Current status of childhood interstitial lung disease in Turkiye: 5-year data from ChILD-TR

Kekeç, Handan; ASLAN, AYŞE; ŞİŞMANLAR EYÜBOĞLU, TUĞBA; ÜNLÜ, TUĞÇE; Can Oksay, Sinem; Mocan Çağlar, Yasemin; Ülgen, Özge; Ayman, Fatma; Ercan, Fatih; Ünal, Gökçen; Korkmaz, Çiğdem; Kılıç Başkan, Azer; Çollak, Abdulhamit; Ozcelik, Ugur; DOĞRU ERSÖZ, DENİZ; Atan, Raziye; Gülen, Figen; Girgin Dindar, Bahar; Halis, Ece; Gizem Olgun, Esin; ZİREK, FAZILCAN; FİTOZ, ÖMER; Kürtül Çakar, Meltem; Eryilmaz Polat, Sanem; Tural, Dilber; Bingöl, Ayşen; Başaran, Abdurrahman; Bankoğlu Parlak, Betül; Erdem Eralp, Ela; KARADAĞ, BÜLENT; Ergenekon, Pınar; Köse, Mehmet; Ersoy, Ali; HANGÜL, Melih; Asfuroğlu, Pelin; Cakir, Erkan; Yazan, Hakan; Ozesen, Beste; Gönüllü, Erdem; Uyan, Zeynep; Ocak, Ece; Yılmaz, Aslı; Yılmaz Yegit, Cansu; Nayır Büyükşahin, Halime; Kartal Öztürk, Gökçen; Serbes, Mahir; Ramasli Gursoy, Tugba; Gharibzadeh Hizal, Mina; Cavıldak Karaaslan, Gözde; ÖZCAN, GİZEM; Özdemir, Ali; Pekcan, Sevgi; Kılınç Sakallı, Ayşe; GÖKDEMİR, YASEMİN; Cinel, Güzin; ÇOBANOĞLU, FATMA; Girit, Saniye; EMİRALİOĞLU ORDUKAYA, NAGEHAN; Yalcin, Ebru; Oğuz, Berna; Orhan, Diclehan; Ceylan, Ahmet; Kiper, Nural

doi:10.1136/bmjresp-2026-004626

Current status of childhood interstitial lung disease in Turkiye: 5-year data from ChILD-TR

Kekeç H., ASLAN A. T., ŞİŞMANLAR EYÜBOĞLU T., ÜNLÜ T., Can Oksay S., Mocan Çağlar Y., ...Daha Fazla

BMJ Open Respiratory Research, cilt.13, sa.1, 2026 (SCI-Expanded, Scopus)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 13 Sayı: 1
Basım Tarihi: 2026
Doi Numarası: 10.1136/bmjresp-2026-004626
Dergi Adı: BMJ Open Respiratory Research
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, EMBASE, MEDLINE, Directory of Open Access Journals, Health Research Premium Collection (ProQuest)
Anahtar Kelimeler: Rare lung diseases
Ankara Üniversitesi Adresli: Evet

Özet

Introduction: Childhood interstitial lung disease (chILD) encompasses various diffuse parenchymal lung diseases (DPLDs) in children. We aimed to assess the current status and temporal changes of chILD in Türkiye using updated data from the chILD-Türkiye (chILD-TR) registry. Method: By May 2025, all patients registered in the chILD-TR were included in the study. Patients with missing data were excluded. Patients were divided into two groups: disorders primarily appearing in infancy (DPLD-A) and disorders that can occur at any age (DPLD-B). Clinical and demographic data were compared between the groups. Results: 711 patients (44.7% female) were included. The registry-based prevalence estimate of chILD in Türkiye was approximately 30–35 cases per million children. The current age was 135 (16-383) months. The time from first symptoms to admission was 3 (0–217) months. Age at diagnosis was 59 (11-273) months. Common symptoms were cough (59.5%) and dyspnoea (35.4%). 136 patients (19.1%) required oxygen. 25 patients (3.5%) died. Among patients, 126 (17.7%) had DPLD-A, 549 (77.2%) had DPLD-B and 36 (5.1%) had uncategorised disorders. The DPLD-A group was younger and had earlier symptom onset and older diagnosis age (p<0.05). Growth z-scores were lower in the DPLD-A group (p<0.05). Parental consanguinity and mechanical ventilation use were higher in the DPLD-A group (p<0.05). No differences in spirometry and diffusing capacity of the lungs for carbon monoxide were found between DPLD-A and DPLD-B (p>0.05). Chest CT features varied: rates of ground-glass opacity and pectus excavatum were higher in the DPLD-A group, whereas nodules, peribronchial thickening, mosaic perfusion and bronchiectasis were more predominant in the DPLD-B group (p<0.05). Conclusion: This study shows that children with DPLD-A are diagnosed later despite earlier symptoms and growth issues, revealing a recognition gap and highlighting the need for early genetic screening and support. In DPLD-B, airway findings emphasise the need for long-term monitoring. These insights support phenotype-based diagnosis and treatment to improve outcomes in children with chILD.