Exocrine pancreatic dysfunction is common in hepatocyte nuclear factor 1β-associated renal disease and can be symptomatic | Zendy

Rhian Clissold | Zendy; Jonathan Fulford | Zendy; Michelle Hudson | Zendy; Beverley M. Shields | Zendy; Timothy J. McDonald | Zendy; Sian Ellard | Zendy; Andrew T. Hattersley | Zendy; Coralie Bingham | Zendy

Open Access

Exocrine pancreatic dysfunction is common in hepatocyte nuclear factor 1β-associated renal disease and can be symptomatic

Author(s) -

Rhian Clissold,

Jonathan Fulford,

Michelle Hudson,

Beverley M. Shields,

Timothy J. McDonald,

Sian Ellard,

Andrew T. Hattersley,

Coralie Bingham

Publication year - 2017

Publication title -

clinical kidney journal

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.033

H-Index - 40

eISSN - 2048-8513

pISSN - 2048-8505

DOI - 10.1093/ckj/sfx150

Subject(s) - medicine , hnf1b , gastroenterology , diabetes mellitus , exocrine pancreatic insufficiency , pancreatitis , hyperglucagonemia , steatorrhea , endocrinology , insulin , glucagon , biology , biochemistry , gene expression , gene , homeobox

Heterozygous mutations in the HNF1B gene are the most common monogenic cause of developmental kidney disease. Extrarenal phenotypes frequently occur, including diabetes mellitus and pancreatic hypoplasia; the latter is associated with subclinical exocrine dysfunction. We measured faecal elastase-1 in patients with HNF1B-associated disease regardless of diabetes status and assessed the degree of symptoms associated with pancreatic exocrine deficiency.

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