{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T12:40:09Z","timestamp":1793796009901,"version":"4.6.1"},"reference-count":17,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,12,7]],"date-time":"2023-12-07T00:00:00Z","timestamp":1701907200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:p>With the introduction of elexacaftor\/tezacaftor\/ivacaftor (ETI), more women with cystic fibrosis (CF) are likely to grow families. Hence, an understanding long-term safety and effects of CFTR modulators on fertile women and children while monitoring their concentrations is crucial. Here, we report on the development of an improved LC\u2013MS\/MS methodology to measure ETI concentrations in maternal and child blood and breastmilk, applied in one case of successful pregnancy of a 30-year-old woman with CF (F508del\/R334W). We observed that ETI remains stable in breastmilk, is absorbed by the infant and can be detected in child plasma. Our results confirm accumulating evidence of a successful pregnancy in women treated with CFTR modulators without significant side effects on the child and provide valuable analytical procedures suitable for the post-marketing evaluation of CFTR modulators in pregnant and lactating women, as well as in their infants.<\/jats:p>","DOI":"10.3389\/fmed.2023.1274303","type":"journal-article","created":{"date-parts":[[2023,12,10]],"date-time":"2023-12-10T23:59:55Z","timestamp":1702252795000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":20,"title":["Maternal, newborn and breast milk concentrations of elexacaftor\/tezacaftor\/ivacaftor in a F508del heterozygous woman with cystic fibrosis following successful pregnancy"],"prefix":"10.3389","volume":"10","author":[{"given":"Pietro","family":"Ripani","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matteo","family":"Mucci","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Pantano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Di Sabatino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Collini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Ferri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Romano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Recchiuti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2023,12,7]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"1243","DOI":"10.3390\/cells11071243","article-title":"CFTR modulator therapies: potential impact on airway infections in cystic fibrosis","volume":"11","author":"Saluzzo","year":"2022","journal-title":"Cells"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"529","DOI":"10.1164\/rccm.202108-1986OC","article-title":"Clinical effectiveness of elexacaftor\/tezacftor\/ivacaftor in people with cystic fibrosis","volume":"205","author":"Nichols","year":"2021","journal-title":"Am J Respir Crit Care Med [Internet]"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"2706","DOI":"10.3390\/jcm9092706","article-title":"CFTR modulators: impact on fertility, pregnancy, and lactation in women with cystic fibrosis","volume":"9","author":"Taylor-Cousar","year":"2020","journal-title":"J Clin Med"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"721","DOI":"10.18553\/jmcp.2022.28.7.721","article-title":"Analyzing the use and impact of elexacaftor\/tezacaftor\/ivacaftor on total cost of care and other health care resource utilization in a commercially insured population","volume":"28","author":"Smith","year":"2022","journal-title":"JMCP"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"725","DOI":"10.1016\/j.jcf.2021.12.004","article-title":"Drug exposure to infants born to mothers taking Elexacaftor, Tezacaftor, and Ivacaftor","volume":"21","author":"Collins","year":"2022","journal-title":"J Cyst Fibros"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"101778","DOI":"10.1016\/j.rmcr.2022.101778","article-title":"Experience to date with CFTR modulators during pregnancy and breastfeeding in the British Columbia cystic fibrosis clinic","volume":"40","author":"Goodwin","year":"2022","journal-title":"Respir Med Case Rep"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"402","DOI":"10.1016\/j.jcf.2021.03.006","article-title":"Maternal and fetal outcomes following elexacaftor-tezacaftor-ivacaftor use during preg-nancy and lactation","volume":"20","author":"Taylor-Cousar","year":"2021","journal-title":"J Cyst Fibros"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"779","DOI":"10.1016\/j.jcf.2018.05.009","article-title":"Measured fetal and neonatal exposure to Lumacaftor and Ivacaftor during pregnancy and while breastfeeding","volume":"17","author":"Trimble","year":"2018","journal-title":"J Cyst Fibros"},{"key":"ref9","first-page":"906","article-title":"Differential cellular expression of cystic fibrosis transmembrane regulator in human reproductive tissues. Clues for the infertility in patients with cystic fibrosis","volume":"144","author":"Tizzano","year":"1994","journal-title":"Am J Pathol"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"167","DOI":"10.1097\/GCO.0b013e32835f1745","article-title":"Cystic fibrosis and fertility","volume":"25","author":"Ahmad","year":"2013","journal-title":"Curr Opin Obstet Gynecol"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"1","DOI":"10.19191\/EP22.4S2.060","article-title":"Registro italiano Fibrosi Cistica (RIFC). Rap-porto 2019-2020","volume":"46","author":"Campagna","year":"2021","journal-title":"E&P"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"1074","DOI":"10.1016\/j.jcf.2022.10.004","article-title":"Congenital bilateral cataracts in newborns ex-posed to elexacaftor-tezacaftor-ivacaftor in utero and while breast feeding","volume":"21","author":"Jain","year":"2022","journal-title":"J Cyst Fibros"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"721","DOI":"10.1016\/j.jcf.2022.04.005","article-title":"A case report of CFTR modulator administration via carrier mother to treat meconium ileus in a F508del homozygous fetus","volume":"21","author":"Szentpetery","year":"2022","journal-title":"J Cyst Fibros"},{"key":"ref14","volume-title":"Nutrient adequacy of exclusive breastfeeding for the term infant during the first six months of life.","author":"Butte","year":"2002"},{"key":"ref15","doi-asserted-by":"publisher","first-page":"82","DOI":"10.1515\/cclm-2021-0724","article-title":"Isotope dilution LC-MS\/MS quantification of the cystic fibrosis transmembrane conductance regulator (CFTR) modulators ivacaftor, lumacaftor, tezacaftor, elexacaftor, and their major metabolites in human serum","volume":"60","author":"Habler","year":"2022","journal-title":"Clin Chem Lab Med"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"628","DOI":"10.3390\/biomedicines11020628","article-title":"Simultaneous quantification of Ivacaftor, Te-zacaftor, and Elexacaftor in cystic fibrosis patients\u2019 plasma by a novel LC\u2013MS\/MS method","volume":"11","author":"Pigliasco","year":"2023","journal-title":"Biomedicine"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"e001289","DOI":"10.1136\/bmjresp-2022-001289","article-title":"Prospectively evaluating maternal and fetal out-comes in the era of CFTR modulators: the MAYFLOWERS observational clinical trial study design","volume":"9","author":"Jain","year":"2022","journal-title":"BMJ Open Resp Res"}],"container-title":["Frontiers in Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2023.1274303\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,12,11]],"date-time":"2023-12-11T00:00:01Z","timestamp":1702252801000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fmed.2023.1274303\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,12,7]]},"references-count":17,"alternative-id":["10.3389\/fmed.2023.1274303"],"URL":"https:\/\/doi.org\/10.3389\/fmed.2023.1274303","relation":{},"ISSN":["2296-858X"],"issn-type":[{"value":"2296-858X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,12,7]]},"article-number":"1274303"}}