학술논문
Childhood interstitial lung disease in Turkey: first data from the national registry
Document Type
Original Paper
Author
Nayır-Büyükşahin, Halime; Emiralioğlu, Nagehan; Kılınç, Ayşe Ayzıt; Girit, Saniye; Yalçın, Ebru; Şişmanlar Eyüboğlu, Tuğba; Çobanoğlu, Nazan; Cinel, Güzin; Pekcan, Sevgi; Gökdemir, Yasemin; Oğuz, Berna; Orhan, Diclehan; Doğru, Deniz; Özçelik, Uğur; Başkan, Azer Kılıç; Arslan, Hüseyin; Çokuğraş, Haluk; Onay, Zeynep Reyhan; Oksay, Sinem Can; Tortop, Deniz Mavi; Aslan, Ayşe Tana; Kekeç, Handan; Zirek, Fazılcan; Tekin, Merve Nur; Gülen, Figen; Girgin Dindar, Bahar; Eryılmaz Polat, Sanem; Uytun, Salih; Yılmaz, Aslı İmran; Ünal, Gökçen; Eralp, Ela Erdem; Karadağ, Bülent; Hangül, Melih; Köse, Mehmet; Özsezen, Beste; Çakır, Erkan; Bingöl, Ayşen; Hızal, Mina; Kartal Öztürk, Gökçen; Uyan, Zeynep Seda; Ramaslı Gürsoy, Tuğba; Serbes, Mahir; Kiper, Nural
Source
European Journal of Pediatrics. 183(1):295-304
Subject
Language
English
ISSN
1432-1076
Abstract
The childhood interstitial lung diseases (chILD) Turkey registry (chILD-TR) was established in November 2021 to increase awareness of disease, and in collaboration with the centers to improve the diagnostic and treatment standards. Here, the first results of the chILD registry system were presented. In this prospective cohort study, data were collected using a data‐entry software system. The demographic characteristics, clinical, laboratory, radiologic findings, diagnoses, and treatment characteristics of the patients were evaluated. Clinical characteristics were compared between two main chILD groups ((A) diffuse parenchymal lung diseases (DPLD) disorders manifesting primarily in infancy [group1] and (B) DPLD disorders occurring at all ages [group 2]). There were 416 patients registered from 19 centers. Forty-six patients were excluded due to missing information. The median age of diagnosis of the patients was 6.05 (1.3–11.6) years. Across the study population (n = 370), 81 (21.8%) were in group 1, and 289 (78.1%) were in group 2. The median weight z-score was significantly lower in group 1 (− 2.0 [− 3.36 to − 0.81]) than in group 2 (− 0.80 [− 1.7 to 0.20]) (p < 0.001). When we compared the groups according to chest CT findings, ground-glass opacities were significantly more common in group 1, and nodular opacities, bronchiectasis, mosaic perfusion, and mediastinal lymphadenopathy were significantly more common in group 2. Out of the overall study population, 67.8% were undergoing some form of treatment. The use of oral steroids was significantly higher in group 2 than in group 1 (40.6% vs. 23.3%, respectively; p = 0.040). Conclusion: This study showed that national registry allowed to obtain information about the frequency, types, and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients.[Table Removed]