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This version published online on July 1, 2008
Journal of Clinical Endocrinology & Metabolism, doi:10.1210/jc.2008-0340
A more recent version of this article appeared on September 1, 2008
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Submitted on February 11, 2008
Accepted on June 19, 2008

Reduced Pancreatic Volume in HNF1A-MODY

Mette Vesterhus, Ingfrid S. Haldorsen, Helge Ræder, Anders Molven, and Pål R. Njølstad*

Department of Pediatrics, Haukeland University Hospital, Bergen, Norway (M.V., H.R., P.R.N.); Department of Clinical Medicine, University of Bergen, Bergen, Norway (M.V., H.R., P.R.N.); Department of Radiology, Haukeland University Hospital, Bergen, Norway (I.S.H.); Section for Radiology, Department of Surgical Sciences, University of Bergen, Bergen, Norway (I.S.H.); The Gade Institute, University of Bergen, Bergen, Norway (A.M.); and Department of Pathology, Haukeland University Hospital, Bergen, Norway (A.M.)

* To whom correspondence should be addressed. E-mail: pal.njolstad{at}uib.no.

CONTEXT: There are interplays between the endocrine and exocrine pancreas. We have recently reported an increased frequency of exocrine dysfunction in HNF1A-MODY (MODY3) patients compared to controls. Reduced pancreatic volume is seen in HNF1B-MODY (MODY5) and in diabetes type 1 and 2.

OBJECTIVE: The aim of this study was to investigate whether HNF1A mutation carriers have reduced pancreatic volume or abnormal pancreatic structure, and whether any changes are associated with exocrine dysfunction.

METHODS: Fifteen HNF1A mutation carriers recruited from the Norwegian MODY Registry, 31 subjects with type 1 diabetes, 10 subjects with type 2 diabetes and 11 controls underwent computed tomography of the pancreas. We measured pancreatic volume and X-ray attenuation. Pancreatic volume index was defined as pancreatic volume divided by body surface area.

RESULTS: Pancreatic volume index was reduced in subjects with HNF1A-MODY (34.5 mL/m2; P < 0.02) and type 1 diabetes (21.4 mL/m2; P < 0.001) as compared to non-diabetic controls (45.7 mL/m2), and was reduced in subjects with diabetes in combination with fecal elastase deficiency (P= 0.03). Subjects with type 1 diabetes had smaller pancreatic volume index compared to HNF1A mutation carriers (P < 0.001). Reduced pancreatic volume index was associated with increasing duration of diabetes. Pancreatic X-ray attenuation in HNF1A mutation carriers was not significantly different from that of non-diabetic controls.

CONCLUSIONS: HNF1A mutation carriers have reduced pancreatic volume, but less reduced than in patients with type 1 diabetes. Insulinopenia could explain both the pancreatic volume reduction and the associated pancreatic dysfunction.
Key words: Developmental Biology • Diabetes • Digestive Tract • Genetic Disease • Pancreas






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