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Dilek Yapar

and 8 more

Background While the Pediatric Quality of Life Inventory™ (PedsQL™) 3.0 eosinophilic esophagitis (EoE) Module is widely used for EoE assessment, no comprehensive transcultural adaptation has been completed, except for one linguistic validation study in Spanish. To assess the transcultural adaptation of the Turkish version of PedsQL™ (Tr-PedsQL™) 3.0 EoE Module for teens and parent reports through validity and reliability tests, marking a first in the field. Methods Teens with EoE and their parents were included in the study. Linguistic validation, content validity (CnV) and field test for construct validity (CsV) and reliability were completed in the adaptation of the Tr-PedsQL™ 3.0 EoE Module. Convergent and divergent validity (CgV and DgV) were examined for CsV by correlation analysis between Turkish version of Pediatric EoE Symptom Scores® (Tr-PEESS) v2.0 and Tr-PedsQL TM 3.0 EoE Module scores. Reliability was determined through internal consistency (Cronbach-α) and test-retest reliability (intraclass correlation coefficients: ICC). Results Thirty-three teens and their parents completed the study. CnV indexes were > 0.8 for all items. Good correlations between Tr-PEESS v2.0 and Tr-PedsQL TM 3.0 EoE Module Total, Symptoms I, II and Total scores substantiated CgV, while low or absent correlations in certain dimensions evidenced DgV. Tr-PedsQL TM 3.0 EoE Module showed good internal consistency (Cronbach-α: 0.61-0.90) and good to excellent test-retest reliability (ICC: 0.713-0.935). Conclusions This study is the first to adapt the PedsQL™ 3.0 EoE Module for another language with its validity and reliability in assessing the health-related quality of life among Turkish-speaking teens with EoE and their parents.

Hasan Satış

and 15 more

Objectives: Treatment of connective tissue disease-related interstitial lung disease (CTD-ILD) remains challenging. The literature related to the immunosuppressive drugs is very limited in most CTDs and there is no previously reported study comparing induction regimens in patients with newly defined ‘interstitial pneumonia with autoimmune features (IPAF), We aimed to investigate the efficacy of azathioprine (AZA) used in induction regimens for interstitial lung disease in a variety of connective tissue disorders including IPAF, and compare it with cyclophosphamide (CYC) Methods. In a retrospective study, all patients presented with interstitial lung disease to the rheumatology and/or pulmonology departments in a tertiary referral centre, between 2009 and 2019. Five major CTD groups were defined; systemic sclerosis, rheumatoid arthritis (RA), primary Sjögren syndrome (pSS), inflammatory myositis (IMS) and interstitial pneumonia with autoimmune features (IPAF). All patients who satisfied one of these and received AZA or CYC for induction therapy for at least 6 months were included in the study. Treatment responses at six months and side effects were analyzed. Results: There were 1351 patients diagnosed with ILD. Of these, 328 patients were identified as CTD-ILD, satisfying the classification criteria of one of the CTDs. Among these, 57 patients received AZA and 79 patients received CYC for induction therapy. CYC treatment resulted in a 2.41 % increase in FVC, however, AZA resulted in a 1.44% decline in FVC predicted (p=0.041). Propensity score matching was used to reduce selection bias, AZA treatment was related to increased risk of progression (50% vs 13.5%, p:0.002). Conclusion: CYC is superior to AZA in induction therapy of CTD-ILD.