Skip to main content

Advertisement

Log in

USIDNET: A Strategy to Build a Community of Clinical Immunologists

  • Original Research
  • Published:
Journal of Clinical Immunology Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. 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.

    Article  CAS  PubMed  Google Scholar 

  2. 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.

    Google Scholar 

  3. 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.

    Article  CAS  PubMed  Google Scholar 

  4. 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.

    Article  CAS  PubMed  Google Scholar 

  5. 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.

    Article  CAS  PubMed  Google Scholar 

  6. 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.

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  8. 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.

    Article  CAS  Google Scholar 

  9. 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  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed Central  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  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.

    Article  PubMed Central  PubMed  Google Scholar 

  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.

    Article  PubMed Central  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

The authors thank the patients and many physicians whose time and effort made the tools provided by USIDNET possible. This work was supported by U24 AI086037, Baxter, Talecris, CSL Behring, Sigma Tau, and AxelaCare.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Kathleen E. Sullivan.

Electronic supplementary material

Below is the link to the electronic supplementary material.

ESM 1

(DOCX 106 kb)

Appendix

Appendix

Medical Centers

Advocate Hope Children’s Hospital

Ann & Robert H. Lurie Children’s Hospital of Chicago

Boston Children’s Hospital

Children’s Hospital of Pennsylvania

CHU Sainte-Justine Montreal, Canada

Cincinnati Children’s Hospital Medical Center

Cincinnati Children’s Hospital Medical Center

Cleveland Clinic

Duke University Medical Center

Emory University

Levine Children’s Hospital

Louisiana State University

Mattel Children’s Hospital Univ. of CA, LA Mayo Clinic

Medical College of Wisconsin

Miami Children’s Hospital

Mt. Sinai School of Medicine

National Institute of Allergy and Infectious Diseases

Rainbow Babies and Children’s Hospital Ohio

Seattle Children’s Hospital

Texas Children’s Hospital

University of California, San Francisco

University of Colorado, Denver

University of Iowa

University Texas (UT) Southwestern Medical Center

University of Utah

Wake Forest University

Wayne State University

Women and Children’s Hospital of Buffalo

Yale University

Children’s Hospital Orange County

Joe DiMaggio Children’s Hospital

John Hopkins Hospital

Massachusetts’s General Hospital

NIH – NHGRI

University of Wisconsin (New 2013)

Hackensack University Medical Center (New 2013)

Enrolling Institutions Under USIDNET IIRB

Allergy Associate of the Palm Beaches

Daniel Suez Allergy, Asthma, & Immunology

Midwest Immunology Clinic

United States Immunodeficiency Network (USIDNET)

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sullivan, K.E., Puck, J.M., Notarangelo, L.D. et al. USIDNET: A Strategy to Build a Community of Clinical Immunologists. J Clin Immunol 34, 428–435 (2014). https://doi.org/10.1007/s10875-014-0028-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10875-014-0028-1

Keywords

Navigation