ICON: the early diagnosis of congenital immunodeficiencies.

Journal Article (Review)

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.

Full Text

Duke Authors

Cited Authors

  • 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

Published Date

  • May 2014

Published In

Volume / Issue

  • 34 / 4

Start / End Page

  • 398 - 424

PubMed ID

  • 24619621

Electronic International Standard Serial Number (EISSN)

  • 1573-2592

International Standard Serial Number (ISSN)

  • 0271-9142

Digital Object Identifier (DOI)

  • 10.1007/s10875-014-0003-x

Language

  • eng