{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T00:00:18Z","timestamp":1793059218604,"version":"4.5.0"},"reference-count":58,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2024,5,1]],"date-time":"2024-05-01T00:00:00Z","timestamp":1714521600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,5,1]],"date-time":"2024-05-01T00:00:00Z","timestamp":1714521600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,3,15]],"date-time":"2024-03-15T00:00:00Z","timestamp":1710460800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100003785","name":"Fund for Folk Culture","doi-asserted-by":"publisher","award":["6\/2018"],"award-info":[{"award-number":["6\/2018"]}],"id":[{"id":"10.13039\/100003785","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100003785","name":"Fund for Folk Culture","doi-asserted-by":"publisher","award":["3\/2016"],"award-info":[{"award-number":["3\/2016"]}],"id":[{"id":"10.13039\/100003785","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100003785","name":"Fund for Folk Culture","doi-asserted-by":"publisher","award":["5\/2020"],"award-info":[{"award-number":["5\/2020"]}],"id":[{"id":"10.13039\/100003785","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008385","name":"Fondazione per La Ricerca sulla Fibrosi Cistica","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008385","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.fr","clinicalkey.jp","clinicalkey.es","clinicalkey.com.au","clinicalkey.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Pancreatology"],"published-print":{"date-parts":[[2024,5]]},"DOI":"10.1016\/j.pan.2024.03.005","type":"journal-article","created":{"date-parts":[[2024,3,11]],"date-time":"2024-03-11T01:18:07Z","timestamp":1710119887000},"page":"394-403","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":9,"title":["CFTR function is impaired in a subset of patients with pancreatitis carrying rare CFTR variants"],"prefix":"10.1016","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6663-8918","authenticated-orcid":false,"given":"Dora","family":"Angyal","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1267-4814","authenticated-orcid":false,"given":"Karina","family":"Kleinfelder","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1493-9767","authenticated-orcid":false,"given":"Fabiana","family":"Ciciriello","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tessa A.","family":"Groeneweg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5961-8281","authenticated-orcid":false,"given":"Giulia","family":"De Marchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3558-681X","authenticated-orcid":false,"given":"Nicol\u00f2","family":"de Pretis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1712-4582","authenticated-orcid":false,"given":"Laura","family":"Bernardoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Rodella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Tomba","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"De Angelis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cecilia","family":"Surace","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily","family":"Pintani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4333-791X","authenticated-orcid":false,"given":"Federico","family":"Alghisi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hugo R.","family":"de Jonge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Melotti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2739-4014","authenticated-orcid":false,"given":"Claudio","family":"Sorio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincenzina","family":"Lucidi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0814-188X","authenticated-orcid":false,"given":"Marcel J.C.","family":"Bijvelds","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7417-2655","authenticated-orcid":false,"given":"Luca","family":"Frulloni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.pan.2024.03.005_bib1","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1053\/j.gastro.2021.10.017","article-title":"Burden