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

Journal of Clinical Immunology volume 37, pages 319–328 (2017)Cite this 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.

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Fig. 1
Fig. 2

Abbreviations

XL:

X-linked

AR:

Autosomal recessive

MALDI-TOF:

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

SI:

Stimulation index

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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.

Author information

Affiliations

  1. Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India

    Amit Rawat, Pandiarajan Vignesh, Avinash Sharma, Jitendra K. Shandilya, Madhubala Sharma, Deepti Suri, Anju Gupta & Surjit Singh

  2. Department of Medical Microbiology, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India

    Vikas Gautam, Pallab Ray, Shivaprakash M. Rudramurthy & Arunaloke Chakrabarti

  3. Department of Pediatrics, National Defense Medical College, Tokorozawa, Saitama, Japan

    Kohsuke Imai & Shigeaki Nonoyama

  4. Kazusa DNA Research Institute, Kisarazu, Chiba, Japan

    Osamu Ohara

  5. Department of Pediatrics and Adolescent Medicine, Queen Mary Hospital, LKS Faculty of Medicine, The University of Hong Kong, Hong Kong, Hong Kong Special Administrative Region, China

    Yu L. Lau

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  1. Amit Rawat
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Corresponding author

Correspondence to Amit Rawat.

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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.

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

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Rawat, A., Vignesh, P., Sharma, A. et al. Infection Profile in Chronic Granulomatous Disease: a 23-Year Experience from a Tertiary Care Center in North India. J Clin Immunol 37, 319–328 (2017). https://doi.org/10.1007/s10875-017-0382-x

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  • DOI: https://doi.org/10.1007/s10875-017-0382-x

Keywords

  • Chronic granulomatous disease
  • North India
  • infections
  • bacteria
  • fungi
  • mortality