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


METHODS
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.
Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India. rawatamit@yahoo.com.
Amit
A
Rawat
Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India.
Pandiarajan
P
Vignesh
Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India.
Avinash
A
Sharma
Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India.
Jitendra K
JK
Shandilya
Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India.
Madhubala
M
Sharma
Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India.
Deepti
D
Suri
Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India.
Anju
A
Gupta
Department of Medical Microbiology, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India.
Vikas
V
Gautam
Department of Medical Microbiology, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India.
Pallab
P
Ray
Department of Medical Microbiology, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India.
Shivaprakash M
SM
Rudramurthy
Department of Medical Microbiology, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India.
Arunaloke
A
Chakrabarti
Department of Pediatrics, National Defense Medical College, Tokorozawa, Saitama, Japan.
Kohsuke
K
Imai
Department of Pediatrics, National Defense Medical College, Tokorozawa, Saitama, Japan.
Shigeaki
S
Nonoyama
Kazusa DNA Research Institute, Kisarazu, Chiba, Japan.
Osamu
O
Ohara
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
YL
Lau
Pediatric Allergy and Immunology Unit, Advanced Pediatrics Centre, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigargh, 160012, India.
Surjit
S
Singh
D017668:Age of Onset; D000890:Anti-Infective Agents / Q000627:therapeutic use; D019072:Antibiotic Prophylaxis; D002675:Child, Preschool; D060085:Coinfection; D004252:DNA Mutational Analysis; D005260:Female; D005500:Follow-Up Studies; D006105:Granulomatous Disease, Chronic / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology; D006801:Humans; D016130:Immunophenotyping; D007194:India / Q000453:epidemiology; D007223:Infant; D017053:Infection Control; D007239:Infections / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000209:etiology*; D008297:Male; D009026:Mortality; D009154:Mutation; D010641:Phenotype; D062606:Tertiary Care Centers
D000890:Anti-Infective Agents
Chronic granulomatous disease; North India; bacteria; fungi; infections; mortality