and cost of gastrointestinal, liver, and pancreatic diseases in the United States: update 2021","volume":"162","author":"Peery","year":"2022","journal-title":"Gastroenterology"},{"key":"10.1016\/j.pan.2024.03.005_bib2","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1038\/mi.2016.101","article-title":"Immunopathogenesis of pancreatitis","volume":"10","author":"Watanabe","year":"2017","journal-title":"Mucosal Immunol"},{"key":"10.1016\/j.pan.2024.03.005_bib3","doi-asserted-by":"crossref","first-page":"1941","DOI":"10.1053\/j.gastro.2018.11.082","article-title":"Acute pancreatitis: a multifaceted set of organelle and cellular interactions","volume":"156","author":"Habtezion","year":"2019","journal-title":"Gastroenterology"},{"key":"10.1016\/j.pan.2024.03.005_bib4","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1016\/j.pan.2016.02.001","article-title":"Chronic pancreatitis: an international draft consensus proposal for a new mechanistic definition","volume":"16","author":"Whitcomb","year":"2016","journal-title":"Pancreatology"},{"key":"10.1016\/j.pan.2024.03.005_bib5","doi-asserted-by":"crossref","DOI":"10.3389\/fped.2022.941852","article-title":"Central role of the sentinel acute pancreatitis event (SAPE) model in understanding recurrent acute pancreatitis (RAP): implications for precision medicine","volume":"10","author":"Whitcomb","year":"2022","journal-title":"Front Pediatr"},{"key":"10.1016\/j.pan.2024.03.005_bib6","doi-asserted-by":"crossref","first-page":"720","DOI":"10.1016\/S2468-1253(18)30211-5","article-title":"Idiopathic recurrent acute pancreatitis","volume":"3","author":"Guda","year":"2018","journal-title":"Lancet Gastroenterol Hepatol"},{"key":"10.1016\/j.pan.2024.03.005_bib7","first-page":"190","article-title":"Acute pancreatitis: genetic risk and clinical implications","volume":"10","author":"Weiss","year":"2021","journal-title":"J\u00a0Clin Med"},{"key":"10.1016\/j.pan.2024.03.005_bib8","doi-asserted-by":"crossref","first-page":"1292","DOI":"10.1053\/j.gastro.2013.01.069","article-title":"Genetic risk factors for pancreatic disorders","volume":"144","author":"Whitcomb","year":"2013","journal-title":"Gastroenterology"},{"key":"10.1016\/j.pan.2024.03.005_bib9","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1186\/s10020-018-0041-6","article-title":"Trans-heterozygosity for mutations enhances the risk of recurrent\/chronic pancreatitis in patients with cystic fibrosis","volume":"24","author":"Sofia","year":"2018","journal-title":"Mol Med"},{"key":"10.1016\/j.pan.2024.03.005_bib10","doi-asserted-by":"crossref","first-page":"888","DOI":"10.1097\/MPA.0000000000001350","article-title":"Acute recurrent and chronic pancreatitis as initial manifestations of cystic fibrosis and cystic fibrosis transmembrane conductance regulator-related disorders","volume":"48","author":"Baldwin","year":"2019","journal-title":"Pancreas"},{"key":"10.1016\/j.pan.2024.03.005_bib11","first-page":"G961","article-title":"Pancreatic and biliary secretion are both altered in cystic fibrosis pigs","volume":"303","author":"Uc","year":"2012","journal-title":"Am J Physiol"},{"key":"10.1016\/j.pan.2024.03.005_bib12","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1152\/physrev.00011.2011","article-title":"Molecular mechanism of pancreatic and salivary gland fluid and HCO3- secretion","volume":"92","author":"Lee","year":"2012","journal-title":"Physiol Rev"},{"key":"10.1016\/j.pan.2024.03.005_bib13","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1016\/j.ccm.2015.11.004","article-title":"Gastrointestinal disorders in cystic fibrosis","volume":"37","author":"Assis","year":"2016","journal-title":"Clin Chest Med"},{"key":"10.1016\/j.pan.2024.03.005_bib14","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1016\/j.jcf.2012.05.001","article-title":"Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in