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.

Agency
National Institute of Health (NIH)
Institute
National Institute of Allergy and Infectious Diseases (NIAID)
Type
Resource-Related Research Projects--Cooperative Agreements (U24)
Project #
5U24AI086037-10
Application #
9653968
Study Section
Special Emphasis Panel (ZAI1)
Program Officer
Voulgaropoulou, Frosso
Project Start
2010-04-01
Project End
2020-03-31
Budget Start
2019-04-01
Budget End
2020-03-31
Support Year
10
Fiscal Year
2019
Total Cost
Indirect Cost
Name
Immune Deficiency Foundation
Department
Type
DUNS #
607938610
City
Towson
State
MD
Country
United States
Zip Code
21204
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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
Ho, Hsi-En; Byun, Minji; Cunningham-Rundles, Charlotte (2018) Disseminated Cutaneous Warts in X-Linked Hyper IgM Syndrome. J Clin Immunol 38:454-456
Ruffner, Melanie A; USIDNET Body Weight Group; Sullivan, Kathleen E (2018) Complications Associated with Underweight Primary Immunodeficiency Patients: Prevalence and Associations Within the USIDNET Registry. J Clin Immunol 38:283-293
Smith, Tukisa D; Cunningham-Rundles, Charlotte (2018) Detection of anti-glutamic acid decarboxylase antibodies in immunoglobulin products. J Allergy Clin Immunol Pract 6:260-261
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Feuille, Elizabeth J; Anooshiravani, Niloofar; Sullivan, Kathleen E et al. (2018) Autoimmune Cytopenias and Associated Conditions in CVID: a Report From the USIDNET Registry. J Clin Immunol 38:28-34
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Picard, Capucine; Bobby Gaspar, H; Al-Herz, Waleed et al. (2018) International Union of Immunological Societies: 2017 Primary Immunodeficiency Diseases Committee Report on Inborn Errors of Immunity. J Clin Immunol 38:96-128
Farmer, Jocelyn R; Ong, Mei-Sing; Barmettler, Sara et al. (2017) Common Variable Immunodeficiency Non-Infectious Disease Endotypes Redefined Using Unbiased Network Clustering in Large Electronic Datasets. Front Immunol 8:1740

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