Vesterhus Mette, Haldorsen Ingfrid S, Raeder Helge, Molven Anders, Njølstad Pål R
Department of Pediatrics, Haukeland University Hospital, N-5021 Bergen, Norway.
J Clin Endocrinol Metab. 2008 Sep;93(9):3505-9. doi: 10.1210/jc.2008-0340. Epub 2008 Jul 1.
There are interplays between the endocrine and exocrine pancreas. We recently reported an increased frequency of exocrine dysfunction in HNF1A-maturity-onset diabetes of the young (MODY3) patients, compared with controls. Reduced pancreatic volume is seen in HNF1B-MODY (MODY5) and diabetes types 1 and 2.
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.
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.
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 with nondiabetic 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 with 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 nondiabetic controls.
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.
内分泌胰腺和外分泌胰腺之间存在相互作用。我们最近报告称，与对照组相比，年轻的HNF1A - 成年发病型糖尿病（MODY3）患者外分泌功能障碍的发生率增加。在HNF1B - MODY（MODY5）以及1型和2型糖尿病患者中可见胰腺体积减小。
本研究的目的是调查HNF1A突变携带者的胰腺体积是否减小或胰腺结构是否异常，以及是否有任何变化与外分泌功能障碍相关。
从挪威MODY登记处招募了15名HNF1A突变携带者、31名1型糖尿病患者、10名2型糖尿病患者和11名对照者，对其进行胰腺计算机断层扫描。我们测量了胰腺体积和X线衰减。胰腺体积指数定义为胰腺体积除以体表面积。
与非糖尿病对照组（45.7 ml/m²）相比，HNF1A - MODY患者（34.5 ml/m²；P < 0.02）和1型糖尿病患者（21.4 ml/m²；P < 0.001）的胰腺体积指数降低，糖尿病合并粪便弹性蛋白酶缺乏的患者胰腺体积指数也降低（P = 0.03）。与HNF1A突变携带者相比，1型糖尿病患者的胰腺体积指数更小（P < 0.001）。胰腺体积指数降低与糖尿病病程延长有关。HNF1A突变携带者的胰腺X线衰减与非糖尿病对照组无显著差异。
HNF1A突变携带者的胰腺体积减小，但比1型糖尿病患者减小程度小。胰岛素缺乏可以解释胰腺体积减小及其相关的胰腺功能障碍。