pancreatitis","volume":"11","author":"Ooi","year":"2012","journal-title":"J\u00a0Cyst Fibros"},{"key":"10.1016\/j.pan.2024.03.005_bib15","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1016\/j.jcf.2022.09.005","article-title":"ECFS standards of care on CFTR-related disorders: diagnostic criteria of CFTR dysfunction","volume":"21","author":"Sermet-Gaudelus","year":"2022","journal-title":"J\u00a0Cyst Fibros"},{"issue":"Suppl 2","key":"10.1016\/j.pan.2024.03.005_bib16","doi-asserted-by":"crossref","first-page":"S86","DOI":"10.1016\/S1569-1993(11)60014-3","article-title":"Recommendations for the classification of diseases as CFTR-related disorders","volume":"10","author":"Bombieri","year":"2011","journal-title":"J\u00a0Cyst Fibros"},{"key":"10.1016\/j.pan.2024.03.005_bib17","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1038\/sj.ejhg.5200786","article-title":"Determination of the relative contribution of three genes-the cystic fibrosis transmembrane conductance regulator gene, the cationic trypsinogen gene, and the pancreatic secretory trypsin inhibitor gene-to the etiology of idiopathic chronic pancreatitis","volume":"10","author":"Audr\u00e9zet","year":"2002","journal-title":"Eur J Hum Genet"},{"key":"10.1016\/j.pan.2024.03.005_bib18","doi-asserted-by":"crossref","DOI":"10.1101\/cshperspect.a009480","article-title":"Assessing the disease-liability of mutations in CFTR","volume":"2","author":"Ferec","year":"2012","journal-title":"Cold Spring Harb Perspect Med"},{"key":"10.1016\/j.pan.2024.03.005_bib19","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1053\/j.gastro.2014.11.002","article-title":"Alcohol disrupts levels and function of the cystic fibrosis transmembrane conductance regulator to promote development of pancreatitis","volume":"148","author":"Maleth","year":"2015","journal-title":"Gastroenterology"},{"key":"10.1016\/j.pan.2024.03.005_bib20","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.1136\/gut.2007.134361","article-title":"Effects of bile acids on pancreatic ductal bicarbonate secretion in Guinea pig","volume":"57","author":"Venglovecz","year":"2008","journal-title":"Gut"},{"key":"10.1016\/j.pan.2024.03.005_bib21","doi-asserted-by":"crossref","first-page":"2228","DOI":"10.1053\/j.gastro.2011.08.039","article-title":"Trypsin reduces pancreatic ductal bicarbonate secretion by inhibiting CFTR Cl\u207b channels and luminal anion exchangers","volume":"141","author":"Pallagi","year":"2011","journal-title":"Gastroenterology"},{"key":"10.1016\/j.pan.2024.03.005_bib22","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1007\/112_2015_5002","article-title":"CFTR: a new horizon in the pathomechanism and treatment of pancreatitis","volume":"170","author":"Hegyi","year":"2016","journal-title":"Rev Physiol Biochem Pharmacol"},{"key":"10.1016\/j.pan.2024.03.005_bib23","doi-asserted-by":"crossref","first-page":"1148","DOI":"10.1053\/j.gastro.2017.06.011","article-title":"Correction of ductal CFTR activity rescues acinar cell and pancreatic and salivary gland functions in mouse models of autoimmune disease","volume":"153","author":"Zeng","year":"2017","journal-title":"Gastroenterology"},{"key":"10.1016\/j.pan.2024.03.005_bib24","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/j.cell.2019.12.032","article-title":"A\u00a0therapy for most with cystic fibrosis","volume":"180","author":"Bear","year":"2020","journal-title":"Cell"},{"key":"10.1016\/j.pan.2024.03.005_bib25","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.aau7531","article-title":"In utero and postnatal VX-770 administration rescues multiorgan disease in a ferret model of cystic fibrosis","volume":"11","author":"Sun","year":"2019","journal-title":"Sci Transl Med"},{"key":"10.1016\/j.pan.2024.03.005_bib26","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1016\/S2213-2600(18)30202-9","article-title":"Ivacaftor treatment of cystic fibrosis in children aged 12 to <24 