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Childhood interstitial lung disease in Turkey: first data from the national registry
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....
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Published in: | European journal of pediatrics 2024-01, Vol.183 (1), p.295-304 |
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creator | 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 |
description | 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
|
doi_str_mv | 10.1007/s00431-023-05290-9 |
format | article |
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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.
What is Known:
• Childhood interstitial lung diseases comprise many diverse entities which are challenging to diagnose and manage.
What is New:
• 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. Also, our findings reveal that nutrition should be considered in all patients with chILD, especially in A-DPLD disorders manifesting primarily in infancy.</description><identifier>ISSN: 1432-1076</identifier><identifier>ISSN: 0340-6199</identifier><identifier>EISSN: 1432-1076</identifier><identifier>DOI: 10.1007/s00431-023-05290-9</identifier><identifier>PMID: 37875631</identifier><language>eng</language><publisher>Berlin/Heidelberg: Springer Berlin Heidelberg</publisher><subject>Bronchiectasis ; Child ; Childhood ; Children ; Diagnosis ; Humans ; Lung ; Lung diseases ; Lung Diseases, Interstitial - diagnosis ; Lung Diseases, Interstitial - epidemiology ; Lung Diseases, Interstitial - therapy ; Lymphadenopathy ; Medicine ; Medicine & Public Health ; Patients ; Pediatrics ; Population studies ; Prospective Studies ; Registries ; Steroid hormones ; Turkey - epidemiology</subject><ispartof>European journal of pediatrics, 2024-01, Vol.183 (1), p.295-304</ispartof><rights>The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.</rights><rights>2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c375t-5b0d0df7ae30d7a57a086aa41c3c1f8a3648d3067d5fea8dea4f0da98e99cf803</citedby><cites>FETCH-LOGICAL-c375t-5b0d0df7ae30d7a57a086aa41c3c1f8a3648d3067d5fea8dea4f0da98e99cf803</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27923,27924</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/37875631$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Nayır-Büyükşahin, Halime</creatorcontrib><creatorcontrib>Emiralioğlu, Nagehan</creatorcontrib><creatorcontrib>Kılınç, Ayşe Ayzıt</creatorcontrib><creatorcontrib>Girit, Saniye</creatorcontrib><creatorcontrib>Yalçın, Ebru</creatorcontrib><creatorcontrib>Şişmanlar Eyüboğlu, Tuğba</creatorcontrib><creatorcontrib>Çobanoğlu, Nazan</creatorcontrib><creatorcontrib>Cinel, Güzin</creatorcontrib><creatorcontrib>Pekcan, Sevgi</creatorcontrib><creatorcontrib>Gökdemir, Yasemin</creatorcontrib><creatorcontrib>Oğuz, Berna</creatorcontrib><creatorcontrib>Orhan, Diclehan</creatorcontrib><creatorcontrib>Doğru, Deniz</creatorcontrib><creatorcontrib>Özçelik, Uğur</creatorcontrib><creatorcontrib>Başkan, Azer Kılıç</creatorcontrib><creatorcontrib>Arslan, Hüseyin</creatorcontrib><creatorcontrib>Çokuğraş, Haluk</creatorcontrib><creatorcontrib>Onay, Zeynep Reyhan</creatorcontrib><creatorcontrib>Oksay, Sinem Can</creatorcontrib><creatorcontrib>Tortop, Deniz Mavi</creatorcontrib><creatorcontrib>Aslan, Ayşe Tana</creatorcontrib><creatorcontrib>Kekeç, Handan</creatorcontrib><creatorcontrib>Zirek, Fazılcan</creatorcontrib><creatorcontrib>Tekin, Merve