{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,14]],"date-time":"2026-02-14T05:02:58Z","timestamp":1771045378365,"version":"3.50.1"},"reference-count":14,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2024,7,11]],"date-time":"2024-07-11T00:00:00Z","timestamp":1720656000000},"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":[[2024,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>New York (NY) State implemented a new cystic fibrosis (CF) newborn screen (NBS) algorithm in December 2017 with improvement in positive predictive value and unanticipated increased identification of infants with cystic fibrosis transmembrane conductance regulator (<jats:italic>CFTR<\/jats:italic>)\u2010related metabolic syndrome (CRMS). Repeat sweat testing is recommended in infants with CRMS. During the COVID\u201019 pandemic infants with CRMS were lost to follow up. With this quality improvement (QI) initiative, we aimed to perform repeat sweat testing in 25% of infants lost to follow up. We also describe consensus recommendations for CRMS from the NY CF NBS Consortium.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Our QI team identified the primary drivers contributing to absent follow up, outreached to families, and created a questionnaire to evaluate parental understanding of CRMS using QI\u2010based strategies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 350 infants diagnosed with CRMS during the study period, 179 (51.1%) infants were lost to follow up. A total of 31 (17.3%) were scheduled for repeat sweat tests and followed up at CF Centers. Families reported high satisfaction with the CRMS knowledge questionnaire.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>With this QI\u2010based approach, we effectively recaptured infants with CRMS previously lost to follow up during the COVID\u201019 pandemic. Ongoing concerns about infection risk and lack of understanding on the part of families and pediatricians likely contributed to patients with CRMS lost to follow up. Consensus recommendations for CRMS include annual visits with repeat sweat testing until 2\u20136 years of age and education for adolescents about clinical and reproductive implications of CRMS.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.27160","type":"journal-article","created":{"date-parts":[[2024,7,11]],"date-time":"2024-07-11T13:45:58Z","timestamp":1720705558000},"page":"2932-2938","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["New York cystic fibrosis consortium newborn screening quality improvement: Development and implementation of a statewide consensus recommendations for management of infants with <i>CFTR<\/i>\u2010related metabolic syndrome"],"prefix":"10.1002","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-7871-2574","authenticated-orcid":false,"given":"Saroj","family":"Choudhary","sequence":"first","affiliation":[{"name":"Division of Pulmonary Medicine, Department of Pediatrics New York University Langone Hospital\u2010Long Island Long Island New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eleanor D.","family":"Muise","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine, Department of Pediatrics New York University New York City New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Soumia","family":"Hammouda","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine, Department of Pediatrics New York University New York City New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5467-5423","authenticated-orcid":false,"given":"Danielle","family":"Goetz","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine, Department of Pediatrics University of Buffalo Buffalo New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert","family":"Giusti","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Medicine, Department of Pediatrics New York University New York City New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2024,7,11]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.3390\/life13081646"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0b013e328353489a"},{"key":"e_1_2_11_4_1","unstructured":"The Clinical and Functional TRanslation of CFTR (CFTR2); available athttp:\/\/cftr2.org"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijns7040073"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2009.09.003"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2015-0811"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2015.01.052"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2014-3698"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2014-2081"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2019.04.002"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1089\/gtmb.2016.0102"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24634"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2020.11.006"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2022.12.002"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.27160","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,10]],"date-time":"2024-10-10T15:04:46Z","timestamp":1728572686000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.27160"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,7,11]]},"references-count":14,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2024,11]]}},"alternative-id":["10.1002\/ppul.27160"],"URL":"https:\/\/doi.org\/10.1002\/ppul.27160","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,7,11]]},"assertion":[{"value":"2023-08-04","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-06-20","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-07-11","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}