{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T08:43:43Z","timestamp":1776933823498,"version":"3.51.2"},"reference-count":20,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100003356","name":"Gazi University","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100003356","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Eur J Pediatr"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Childhood interstitial lung diseases (chILD) include a diverse range of rare disorders, complex clinical progressions, and high rates of morbidity and mortality. This study aims to assess the clinical and psychosocial impacts of chILD in children registered in the National chILD-Turkiye database.\u00a0Data from 17 centers were analyzed as of January 2024. Patients aged 1\u00a0month to 18\u00a0years were included in the study. Through face-to-face interviews, patients and their families completed the Pediatric Quality of Life Inventory (PedsQL\u2122 4.0 Generic Core Scales) and PedsQL\u2122 2.0 Family Impact Module. Higher scores suggest better performance and higher health-related quality of life (HRQoL). Patient clinic data, demographic information, pulmonary function tests, Fan scores, and household income were recorded. Patients were categorized into two groups according to their use of O\n                    <jats:sub>2<\/jats:sub>\n                    therapy and diffuse progressive lung disease (DPLD) groups A and B.\u00a0The study included 175 patients (90 girls), with a median age of 7.7 (1.0\u201317.8) years. A strong positive correlation was found between the FEV1% value and both the Physical Health Summary Score and the Total Score (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.007,\n                    <jats:italic>r<\/jats:italic>\n                    \u2009=\u20090.608;\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.008,\n                    <jats:italic>r<\/jats:italic>\n                    \u2009=\u20090.607). The Fan score negatively correlated with PedsQL\u2122 scores (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.001). Patients who use O\n                    <jats:sub>2<\/jats:sub>\n                    have an earlier diagnosis of age of chILD, lower FEV1%, higher Fan scores, and lower Child Self-Reports and Parent Reports scores (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.05). The group with DPLD-A disorders has an earlier diagnosis age, lower weight and BMI z-scores, higher Fan score, and higher Family Impact Module score (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.05).\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:italic>Conclusion<\/jats:italic>\n                    :\u00a0Lung function and Fan score strongly correlate with HRQoL, emphasizing the impact of disease severity on physical and psychosocial well-being. These findings highlight the need for multidisciplinary care and psychosocial support to enhance the QoL in children with chILD and their families.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:table-wrap>\n                      <jats:table>\n                        <jats:tbody>\n                          <jats:tr>\n                            <jats:td align=\"left\">\n                              <jats:bold>What is Known:<\/jats:bold>\n                              \u2022\n                              <jats:italic>Childhood interstitial lung diseases (chILD) are rare and diverse disorders associated with significant morbidity and complex management.<\/jats:italic>\n                              \u2022\n                              <jats:italic>Evidence regarding health-related quality of life (HRQoL) and the psychosocial burden on affected families remains limited.<\/jats:italic>\n                            <\/jats:td>\n                          <\/jats:tr>\n                          <jats:tr>\n                            <jats:td align=\"left\">\n                              <jats:bold>What is New:<\/jats:bold>\n                              \u2022\n                              <jats:italic>This multicenter national registry\u2013based study provides the first comprehensive evaluation of HRQoL and family impact in children with chILD in T\u00fcrkiye.<\/jats:italic>\n                              \u2022\n                              <jats:italic>Lung function impairment and higher Fan severity scores were strongly linked to poorerHRQoL, highlighting the influence of disease severity on physical and psychosocial well-being.