{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Wongjarupong N"],"funding":["National Institutes of Health&apos;s National Center for Advancing Translational Sciences","Cystic Fibrosis Foundation"],"pagination":["e0328015"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC12303333"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["20(7)"],"pubmed_abstract":["<h4>Background</h4>Gastrointestinal (GI) complications are the second most common disorders in persons with cystic fibrosis (PwCF). There is limited data on how having a dedicated CF-GI clinic and cystic fibrosis transmembrane conductance regulator (CFTR) modulators may affect rates of GI complications. Our aim was to assess the effect of the CF-GI clinic and CFTR modulators on GI complications with incidence of distal intestinal obstructive syndrome (DIOS).<h4>Methods</h4>This was a retrospective study of adult PwCF who were seen in a CF-GI clinic from 2000-2023. Comparisons were made between the numbers of admissions and emergency department (ED) visits for DIOS at three years before and after CFTR modulator use and the first CF-GI clinic visit.<h4>Results</h4>Of the 1,076 PwCF identified, 242 were seen in CF-GI clinic. Of this, 126 (52.1%) were female, with a median age of 40 (IQR: 30-47) years. There were 146 (60.3%) with regular use of laxatives. Of the 59 PwCF with CF-GI clinic visits for constipation, hospital admissions decreased in 16, were unchanged in 32, and increased in 11 (p = 0.402) while ED visits decreased in 9, remained the same in 40, and increased in 10 (p = 0.862). Of the 125 PwCF with CFTR modulator use, DIOS-related hospital admissions decreased in 15 patients, remained unchanged in 89, and increased in 21 (p = 0.021) while ED visits were fewer in 8, unchanged in 97, and increased in 20 (p = 0.587).<h4>Conclusion</h4>PwCF had high burden of constipation with a majority of patients regularly using laxatives, and almost half had a history of DIOS. CFTR modulator use and CF-GI clinic were not associated with a decrease of DIOS incidence."],"journal":["PloS one"],"pubmed_title":["Effect of cystic fibrosis transmembrane conductance regulator modulators and dedicated cystic fibrosis gastrointestinal clinic visits on the incidence of distal intestinal obstructive syndrome in persons with cystic fibrosis."],"pmcid":["PMC12303333"],"funding_grant_id":["UM1TR004405","00451A121","NICE-CF, PROMISE-OB-18, DIGEST 4, and STRONG-CF"],"pubmed_authors":["Benner A","Moshiree B","Alp J","Dunitz JM","Billings JL","Sultan S","Wongjarupong N","Delbrune MF","Moutsoglou DM","Wiggen T","Schwarzenberg SJ"],"additional_accession":[]},"is_claimable":false,"name":"Effect of cystic fibrosis transmembrane conductance regulator modulators and dedicated cystic fibrosis gastrointestinal clinic visits on the incidence of distal intestinal obstructive syndrome in persons with cystic fibrosis.","description":"<h4>Background</h4>Gastrointestinal (GI) complications are the second most common disorders in persons with cystic fibrosis (PwCF). There is limited data on how having a dedicated CF-GI clinic and cystic fibrosis transmembrane conductance regulator (CFTR) modulators may affect rates of GI complications. Our aim was to assess the effect of the CF-GI clinic and CFTR modulators on GI complications with incidence of distal intestinal obstructive syndrome (DIOS).<h4>Methods</h4>This was a retrospective study of adult PwCF who were seen in a CF-GI clinic from 2000-2023. Comparisons were made between the numbers of admissions and emergency department (ED) visits for DIOS at three years before and after CFTR modulator use and the first CF-GI clinic visit.<h4>Results</h4>Of the 1,076 PwCF identified, 242 were seen in CF-GI clinic. Of this, 126 (52.1%) were female, with a median age of 40 (IQR: 30-47) years. There were 146 (60.3%) with regular use of laxatives. Of the 59 PwCF with CF-GI clinic visits for constipation, hospital admissions decreased in 16, were unchanged in 32, and increased in 11 (p = 0.402) while ED visits decreased in 9, remained the same in 40, and increased in 10 (p = 0.862). Of the 125 PwCF with CFTR modulator use, DIOS-related hospital admissions decreased in 15 patients, remained unchanged in 89, and increased in 21 (p = 0.021) while ED visits were fewer in 8, unchanged in 97, and increased in 20 (p = 0.587).<h4>Conclusion</h4>PwCF had high burden of constipation with a majority of patients regularly using laxatives, and almost half had a history of DIOS. CFTR modulator use and CF-GI clinic were not associated with a decrease of DIOS incidence.","dates":{"release":"2025-01-01T00:00:00Z","publication":"2025","modification":"2026-03-27T17:01:14.807Z","creation":"2025-09-01T03:05:24.775Z"},"accession":"S-EPMC12303333","cross_references":{"pubmed":["40720490"],"doi":["10.1371/journal.pone.0328015"]}}