Nur</creatorcontrib><creatorcontrib>Gülen, Figen</creatorcontrib><creatorcontrib>Girgin Dindar, Bahar</creatorcontrib><creatorcontrib>Eryılmaz Polat, Sanem</creatorcontrib><creatorcontrib>Uytun, Salih</creatorcontrib><creatorcontrib>Yılmaz, Aslı İmran</creatorcontrib><creatorcontrib>Ünal, Gökçen</creatorcontrib><creatorcontrib>Eralp, Ela Erdem</creatorcontrib><creatorcontrib>Karadağ, Bülent</creatorcontrib><creatorcontrib>Hangül, Melih</creatorcontrib><creatorcontrib>Köse, Mehmet</creatorcontrib><creatorcontrib>Özsezen, Beste</creatorcontrib><creatorcontrib>Çakır, Erkan</creatorcontrib><creatorcontrib>Bingöl, Ayşen</creatorcontrib><creatorcontrib>Hızal, Mina</creatorcontrib><creatorcontrib>Kartal Öztürk, Gökçen</creatorcontrib><creatorcontrib>Uyan, Zeynep Seda</creatorcontrib><creatorcontrib>Ramaslı Gürsoy, Tuğba</creatorcontrib><creatorcontrib>Serbes, Mahir</creatorcontrib><creatorcontrib>Kiper, Nural</creatorcontrib><title>Childhood interstitial lung disease in Turkey: first data from the national registry</title><title>European journal of pediatrics</title><addtitle>Eur J Pediatr</addtitle><addtitle>Eur J Pediatr</addtitle><description>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.
What is Known:
• Childhood interstitial lung diseases comprise many diverse entities which are challenging to diagnose and manage.
What is New:
• 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. Also, our findings reveal that nutrition should be considered in all patients with chILD, especially in A-DPLD disorders manifesting primarily in infancy.</description><subject>Bronchiectasis</subject><subject>Child</subject><subject>Childhood</subject><subject>Children</subject><subject>Diagnosis</subject><subject>Humans</subject><subject>Lung</subject><subject>Lung diseases</subject><subject>Lung Diseases, Interstitial - diagnosis</subject><subject>Lung Diseases, Interstitial - epidemiology</subject><subject>Lung Diseases, Interstitial - therapy</subject><subject>Lymphadenopathy</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Patients</subject><subject>Pediatrics</subject><subject>Population studies</subject><subject>Prospective Studies</subject><subject>Registries</subject><subject>Steroid hormones</subject><subject>Turkey - epidemiology</subject><issn>1432-1076</issn><issn>0340-6199</issn><issn>1432-1076</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><recordid>eNp9kLFu2zAQhokiRe24fYEOAYEsXZQeRUmksgVGmgQw0MWdiYt4tJnKkktSg98-bJykQYZOJHDf__P4MfZVwIUAUN8jQCVFAaUsoC5bKNoPbC4qWRYCVHPy5j5jpzE-QA61Qn9iM6m0qhsp5my93PrebsfRcj8kCjH55LHn_TRsuPWRMFKe8PUUftPhkjufEW4xIXdh3PG0JT5g8uOQQ4E2PqZw-Mw-OuwjfXk-F-zXj-v18rZY_by5W16tik6qOhX1PViwTiFJsAprhaAbxEp0shNOo2wqbSU0ytaOUFvCyoHFVlPbdk6DXLBvx959GP9MFJPZ-dhR3-NA4xRNqbVQ-f9lndHzd-jDOIW8dKbasnoSU2WqPFJdGGMM5Mw--B2GgxFg_jo3R-cmOzdPzk2bQ2fP1dP9juxr5EVyBuQRiHk0bCj8e_s_tY_Uy4zP</recordid><startdate>20240101</startdate><enddate>20240101</enddate><creator>Nayır-Büyükşahin, Halime</creator><creator>Emiralioğlu, Nagehan</creator><creator>Kılınç, Ayşe Ayzıt</creator><creator>Girit, Saniye</creator><creator>Yalçın, Ebru</creator><creator>Şişmanlar Eyüboğlu, Tuğba</creator><creator>Çobanoğlu, Nazan</creator><creator>Cinel, Güzin</creator><creator>Pekcan, Sevgi</creator><creator>Gökdemir, Yasemin</creator><creator>Oğuz, Berna</creator><creator>Orhan, Diclehan</creator><creator>Doğru, Deniz</creator><creator>Özçelik, Uğur</creator><creator>Başkan, Azer