{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T16:09:17Z","timestamp":1789056557801,"version":"build-2803163510"},"reference-count":11,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/100011022","name":"Vertex Pharmaceuticals Incorporated","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100011022","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","resmedjournal.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Respiratory Medicine"],"published-print":{"date-parts":[[2026,10]]},"DOI":"10.1016\/j.rmed.2026.109079","type":"journal-article","created":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T06:47:50Z","timestamp":1785826070000},"page":"109079","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":0,"special_numbering":"C","title":["Clinical characteristics and outcomes of people with cystic fibrosis with homozygous minimal function genotypes compared to those with F508del\/minimal function genotypes"],"prefix":"10.1016","volume":"262","author":[{"given":"Michael W.","family":"Konstan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-3841-4564","authenticated-orcid":false,"given":"Brandon","family":"Lumsden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prema","family":"Menon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maral","family":"DerSarkissian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megan","family":"Rice","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.rmed.2026.109079_bib1","doi-asserted-by":"crossref","DOI":"10.1016\/j.jcf.2023.03.002","article-title":"Real-world safety and effectiveness of elexacaftor\/tezacaftor\/ivacaftor in people with cystic fibrosis: interim results of a long-term registry-based study","author":"Bower","year":"2023","journal-title":"J. Cyst. Fibros."},{"issue":"19","key":"10.1016\/j.rmed.2026.109079_bib2","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"Elexacaftor-tezacaftor-ivacaftor for cystic fibrosis with a single Phe508del allele","volume":"381","author":"Middleton","year":"2019","journal-title":"N. Engl. J. Med."},{"issue":"8","key":"10.1016\/j.rmed.2026.109079_bib3","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1136\/thoraxjnl-2017-210394","article-title":"Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor","volume":"73","author":"Bessonova","year":"2018","journal-title":"Thorax"},{"issue":"2","key":"10.1016\/j.rmed.2026.109079_bib4","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1016\/j.jcf.2024.07.015","article-title":"Characteristics of individuals with cystic fibrosis in the United States ineligible for ivacaftor and elexacaftor\/tezacaftor\/ivacaftor","volume":"24","author":"Sanders","year":"2025","journal-title":"J. Cyst. Fibros."},{"issue":"3","key":"10.1016\/j.rmed.2026.109079_bib5","doi-asserted-by":"crossref","first-page":"414","DOI":"10.1016\/j.jcf.2022.12.007","article-title":"Lived experiences of people with cystic fibrosis that were not eligible for elexacaftor-tezacaftor-ivacaftor (ETI): a qualitative study","volume":"22","author":"Milo","year":"2023","journal-title":"J. Cyst. Fibros."},{"issue":"3","key":"10.1016\/j.rmed.2026.109079_bib6","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1016\/j.jcf.2011.01.005","article-title":"Association between genotype and pulmonary phenotype in cystic fibrosis patients with severe mutations","volume":"10","author":"Geborek","year":"2011","journal-title":"J. Cyst. Fibros."},{"key":"10.1016\/j.rmed.2026.109079_bib7","doi-asserted-by":"crossref","first-page":"S3","DOI":"10.1016\/S1569-1993(24)00115-2","article-title":"WS02.03 disease burden in people with cystic fibrosis according to CFTR genotype and eligibility to CFTR modulator therapy: a ECFS patient registry analysis","volume":"23","author":"Tomarelli","year":"2024","journal-title":"J. Cyst. Fibros."},{"issue":"6","key":"10.1016\/j.rmed.2026.109079_bib8","doi-asserted-by":"crossref","first-page":"1324","DOI":"10.1183\/09031936.00080312","article-title":"Multi-ethnic reference values for spirometry for the 3-95-yr age range: the global lung function 2012 equations","volume":"40","author":"Quanjer","year":"2012","journal-title":"Eur. Respir. J."},{"issue":"6","key":"10.1016\/j.rmed.2026.109079_bib9","doi-asserted-by":"crossref","first-page":"768","DOI":"10.1164\/rccm.202205-0963OC","article-title":"A race-neutral approach to the interpretation of lung function measurements","volume":"207","author":"Bowerman","year":"2023","journal-title":"Am. J. Respir. Crit. Care Med."},{"issue":"3","key":"10.1016\/j.rmed.2026.109079_bib10","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1016\/j.jcf.2022.12.009","article-title":"Effect of Elexacaftor\/Tezacaftor\/Ivacaftor on annual rate of lung function decline in people with cystic fibrosis","volume":"22","author":"Lee","year":"2023","journal-title":"J. Cyst. Fibros."},{"issue":"3","key":"10.1016\/j.rmed.2026.109079_bib11","doi-asserted-by":"crossref","first-page":"436","DOI":"10.1016\/j.jcf.2023.02.009","article-title":"Cystic fibrosis prevalence in the United States and participation in the Cystic Fibrosis Foundation Patient Registry in 2020","volume":"22","author":"Cromwell","year":"2023","journal-title":"J. Cyst. Fibros."}],"container-title":["Respiratory Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0954611126004476?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0954611126004476?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T15:42:03Z","timestamp":1789054923000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0954611126004476"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10]]},"references-count":11,"alternative-id":["S0954611126004476"],"URL":"https:\/\/doi.org\/10.1016\/j.rmed.2026.109079","relation":{},"ISSN":["0954-6111"],"issn-type":[{"value":"0954-6111","type":"print"}],"subject":[],"published":{"date-parts":[[2026,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Clinical characteristics and outcomes of people with cystic fibrosis with homozygous minimal function genotypes compared to those with F508del\/minimal function genotypes","name":"articletitle","label":"Article Title"},{"value":"Respiratory Medicine","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.rmed.2026.109079","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2026 Elsevier Ltd. All rights are reserved, including those for text and data mining, AI training, and similar technologies.","name":"copyright","label":"Copyright"}],"article-number":"109079"}}