{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T17:02:50Z","timestamp":1790528570597,"version":"4.1.0"},"reference-count":112,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2021,2,22]],"date-time":"2021-02-22T00:00:00Z","timestamp":1613952000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Senthilkumar Sankararaman is supported by the Developing Innovative Gastroenterology Specialty Training Program grant from the Cystic Fibrosis Foundation","award":["SANKAR19GE0"],"award-info":[{"award-number":["SANKAR19GE0"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Metabolites"],"abstract":"<jats:p>Cystic fibrosis (CF) is the most common lethal, multisystemic genetic disorder in Caucasians. Mutations in the gene encoding the cystic fibrosis transmembrane regulator (CFTR) protein are responsible for impairment of epithelial anionic transport, leading to impaired fluid regulation and pH imbalance across multiple organs. Gastrointestinal (GI) manifestations in CF may begin in utero and continue throughout the life, resulting in a chronic state of an altered intestinal milieu. Inherent dysfunction of CFTR leads to dysbiosis of the gut. This state of dysbiosis is further perpetuated by acquired factors such as use of antibiotics for recurrent pulmonary exacerbations. Since the gastrointestinal microbiome and their metabolites play a vital role in nutrition, metabolic, inflammatory, and immune functions, the gut dysbiosis will in turn impact various manifestations of CF\u2014both GI and extra-GI. This review focuses on the consequences of gut dysbiosis and its metabolic implications on CF disease and possible ways to restore homeostasis.<\/jats:p>","DOI":"10.3390\/metabo11020123","type":"journal-article","created":{"date-parts":[[2021,2,22]],"date-time":"2021-02-22T20:42:51Z","timestamp":1614026571000},"page":"123","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":64,"title":["Impact of Altered Gut Microbiota and Its Metabolites in Cystic Fibrosis"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4966-5113","authenticated-orcid":false,"given":"Aravind","family":"Thavamani","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Gastroenterology, UH Rainbow Babies &amp; Children\u2019s Hospital, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iman","family":"Salem","sequence":"additional","affiliation":[{"name":"Center for Medial Mycology, Case Western Reserve University School of Medicine, UH Cleveland Medical Center, Cleveland, OH 44106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas J.","family":"Sferra","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Gastroenterology, UH Rainbow Babies &amp; Children\u2019s Hospital, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3094-9703","authenticated-orcid":false,"given":"Senthilkumar","family":"Sankararaman","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Gastroenterology, UH Rainbow Babies &amp; Children\u2019s Hospital, Case Western Reserve University School of Medicine, Cleveland, OH 44106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,2,22]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1146\/annurev.genom.6.080604.162254","article-title":"Modifier Genetics: Cystic Fibrosis","volume":"6","author":"Cutting","year":"2005","journal-title":"Annu. 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