Serum and Fiberoptic Derived Bronchoalveolar Lavage Fibulin-1 in Relation to BAL Differential Cell Count among Children with Interstitial Lung Diseases and its Correlation with Clinical and Functional Grading

Abstract

ibulin-1 is a novel potential biomarker for disease severity in children with ILD and it could be used as a target for the development of new treatments. The aim of this study was to measure serum and BAL fibulin-1 in children with interstitial pulmonary fibrosis and to determine the role of fibulin-1 in assessment of disease severity. 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 children with non pulmonary diseases after a high risk written consent was taken from all patients’ care givers included in the study. The patients were divided according to the possible etiology of ILD into two groups: ChILD group included 70% ILD cases with unknown etiology (ChILD syndrome),Their ages was ranging from 1 to 15 years old, with mean of age 8.40 ± 4.76, The patients were 6 males and 15 females. Non ChILD group included 30% of ILD patients diagnosed as diffuse alveolar hemoraghe syndrome, Aspiration syndrome, tracheoesophageal fistula, sacoidosis, Drug induced ILD, and Post-infectious bronchiolitis obliteran. Their a