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Examining the Use of ICD-9 Diagnosis Codes for Primary Immune Deficiency Diseases in New York State

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Abstract

Purpose

To use International Classification of Disease Codes (ICD-9) codes to investigate primary immune deficiency (PID) in New York State.

Methods

We investigated the diagnosis of Primary Immune Deficiency (PID) in New York State (NYS) using the Statewide Planning and Research Cooperative System (SPARCS) database, a comprehensive data reporting system that collects ICD-9 codes for each patient hospitalized in NYS.

Results

From 2000–2004 there were 13,539,358 hospitalizations for 4,777,295 patients; of these, 2,361 patients (0.05 %) were diagnosed with one or more of the ICD-9 codes for PID. Antibody defects were the most common diagnoses made. The PID population had significantly more Caucasians, and fewer African American or Hispanic subjects compared to the general population. Subjects with PID codes were younger, had longer hospitalizations, were less likely to have Medicare and more likely to have Medicaid or Blue Cross insurance. Most hospitalizations were due to respiratory and infectious diseases. Most patients resided in the most populous counties, Kings, New York and Queens, but the distribution of home zip codes was not proportional to county populations.

Conclusions

These data provide useful information on incidence and complications of selected PID diagnoses in one large state.

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Abbreviations

PID:

Primary immune deficiency diseases

NYS:

New York State

SPARCS:

Statewide Planning and Research Cooperative System

XLA:

X-linked agammaglobulinemia

CVID:

Common Variable Immune Deficiency

HIV:

Human Immune Deficiency Virus

CGD:

Chronic Granulomatous Disease

SAS:

Statistical Analysis Software

SCID:

Severe Combined Immune Deficiency

WAS:

Wiskott Aldrich Syndrome

References

  1. Notarangelo LD, Fischer A, Geha RS, Casanova JL, Chapel H, Conley ME, et al. Primary immunodeficiencies: 2009 update. J Allergy Clin Immunol. 2009;124:1161–78.

    Article  PubMed  CAS  Google Scholar 

  2. Bruton OC. Agammaglobulinemia. Pediatrics. 1952;9:722–8.

    PubMed  CAS  Google Scholar 

  3. Resnick ES, Moshier EL, Godbold JH, Cunningham-Rundles C. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood. 2012;119:1650–7.

    Article  PubMed  CAS  Google Scholar 

  4. Bellanti JA, Immunology IV. Clinical applications in health and disease. Bethesda: Saunders; 2012.

    Google Scholar 

  5. Joshi AY, Iyer VN, Hagan JB, St Sauver JL, Boyce TG. Incidence and temporal trends of primary immunodeficiency: a population-based cohort study. Mayo Clin Proc. 2009;84:16–22.

    Article  PubMed  Google Scholar 

  6. Stiehm ER, Ochs H, Winkelstein JA. Immunologic disorders in infants and children. 5th ed. Philadelphia: Elsevier Saunders; 2004.

    Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  8. Samarghitean C, Ortutay C, Vihinen M. Systematic classification of primary immunodeficiencies based on clinical, pathological, and laboratory parameters. J Immunol. 2009;183:7569–75.

    Article  PubMed  CAS  Google Scholar 

  9. Yarmohammadi H, Estrella L, Doucette J, Cunningham-Rundles C. Recognizing primary immune deficiency in clinical practice. Clin Vaccine Immunol. 2006;13:329–32.

    Article  PubMed  CAS  Google Scholar 

  10. Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immunol. 1999;92:34–48.

    Article  PubMed  CAS  Google Scholar 

  11. Fasano MB, Sullivan KE, Sarpong SB, Wood RA, Jones SM, Johns CJ, et al. Sarcoidosis and common variable immunodeficiency. Report of 8 cases and review of the literature. Medicine (Baltimore). 1996;75:251–61.

    Article  CAS  Google Scholar 

  12. Mechanic LJ, Dikman S, Cunningham-Rundles C. Granulomatous disease in common variable immunodeficiency. Ann Intern Med. 1997;127:613–7.

    PubMed  CAS  Google Scholar 

  13. Thickett KM, Kumararatne DS, Banerjee AK, Dudley R, Stableforth DE. Common variable immune deficiency: respiratory manifestations, pulmonary function and high-resolution CT scan findings. QJM. 2002;95:655–62.

    Article  PubMed  CAS  Google Scholar 

  14. Chapel H, Lucas M, Lee M, Bjorkander J, Webster D, Grimbacher B, et al. Common variable immunodeficiency disorders: division into distinct clinical phenotypes. Blood. 2008;112:277–86.

