Swati Sagwal1, Anil Chauhan1,2, Jyotdeep Kaur3, Rajendra Prasad3,4, Meenu Singh5,6, Manvi Singh1. 1. Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. 2. Evidence Based Health Informatics Unit, Regional Resource Centre, Dept. of Telemedicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India. 3. Department of Biochemistry, Postgraduate Institute of Medical Education and Research, Chandigarh, India. 4. Department of Biochemistry, Maharishi Markandeswar Institute of Medical Sciences and Research (Deemed to be University), Ambala, Haryana, India. 5. Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. meenusingh4@gmail.com. 6. Evidence Based Health Informatics Unit, Regional Resource Centre, Dept. of Telemedicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India. meenusingh4@gmail.com.
Abstract
PURPOSE: Cystic Fibrosis (CF) is a multi-organ genetic disorder and Transforming Growth Factor (TGF-β1) is a modifier gene which modulates lung pathology in CF. There is great phenotypic variability among CF patients who even have similar genotype. The aim of the present study was to associate the serum levels of TGF-β1 with several clinical phenotypes of CF. METHODS: The diagnosed cases of CF were recruited and the blood sample was withdrawn at different time points: during exacerbation (n = 26), non-exacerbation (n = 9) and after antibiotic therapy (n = 11). The concentration of the total TGF-β1 in serum was measured with commercial ELISA kit. The ΔF508 mutation was assessed by the Amplification Refractory Mutation System (ARMS-PCR). RESULTS: The levels of TGF-β1 were increased in exacerbation phase (119.89 ± 29.64 ng/mL), infection with P. aeruginosa (121.8 ± 28.83 ng/mL) and in subjects with ΔF508 mutation (139.2 ± 19.59 ng/mL). The levels of TGF-β1 in CF patients with Allergic Bronchopulmonary Aspergillosis (ABPA) (109.97 ± 27.71 ng/mL) were decreased as compared to CF patients without ABPA (123.55 ± 30.20 ng/mL). It was observed that the serum levels of TGF-β1 were decreased significantly after antibiotic therapy (p < 0.05). CONCLUSIONS: The present study has determined that the serum levels of TGF-β1 vary with the type of infections, ΔF508 CFTR mutation, presence of ABPA and response to therapy.
PURPOSE:Cystic Fibrosis (CF) is a multi-organ genetic disorder and Transforming Growth Factor (TGF-β1) is a modifier gene which modulates lung pathology in CF. There is great phenotypic variability among CFpatients who even have similar genotype. The aim of the present study was to associate the serum levels of TGF-β1 with several clinical phenotypes of CF. METHODS: The diagnosed cases of CF were recruited and the blood sample was withdrawn at different time points: during exacerbation (n = 26), non-exacerbation (n = 9) and after antibiotic therapy (n = 11). The concentration of the total TGF-β1 in serum was measured with commercial ELISA kit. The ΔF508 mutation was assessed by the Amplification Refractory Mutation System (ARMS-PCR). RESULTS: The levels of TGF-β1 were increased in exacerbation phase (119.89 ± 29.64 ng/mL), infection with P. aeruginosa (121.8 ± 28.83 ng/mL) and in subjects with ΔF508 mutation (139.2 ± 19.59 ng/mL). The levels of TGF-β1 in CFpatients with Allergic Bronchopulmonary Aspergillosis (ABPA) (109.97 ± 27.71 ng/mL) were decreased as compared to CFpatients without ABPA (123.55 ± 30.20 ng/mL). It was observed that the serum levels of TGF-β1 were decreased significantly after antibiotic therapy (p < 0.05). CONCLUSIONS: The present study has determined that the serum levels of TGF-β1 vary with the type of infections, ΔF508 CFTR mutation, presence of ABPA and response to therapy.
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