{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T05:09:56Z","timestamp":1790744996240,"version":"4.1.0"},"reference-count":27,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2024,5,9]],"date-time":"2024-05-09T00:00:00Z","timestamp":1715212800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100010787","name":"Associazione Italiana Rett","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010787","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2024,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Despite the publication of the 2020 guidelines on how to manage Rett Syndrome (RS), some fundamental topics are still open, in particular respiratory problems.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>Identification and reinforcement of current recommendations concerning the management of respiratory issues in RS patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Materials and Methods<\/jats:title><jats:p>Using a Delphi approach, the leading group reviewed the literature and formulated 14 statements. A multidisciplinary panel of 29 experts were invited to score, for each statement, their agreement on a 1\u20135 scale. The cut\u2010off level for consensus was 75%, obtained through multiple rounds.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The panel agreed that in all RS types, respiratory issues should be faced at an early stage, regardless of epilepsy onset. It is recommended to perform periodically sleep studies in all Congenital Rett Syndrome,\u00a0and in selected cases with other RS types. Noninvasive ventilation\u00a0should be considered in all RS subjects with sleep respiratory disorders and in those with hypotonia associated with hypercapnia. Chest physiotherapy should be performed in all RS patients with difficult management of the accumulation of respiratory secretions, using airway clearance techniques and devices (PEP\u2010mask, AMBU bag, or cough machine), more appropriate\u00a0and tolerated by the patients. The panel recommended individualized programs for the management of scoliosis, and to consider performing gastrostomy\u00a0in patients at increased risk of <jats:italic>ab ingestis<\/jats:italic> pneumonia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This consensus could support everyday clinical practice on respiratory issues in RS patients, complementary to existing recommendations by regulatory agencies and guidelines.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.27030","type":"journal-article","created":{"date-parts":[[2024,5,9]],"date-time":"2024-05-09T10:48:08Z","timestamp":1715251688000},"page":"1970-1978","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Management of respiratory issues in patients with Rett syndrome: Italian experts' consensus using a Delphi approach"],"prefix":"10.1002","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1628-5488","authenticated-orcid":false,"given":"Claudio","family":"Cherchi","sequence":"first","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit Bambino Ges\u00f9 Children's Hospital, IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1476-4752","authenticated-orcid":false,"given":"Elena","family":"Chiappini","sequence":"additional","affiliation":[{"name":"Meyer Children's University Hospital, IRCCS Florence Italy"},{"name":"Division of Pediatric Infectious Diseases Infective Diseases, Anna Meyer Children's University Hospital Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Amaddeo","sequence":"additional","affiliation":[{"name":"Institute for Maternal and Child Health \u2013 IRCCS \u201cBurlo Garofolo\u201d Trieste Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Beatrice","family":"Chiarini Testa","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit Bambino Ges\u00f9 Children's Hospital, IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Banfi","sequence":"additional","affiliation":[{"name":"Heart\u2010Respiratory Rehabilitation Unit IRCCS Fondazione Don Carlo Gnocchi Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edvige","family":"Veneselli","sequence":"additional","affiliation":[{"name":"Child Neuropsychiatry, Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics and Maternal and Child Health DINOG\u2010MI University of Genoa Genoa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7711-5672","authenticated-orcid":false,"given":"Renato","family":"Cutrera","sequence":"additional","affiliation":[{"name":"Pediatric Pulmonology and Cystic Fibrosis Unit Bambino Ges\u00f9 Children's Hospital, IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the panel for the Problems in Patients with Rett Syndrome","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,5,9]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/13810"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2007.10.001"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.63.9.1039"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.abb.2021.108768"},{"key":"e_1_2_13_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.braindev.2007.04.001"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2018.09.003"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.5664\/jcsm.7974"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.37784"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11325-012-0654-x"},{"key":"e_1_2_13_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/phar.1686"},{"key":"e_1_2_13_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjpo-2020-000717"},{"key":"e_1_2_13_13_1","doi-asserted-by":"publisher","DOI":"10.3310\/hta2030"},{"key":"e_1_2_13_14_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2648.2000.t01-1-01567.x"},{"key":"e_1_2_13_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23114"},{"key":"e_1_2_13_16_1","doi-asserted-by":"publisher","DOI":"10.1155\/2019\/6956934"},{"key":"e_1_2_13_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2016.03.016"},{"key":"e_1_2_13_18_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13052-020-0778-8"},{"key":"e_1_2_13_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-323-44887-1.00017-1"},{"key":"e_1_2_13_20_1","doi-asserted-by":"publisher","DOI":"10.12968\/bjon.2009.18.19.44820"},{"key":"e_1_2_13_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2017.08.003"},{"key":"e_1_2_13_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2018.01.012"},{"key":"e_1_2_13_23_1","doi-asserted-by":"publisher","DOI":"10.1097\/BRS.0000000000001399"},{"key":"e_1_2_13_24_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2017-1233"},{"key":"e_1_2_13_25_1","doi-asserted-by":"publisher","DOI":"10.3148\/66.1.2005.19"},{"key":"e_1_2_13_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0b013e31829e0b65"},{"key":"e_1_2_13_27_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijerph182413372"},{"key":"e_1_2_13_28_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2648.2003.02537.x"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.27030","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,6,21]],"date-time":"2024-06-21T13:35:11Z","timestamp":1718976911000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.27030"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5,9]]},"references-count":27,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2024,7]]}},"alternative-id":["10.1002\/ppul.27030"],"URL":"https:\/\/doi.org\/10.1002\/ppul.27030","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,5,9]]},"assertion":[{"value":"2023-12-03","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-04-14","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-05-09","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}