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Liver Abscess in Chronic Granulomatous Disease—Two Decades of Experience from a Tertiary Care Centre in North-West India

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Abstract

Purpose

Most of the literature on liver abscess in chronic granulomatous disease (CGD) emanates from developed countries. Data from developing countries are scarce. In this study, we report clinical features, microbiological profile, and treatment difficulties encountered while managing liver abscesses in patients with CGD at a tertiary care centre in North-West India.

Methodology

Case records of children with CGD and liver abscesses at Pediatric Immunodeficiency Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India were analyzed.

Results

Seven of 68 patients (10.29%) with CGD presented with hepatic abscess. One patient had 2 recurrences. All were males and age-range at presentation was 7 months–22 years. Mutation analysis was carried out in all patients—3 had defects in CYBB gene; 2 in NCF1; 2 in NCF2 gene. Staphylococcus aureus was isolated from 5 patients. Duration of antimicrobial treatment ranged from 3 weeks to 7 months. Open drainage was required in 1 patient, and 1 patient was treated with a prolonged course of prednisolone. Two children succumbed to the illness.

Conclusions

This is the largest reported experience of liver abscesses in patients with CGD from the developing world. Staphylococcus aureus was the commonest pathogen isolated. In our experience, prolonged courses of antimicrobials are usually necessary in these patients. Glucocorticoids can reduce inflammatory response and facilitate early resolution of abscesses in CGD.

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Abbreviations

AR:

Autosomal recessive

BAL:

Bronchoalveolar lavage

CECT:

Contrast-enhanced computerized tomography

CGD:

Chronic granulomatous disease

CRP:

C-reactive protein

DHR:

Dihydrorhodamine

ESR:

Erythrocyte sedimentation rate

IUIS:

International Union of Immunological Societies

IV:

Intravenous

MRI:

Magnetic resonance imaging

MRSA:

Methicillin-resistant Staphylococcus aureus

MSSA:

Methicillin-sensitive Staphylococcus aureus

NBT:

Nitro blue tetrazolium test

SI:

Stimulation index

XL:

X-linked

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Acknowledgments

We sincerely thank the patients and their parents for their cooperation.

Disclaimer

The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

Funding

This study is funded by the Indian Council of Medical Research, New Delhi and Department of Health Research, Ministry of Health and Family Welfare, Government of India, New Delhi Grant No GIA/48/2014-DHR to SS, and The Foundation for Primary Immunodeficiencies (FPID), USA.

YLL was supported by The Society for Relief of Disabled Children for genetic sequencing.

Author information

Authors and Affiliations

  1. Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India

    Rakesh Kumar Pilania, Amit Rawat, Pandiarajan Vignesh, Sandesh Guleria, Ankur Kumar Jindal, Gargi Das, Deepti Suri, Anju Gupta & Surjit Singh

  2. Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012, India

    Kirti Gupta

  3. Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, Hong Kong

    Koon-Wing Chan & Yu-Lung Lau

  4. Community Pediatrics, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan

    Kohsuke Imai

Authors
  1. Rakesh Kumar Pilania
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  2. Amit Rawat
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  3. Pandiarajan Vignesh
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  5. Ankur Kumar Jindal
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Contributions

RKP and AR designed the research.

RKP, AR, PV, GD, SS, AKJ, GD, DS, AG, KG, KWC, YLL, KI, and SS performed the research.

RKP, AR, PV, and SS wrote the paper.

AR, KWY, YLL, and KI performed the genetic analysis of these patients.

KG performed histopathological examination. AR and SS critically supervised the manuscript.

All the authors read and approve the final manuscript.

Corresponding author

Correspondence to Amit Rawat.

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Conflict of Interest

The authors declare that they have no conflict of interest

Ethical Approval

This study was approved by Departmental Review Board at the Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

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Supplementary Information

Supplementary Figure 1

Course of illness in case 1 (Abbreviations: CGD: Chronic granulomatous disease; IV: Intravenous; MSSA: Methicillin sensitive Staphylococcus aureus; MRSA; Methicillin resistant Staphylococcus aureus; USG: Ultrasonogram) (JPG 145 kb)

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Pilania, R.K., Rawat, A., Vignesh, P. et al. Liver Abscess in Chronic Granulomatous Disease—Two Decades of Experience from a Tertiary Care Centre in North-West India. J Clin Immunol 41, 552–564 (2021). https://doi.org/10.1007/s10875-020-00938-9

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  • DOI: https://doi.org/10.1007/s10875-020-00938-9

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