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Prevalence of inherited errors of immunity in a cohort of Egyptian children with childhood interstitial lung disease

Year: 2025
Type: article
Study design: Observational Study
Abstract: Background Childhood interstitial lung disease (ChILD) represents a heterogeneous group of diseases, typically with a restrictive lung disease pattern. Patients with inherited errors of immunity (IEI) display many symptoms. Beyond recurrent infections, non-infectious manifestations, such as interstitial lung disease (ILD), may occur. Aim of the study To estimate the prevalence of IEI among a group of Egyptian children with ChILD. Methods This study enrolled 30 patients, both males and females, aged 1 to 18 years, who were clinically and radiologically diagnosed with ChILD. All participants underwent comprehensive evaluations, including a full medical history, clinical examination, laboratory investigations (complete blood count [CBC], absolute lymphocyte count [ALC], immunoglobulins [Igs], and clusters of differentiation [CDs], among others as indicated), imaging studies (high-resolution computed tomography [HR-CT] of the chest), and pulmonary function tests, when feasible. Results The prevalence of IEI among ChILD cases was 20% in the studied population. Chronic granulomatous disease (CGD) was the most frequently diagnosed IEI in this cohort. Significant differences between the immunocompetent and immunodeficient groups were observed in consanguinity (P = 0.017) and the occurrence of unusual infections (P < 0.001), with both factors strongly correlating with IEI. Conclusion The prevalence of IEI among ChILD cases in this cohort was 20%. IEI should be investigated in ChILD, particularly in cases with a suggestive history and the absence of other pulmonary or systemic causes.
DOI: 10.1186/s43054-025-00357-8
Language: English
Subfield: Immunology
Open Access status: diamond
OpenAlex ID: W4408460292
FWCI:
Cites: 25
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Related to: 10