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Ten warning signs of primary immunodeficiency: a new paradigm is needed for the 21st century

Authors


  • Preferred citation: Arkwright, P.D. & A.R. Gennery. 2011. Ten warning signs of primary immunodeficiency: a new paradigm is needed for the 21st century. In “The Year in Human and Medical Genetics: Inborn Errors of Immunity I.” Jean-Laurent Casanova, Mary Ellen Conley & Luigi Notarangelo, Eds. Ann. N.Y. Acad. Sci.1238: 7–14.

Peter D. Arkwright, Senior Lecturer in Paediatric Immunology, Department of Paediatric Allergy and Immunology, Royal Manchester Children's Hospital, Oxford Road, Manchester M13 9WL. peter.arkwright@nhs.net

Abstract

The 10 warning signs of primary immunodeficiency are being promoted as a screening tool for use by both the general public and physicians. A recent study, however, shows that except for family history, need for intravenous antibiotics and failure to thrive, the 10 warning signs are not a useful screen of primary immunodeficiency diseases (PIDs). Over the last few decades, there has been a revolution in our understanding of PID. The 10 warning signs do not take into account the fact that PIDs now include diseases that present with sporadic infections, autoimmunity, autoinflammation, and malignancy. This review focuses on the advances in our understanding of PID, the current limitations of the 10 warning signs, and recommendations to ensure that patients with PID are diagnosed in a timely fashion in the future.

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