<\/jats:italic>\n                            <\/jats:td>\n                          <\/jats:tr>\n                        <\/jats:tbody>\n                      <\/jats:table>\n                    <\/jats:table-wrap>\n                  <\/jats:p>","DOI":"10.1007\/s00431-026-06834-5","type":"journal-article","created":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T04:26:02Z","timestamp":1775017562000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Quality of life in Turkish children with interstitial lung disease: Insights from the National chILD Registry"],"prefix":"10.1007","volume":"185","author":[{"given":"Handan","family":"Kekec","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tugba","family":"Sismanlar Eyuboglu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayse Tana","family":"Aslan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ozge","family":"Ulgen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Asl\u0131han","family":"Ozcaglar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zeliha Ba\u015fak","family":"Polat","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bet\u00fcl","family":"Bankoglu Parlak","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aysen","family":"Bingol","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seyda","family":"Karabulut","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ela Erdem","family":"Eralp","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cigdem","family":"Korkmaz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Berrak","family":"Oztosun","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gokcen","family":"Kartal Ozturk","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bahar","family":"Girgin Dindar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meltem","family":"Akgul Erdal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Umay","family":"Kavgac\u0131","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Esin Gizem","family":"Olgun","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fatma Nur","family":"Ayman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zeynep Seda","family":"Uyan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beste","family":"Ozsezen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tu\u011fba","family":"Ramasl\u0131 Gursoy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pelin","family":"Asfuroglu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mahir","family":"Serbes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mina","family":"H\u0131zal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Halime","family":"Nay\u0131r Buyuksah\u0131n","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G\u00f6zde","family":"Cav\u0131ldak Karaaslan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ebru","family":"Yalc\u0131n","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nagehan","family":"Emiralioglu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasemin","family":"G\u00f6kdemir","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sevgi","family":"Pekcan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ay\u015fe Ayz\u0131t","family":"K\u0131l\u0131nc Sakall\u0131","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saniye","family":"Girit","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nazan","family":"Cobanoglu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G\u00fczin","family":"Cinel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Berna","family":"Oguz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Diclehan","family":"Orhan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ahmet Cevdet","family":"Ceylan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nural","family":"Kiper","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,4,1]]},"reference":[{"key":"6834_CR1","doi-asserted-by":"publisher","unstructured":"Bush A, Cunningham S, de Blic J, Barbato A, Clement A, Epaud R et al (2015) European protocols for the diagnosis and initial treatment of interstitial lung disease in children. Thorax 70:1078\u20131084. https:\/\/doi.org\/10.1136\/thoraxjnl-2015-207349","DOI":"10.1136\/thoraxjnl-2015-207349"},{"key":"6834_CR2","doi-asserted-by":"publisher","first-page":"1219","DOI":"10.1164\/rccm.201903-0544CI","volume":"200","author":"RR Deterding","year":"2019","unstructured":"Deterding RR, DeBoer EM, Cidon MJ, Robinson TE, Warburton D, Deutsch GH et al (2019) Approaching clinical trials in childhood interstitial