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Common variable immune deficiency: reviews, continued puzzles, and a new registry

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Abstract

Background

Common variable immune deficiency (CVID) is a clinically and immunologically heterogenous primary immune deficiency first described more than 50 years ago. The main features are hypogammaglobulinemia, recurrent infections, and other complications. While CVID is considered as a genetic immune defect, and several genes have been reported as leading to the CVID phenotype, one of the most puzzling features of CVID is the sporadic inheritance pattern and the relatively late onset. In most cases, no other family members have any immune defect. The mean age at diagnosis is between 25 and 45 years of age. These features suggest the interplay between either several or numerous genes with or without potential environmental factors.

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Abbreviations

CVID:

Common variable immune deficiency

ICOS:

Inducible costimulator

IDF:

Immune Deficiency Foundation

ITP:

Idiopathic thrombocytopenia purpura

TACI:

Transmembrane activator and calcium-modulator and cyclophilin-ligand interactor

USID Net:

United States Immune Deficiency Network

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Acknowledgment

This work was supported by grants from the National Institutes of Health, AI 101093, AI-467320, AI-48693 and NIAID Contract 03-22.

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Authors and Affiliations

  1. Department of Medicine, Mount Sinai Medical Center, The Mount Sinai School of Medicine, New York, NY, USA

    Charlotte Cunningham-Rundles & Adina Kay Knight

  2. Department of Pediatrics, Mount Sinai Medical Center, The Mount Sinai School of Medicine, New York, NY, USA

    Charlotte Cunningham-Rundles & Adina Kay Knight

  3. The Immunobiology Center, Mount Sinai Medical Center, The Mount Sinai School of Medicine, New York, NY, USA

    Charlotte Cunningham-Rundles & Adina Kay Knight

  4. 1450 Madison Avenue, Box 1089, New York, NY, 10029, USA

    Charlotte Cunningham-Rundles

  5. 10 Old Montgomery Highway, Suite 100, Birmingham, AL, 35209, USA

    Adina Kay Knight

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  1. Charlotte Cunningham-Rundles
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Correspondence to Charlotte Cunningham-Rundles.

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Presented at the First Robert A Good Society Symposium, St. Petersburg, FL 2006.

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Cunningham-Rundles, C., Knight, A.K. Common variable immune deficiency: reviews, continued puzzles, and a new registry. Immunol Res 38, 78–86 (2007). https://doi.org/10.1007/s12026-007-0024-0

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