Top

Published in:

01-05-2014 | Original Research

USIDNET: A Strategy to Build a Community of Clinical Immunologists

Authors: Kathleen E. Sullivan, Jennifer M. Puck, Luigi D. Notarangelo, Ramsay Fuleihan, Tara Caulder, Connie Wang, Marcia Boyle, Charlotte Cunningham-Rundles

Published in: Journal of Clinical Immunology | Issue 4/2014

Abstract

Objectives

Information about patients with primary immune deficiencies can be scarce because of the rarity of the disorders. Individual centers rarely have sufficient patients to educate trainees and garner collective wisdom. Registries for many diseases have proven their worth by providing essential information on disease spectrum, treatments and natural history. This study describes the construction and use of a registry for patients with primary immune deficiencies and other efforts to improve knowledge and care for affected patients and their families.

Methods

Registry demographics and data were extracted using proprietary reporting tools. Educational efforts and cell repository data were collected from centralized source material.

Results

The USIDNET Registry contains 3,459 patients, with common variable immune deficiency being the most represented. Pilot studies identified strengths and weaknesses of the data. Visiting Professor and Visiting Scholar Programs have been successful, encouraging trainees at all levels to pursue a career in Immunology.

Conclusions

USIDNET’s comprehensive and integrated approach provides resources that strengthen the field of primary immune deficiencies, as shown by utilization by 312 distinct sites or individuals. The reach of USIDNET’s efforts is extended through the educational resources.

Available only for authorised users

Winkelstein JA, Marino MC, Johnston RB, Boyle J, Curnutte J, Gallin JI, et al. Chronic granulomatous disease. Report on a national registry of 368 patients. Medicine. 2000;79:155–69.PubMedCrossRef

A controlled trial of interferon gamma to prevent infection in chronic granulomatous disease. The International Chronic Granulomatous Disease Cooperative Study Group. N Engl J Med. 1991;324(8):509–16.

Gallin JI, Alling DW, Malech HL, Wesley R, Koziol D, Marciano B, et al. Itraconazole to prevent fungal infections in chronic granulomatous disease. N Engl J Med. 2003;348(24):2416–22.PubMedCrossRef

Levy J, Espanol-Boren T, Thomas C, Fischer A, Tovo P, Bordigoni P, et al. Clinical spectrum of X-linked hyper-IgM syndrome. J Pediatr. 1997;131(1 Pt 1):47–54.PubMedCrossRef

Guzman D, Veit D, Knerr V, Kindle G, Gathmann B, Eades-Perner AM, et al. The ESID Online Database network. Bioinformatics. 2007;23(5):654–5.PubMedCrossRef

Ishimura M, Takada H, Doi T, Imai K, Sasahara Y, Kanegane H, et al. Nationwide survey of patients with primary immunodeficiency diseases in Japan. J Clin Immunol. 2011;31(6):968–76.PubMedCrossRef

Kirkpatrick P, Riminton S. Primary immunodeficiency diseases in Australia and New Zealand. J Clin Immunol. 2007;27(5):517–24.PubMedCrossRef

Nozaki T, Takada H, Ishimura M, Ihara K, Imai K, Morio T, et al. Endocrine complications in primary immunodeficiency diseases in Japan. Clin Endocrinol. 2012;77(4):628–34.CrossRef

Mir Saeid Ghazi B, Aghamohammadi A, Kouhi A, Farhoudi A, Moin M, Rezaei N, et al. Mortality in primary immunodeficient patients, registered in Iranian primary immunodeficiency registry. Iran J Allergy Asthma Immunol. 2004;3(1):31–6.PubMed

10.

Aghamohammadi A, Moein M, Farhoudi A, Pourpak Z, Rezaei N, Abolmaali K, et al. Primary immunodeficiency in Iran: first report of the National Registry of PID in Children and Adults. J Clin Immunol. 2002;22(6):375–80.PubMedCrossRef

11.

Shah BR, Drozda J, Peterson ED. Leveraging observational registries to inform comparative effectiveness research. Am Heart J. 2010;160(1):8–15.PubMedCrossRef

12.

Frobert O, Lagerqvist B, Olivecrona GK, Omerovic E, Gudnason T, Maeng M, et al. Thrombus aspiration during ST-segment elevation myocardial infarction. N Engl J Med. 2013;369(17):1587–97.PubMedCrossRef

13.

Pollard C, Bailey KA, Petitte T, Baus A, Swim M, Hendryx M. Electronic patient registries improve diabetes care and clinical outcomes in rural community health centers. J Rural Health Off J Am Rural Health Assoc Natl Rural Health Care Assoc. 2009;25(1):77–84.CrossRef

14.

Fonarow GC, Albert NM, Curtis AB, Stough WG, Gheorghiade M, Heywood JT, et al. Improving evidence-based care for heart failure in outpatient cardiology practices: primary results of the Registry to Improve the Use of Evidence-Based Heart Failure Therapies in the Outpatient Setting (IMPROVE HF). Circulation. 2010;122(6):585–96.PubMedCrossRef

15.

