{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T15:57:00Z","timestamp":1792166220371,"version":"4.4.0"},"reference-count":28,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2023,1,9]],"date-time":"2023-01-09T00:00:00Z","timestamp":1673222400000},"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":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>There are no recent data on primary ciliary dyskinesia (PCD) distribution, diagnosis and treatment in Italy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A descriptive study based on a survey questionnaire. It consisted of three sections (patients, diagnosis, and treatment), and sent to all the Italian PCD Centers.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Questionnaires obtained from 20\/22 centers in 12\/20 regions showed that the total number of PCD patients treated at the participating centers was of 416. Out of all centers, 55% follow &lt;20 patients, two centers have &gt;40 patients, and 75% follow both pediatric and adults. Age at diagnosis was between 4 and 8 years in 45% of the centers, &lt;3 years in three centers. Nasal nitric oxide, transmission electron microscopy and ciliary high\u2010speed video microscopy are performed in 75%, 90%, and 40% of centers, respectively. Immunofluorescence is available in five centers. Genetic analysis is offered in 55% of the centers, and in seven centers &gt;50% of the patients have a known genetic profile. Patients treated at all centers receive inhaled saline solutions, corticosteroids and chest physiotherapy. Prophylactic antibiotics and mucolytics are prescribed in 95% and 50% of the centers, respectively. Pseudomonas infection is treated with oral or inhaled antibiotics.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Many Italian centers care for a small number of pediatric and adult patients, and diagnosis is often delayed. We found a great variability in the available diagnostic procedures, as well in the prescribed therapies. Our study will help to uniform diagnostic algorithm and share treatments protocols for PCD in Italy and allowed to set specific national goals.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.26303","type":"journal-article","created":{"date-parts":[[2023,1,1]],"date-time":"2023-01-01T22:59:41Z","timestamp":1672613981000},"page":"1127-1135","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Primary ciliary dyskinesia: A multicenter survey on clinical practice and patient management in Italy"],"prefix":"10.1002","volume":"58","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1111-5690","authenticated-orcid":false,"given":"Nicola","family":"Ullmann","sequence":"first","affiliation":[{"name":"Academic Department of Pediatrics (DPUO) Pediatric Pulmonology &amp; Respiratory Intermediate Care Unit, Sleep and Long Term Ventilation Unit, Bambino Ges\u00f9 Children's Hospital, IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9375-7429","authenticated-orcid":false,"given":"Francesca","family":"Santamaria","sequence":"additional","affiliation":[{"name":"Department of Translational Medical Sciences, Pediatric Pulmonology Federico II University  Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4685-7205","authenticated-orcid":false,"given":"Annalisa","family":"Allegorico","sequence":"additional","affiliation":[{"name":"Academic Department of Pediatrics (DPUO) Pediatric Pulmonology &amp; Respiratory Intermediate Care Unit, Sleep and Long Term Ventilation Unit, Bambino Ges\u00f9 Children's Hospital, IRCCS  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4601-7484","authenticated-orcid":false,"given":"Valentina","family":"Fainardi","sequence":"additional","affiliation":[{"name":"Department of Medicine and Surgery, Pediatric Clinic, Pietro Barilla Children's Hospital University of Parma  Parma Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melissa","family":"Borrelli","sequence":"additional","affiliation":[{"name":"Department of Translational Medical Sciences, Pediatric Pulmonology Federico II University  Naples Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina A.","family":"Ferraro","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health University of Padua  Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Proietti","sequence":"additional","affiliation":[{"name":"Pediatric Unit, Rovereto Hospital Rovereto Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4291-0195","authenticated-orcid":false,"given":"Giuseppe F.","family":"Parisi","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine University of Catania  Catania Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2047-5340","authenticated-orcid":false,"given":"Vittorio","family":"Romagnoli","sequence":"additional","affiliation":[{"name":"Department of Mother and Child Health Pediatric Unit,\u00a0G. Salesi Children's Hospital Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Lucca","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Centre, Azienda Ospedaliero\u2010Universitaria Integrata di Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcella","family":"Gallucci","sequence":"additional","affiliation":[{"name":"Pediatric Unit\u2010IRCCS Azienda Ospedaliero\u2010Universitaria di Bologna  Bologna Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luigi","family":"Mappa","sequence":"additional","affiliation":[{"name":"Department of Interdisciplinary Medicine (D.I.M.), Pediatric Unit \u201cTrambusti\u201d Cystic Fibrosis University of Bari Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mara","family":"Lelii","sequence":"additional","affiliation":[{"name":"Pediatric Highly Intensive Care Unit, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doriana","family":"Amato","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Hospital Giovanni XXIII University of Bari  Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Petrarca","sequence":"additional","affiliation":[{"name":"Department of Maternal Infantile and Urological Sciences Sapienza University of Rome  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Cimino","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Regional Reference Center, A.O.U. 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