Prevalence and Microbial Associations in Pediatric Chronic Granulomatous Disease: A Retrospective Case Series at Mofid and Masih Daneshvari Hospitals (2014 - 2019)

Abstract

Background: Chronic granulomatous disease (CGD) is an immune system disorder caused by NADPH oxidase dysfunction. This dysfunction reduces the ability of neutrophils and monocytes to combat infections, leading to recurrent infections with microorganisms such as Staphylococcus, Salmonella, and BCG. CGD is commonly diagnosed in children younger than 5 years and can cause a range of infectious and noninfectious complications. Objectives: This study aimed to describe the clinical and microbial profiles of infections in pediatric patients with CGD at two Iranian referral hospitals over a 5-year period. Methods: This retrospective consecutive case series included patients with CGD admitted to two hospitals between 2014 and 2019. All patients with confirmed CGD (nitroblue tetrazolium [NBT] < 5% or dihydrorhodamine [DHR] stimulation index < 10) were eligible. Six patients were excluded because of incomplete data or declined retrospective consent. Culture, polymerase chain reaction (PCR), and BacTec were used. Causative pathogens were defined by sterile-site isolation or clinical assessment. Fisher's exact test was used, and P < 0.05 was considered statistically significant. Results: The study included 30 patients with CGD who presented to the two hospitals between April 2014 and April 2019. Most patients were male. The mean age was 107.96 ± 59.08 months, and the median age at diagnosis was 60 months (interquartile range [IQR], 24 - 108). The mean number of hospital admissions was 1.89 (IQR, 1 - 2), and the median length of hospital stay was 25 days (IQR, 14 - 42). The most frequent clinical manifestations were pneumonia and lymphadenopathy. Streptococcus (33.3%) and Candida (30%) were the most common pathogens. No significant association was observed between the infecting microorganisms and growth status, number of admissions, immunoglobulin levels, or lesion type (Fisher's exact P > 0.05). Conclusions: Streptococcus and Candida predominated among Iranian pediatric patients with CGD, differing from global patterns. These findings support consideration of local microbial patterns when evaluating prophylactic strategies.