lung disease and pediatric pulmonary fibrosis. Am J Respir Crit Care Med 200:1219\u20131227. https:\/\/doi.org\/10.1164\/rccm.201903-0544CI","journal-title":"Am J Respir Crit Care Med"},{"key":"6834_CR3","doi-asserted-by":"publisher","first-page":"954","DOI":"10.1002\/ppul.24018","volume":"53","author":"M Niemitz","year":"2018","unstructured":"Niemitz M, Schwerk N, Goldbeck L, Griese M (2018) Development and validation of a health-related quality of life questionnaire for pediatric patients with interstitial lung disease. Pediatr Pulmonol 53:954\u2013963. https:\/\/doi.org\/10.1002\/ppul.24018","journal-title":"Pediatr Pulmonol"},{"key":"6834_CR4","doi-asserted-by":"publisher","unstructured":"Varni\u00a0JW, Limbers\u00a0CA, Neighbors\u00a0K, Schulz\u00a0K, Lieu\u00a0JEC, Robert W, et al (2011) The PedsQL\u2122 infant scales: feasibility internal consistency reliability and validity in healthy and ill infants quality of life research 20(1):45\u201355. https:\/\/doi.org\/10.1007\/s11136-010-9730-5","DOI":"10.1007\/s11136-010-9730-5"},{"key":"6834_CR5","doi-asserted-by":"publisher","first-page":"828","DOI":"10.1002\/ppul.24308","volume":"54","author":"C Lauby","year":"2019","unstructured":"Lauby C, Boelle PY, Abou Taam R, Bessaci K, Brouard J, Dalphin ML et al (2019) Health-related quality of life in infants and children with interstitial lung disease. Pediatr Pulmonol 54:828\u2013836. https:\/\/doi.org\/10.1002\/ppul.24308","journal-title":"Pediatr Pulmonol"},{"key":"6834_CR6","doi-asserted-by":"publisher","first-page":"1336","DOI":"10.1002\/ppul.24085","volume":"53","author":"RJ Nevel","year":"2018","unstructured":"Nevel RJ, Young LR (2018) Measures that matter: health-related quality of life in children with interstitial lung disease. Pediatr Pulmonol 53:1336\u20131337. https:\/\/doi.org\/10.1002\/ppul.24085","journal-title":"Pediatr Pulmonol"},{"key":"6834_CR7","doi-asserted-by":"publisher","first-page":"2572","DOI":"10.1002\/ppul.27069","volume":"59","author":"M Griese","year":"2024","unstructured":"Griese M, Schwerk N, Carlens J, Wetzke M, Emiralioglu N, Kiper N et al (2024) Health-related quality scores in childhood interstitial lung disease: good agreement between patient and caregiver reports. Pediatr Pulmonol 59:2572\u20132579. https:\/\/doi.org\/10.1002\/ppul.27069","journal-title":"Pediatr Pulmonol"},{"key":"6834_CR8","doi-asserted-by":"publisher","DOI":"10.1186\/1477-7525-2-55","volume":"2","author":"JW Varni","year":"2004","unstructured":"Varni JW, Sherman SA, Burwinkle TM, Dickinson PE, Dixon P (2004) The PedsQL family impact module: preliminary reliability and validity. Health Qual Life Outcomes 2:55. https:\/\/doi.org\/10.1186\/1477-7525-2-55","journal-title":"Health Qual Life Outcomes"},{"key":"6834_CR9","doi-asserted-by":"publisher","first-page":"800","DOI":"10.1097\/00005650-200108000-00006","volume":"39","author":"JW Varni","year":"2001","unstructured":"Varni JW, Seid M, Kurtin PS (2001) PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient populations. Med Care 39:800\u2013812. https:\/\/doi.org\/10.1097\/00005650-200108000-00006","journal-title":"Med Care"},{"key":"6834_CR10","doi-asserted-by":"publisher","first-page":"295","DOI":"10.1007\/s00431-023-05290-9","volume":"183","author":"H Nay\u0131r-B\u00fcy\u00fck\u015fahin","year":"2024","unstructured":"Nay\u0131r-B\u00fcy\u00fck\u015fahin H, Emiralio\u011flu N, K\u0131l\u0131n\u00e7 AA, Girit S, Yal\u00e7\u0131n E, \u015ei\u015fmanlar Ey\u00fcbo\u011flu T et al (2024) Childhood interstitial lung disease in Turkey: first data from the national registry. Eur J Pediatr 183:295\u2013304. https:\/\/doi.org\/10.1007\/s00431-023-05290-9","journal-title":"Eur J Pediatr"},{"key":"6834_CR11","doi-asserted-by":"publisher","first-page":"376","DOI":"10.1164\/rccm.201305-0923ST","volume":"188","author":"G Kurland","year":"2013","unstructured":"Kurland G, Deterding RR, Hagood JS, Young LR, Brody AS, Castile RG et al (2013) An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med 188:376\u2013394. https:\/\/doi.org\/10.1164\/rccm.201305-0923ST","journal-title":"Am J Respir Crit Care Med"},{"key":"6834_CR12","doi-asserted-by":"publisher","first-page":"1120","DOI":"10.1164\/rccm.200703-393OC","volume":"176","author":"GH Deutsch","year":"2007","unstructured":"Deutsch GH, Young LR, Deterding RR, Fan LL, Dell SD, Bean JA et al (2007) Diffuse lung disease in young children: application of a novel classification scheme. Am J Respir Crit Care Med 