Kılıç</creator><creator>Arslan, Hüseyin</creator><creator>Çokuğraş, Haluk</creator><creator>Onay, Zeynep Reyhan</creator><creator>Oksay, Sinem Can</creator><creator>Tortop, Deniz Mavi</creator><creator>Aslan, Ayşe Tana</creator><creator>Kekeç, Handan</creator><creator>Zirek, Fazılcan</creator><creator>Tekin, Merve Nur</creator><creator>Gülen, Figen</creator><creator>Girgin Dindar, Bahar</creator><creator>Eryılmaz Polat, Sanem</creator><creator>Uytun, Salih</creator><creator>Yılmaz, Aslı İmran</creator><creator>Ünal, Gökçen</creator><creator>Eralp, Ela Erdem</creator><creator>Karadağ, Bülent</creator><creator>Hangül, Melih</creator><creator>Köse, Mehmet</creator><creator>Özsezen, Beste</creator><creator>Çakır, 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B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7RV</scope><scope>7TK</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8C1</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9-</scope><scope>K9.</scope><scope>KB0</scope><scope>M0R</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>20240101</creationdate><title>Childhood interstitial lung disease in Turkey: first data from the national registry</title><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</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c375t-5b0d0df7ae30d7a57a086aa41c3c1f8a3648d3067d5fea8dea4f0da98e99cf803</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Bronchiectasis</topic><topic>Child</topic><topic>Childhood</topic><topic>Children</topic><topic>Diagnosis</topic><topic>Humans</topic><topic>Lung</topic><topic>Lung diseases</topic><topic>Lung Diseases, Interstitial - diagnosis</topic><topic>Lung Diseases, Interstitial - epidemiology</topic><topic>Lung Diseases, Interstitial - therapy</topic><topic>Lymphadenopathy</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Patients</topic><topic>Pediatrics</topic><topic>Population studies</topic><topic>Prospective Studies</topic><topic>Registries</topic><topic>Steroid hormones</topic><topic>Turkey - 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Sinem Can</creatorcontrib><creatorcontrib>Tortop, Deniz Mavi</creatorcontrib><creatorcontrib>Aslan, Ayşe Tana</creatorcontrib><creatorcontrib>Kekeç, Handan</creatorcontrib><creatorcontrib>Zirek, Fazılcan</creatorcontrib><creatorcontrib>Tekin, Merve Nur</creatorcontrib><creatorcontrib>Gülen, Figen</creatorcontrib><creatorcontrib>Girgin Dindar, Bahar</creatorcontrib><creatorcontrib>Eryılmaz Polat, Sanem</creatorcontrib><creatorcontrib>Uytun, Salih</creatorcontrib><creatorcontrib>Yılmaz, Aslı İmran</creatorcontrib><creatorcontrib>Ünal, Gökçen</creatorcontrib><creatorcontrib>Eralp, Ela Erdem</creatorcontrib><creatorcontrib>Karadağ, Bülent</creatorcontrib><creatorcontrib>Hangül, Melih</creatorcontrib><creatorcontrib>Köse, Mehmet</creatorcontrib><creatorcontrib>Özsezen, Beste</creatorcontrib><creatorcontrib>Çakır, Erkan</creatorcontrib><creatorcontrib>Bingöl, Ayşen</creatorcontrib><creatorcontrib>Hızal, Mina</creatorcontrib><creatorcontrib>Kartal Öztürk, Gökçen</creatorcontrib><creatorcontrib>Uyan, Zeynep Seda</creatorcontrib><creatorcontrib>Ramaslı Gürsoy, Tuğba</creatorcontrib><creatorcontrib>Serbes, Mahir</creatorcontrib><creatorcontrib>Kiper, Nural</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Nursing & Allied Health