months and with a CFTR gating mutation (ARRIVAL): a phase 3 single-arm study","volume":"6","author":"Rosenfeld","year":"2018","journal-title":"Lancet Respir Med"},{"key":"10.1016\/j.pan.2024.03.005_bib27","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1016\/j.pan.2019.09.014","article-title":"Cystic fibrosis transmembrane conductance regulator modulators reduce the risk of recurrent acute pancreatitis among adult patients with pancreas sufficient cystic fibrosis","volume":"19","author":"Akshintala","year":"2019","journal-title":"Pancreatology"},{"key":"10.1016\/j.pan.2024.03.005_bib28","doi-asserted-by":"crossref","first-page":"614","DOI":"10.1016\/j.jcf.2019.10.022","article-title":"Forskolin-induced swelling of intestinal organoids correlates with disease severity in adults with cystic fibrosis and homozygous F508del mutations","volume":"19","author":"De Winter-de Groot","year":"2020","journal-title":"J\u00a0Cyst Fibros"},{"key":"10.1016\/j.pan.2024.03.005_bib29","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.02529-2017","article-title":"Stratifying infants with cystic fibrosis for disease severity using intestinal organoid swelling as a biomarker of CFTR function","volume":"52","author":"De Winter-de Groot","year":"2018","journal-title":"Eur Respir J"},{"key":"10.1016\/j.pan.2024.03.005_bib30","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.jcmgh.2019.09.003","article-title":"Regulation of CFTR bicarbonate channel activity by WNK1: implications for pancreatitis and CFTR-related disorders","volume":"9","author":"Kim","year":"2020","journal-title":"Cell Mol Gastroenterol Hepatol"},{"key":"10.1016\/j.pan.2024.03.005_bib31","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1004376","article-title":"Mechanisms of CFTR functional variants that impair regulated bicarbonate permeation and increase risk for pancreatitis but not for cystic fibrosis","volume":"10","author":"LaRusch","year":"2014","journal-title":"PLoS Genet"},{"key":"10.1016\/j.pan.2024.03.005_bib32","doi-asserted-by":"crossref","first-page":"5317","DOI":"10.1113\/jphysiol.2012.232124","article-title":"Rescue of epithelial HCO3- secretion in murine intestine by apical membrane expression of the cystic fibrosis transmembrane conductance regulator mutant F508del","volume":"590","author":"Xiao","year":"2012","journal-title":"J\u00a0Physiol"},{"key":"10.1016\/j.pan.2024.03.005_bib33","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1038\/35065099","article-title":"Aberrant CFTR-dependent HCO3- transport in mutations associated with cystic fibrosis","volume":"410","author":"Choi","year":"2001","journal-title":"Nature"},{"key":"10.1016\/j.pan.2024.03.005_bib34","doi-asserted-by":"crossref","first-page":"16891","DOI":"10.3748\/wjg.v20.i45.16891","article-title":"Acute recurrent pancreatitis: etiopathogenesis, diagnosis and treatment","volume":"20","author":"Testoni","year":"2014","journal-title":"World J Gastroenterol"},{"key":"10.1016\/j.pan.2024.03.005_bib35","doi-asserted-by":"crossref","first-page":"908","DOI":"10.1016\/j.jcf.2022.09.011","article-title":"ECFS standards of care on CFTR-related disorders: updated diagnostic criteria","volume":"21","author":"Castellani","year":"2022","journal-title":"J\u00a0Cyst Fibros"},{"key":"10.1016\/j.pan.2024.03.005_bib36","doi-asserted-by":"crossref","first-page":"2023","DOI":"10.1002\/ppul.25395","article-title":"Role of sweat ion ratios in diagnosing cystic fibrosis","volume":"56","author":"Treggiari","year":"2021","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.pan.2024.03.005_bib37","doi-asserted-by":"crossref","first-page":"S4","DOI":"10.1016\/j.jpeds.2016.09.064","article-title":"Diagnosis of cystic fibrosis: consensus guidelines from the cystic fibrosis foundation","volume":"181S","author":"Farrell","year":"2017","journal-title":"J\u00a0Pediatr"},{"key":"10.1016\/j.pan.2024.03.005_bib38","doi-asserted-by":"crossref","first-page":"824","DOI":"10.1016\/j.jcf.2021.03.003","article-title":"Optical