Diagnostic Evaluation and Clinical Findings in Children With Persistent Tachypnea of Infancy and Neuroendocrine Cell Hyperplasia of Infancy: A European Multicenter Retrospective Study.
Honorata Marczak, Katarzyna Krenke, Matthias Griese, Joanna Peradzyńska, Joanna Lange, Marek Kulus, Magdalena Grochowska, Elias Seidl, Jean-Christophe Dubus, Julia Rodler, Nicolaus Schwerk, Julia Carlens, Oded Breuer, Avigdor Hevroni, Silvia Castillo-Corullón, Malena Aldeco, Frederik Fouirnaies Buchvald, Kim G Nielsen, Sarah Mayell, Alba Torrent, Maynor Bravo-López, Nicolas Regamey, Florian Stehling, Philipp Latzin, Anna Zschocke, Elpis Hatziagorou, Roser Ayats, Tuğba Şişmanlar Eyüboğlu, Ela Erdem, Marijke Proesmans, Steve Cunningham, Dafni Moriki, Sevgi Pekcan, Nazan Cobanoglu, Lutz Naehrlich, Christiane Lex, Nicola Ullmann, Winfried Baden, Dora Krikovszky, Mirella Gaboli, Nuria Diez Monge, David Naranjo Vivas, Sune Leisgaard Mørck Rubak, Brigitte Willemse, Laura Petrarca, Anna Wiemers, Dirk Schramm, Christoph Mueller, Freerk Prenzel, Vaclav Koucky, Juan A López-Andreu, Nadia Nathan
{"title":"Diagnostic Evaluation and Clinical Findings in Children With Persistent Tachypnea of Infancy and Neuroendocrine Cell Hyperplasia of Infancy: A European Multicenter Retrospective Study.","authors":"Honorata Marczak, Katarzyna Krenke, Matthias Griese, Joanna Peradzyńska, Joanna Lange, Marek Kulus, Magdalena Grochowska, Elias Seidl, Jean-Christophe Dubus, Julia Rodler, Nicolaus Schwerk, Julia Carlens, Oded Breuer, Avigdor Hevroni, Silvia Castillo-Corullón, Malena Aldeco, Frederik Fouirnaies Buchvald, Kim G Nielsen, Sarah Mayell, Alba Torrent, Maynor Bravo-López, Nicolas Regamey, Florian Stehling, Philipp Latzin, Anna Zschocke, Elpis Hatziagorou, Roser Ayats, Tuğba Şişmanlar Eyüboğlu, Ela Erdem, Marijke Proesmans, Steve Cunningham, Dafni Moriki, Sevgi Pekcan, Nazan Cobanoglu, Lutz Naehrlich, Christiane Lex, Nicola Ullmann, Winfried Baden, Dora Krikovszky, Mirella Gaboli, Nuria Diez Monge, David Naranjo Vivas, Sune Leisgaard Mørck Rubak, Brigitte Willemse, Laura Petrarca, Anna Wiemers, Dirk Schramm, Christoph Mueller, Freerk Prenzel, Vaclav Koucky, Juan A López-Andreu, Nadia Nathan","doi":"10.1016/j.chest.2025.02.023","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Persistent tachypnea of infancy (PTI) or neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease (chILD) that predominantly affects young children. Although it is one of the most common chILDs, no unified diagnostic approach specific to this condition exists.</p><p><strong>Research question: </strong>Are the clinical presentation and the diagnostic approach different in patients with PTI or NEHI among the European countries?</p><p><strong>Study design and methods: </strong>This was a European multicenter, retrospective, observational study. Data on clinical characteristics and diagnostic strategies in patients with PTI or NEHI were analyzed and compared across participating countries.</p><p><strong>Results: </strong>The study included 378 children with PTI or NEHI from 17 countries (63.5% male, 97.4% White) who received a diagnosis at a median age of 9 months (interquartile range, 6-13 months). The most common baseline symptoms were tachypnea, chest retractions, crackles on auscultation, hypoxemia, and failure to thrive. High-resolution CT (HRCT) imaging was performed in all patients, with most undergoing chest radiography, echocardiography, and immunology tests. Lung biopsy was carried out in 23.5% of patients, with a decreasing trend over time and variation by country; its use was associated with longer diagnostic delay. Histopathologic examination showed a hyperplasia of pulmonary neuroendocrine cells in 52.8% of patients. Genetic testing was rare, and its application varied significantly among countries. Additional investigations that do not have an established role, such as assessment for gastroesophageal reflux disease and OSA, infant pulmonary function tests, and lung ultrasound, were limited to single countries.</p><p><strong>Interpretation: </strong>Diagnosis of PTI or NEHI relies on clinical symptoms and HRCT imaging results, with lung biopsies less commonly performed. Differences exist among countries regarding the number and type of investigations. A need exists for guidelines that will uniform the diagnostic approach.</p>","PeriodicalId":9782,"journal":{"name":"Chest","volume":" ","pages":""},"PeriodicalIF":9.5000,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Chest","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.chest.2025.02.023","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CRITICAL CARE MEDICINE","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Persistent tachypnea of infancy (PTI) or neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease (chILD) that predominantly affects young children. Although it is one of the most common chILDs, no unified diagnostic approach specific to this condition exists.
Research question: Are the clinical presentation and the diagnostic approach different in patients with PTI or NEHI among the European countries?
Study design and methods: This was a European multicenter, retrospective, observational study. Data on clinical characteristics and diagnostic strategies in patients with PTI or NEHI were analyzed and compared across participating countries.
Results: The study included 378 children with PTI or NEHI from 17 countries (63.5% male, 97.4% White) who received a diagnosis at a median age of 9 months (interquartile range, 6-13 months). The most common baseline symptoms were tachypnea, chest retractions, crackles on auscultation, hypoxemia, and failure to thrive. High-resolution CT (HRCT) imaging was performed in all patients, with most undergoing chest radiography, echocardiography, and immunology tests. Lung biopsy was carried out in 23.5% of patients, with a decreasing trend over time and variation by country; its use was associated with longer diagnostic delay. Histopathologic examination showed a hyperplasia of pulmonary neuroendocrine cells in 52.8% of patients. Genetic testing was rare, and its application varied significantly among countries. Additional investigations that do not have an established role, such as assessment for gastroesophageal reflux disease and OSA, infant pulmonary function tests, and lung ultrasound, were limited to single countries.
Interpretation: Diagnosis of PTI or NEHI relies on clinical symptoms and HRCT imaging results, with lung biopsies less commonly performed. Differences exist among countries regarding the number and type of investigations. A need exists for guidelines that will uniform the diagnostic approach.
期刊介绍：
At CHEST, our mission is to revolutionize patient care through the collaboration of multidisciplinary clinicians in the fields of pulmonary, critical care, and sleep medicine. We achieve this by publishing cutting-edge clinical research that addresses current challenges and brings forth future advancements. To enhance understanding in a rapidly evolving field, CHEST also features review articles, commentaries, and facilitates discussions on emerging controversies. We place great emphasis on scientific rigor, employing a rigorous peer review process, and ensuring all accepted content is published online within two weeks.
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号
