peptides and Proteins
  • Toll-Like Receptors
  • T-Lymphocytes
  • Signal Transduction
  • Opportunistic Infections
  • Neonatal Screening
  • Mutation
  • Infant, Newborn
  • Immunology
  • Immunologic Factors
 

Citation

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Routes, J., Abinun, M., Al-Herz, W., Bustamante, J., Condino-Neto, A., De La Morena, M. T., … Torgerson, T. (2014). ICON: the early diagnosis of congenital immunodeficiencies. J Clin Immunol, 34(4), 398–424. https://doi.org/10.1007/s10875-014-0003-x
Routes, John, Mario Abinun, Waleed Al-Herz, Jacinta Bustamante, Antonio Condino-Neto, Maria Teresa De La Morena, Amos Etzioni, et al. “ICON: the early diagnosis of congenital immunodeficiencies.J Clin Immunol 34, no. 4 (May 2014): 398–424. https://doi.org/10.1007/s10875-014-0003-x.
Routes J, Abinun M, Al-Herz W, Bustamante J, Condino-Neto A, De La Morena MT, et al. ICON: the early diagnosis of congenital immunodeficiencies. J Clin Immunol. 2014 May;34(4):398–424.
Routes, John, et al. “ICON: the early diagnosis of congenital immunodeficiencies.J Clin Immunol, vol. 34, no. 4, May 2014, pp. 398–424. Pubmed, doi:10.1007/s10875-014-0003-x.
Routes J, Abinun M, Al-Herz W, Bustamante J, Condino-Neto A, De La Morena MT, Etzioni A, Gambineri E, Haddad E, Kobrynski L, Le Deist F, Nonoyama S, Oliveira JB, Perez E, Picard C, Rezaei N, Sleasman J, Sullivan KE, Torgerson T. ICON: the early diagnosis of congenital immunodeficiencies. J Clin Immunol. 2014 May;34(4):398–424.
Journal cover image

Published In

J Clin Immunol

DOI

EISSN

1573-2592

Publication Date

May 2014

Volume

34

Issue

4

Start / End Page

398 / 424

Location

Netherlands

Related Subject Headings

  • Tumor Necrosis Factor Receptor-Associated Peptides and Proteins
  • Toll-Like Receptors
  • T-Lymphocytes
  • Signal Transduction
  • Opportunistic Infections
  • Neonatal Screening
  • Mutation
  • Infant, Newborn
  • Immunology
  • Immunologic Factors