measurements of sweat for in\u00a0vivo quantification of CFTR function in individual sweat glands","volume":"20","author":"Treggiari","year":"2021","journal-title":"J\u00a0Cyst Fibros"},{"key":"10.1016\/j.pan.2024.03.005_bib39","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1016\/j.jcf.2017.12.003","article-title":"Ratiometric sweat secretion optical test in cystic fibrosis, carriers and healthy subjects","volume":"17","author":"Bergamini","year":"2018","journal-title":"J\u00a0Cyst Fibros"},{"issue":"Suppl 2","key":"10.1016\/j.pan.2024.03.005_bib40","doi-asserted-by":"crossref","first-page":"S53","DOI":"10.1016\/S1569-1993(11)60009-X","article-title":"New clinical diagnostic procedures for cystic fibrosis in Europe","volume":"10","author":"De Boeck","year":"2011","journal-title":"J\u00a0Cyst Fibros"},{"key":"10.1016\/j.pan.2024.03.005_bib41","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1016\/j.jcf.2016.06.009","article-title":"Nasal potential difference outcomes support diagnostic decisions in cystic fibrosis","volume":"15","author":"Tridello","year":"2016","journal-title":"J\u00a0Cyst Fibros"},{"key":"10.1016\/j.pan.2024.03.005_bib42","first-page":"939","article-title":"A\u00a0functional CFTR assay using primary cystic fibrosis intestinal organoids","volume":"19","author":"Dekkers","year":"2013","journal-title":"Nat Med (N Y, NY, U S)"},{"key":"10.1016\/j.pan.2024.03.005_bib43","doi-asserted-by":"crossref","first-page":"594","DOI":"10.1136\/thx.2009.125088","article-title":"Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference data","volume":"65","author":"Derichs","year":"2010","journal-title":"Thorax"},{"key":"10.1016\/j.pan.2024.03.005_bib44","article-title":"Intestinal current measurement and nasal potential difference to make a diagnosis of cases with inconclusive CFTR genetics and sweat test","volume":"7","author":"Minso","year":"2020","journal-title":"BMJ Open Respir Res"},{"key":"10.1016\/j.pan.2024.03.005_bib45","doi-asserted-by":"crossref","first-page":"S27","DOI":"10.1016\/j.jpeds.2016.09.063","article-title":"Applying cystic fibrosis transmembrane conductance regulator genetics and CFTR2 data to facilitate diagnoses","volume":"181S","author":"Sosnay","year":"2017","journal-title":"J\u00a0Pediatr"},{"key":"10.1016\/j.pan.2024.03.005_bib46","doi-asserted-by":"crossref","first-page":"1160","DOI":"10.1038\/ng.2745","article-title":"Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene","volume":"45","author":"Sosnay","year":"2013","journal-title":"Nat Genet"},{"key":"10.1016\/j.pan.2024.03.005_bib47","doi-asserted-by":"crossref","first-page":"1376","DOI":"10.1038\/s41395-018-0194-5","article-title":"Hereditary pancreatitis in the United States: survival and rates of pancreatic cancer","volume":"113","author":"Shelton","year":"2018","journal-title":"Am J Gastroenterol"},{"key":"10.1016\/j.pan.2024.03.005_bib48","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1086\/381001","article-title":"Variation in a repeat sequence determines whether a common variant of the cystic fibrosis transmembrane conductance regulator gene is pathogenic or benign","volume":"74","author":"Groman","year":"2004","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.pan.2024.03.005_bib49","doi-asserted-by":"crossref","first-page":"1422","DOI":"10.1086\/316911","article-title":"Active intestinal chloride secretion in human carriers of cystic fibrosis mutations: an evaluation of the hypothesis that heterozygotes have subnormal active intestinal chloride secretion","volume":"67","author":"Hogenauer","year":"2000","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.pan.2024.03.005_bib50","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1016\/j.jcf.2018.02.007","article-title":"Comparison of ex\u00a0vivo and in\u00a0vitro intestinal cystic fibrosis models to measure CFTR-dependent ion channel activity","volume":"17","author":"Zomer-van