    Article  PubMed  CAS  Google Scholar 

  15. Urschel S, Kayikci L, Wintergerst U, Notheis G, Jansson A, Belohradsky BH. Common variable immunodeficiency disorders in children: delayed diagnosis despite typical clinical presentation. J Pediatr. 2009;154:888–94.

    Article  PubMed  Google Scholar 

  16. Grimbacher B, Holland SM, Gallin JI, Greenberg F, Hill SC, Malech HL, et al. Hyper-IgE syndrome with recurrent infections–an autosomal dominant multisystem disorder. N Engl J Med. 1999;340:692–702.

    Article  PubMed  CAS  Google Scholar 

  17. Winkelstein JA, Marino MC, Ochs H, Fuleihan R, Scholl PR, Geha R, et al. The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients. Medicine (Baltimore). 2003;82:373–84.

    Article  CAS  Google Scholar 

  18. Johnston DT, Mehaffey G, Thomas J, Young Jr KR, Wiener H, Li J, et al. Increased frequency of HLA-B44 in recurrent sinopulmonary infections (RESPI). Clin Immunol. 2006;119:346–50.

    Article  PubMed  CAS  Google Scholar 

  19. Winkelstein JA, Marino MC, Lederman HM, Jones SM, Sullivan K, Burks AW, et al. X-linked agammaglobulinemia: report on a United States registry of 201 patients. Medicine (Baltimore). 2006;85:193–202.

    Article  Google Scholar 

  20. Abuzakouk M, Feighery C. Primary immunodeficiency disorders in the Republic of Ireland: first report of the national registry in children and adults. J Clin Immunol. 2005;25:73–7.

    Article  PubMed  CAS  Google Scholar 

  21. Al-Herz W. Primary immunodeficiency disorders in Kuwait: first report from Kuwait National Primary Immunodeficiency Registry (2004–2006). J Clin Immunol. 2008;28:186–93.

    Article  PubMed  Google Scholar 

  22. Carneiro-Sampaio MM, Carbonare SB, Rozentraub RB, de Araujo MN, Riberiro MA, Porto MH. Frequency of selective IgA deficiency among Brazilian blood donors and healthy pregnant women. Allergol Immunopathol (Madr). 1989;17:213–6.

    CAS  Google Scholar 

  23. Gathmann B, Binder N, Ehl S, Kindle G. The European internet-based patient and research database for primary immunodeficiencies: update 2011. Clin Exp Immunol. 2011;167:479–91.

    Article  Google Scholar 

  24. Knerr V, Grimbacher B. Primary immunodeficiency registries. Curr Opin Allergy Clin Immunol. 2007;7:475–80.

    Article  PubMed  Google Scholar 

  25. Leiva LE, Zelazco M, Oleastro M, Carneiro-Sampaio M, Condino-Neto A, Costa-Carvalho BT, et al. Primary immunodeficiency diseases in Latin America: the second report of the LAGID registry. J Clin Immunol. 2007;27:101–8.

    Article  PubMed  Google Scholar 

  26. Plebani A, Soresina A, Rondelli R, Amato GM, Azzari C, Cardinale F, et al. Clinical, immunological, and molecular analysis in a large cohort of patients with X-linked agammaglobulinemia: an Italian multicenter study. Clin Immunol. 2002;104:221–30.

    Article  PubMed  CAS  Google Scholar 

  27. Rezaei N, Aghamohammadi A, Moin M, Pourpak Z, Movahedi M, Gharagozlou M, et al. Frequency and clinical manifestations of patients with primary immunodeficiency disorders in Iran: update from the Iranian Primary Immunodeficiency Registry. J Clin Immunol. 2006;26:519–32.

    Article  PubMed  Google Scholar 

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

    Article  CAS  Google Scholar 

  29. Waltenburg R, Kobrynski L, Reyes M, Bowen S, Khoury MJ. Primary immunodeficiency diseases: practice among primary care providers and awareness among the general public, United States, 2008. Genet Med. 2010;12:792–800.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  31. SPARCS Database. New York, 2009: SPARCS Database. Available from: http://www.health.state.ny.us/statistics/sparcs/.

  32. Perry NW. Empire State Roads. New York Highways Website. http://www.empirestateroads.com/index.html

  33. State & County QuickFacts. 2010. Available from: http://quickfacts.census.gov/qfd/states/36000.html

  34. Gennery AR. Immunological aspects of 22q11.2 deletion syndrome. Cell Mol Life Sci. 2012;69:17–27.

    Article  PubMed  CAS  Google Scholar 

  35. Cunningham-Rundles C, Sidi P, Estrella L, Doucette J. Identifying undiagnosed primary immunodeficiency diseases in minority subjects by using computer sorting of diagnosis codes. J Allergy Clin Immunol. 2004;113:747–55.