Holmes JA, Carpenter WR, Wu Y, Hendrix LH, Peacock S, Massing M, et al. Impact of distance to a urologist on early diagnosis of prostate cancer among black and white patients. J Urol. 2012;187(3):883–8.PubMedCrossRef

16.

Rogler LE, Kosmyna B, Moskowitz D, Bebawee R, Rahimzadeh J, Kutchko K, et al. Small RNAs derived from lncRNA RNase MRP have gene-silencing activity relevant to human cartilage-hair hypoplasia. Hum Mol Genet. 2014;23(2):368–82.PubMedCrossRef

17.

Al-Herz W, Bousfiha A, Casanova JL, Chapel H, Conley ME, Cunningham-Rundles C, et al. Primary immunodeficiency diseases: an update on the classification from the international union of immunological societies expert committee for primary immunodeficiency. Front Immunol. 2011;2:54.PubMedCentralPubMedCrossRef

18.

Bousfiha AA, Jeddane L, Ailal F, Al Herz W, Conley ME, Cunningham-Rundles C, et al. A phenotypic approach for IUIS PID classification and diagnosis: guidelines for clinicians at the bedside. J Clin Immunol. 2013;33(6):1078–87.PubMedCrossRef

19.

Teutsch SM, Fielding JE. Comparative effectiveness—looking under the lamppost. JAMA. 2011;305(21):2225–6.PubMedCrossRef

20.

Wehr C, Kivioja T, Schmitt C, Ferry B, Witte T, Eren E, et al. The EUROclass trial: defining subgroups in common variable immunodeficiency. Blood. 2008;111(1):77–85.PubMedCrossRef

21.

Rakhmanov M, Keller B, Gutenberger S, Foerster C, Hoenig M, Driessen G, et al. Circulating CD21low B cells in common variable immunodeficiency resemble tissue homing, innate-like B cells. Proc Natl Acad Sci U S A. 2009;106(32):13451–6.PubMedCentralPubMedCrossRef

22.

Kappelman MD, Crandall WV, Colletti RB, Goudie A, Leibowitz IH, Duffy L, et al. Short pediatric Crohn’s disease activity index for quality improvement and observational research. Inflamm Bowel Dis. 2011;17(1):112–7.PubMedCentralPubMedCrossRef

23.

Crandall WV, Margolis PA, Kappelman MD, King EC, Pratt JM, Boyle BM, et al. Improved outcomes in a quality improvement collaborative for pediatric inflammatory bowel disease. Pediatrics. 2012;129(4):e1030–41.PubMedCentralPubMedCrossRef

24.

Tuchman L, Schwartz M. Health outcomes associated with transition from pediatric to adult cystic fibrosis care. Pediatrics. 2013;132(5):847–53.PubMedCrossRef

25.

Boyle JM, Buckley RH. Population prevalence of diagnosed primary immunodeficiency diseases in the United States. J Clin Immunol. 2007;27(5):497–502.PubMedCrossRef

26.

Modell V, Gee B, Lewis DB, Orange JS, Roifman CM, Routes JM, et al. Global study of primary immunodeficiency diseases (PI)—diagnosis, treatment, and economic impact: an updated report from the Jeffrey Modell Foundation. Immunol Res. 2011;51(1):61–70.PubMedCrossRef

Title: USIDNET: A Strategy to Build a Community of Clinical Immunologists
Authors: Kathleen E. Sullivan
Jennifer M. Puck
Luigi D. Notarangelo
Ramsay Fuleihan
Tara Caulder
Connie Wang
Marcia Boyle
Charlotte Cunningham-Rundles
Publication date: 01-05-2014
Publisher: Springer US
Published in: Journal of Clinical Immunology / Issue 4/2014
Print ISSN: 0271-9142
Electronic ISSN: 1573-2592
DOI: https://doi.org/10.1007/s10875-014-0028-1

ACC 2024 Congress Coverage

Heart Failure Video

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

USIDNET: A Strategy to Build a Community of Clinical Immunologists

Abstract

Objectives

Methods

Results

Conclusions

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Objectives

Methods

Results

Conclusions

Please log in to get access to this content

Other articles of this Issue 4/2014

Chronic Granulomatous Disease in Morocco: Genetic, Immunological, and Clinical Features of 12 Patients from 10 Kindreds

The Case for Mandatory Newborn Screening for Severe Combined Immunodeficiency (SCID)

Erratum to: Efficacy and Safety of IgPro20, a Subcutaneous Immunoglobulin, in Japanese Patients with Primary Immunodeficiency Diseases

Correlating Interleukin-12 Stimulated Interferon-γ Production and the Absence of Ectodermal Dysplasia and Anhidrosis (EDA) in Patients with Mutations in NF-κB Essential Modulator (NEMO)

First Report on the Moroccan Registry of Primary Immunodeficiencies: 15 Years of Experience (1998–2012)

Retrospective Analysis of TREC Based Newborn Screening Results and Clinical Phenotypes in Infants with the 22q11 Deletion Syndrome

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page