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Journal of Clinical Immunology

, Volume 34, Issue 4, pp 428–435 | Cite as

USIDNET: A Strategy to Build a Community of Clinical Immunologists

  • Kathleen E. SullivanEmail author
  • Jennifer M. Puck
  • Luigi D. Notarangelo
  • Ramsay Fuleihan
  • Tara Caulder
  • Connie Wang
  • Marcia Boyle
  • Charlotte Cunningham-Rundles
Original Research

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.

Keywords

Primary immune deficiency registry comparative effectiveness immunoglobulin 

Notes

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.

Supplementary material

10875_2014_28_MOESM1_ESM.docx (106 kb)
ESM 1 (DOCX 106 kb)

References

  1. 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.PubMedCrossRefGoogle Scholar
  2. 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. 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.PubMedCrossRefGoogle Scholar
  4. 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.PubMedCrossRefGoogle Scholar
  5. 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.PubMedCrossRefGoogle Scholar
  6. 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.PubMedCrossRefGoogle Scholar
  7. 7.
    Kirkpatrick P, Riminton S. Primary immunodeficiency diseases in Australia and New Zealand. J Clin Immunol. 2007;27(5):517–24.PubMedCrossRefGoogle Scholar
  8. 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.CrossRefGoogle Scholar
  9. 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.PubMedGoogle Scholar
  10. 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.PubMedCrossRefGoogle Scholar
  11. 11.
    Shah BR, Drozda J, Peterson ED. Leveraging observational registries to inform comparative effectiveness research. Am Heart J. 2010;160(1):8–15.PubMedCrossRefGoogle Scholar
  12. 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.PubMedCrossRefGoogle Scholar
  13. 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.CrossRefGoogle Scholar
  14. 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.PubMedCrossRefGoogle Scholar
  15. 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.PubMedCrossRefGoogle Scholar
  16. 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.PubMedCrossRefGoogle Scholar
  17. 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.PubMedCentralPubMedCrossRefGoogle Scholar
  18. 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.PubMedCrossRefGoogle Scholar
  19. 19.
    Teutsch SM, Fielding JE. Comparative effectiveness—looking under the lamppost. JAMA. 2011;305(21):2225–6.PubMedCrossRefGoogle Scholar
  20. 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.PubMedCrossRefGoogle Scholar
  21. 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.PubMedCentralPubMedCrossRefGoogle Scholar
  22. 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.PubMedCentralPubMedCrossRefGoogle Scholar
  23. 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.PubMedCentralPubMedCrossRefGoogle Scholar
  24. 24.
    Tuchman L, Schwartz M. Health outcomes associated with transition from pediatric to adult cystic fibrosis care. Pediatrics. 2013;132(5):847–53.PubMedCrossRefGoogle Scholar
  25. 25.
    Boyle JM, Buckley RH. Population prevalence of diagnosed primary immunodeficiency diseases in the United States. J Clin Immunol. 2007;27(5):497–502.PubMedCrossRefGoogle Scholar
  26. 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.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  • Kathleen E. Sullivan
    • 1
    Email author
  • Jennifer M. Puck
    • 2
  • Luigi D. Notarangelo
    • 3
  • Ramsay Fuleihan
    • 4
  • Tara Caulder
    • 5
  • Connie Wang
    • 5
  • Marcia Boyle
    • 5
  • Charlotte Cunningham-Rundles
    • 6
  1. 1.Department of PediatricsThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  2. 2.Department of Pediatrics, University of California San Francisco and UCSF Benioff Children’s HospitalSan FranciscoUSA
  3. 3.Division of ImmunologyBoston Children’s HospitalBostonUSA
  4. 4.Division of Allergy & ImmunologyAnn & Robert H. Lurie Children’s Hospital of ChicagoChicagoUSA
  5. 5.The Immune Deficiency FoundationTowsonUSA
  6. 6.Departments of Medicine and Pediatrics, The Immunology InstituteMount Sinai School of MedicineNew YorkUSA

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