    Article  PubMed  Google Scholar 

  36. Derose KP, Gresenz CR, Ringel JS. Understanding disparities in health care access–and reducing them–through a focus on public health. Health Aff (Millwood). 2011;30:1844–51.

    Article  Google Scholar 

  37. Mehra A, Sidi P, Doucette J, Estrella L, Rouvelas H, Cunningham-Rundles C. Subspecialty evaluation of chronically ill hospitalized patients with suspected immune defects. Ann Allergy Asthma Immunol. 2007;99:143–50.

    Article  PubMed  Google Scholar 

  38. Estrella L, Foley ME, Cunningham-Rundles C. X-linked agammaglobulinemia in a 10-year-old child: a case study. J Am Acad Nurse Pract. 2007;19:205–11.

    Article  PubMed  Google Scholar 

  39. Griffith LM, Cowan MJ, Kohn DB, Notarangelo LD, Puck JM, Schultz KR, et al. Allogeneic hematopoietic cell transplantation for primary immune deficiency diseases: current status and critical needs. J Allergy Clin Immunol. 2008;122:1087–96.

    Article  PubMed  CAS  Google Scholar 

  40. Chan K, Puck JM. Development of population-based newborn screening for severe combined immunodeficiency. J Allergy Clin Immunol. 2005;115:391–8.

    Article  PubMed  Google Scholar 

  41. Yee A, De Ravin SS, Elliott E, Ziegler JB. Severe combined immunodeficiency: a national surveillance study. Pediatr Allergy Immunol. 2008;19:298–302.

    Article  PubMed  Google Scholar 

  42. Begue RE, Perrin K. Reduction in gastroenteritis with the use of pentavalent rotavirus vaccine in a primary practice. Pediatrics. 2010;126:e40–5.

    Article  PubMed  Google Scholar 

  43. Sircar KD, Bancroft E, Nguyen DM, Mascola L. Hospitalization of paediatric patients for methicillin-resistant Staphylococcus aureus skin and soft-tissue infection, 1998–2006. Epidemiol Infect. 2010;138:677–82.

    Article  PubMed  CAS  Google Scholar 

  44. Jain VD, Moghbeli N, Webb G, Srinivas SK, Elovitz MA, Pare E. Pregnancy in women with congenital heart disease: the impact of a systemic right ventricle. Congenit Heart Dis. 2011;6:147–56.

    Article  PubMed  Google Scholar 

  45. Amari W, Zeringue AL, McDonald JR, Caplan L, Eisen SA, Ranganathan P. Risk of non-melanoma skin cancer in a national cohort of veterans with rheumatoid arthritis. Rheumatology (Oxford) 2011.

  46. Jinjuvadia K, Kwan W, Fontana RJ. Searching for a needle in a haystack: use of ICD-9-CM codes in drug-induced liver injury. Am J Gastroenterol. 2007;102:2437–43.

    Article  PubMed  Google Scholar 

  47. Fillit H, Hess G, Hill J, Bonnet P, Toso C. IV immunoglobulin is associated with a reduced risk of Alzheimer disease and related disorders. Neurology. 2009;73:180–5.

    Article  PubMed  CAS  Google Scholar 

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Acknowledgments

This work was supported by grants from the National Institutes of Health, AI 101093, AI-467320, AI-48693, NIAID Contract 03–22, and the David S Gottesman Immunology Chair and Baxter Healthcare.

Conflicts of Interest

E.S.R, P.B., and P.S. report no conflicts of interest.

C.C.R. reports previous/current grant funding from the N.I.H, Baxter Pharmaceuticals, and Octapharma as well as previous consulting fees/honoraria from Grifols, Merck, and Baxter.

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Authors and Affiliations

  1. Mount Sinai School of Medicine, Immunology Institute, New York, NY, USA

    Elena S. Resnick, Priyanka Bhatt, Peter Sidi & Charlotte Cunningham-Rundles

  2. David S. Gottesman Professor of Immunology, Mount-Sinai Medical Center, 1425 Madison Avenue, New-York, NY, 10029, USA

    Charlotte Cunningham-Rundles

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  1. Elena S. Resnick
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  2. Priyanka Bhatt
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  4. Charlotte Cunningham-Rundles
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Correspondence to Elena S. Resnick.

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Resnick, E.S., Bhatt, P., Sidi, P. et al. Examining the Use of ICD-9 Diagnosis Codes for Primary Immune Deficiency Diseases in New York State. J Clin Immunol 33, 40–48 (2013). https://doi.org/10.1007/s10875-012-9773-1

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  • DOI: https://doi.org/10.1007/s10875-012-9773-1

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