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Journal of Clinical Immunology

, Volume 37, Issue 3, pp 319–328 | Cite as

Infection Profile in Chronic Granulomatous Disease: a 23-Year Experience from a Tertiary Care Center in North India

  • Amit RawatEmail author
  • Pandiarajan Vignesh
  • Avinash Sharma
  • Jitendra K. Shandilya
  • Madhubala Sharma
  • Deepti Suri
  • Anju Gupta
  • Vikas Gautam
  • Pallab Ray
  • Shivaprakash M. Rudramurthy
  • Arunaloke Chakrabarti
  • Kohsuke Imai
  • Shigeaki Nonoyama
  • Osamu Ohara
  • Yu L. Lau
  • Surjit Singh
Original Article

Abstract

Purpose

Chronic granulomatous disease (CGD) is an inherited phagocytic disorder characterized by recurrent infections with usually catalase-positive organisms. Infections in CGD from developing countries are expected to be different from those in the Western countries. We report the profile of infections in children diagnosed with CGD from a tertiary care center in North India.

Methodology

Case records of children diagnosed with CGD at Pediatric Immunodeficiency Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India, from August 1993 to April 2016 (23 years) were analyzed.

Results

Thirty-eight children were diagnosed to have CGD. Median follow-up of patients was 2 years (interquartile range 0.75, 6.0). Staphylococcus aureus and Pseudomonas spp. were the two most common causative bacteria isolated. Aspergillus was the most common fungus isolated. The most common organ involved was the lung (94.7%). Liver abscesses were identified in 5 patients (13.2%), and 20 (52.6%) patients had lymphadenitis. Infections with Pseudomonas spp. were high in our cohort (15.7%) compared to the other studies. Infections with some unusual organisms (e.g., Fusarium dimerium and Chryseobacterium gleum) were also seen in our cohort. Children with X-linked CGD presented earlier and also had a greater number of infections as compared to autosomal recessive CGD.

Conclusions

Various socioeconomic factors coupled with the lack of awareness and paucity of readily available diagnostic facilities for primary immunodeficiencies accounted for a late clinical presentation with severe infections and increased mortality (28.9%) in our cohort. However, mortality was similar in X-linked and autosomal recessive CGD as was the number of fungal infections. The incidence of infections and mortality was significantly lower after initiation of antibacterial and antifungal prophylaxis.

Keywords

Chronic granulomatous disease North India infections bacteria fungi mortality 

Abbreviations

XL

X-linked

AR

Autosomal recessive

MALDI-TOF

Matrix-assisted laser desorption ionization time-of-flight mass spectrometry

SI

Stimulation index

Notes

Acknowledgements

Authors thankfully acknowledge India Council of Medical Research, New Delhi, India, and Department of Health Research, Ministry of Health and Family Welfare, Government of India, New Delhi, India, for funding vide Grant No. GIA/48/2014-DHR and the Foundation for Primary Immunodeficiencies (FPID), USA. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

Compliance with Ethical Standards

Funding

Grant No. GIA/48/2014-DHR to one of co-authors (SS) from Indian Council of Medical Research, New Delhi, and Department of Health Research, Ministry of Health and Family Welfare, Government of India, New Delhi.

Conflict of Interest

The authors declare that they have no conflict of interest.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from all individual participants included in the study.

Supplementary material

10875_2017_382_MOESM1_ESM.docx (21 kb)
Table S1 (DOCX 20 kb)
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Table S2 (DOCX 14 kb)
10875_2017_382_MOESM3_ESM.docx (15 kb)
Table S3 (DOCX 15 kb)
10875_2017_382_MOESM4_ESM.docx (12 kb)
Table S4 (DOCX 12 kb)

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Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  • Amit Rawat
    • 1
    Email author
  • Pandiarajan Vignesh
    • 1
  • Avinash Sharma
    • 1
  • Jitendra K. Shandilya
    • 1
  • Madhubala Sharma
    • 1
  • Deepti Suri
    • 1
  • Anju Gupta
    • 1
  • Vikas Gautam
    • 2
  • Pallab Ray
    • 2
  • Shivaprakash M. Rudramurthy
    • 2
  • Arunaloke Chakrabarti
    • 2
  • Kohsuke Imai
    • 3
  • Shigeaki Nonoyama
    • 3
  • Osamu Ohara
    • 4
  • Yu L. Lau
    • 5
  • Surjit Singh
    • 1
  1. 1.Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Department of PediatricsPostgraduate Institute of Medical Education and ResearchChandigarghIndia
  2. 2.Department of Medical MicrobiologyPostgraduate Institute of Medical Education and ResearchChandigarghIndia
  3. 3.Department of PediatricsNational Defense Medical CollegeTokorozawaJapan
  4. 4.Kazusa DNA Research InstituteKisarazuJapan
  5. 5.Department of Pediatrics and Adolescent Medicine, Queen Mary Hospital, LKS Faculty of MedicineThe University of Hong KongHong KongChina

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