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.

摘要: Objectives To examine the exocrine pancreatic function in carriers of hepatocyte nuclear factor 1β gene (HNF1B) mutation by direct testing. Methods Patients with HNF1B mutations and control subjects were assessed using rapid endoscopic secretin tests secretin-stimulated magnetic resonance imaging. Seven patients 25 controls underwent endoscopy, while eight 20 had Ductal was according to peak bicarbonate concentrations acinar digestive enzyme activities duodenal juice. The association diabetes status gland volume examined. Results The mean increase fluid smaller than (4.0 vs 6.4 ml/min; P = 0.003). We found lower ductal (median concentration: 73 116 mEq/L; P < 0.001) lipase activity: 6.4 33.5 kU/ml; P = 0.01; median elastase 0.056 0.130 U/ml; P = 0.01). Pancreatic outputs correlated significantly volumes (r2 = 0.71, P = 0.008) patients. total output ratios higher (4.5 1.3 ml/cm3; P = 0.03), suggesting compensatory hypersecretion remaining gland. Conclusion Carriers have involving both cells. Compensatory suggests that small pancreas is attributable hypoplasia, not atrophy.