The field of Primary Immune Deficiency Diseases PIDD continues to grow rapidly, with recognition of over 200 different disease-associated genes. To keep pace with these discoveries, the NIH has supported a number of mechanisms to accelerate and expand PIDD research, funding individual grants, contracts, and cooperative agreements. The NIAID has recognized that a number of additional resources are required to advance this work, including data collection, physician education, and access to rare resources and first proposed an RFA for this work in 2003. To answer this need, a group of experienced PIDD investigators formed the U. S. Immunodeficiency Network (USIDNET), in collaboration with the Immune Deficiency Foundation (IDF). This group was awarded the first contract, (2003-2010) and subsequently, as a competitive continuation the USIDNET was awarded a cooperative agreement for this work, receiving funds for 2010-2015. The present application is for the continuation of this work, under RFA-AI-13-054, Resources to Assist Investigations in Primary Immunodeficiency Diseases. To continue and accelerate this momentum, the USIDNET investigators propose to carry forward this initiative, with innovative plans to extend and accelerate this work. The goals of USIDNET are to maintain, improve and extend the needed PIDD resources, including (1) Extend the scope, enrollment and utilization of the secure, web-based, consented USIDNET Registry of individuals with all forms of PIDD, to collect longitudinal data, develop additional PIDD-specific forms, support observational studies, stimulate research collaborations, engage the patient community directly and further define patient outcomes using Quality of Life indicators; (2) Maintain and expand a repository of cells and DNA to aid in the research of PIDD and find ways to make accessible other rare materials and (3) Provide intensive training and educational opportunities for dissemination of information about these diseases, and encourage collaborative PIDD research at all levels.

Public Health Relevance

One of the unique endeavors of the NIH has been to foster innovative research on human Primary Immune Deficiency Diseases (PIDD). These diseases are challenging to diagnose and treat, but research into their underlying gene defects and immunologic pathways extends far beyond the disorders themselves, offering critical and indispensable information for understanding the normal human immune system. The study of these diseases leads to pioneering translational applications, such as gene therapy and identification of targets to treat inflammatory and autoimmune conditions.

National Institute of Health (NIH)
National Institute of Allergy and Infectious Diseases (NIAID)
Resource-Related Research Projects--Cooperative Agreements (U24)
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Special Emphasis Panel (ZAI1)
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Voulgaropoulou, Frosso
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Immune Deficiency Foundation
United States
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Mayor, Paul C; Eng, Kevin H; Singel, Kelly L et al. (2018) Cancer in primary immunodeficiency diseases: Cancer incidence in the United States Immune Deficiency Network Registry. J Allergy Clin Immunol 141:1028-1035
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