{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,5]],"date-time":"2026-01-05T22:00:49Z","timestamp":1767650449714},"reference-count":30,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2021,7,15]],"date-time":"2021-07-15T00:00:00Z","timestamp":1626307200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2021,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background:<\/jats:title><jats:p>Noncystic fibrosis bronchiectasis (NCFB) is still considered an \u201corphan disease\u201d in pediatric age.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective:<\/jats:title><jats:p>The study describes the clinical and functional features, the instrumental, and microbial findings of a large cohort of patients with NCFB, followed in a single tertiary level hospital.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods:<\/jats:title><jats:p>Children and adolescents diagnosed with NCFB from January\u00a01, 2010 to December\u00a031, 2019 were included. Data from the diagnosis and during the years of follow\u2010up were recorded retrospectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p>One hundred and thirty\u2010eight patients were enrolled. The most common cause of NCFB was postinfectious (33%), followed by primary ciliary dyskinesia (PCD) (30%), esophageal atresia (EA) (9.5%), and secondary immunodeficiency (9.5%). Chronic cough was the most frequent symptom. The median age of symptoms presentation was 3 years (interquartile age [IQR]: 12\u201384), with a precocious onset in PCD and EA groups. The median age of CT diagnosis was 9 years for all groups but PCD patients who were diagnosed at older age. Lingula, medium, upper, and lower lobes were more involved in PCD group, while diffuse distribution was observed in the postinfectious one. Microbial exams showed <jats:italic>Pseudomonas aeruginosa<\/jats:italic> colonization higher in PCD patients (22%). Despite microbial differences in airways colonization, no difference in respiratory exacerbation rate was recorded among groups. Lung function tests demonstrated the stability of forced expiratory volume in 1\u2009s (FEV1) and forced vital capacity (FVC) over time, except for the secondary immunodeficiency group.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions:<\/jats:title><jats:p>The role of infections in developed countries should not be underestimated and a major effort to obtain an earlier identification of bronchiectasis should be taken. A prompt diagnosis of NFCB could help to reduce the frequency of exacerbations and improve the stability of lung function over time.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.25553","type":"journal-article","created":{"date-parts":[[2021,7,15]],"date-time":"2021-07-15T20:18:57Z","timestamp":1626380337000},"page":"3026-3034","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Noncystic fibrosis bronchiectasis in children and adolescents: Follow\u2010up over a decade"],"prefix":"10.1002","volume":"56","author":[{"given":"Nicola","family":"Ullmann","sequence":"first","affiliation":[{"name":"Pediatric Pulmonology &amp; Respiratory Intermediate Care Unit, Sleep and Long\u2010Term Ventilation Unit, Academic Department of Pediatrics Bambino Ges\u00f9 Children's Hospital, IRCCS Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federica","family":"Porcaro","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology &amp; Respiratory Intermediate Care Unit, Sleep and Long\u2010Term Ventilation Unit, Academic Department of Pediatrics Bambino Ges\u00f9 Children's Hospital, IRCCS Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesca","family":"Petreschi","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology &amp; Respiratory Intermediate Care Unit, Sleep and Long\u2010Term Ventilation Unit, Academic Department of Pediatrics Bambino Ges\u00f9 Children's Hospital, IRCCS Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michela","family":"Cammerata","sequence":"additional","affiliation":[{"name":"Academic Department of Pediatrics Tor Vergata University Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annalisa","family":"Allegorico","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology &amp; Respiratory Intermediate Care Unit, Sleep and Long\u2010Term Ventilation Unit, Academic Department of Pediatrics Bambino Ges\u00f9 Children's Hospital, IRCCS Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Valentina","family":"Negro","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology &amp; Respiratory Intermediate Care Unit, Sleep and Long\u2010Term Ventilation Unit, Academic Department of Pediatrics Bambino Ges\u00f9 Children's Hospital, IRCCS Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Renato","family":"Cutrera","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology &amp; Respiratory Intermediate Care Unit, Sleep and Long\u2010Term Ventilation Unit, Academic Department of Pediatrics Bambino Ges\u00f9 Children's Hospital, IRCCS Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,7,15]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23380"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2017.00027"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(18)31554-X"},{"key":"e_1_2_10_5_1","first-page":"6","article-title":"Inflammation: a two\u2010edged sword\u2014the model of bronchiectasis","volume":"147","author":"Cole PJ","year":"1986","journal-title":"Eur J Respir Dis Suppl"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12890-020-01214-7"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201303-0411CI"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1183\/20734735.0261-2019"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13052-017-0434-0"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-013-3420-7"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pedneo.2019.09.013"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2016.09.064"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01090-2016"},{"key":"e_1_2_10_14_1","unstructured":"Bronchiectasis: Mechanisms and Imaging Clues of Associated Common and Uncommon Diseases | RadioGraphics;2021.\u00a0https:\/\/pubs.rsna.org\/doi\/full\/10.1148\/rg.2015140214. 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