{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T10:03:16Z","timestamp":1791453796848,"version":"4.3.3"},"reference-count":15,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2025,7,16]],"date-time":"2025-07-16T00:00:00Z","timestamp":1752624000000},"content-version":"vor","delay-in-days":15,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,7,1]],"date-time":"2025-07-01T00:00:00Z","timestamp":1751328000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>With routine newborn screening for cystic fibrosis (CF) now considered standard of care, the designation of CFTR Related Metabolic Syndrome (CRMS) or CF screen positive, indeterminate diagnosis (CFSPID) has been established. The majority of CRMS\/CFSPID patients remain asymptomatic; however, 3.8%\u201344% of these patients may progress to a diagnosis of CF. This raises the question of how to optimally manage CRMS\/CFSPID patients. We set out to gain a better understanding of the past practices employed at CF centers across Canada in the care of patients with a diagnosis of CRMS\/CFSPID.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>An invitation to participate in an online survey was disseminated to CF centers in Canada through the REDCap database. The survey was completed in 2018. It included questions addressing patient population, timing of follow\u2010up of CRMS\/CFSPID patients, and details around specific investigations ordered.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Twelve out of 20 qualifying clinics completed the survey. The total patient population compiled included 1412 patients, of which 171 (12%) were classified as CRMS\/CFSPID. There was wide variability in the timing of follow\u2010up with a median (IQR) of 6 (5.25\u201312) months and a range of 3\u201312 months. There was also wide variability in the timing of repeat investigations such as sweat chloride, respiratory cultures, chest\n                      <jats:italic>x<\/jats:italic>\n                      \u2010rays and spirometry.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>With current evidence showing that a considerable number of CRMS\/CFSPID patients may progress to CF, ensuring these patients are identified as early as possible and followed in a consistent manner is essential. The Cystic Fibrosis Foundation and European Cystic Fibrosis Society have recently developed guidelines regarding the care of these patients. This survey describes historical practices for follow up of CRMS\/CFSPID patients to help inform the development of Canadian consensus guidelines.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71200","type":"journal-article","created":{"date-parts":[[2025,7,17]],"date-time":"2025-07-17T06:55:54Z","timestamp":1752735354000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Variations in the Management of Canadian Patients With CFTR Related Metabolic Syndrome\/Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CRMS\/CFSPID)"],"prefix":"10.1002","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4917-2842","authenticated-orcid":false,"given":"Dana","family":"Chemali","sequence":"first","affiliation":[{"name":"Schulich School of Medicine and Dentistry Western University London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erin","family":"Fleischer","sequence":"additional","affiliation":[{"name":"London Health Sciences Centre Children's Hospital London Ontario Canada"},{"name":"Arthur Labatt Family School of Nursing Western University London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan","family":"Uyeno","sequence":"additional","affiliation":[{"name":"Schulich School of Medicine and Dentistry Western University London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tara","family":"Mullowney","sequence":"additional","affiliation":[{"name":"Schulich School of Medicine and Dentistry Western University London Ontario Canada"},{"name":"London Health Sciences Centre Children's Hospital London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7994-8715","authenticated-orcid":false,"given":"April","family":"Price","sequence":"additional","affiliation":[{"name":"Schulich School of Medicine and Dentistry Western University London Ontario Canada"},{"name":"London Health Sciences Centre Children's Hospital London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,7,16]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2015.01.001"},{"key":"e_1_2_10_3_1","article-title":"Diagnosis of Cystic Fibrosis: Consensus Guidelines From the Cystic Fibrosis Foundation","volume":"181","author":"Farrell P. M.","year":"2017","journal-title":"Journal of Pediatrics"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2019.04.010"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2015-0811"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2014-2081"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-023-05359-5"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24634"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2021-051740"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2019.04.002"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2016.09.066"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2014-3698"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2023-064657"},{"issue":"3","key":"e_1_2_10_14_1","first-page":"40","article-title":"Updated Guidance on the Management of Children With Cystic Fibrosis Transmembrane Conductance Regulator\u2010Related Metabolic Syndrome\/Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CRMS\/CFSPID)","volume":"19","author":"Barben J.","year":"2020","journal-title":"Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2012.3532"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2016.12.012"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71200","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.71200","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71200","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,17]],"date-time":"2026-02-17T12:55:33Z","timestamp":1771332933000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.71200"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,7]]},"references-count":15,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2025,7]]}},"alternative-id":["10.1002\/ppul.71200"],"URL":"https:\/\/doi.org\/10.1002\/ppul.71200","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,7]]},"assertion":[{"value":"2024-12-14","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-07-05","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-07-16","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e71200"}}