Exocrine pancreatic function in hepatocyte nuclear factor 1β‐maturity‐onset diabetes of the young (HNF1B‐MODY) is only moderately reduced: compensatory hypersecretion from a hypoplastic pancreas. Issue 8 (19th April 2013)

• Record Type: Journal Article
• Authors: Tjora, E.; Wathle, G.; Erchinger, F.; Engjom, T.; Molven, A.; Aksnes, L.; Haldorsen, I. S.; Dimcevski, G.; Ræder, H.; Njølstad, P. R.
• Is Part Of: Diabetic medicine. Volume 30:Issue 8(2013:Aug.)
• Page Start: 946
• Page End: 955
• Publication Date: 2013-04-19
• Subjects: Diabetes -- Periodicals; 616.462
• DOI: 10.1111/dme.12190
• Languages: English
• ISSNs: 0742-3071

Abstract

<abstract abstract-type="main" id="dme12190-abs-0001"> <title>Abstract</title> <sec id="dme12190-sec-0001" sec-type="section"> <title>Objectives</title> <p>To examine the exocrine pancreatic function in carriers of the hepatocyte nuclear factor 1β gene (<italic>HNF1B</italic>) mutation by direct testing.</p> </sec> <sec id="dme12190-sec-0002" sec-type="section"> <title>Methods</title> <p>Patients with <italic>HNF1B</italic> mutations and control subjects were assessed using rapid endoscopic secretin tests and secretin‐stimulated 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‐stimulated duodenal juice. The association of pancreatic exocrine function and diabetes status with pancreatic gland volume was examined.</p> </sec> <sec id="dme12190-sec-0003" sec-type="section"> <title>Results</title> <p>The mean increase in secretin‐stimulated duodenal fluid was smaller in patients than controls (4.0 vs 6.4 ml/min; <italic>P </italic>=<italic> </italic>0.003). We found lower ductal function in patients than controls (median peak bicarbonate concentration: 73 vs 116 mEq/L; <italic>P </italic><<italic> </italic>0.001) and lower acinar function (median peak lipase activity: 6.4 vs 33.5 kU/ml; <italic>P </italic>=<italic> </italic>0.01; median peak elastase activity: 0.056 vs 0.130 U/ml; <italic>P </italic>=<italic> </italic>0.01). Pancreatic fluid volume outputs correlated significantly with pancreatic gland volumes (<italic>r</italic><sup>2</sup><italic> </italic>=<italic> </italic>0.71, <italic>P </italic>=<italic> </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<sup>3</sup>; <italic>P </italic>=<italic> </italic>0.03), suggesting compensatory hypersecretion in the remaining gland.</p> </sec> <sec id="dme12190-sec-0004" sec-type="section"> <title>Conclusion</title> <p>Carriers of the <italic>HNF1B</italic> mutation have lower exocrine pancreatic function involving both ductal and acinar cells. Compensatory hypersecretion suggests that the small pancreas of <italic>HNF1B</italic> mutation carriers is attributable to hypoplasia, not atrophy.</p> </sec> </abstract>