176:1120\u20131128. https:\/\/doi.org\/10.1164\/rccm.200703-393OC","journal-title":"Am J Respir Crit Care Med"},{"key":"6834_CR13","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-015-0339-1","volume":"10","author":"M Griese","year":"2015","unstructured":"Griese M, Irnstetter A, Hengst M, Burmester H, Nagel F, Ripper J et al (2015) Categorizing diffuse parenchymal lung disease in children. Orphanet J Rare Dis 10:122. https:\/\/doi.org\/10.1186\/s13023-015-0339-1","journal-title":"Orphanet J Rare Dis"},{"key":"6834_CR14","doi-asserted-by":"publisher","first-page":"e70","DOI":"10.1164\/rccm.201908-1590ST","volume":"200","author":"BL Graham","year":"2019","unstructured":"Graham BL, Steenbruggen I, Miller MR, Barjaktarevic IZ, Cooper BG, Hall GL et al (2019) Standardization of spirometry: 2019 update. An official American Thoracic Society and European Respiratory Society technical statement. Am J Respir Crit Care Med 200:e70\u2013e88. https:\/\/doi.org\/10.1164\/rccm.201908-1590ST","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"6834_CR15","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00016-2016","volume":"49","author":"BL Graham","year":"2017","unstructured":"Graham BL, Brusasco V, Burgos F, Cooper BG, Jensen R, Kendrick A et al (2017) 2017 ERS\/ATS standards for single-breath carbon monoxide uptake in the lung. Eur Respir J 49(1):1600016. https:\/\/doi.org\/10.1183\/13993003.00016-2016","journal-title":"Eur Respir J"},{"key":"6834_CR16","doi-asserted-by":"publisher","first-page":"939","DOI":"10.1164\/ajrccm.156.3.9703051","volume":"156","author":"LL Fan","year":"1997","unstructured":"Fan LL, Kozinetz CA (1997) Factors influencing survival in children with chronic interstitial lung disease. Am J Respir Crit Care Med 156:939\u2013942. https:\/\/doi.org\/10.1164\/ajrccm.156.3.9703051","journal-title":"Am J Respir Crit Care Med"},{"key":"6834_CR17","unstructured":"World Health Organization. Health statistics and information systems.WHOQOL: Measuring Quality of Life http:\/\/www.who.int\/healthinfo\/survey\/whoqol-qualityoflife\/en\/. Accessed 15 June 2025"},{"key":"6834_CR18","doi-asserted-by":"publisher","DOI":"10.1183\/20734735.0004-2025","volume":"21","author":"H Marczak","year":"2025","unstructured":"Marczak H, Krenke K, Griese M, Carlens J, Seidl E, Gilbert C et al (2025) An update on diagnosis and treatments of childhood interstitial lung diseases. Breathe Sheff 21:250004. https:\/\/doi.org\/10.1183\/20734735.0004-2025","journal-title":"Breathe Sheff"},{"key":"6834_CR19","doi-asserted-by":"publisher","DOI":"10.1177\/23779608221094522","volume":"8","author":"J Mattson","year":"2022","unstructured":"Mattson J, Lunnelie J, L\u00f6fholm T, Scheers Andersson E, Aune RE, Bj\u00f6rling G (2022) Quality of life in children with home mechanical ventilation: a scoping review. SAGE Open Nurs 8:23779608221094522. https:\/\/doi.org\/10.1177\/23779608221094522","journal-title":"SAGE Open Nurs"},{"key":"6834_CR20","doi-asserted-by":"publisher","DOI":"10.1186\/1477-7525-6-35","volume":"6","author":"AC Scarpelli","year":"2008","unstructured":"Scarpelli AC, Paiva SM, Pordeus IA, Varni JW, Viegas CM, Allison PJ (2008) The Pediatric Quality of Life Inventory (PedsQL) family impact module: reliability and validity of the Brazilian version. Health Qual Life Outcomes 6:35. https:\/\/doi.org\/10.1186\/1477-7525-6-35","journal-title":"Health Qual Life Outcomes"}],"container-title":["European Journal of Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00431-026-06834-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00431-026-06834-5","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00431-026-06834-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T07:54:42Z","timestamp":1776930882000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00431-026-06834-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,1]]},"references-count":20,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2026,4]]}},"alternative-id":["6834"],"URL":"https:\/\/doi.org\/10.1007\/s00431-026-06834-5","relation":{},"ISSN":["1432-1076"],"issn-type":[{"value":"1432-1076","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,1]]},"assertion":[{"value":"27 June 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 October 2025","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"3 March 2026","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"1 April 2026","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}],"article-number":"233"}}