RESUMEN
This study aimed to evaluate fibrosis and elastin destruction in childhood interstitial lung disease [chILD] patients and to correlate findings with clinical characteristics. Fifty-six patients and apparently healthy twenty children were recruited in this study. Participants were subjected to thorough history taking and clinical examination. All participants provided midstream urine and venus blood samples. Plasma levels of transforming growth factor [TGF]-/ll, connective tissue growth factor [CCN2] and soluble factor related apoptosis [sFas] and urinary desmosome /urinary creatinine [UDes/UCr] were measured by ELISA kits. In patients, clinical findings were crepitation [1.00 %], tachypnea [67. 90%], cardiomegaly [46.40%], digital clubbing [44.60%], cough [33.90%], cyanosis [28.60%], hepatomegaly [28.60%] and wheezes [23.30%]. Fan chILD clinical score was mostly score 3 [n= 20, 35.70%] then score 5 [n= 16, 28.60%], score 2 [n = 9, 16.10%], score 1 [n =8, 14.30% and score [4] [n=3, 5.40%]. TGF-PI, CCN2, sFas and UDes/UCr levels were significantly higher in patients than controls [P =0.0001]. In patients, significant positive correlations were found between TGF-PI and CCN2, sFas and UDes/UCr; between CCN2 and both sFas and UDes/UCr; between UDes/UCr and both sFas and mortality rate. Morbidity and mortality rates were 48.20% and 10.70%.Conclusions: Markers of fibrosis [TGF-B sFas, CCN2] and elastin destruction [UDes/UCr] were increased in chILD. So blockage of their pathways signals may offer novel therapeutic targets