{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T13:33:53Z","timestamp":1789824833429,"version":"4.0.1"},"reference-count":30,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2013,4,19]],"date-time":"2013-04-19T00:00:00Z","timestamp":1366329600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Diabet. Med."],"published-print":{"date-parts":[[2013,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>To examine the exocrine pancreatic function in carriers of the hepatocyte nuclear factor 1\u03b2 gene (<jats:italic><jats:styled-content style=\"fixed-case\">HNF<\/jats:styled-content>1B<\/jats:italic>) mutation by direct testing.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with <jats:italic><jats:styled-content style=\"fixed-case\">HNF<\/jats:styled-content>1B<\/jats:italic> mutations and control subjects were assessed using rapid endoscopic secretin tests and secretin\u2010stimulated magnetic resonance imaging. Seven patients and 25 controls underwent endoscopy, while eight patients and 20 controls had magnetic resonance imaging. Ductal function was assessed according to peak bicarbonate concentrations and acinar function was assessed according to peak digestive enzyme activities in secretin\u2010stimulated duodenal juice. The association of pancreatic exocrine function and diabetes status with pancreatic gland volume was examined.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The mean increase in secretin\u2010stimulated duodenal fluid was smaller in patients than controls (4.0 vs 6.4\u00a0ml\/min; <jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.003). We found lower ductal function in patients than controls (median peak bicarbonate concentration: 73 vs 116 <jats:styled-content style=\"fixed-case\">mE<\/jats:styled-content>q\/L; <jats:italic>P\u00a0<\/jats:italic>&lt;<jats:italic>\u00a0<\/jats:italic>0.001) and lower acinar function (median peak lipase activity: 6.4 vs 33.5 <jats:styled-content style=\"fixed-case\">kU<\/jats:styled-content>\/ml; <jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.01; median peak elastase activity: 0.056 vs 0.130 U\/ml; <jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.01). Pancreatic fluid volume outputs correlated significantly with pancreatic gland volumes (<jats:italic>r<\/jats:italic><jats:sup>2<\/jats:sup><jats:italic>\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.71, <jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.008) in patients. The total fluid output to pancreatic gland volume ratios were higher in patients than controls (4.5 vs 1.3 ml\/cm<jats:sup>3<\/jats:sup>; <jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.03), suggesting compensatory hypersecretion in the remaining gland.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Carriers of the <jats:italic><jats:styled-content style=\"fixed-case\">HNF<\/jats:styled-content>1B<\/jats:italic> mutation have lower exocrine pancreatic function involving both ductal and acinar cells. Compensatory hypersecretion suggests that the small pancreas of <jats:italic><jats:styled-content style=\"fixed-case\">HNF<\/jats:styled-content>1B<\/jats:italic> mutation carriers is attributable to hypoplasia, not atrophy.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dme.12190","type":"journal-article","created":{"date-parts":[[2013,3,28]],"date-time":"2013-03-28T10:09:33Z","timestamp":1364465373000},"page":"946-955","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Exocrine pancreatic function in hepatocyte nuclear factor 1\u03b2\u2010maturity\u2010onset diabetes of the young (<scp>HNF<\/scp>1B\u2010<scp>MODY<\/scp>) is only moderately reduced: compensatory hypersecretion from a hypoplastic pancreas"],"prefix":"10.1111","volume":"30","author":[{"given":"E.","family":"Tjora","sequence":"first","affiliation":[{"name":"KG Jebsen Center for Diabetes Research, Department of Clinical Science University of Bergen Bergen Norway"},{"name":"Department of Pediatrics Haukeland University Hospital Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Wathle","sequence":"additional","affiliation":[{"name":"Department of Radiology Haukeland University Hospital Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F.","family":"Erchinger","sequence":"additional","affiliation":[{"name":"Voss Hospital Haukeland University Hospital Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Engjom","sequence":"additional","affiliation":[{"name":"Department of Medicine Haukeland University Hospital Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Molven","sequence":"additional","affiliation":[{"name":"KG Jebsen Center for Diabetes Research, Department of Clinical Science University of Bergen Bergen Norway"},{"name":"Department of Clinical Medicine University of Bergen Bergen Norway"},{"name":"Islet Cell and Regenerative Biology Joslin Diabetes Center Harvard Medical School Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Aksnes","sequence":"additional","affiliation":[{"name":"KG Jebsen Center for Diabetes Research, Department of Clinical Science University of Bergen Bergen Norway"},{"name":"Department of Pediatrics Haukeland University Hospital Bergen Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I. 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