{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T01:29:44Z","timestamp":1760059784821,"version":"build-2065373602"},"reference-count":15,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2025,7,9]],"date-time":"2025-07-09T00:00:00Z","timestamp":1752019200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: An increase in hemoglobin (Hb) has been reported in subjects with CF treated with the CFTR modulator Ivacaftor and with the combination Lumacaftor\/Ivacaftor (LI), while the literature about the impact of Elexacaftor\/Tezacaftor\/Ivacaftor (ETI) on Hb levels in the pediatric population is lacking. Materials and Methods: We retrospectively evaluated Hb levels in 35 subjects with CF (18 males, median age: 8 years; interquartile range (IQR): 6\u201313 years) treated with LI and 60 (24 males, median age: 10 years; IQR: 6\u201314 years) treated with ETI. For each subject we considered the values of Hb, serum potassium, total bilirubin (TB), and conjugated bilirubin (CB) at baseline, after 3 days, and 1, 3, 6, 9, and 12 months from the start of treatment. Results: In subjects with CF treated with LI, we observed a significant increase in Hb values 3 days after the introduction of the drug, which remained constant throughout the year of treatment. In subjects treated with ETI, a significant decrease in Hb was observed 3 days after the first dose up to 1 month. At 6 months, Hb returned to pre-treatment values remaining stable for up to 12 months. At 3 days of treatment, we also observed a significant increase in serum potassium, which returned to normal at one month, while both TB and CB values significantly increased at 3 days of treatment and remained significantly higher for the whole one-year period of ETI therapy. Conclusions: We confirmed an increase in Hb values over time in subjects treated with LI. While the Hb response in those treated with ETI showed a transient reduction that lasted for one month, this may have depended on hemolysis, and returned to pre-treatment levels. Further studies will clarify the mechanisms that govern changes in Hb in subjects with CF treated with ETI.<\/jats:p>","DOI":"10.3390\/jcm14144856","type":"journal-article","created":{"date-parts":[[2025,7,11]],"date-time":"2025-07-11T10:26:53Z","timestamp":1752229613000},"page":"4856","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Hemoglobin Levels in Children Treated for Cystic Fibrosis with CFTR Modulators: A Single Center Retrospective Study"],"prefix":"10.3390","volume":"14","author":[{"given":"Antonella","family":"Tosco","sequence":"first","affiliation":[{"name":"Paediatric Unit, Cystic Fibrosis Regional Reference Center, Department of Maternal and Child Health, University Hospital Federico II, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-2239-5776","authenticated-orcid":false,"given":"Raffaele","family":"Cerchione","sequence":"additional","affiliation":[{"name":"Paediatric Unit, Cystic Fibrosis Regional Reference Center, Department of Translational Medical Sciences, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1500-0746","authenticated-orcid":false,"given":"Monica","family":"Gelzo","sequence":"additional","affiliation":[{"name":"Centro di Ingegneria Genetica e Biotecnologie Avanzate F. Salvatore (CEINGE) Advanced Biotechnology Franco Salvatore, 80145 Naples, Italy"},{"name":"Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chiara","family":"Cimbalo","sequence":"additional","affiliation":[{"name":"Paediatric Unit, Cystic Fibrosis Regional Reference Center, Department of Translational Medical Sciences, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alice","family":"Castaldo","sequence":"additional","affiliation":[{"name":"Pediatric Pneumology and Sub Intensive Care and Rehabilitation Unit, Santobono-Pausillipon Hospital, 80129 Naples, Italy"},{"name":"Department of Public Health, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rosamaria","family":"Terracciano","sequence":"additional","affiliation":[{"name":"Paediatric Unit, Cystic Fibrosis Regional Reference Center, Department of Translational Medical Sciences, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Valeria","family":"Raia","sequence":"additional","affiliation":[{"name":"Paediatric Unit, Cystic Fibrosis Regional Reference Center, Department of Translational Medical Sciences, University of Naples Federico II, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angela","family":"Sepe","sequence":"additional","affiliation":[{"name":"Paediatric Unit, Cystic Fibrosis Regional Reference Center, Department of Maternal and Child Health, University Hospital Federico II, 80131 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,7,9]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Lopes-Pacheco, M. 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