الفهرس 
? Childhood Interstitial Lung Disease (ChILD)Only 14 pages are availabe for public view
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Abstract
Childhood Interstitial lung disease (ILD) is rising nowdays. Fibulin-1 (Fbln-1) increases in several inflammatory diseases of lung that results in respiratory failure. OBJECTIVES: The study aimed to measure Fbln1 in both serum and bronchoalveolar lavage using fiberoptic bronchoscope to assess its role as a diagnostic marker in children with ILD and its correlation with clinical (Fan scoring) and radiological severity with HRCT of chest. METHODS: The current study included 30 patients who were randomly collected from the patients with interstitial lung diseases attending the Pediatric Chest Clinic, Children’s Hospital, Ain Shams University and 30 children, Age and sex -matched with non pulmonary disease undergoing minor surgery. The patients were divided into cases with unknown etiology (ChILD group) with mean of age 8.40 ± 4.76 and cases secondary to systemic diseases (non ChILD group) with mean of age 9.39 ± 4.06 years. Human Fbln1 in serum and fiberoptic bronchoscopy derived bronchoalveolar lavage was assessed by Immunoassay technique. RESULTS: Fbln1 was significantly increased in both serum and BAL in ILD patients compared to healthy control. The mean value of BAL Fbln1 was significantly increased in ChILD group in comparison to non ChILD group. Mean value of BAL Fbln1 levels correlated with ILD severity represented by serum Fbln1 (r = 0.399, P =0.029) , clinical Fan score (r =0.519, P =0.003) and radiological score (r =0.513, P =0.004) in studied patients. CONCLUSION: Fbln1 could serve as a novel biomarker or ILD disease diagnosis and severity assessment. Fbln1 may be considered as a therapeutic target in respiratory diseases and other conditions involving remodeling and inflammation.