Ommen","year":"2018","journal-title":"J\u00a0Cyst Fibros"},{"key":"10.1016\/j.pan.2024.03.005_bib51","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/j.jcf.2013.06.008","article-title":"Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function","volume":"13","author":"Van Goor","year":"2014","journal-title":"J\u00a0Cyst Fibros"},{"key":"10.1016\/j.pan.2024.03.005_bib52","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.00209-2022","article-title":"Reclassifying inconclusive diagnosis for cystic fibrosis with new generation sweat test","volume":"60","author":"Nguyen-Khoa","year":"2022","journal-title":"Eur Respir J"},{"key":"10.1016\/j.pan.2024.03.005_bib53","doi-asserted-by":"crossref","first-page":"40","DOI":"10.3390\/jpm10020040","article-title":"The CFTR mutation c.3453G > C (D1152H) confers an anion selectivity defect in primary airway tissue that can be rescued by ivacaftor","volume":"10","author":"Laselva","year":"2020","journal-title":"J\u00a0Personalized Med"},{"key":"10.1016\/j.pan.2024.03.005_bib54","doi-asserted-by":"crossref","first-page":"1701","DOI":"10.1016\/j.celrep.2019.01.068","article-title":"Rectal organoids enable personalized treatment of cystic fibrosis","volume":"26","author":"Berkers","year":"2019","journal-title":"Cell Rep"},{"key":"10.1016\/j.pan.2024.03.005_bib55","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1097\/MPG.0000000000001788","article-title":"Reduction of recurrence risk of pancreatitis in cystic fibrosis with ivacaftor: case series","volume":"66","author":"Carrion","year":"2018","journal-title":"J\u00a0Pediatr Gastroenterol Nutr"},{"key":"10.1016\/j.pan.2024.03.005_bib56","doi-asserted-by":"crossref","first-page":"1058","DOI":"10.1038\/s41395-018-0123-7","article-title":"Ivacaftor CFTR potentiator therapy is efficient for pancreatic manifestations in cystic fibrosis","volume":"113","author":"Kounis","year":"2018","journal-title":"Am J Gastroenterol"},{"key":"10.1016\/j.pan.2024.03.005_bib57","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1186\/s12876-019-1044-7","article-title":"The effect of CFTR modulators on a cystic fibrosis patient presenting with recurrent pancreatitis in the absence of respiratory symptoms: a case report","volume":"19","author":"Johns","year":"2019","journal-title":"BMC Gastroenterol"},{"key":"10.1016\/j.pan.2024.03.005_bib58","doi-asserted-by":"crossref","first-page":"1589","DOI":"10.1164\/rccm.202004-1200LE","article-title":"Comparison of organoid swelling and in\u00a0vivo biomarkers of CFTR function to determine effects of lumacaftor-ivacaftor in patients with cystic fibrosis homozygous for the F508del mutation","volume":"202","author":"Graeber","year":"2020","journal-title":"Am J Respir Crit Care Med"}],"container-title":["Pancreatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1424390324000668?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1424390324000668?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2024,7,27]],"date-time":"2024-07-27T09:23:26Z","timestamp":1722072206000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1424390324000668"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5]]},"references-count":58,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024,5]]}},"alternative-id":["S1424390324000668"],"URL":"https:\/\/doi.org\/10.1016\/j.pan.2024.03.005","relation":{},"ISSN":["1424-3903"],"issn-type":[{"value":"1424-3903","type":"print"}],"subject":[],"published":{"date-parts":[[2024,5]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"CFTR function is impaired in a subset of patients with pancreatitis carrying rare CFTR variants","name":"articletitle","label":"Article Title"},{"value":"Pancreatology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.pan.2024.03.005","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 The Authors. Published by Elsevier B.V. on behalf of IAP and EPC.","name":"copyright","label":"Copyright"}]}}