2. Academic Validation
  3. Primary immunodeficiencies: 2009 update

Primary immunodeficiencies: 2009 update

  • J Allergy Clin Immunol. 2009 Dec;124(6):1161-78. doi: 10.1016/j.jaci.2009.10.013.
International Union of Immunological Societies Expert Committee on Primary Immunodeficiencies 1,  Luigi D Notarangelo,  Alain Fischer,  Raif S Geha,  Jean-Laurent Casanova,  Helen Chapel,  Mary Ellen Conley,  Charlotte Cunningham-Rundles,  Amos Etzioni,  Lennart Hammartröm,  Shigeaki Nonoyama,  Hans D Ochs,  Jennifer Puck,  Chaim Roifman,  Reinhard Seger,  Josiah Wedgwood
Affiliations

Affiliation

  • 1 Division of Immunology, Children's Hospital Boston and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA. [email protected]
PMID: 20004777 DOI: 10.1016/j.jaci.2009.10.013
Abstract

More than 50 years after Ogdeon Bruton's discovery of congenital agammaglobulinemia, human primary immunodeficiencies (PIDs) continue to unravel novel molecular and cellular mechanisms that govern development and function of the human immune system. This report provides the updated classification of PIDs that has been compiled by the International Union of Immunological Societies Expert Committee on Primary Immunodeficiencies after its biannual meeting in Dublin, Ireland, in June 2009. Since the appearance of the last classification in 2007, novel forms of PID have been discovered, and additional pathophysiology mechanisms that account for PID in human beings have been unraveled. Careful analysis and prompt recognition of these disorders is essential to prompt effective forms of treatment and thus to improve survival and quality of life in patients affected with PIDs.

Figures