Database</collection><collection>Neurosciences Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Public Health Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>Consumer Health Database (Alumni Edition)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Consumer Health Database</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>PML(ProQuest Medical Library)</collection><collection>Nursing & Allied Health Premium</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>European journal of pediatrics</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Nayır-Büyükşahin, Halime</au><au>Emiralioğlu, Nagehan</au><au>Kılınç, Ayşe Ayzıt</au><au>Girit, Saniye</au><au>Yalçın, Ebru</au><au>Şişmanlar Eyüboğlu, Tuğba</au><au>Çobanoğlu, Nazan</au><au>Cinel, Güzin</au><au>Pekcan, Sevgi</au><au>Gökdemir, Yasemin</au><au>Oğuz, Berna</au><au>Orhan, Diclehan</au><au>Doğru, Deniz</au><au>Özçelik, Uğur</au><au>Başkan, Azer Kılıç</au><au>Arslan, Hüseyin</au><au>Çokuğraş, Haluk</au><au>Onay, Zeynep Reyhan</au><au>Oksay, Sinem Can</au><au>Tortop, Deniz Mavi</au><au>Aslan, Ayşe Tana</au><au>Kekeç, Handan</au><au>Zirek, Fazılcan</au><au>Tekin, Merve Nur</au><au>Gülen, Figen</au><au>Girgin Dindar, Bahar</au><au>Eryılmaz Polat, Sanem</au><au>Uytun, Salih</au><au>Yılmaz, Aslı İmran</au><au>Ünal, Gökçen</au><au>Eralp, Ela Erdem</au><au>Karadağ, Bülent</au><au>Hangül, Melih</au><au>Köse, Mehmet</au><au>Özsezen, Beste</au><au>Çakır, Erkan</au><au>Bingöl, Ayşen</au><au>Hızal, Mina</au><au>Kartal Öztürk, Gökçen</au><au>Uyan, Zeynep Seda</au><au>Ramaslı Gürsoy, Tuğba</au><au>Serbes, Mahir</au><au>Kiper, Nural</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Childhood interstitial lung disease in Turkey: first data from the national registry</atitle><jtitle>European journal of pediatrics</jtitle><stitle>Eur J Pediatr</stitle><addtitle>Eur J Pediatr</addtitle><date>2024-01-01</date><risdate>2024</risdate><volume>183</volume><issue>1</issue><spage>295</spage><epage>304</epage><pages>295-304</pages><issn>1432-1076</issn><issn>0340-6199</issn><eissn>1432-1076</eissn><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.
What is Known:
• Childhood interstitial lung diseases comprise many diverse entities which are challenging to diagnose and manage.
What is New:
• 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. Also, our findings reveal that nutrition should be considered in all patients with chILD, especially in A-DPLD disorders manifesting primarily in infancy.</abstract><cop>Berlin/Heidelberg</cop><pub>Springer Berlin Heidelberg</pub><pmid>37875631</pmid><doi>10.1007/s00431-023-05290-9</doi><tpages>10</tpages></addata></record> |
fulltext | fulltext |
identifier | ISSN: 1432-1076 |
ispartof | European journal of pediatrics, 2024-01, Vol.183 (1), p.295-304 |
issn | 1432-1076 0340-6199 1432-1076 |
language | eng |
recordid | cdi_proquest_miscellaneous_2881710725 |
source | Springer Link |
subjects | Bronchiectasis Child Childhood Children Diagnosis Humans Lung Lung diseases Lung Diseases, Interstitial - diagnosis Lung Diseases, Interstitial - epidemiology Lung Diseases, Interstitial - therapy Lymphadenopathy Medicine Medicine & Public Health Patients Pediatrics Population studies Prospective Studies Registries Steroid hormones Turkey - epidemiology |
title | Childhood interstitial lung disease in Turkey: first data from the national registry |
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