胰腺纤维钙化性糖尿病三例报告及文献复习

Three cases of fibrocalculous pancreatic diabetes and review of literatures

目的分析我院3例胰腺纤维钙化性糖尿病（FCPD）患者的临床特征并复习国内报道的50例FCPD患者资料，以提高对该病的认识。方法采用回顾性分析，对2008至2012年间我院收治的3例FCPD患者及国内报道的50例患者从性别比例、发病年龄、临床特征、实验室检查、影像学特征及治疗方法进行综合分析。结果我院及国内共53例FCPD患者中男37例，女16例，确诊年龄27-60岁，较集中分布于四川（18例）、广西（18例）和广东（12例），散在分布于海南（1例）、贵州（1例）、湖南（1例）、浙江（1例）和内蒙古（1例）。食用木薯者11例，有饮酒史者4例。1例患者被误诊为1型糖尿病达5年，10例患者被误诊为2型糖尿病分别达2～6年。33例患者有腹痛症状，19例患者有脂肪泻。分别通过x线片（11例）、B超（30例）、CT（35例）、磁共振成像（1例）和手术病理（1例）证实存在胰管结石和（或）胰腺纤维化。绝大部分患者（52例）均体形消瘦，入院时随机血糖为14．0～30．8mmol／L，胰岛素及C肽明显降低，仅有2例患者出现酮症。51例患者接受胰岛素治疗，2例患者应用磺脲类口服降糖药物控制血糖。结论（1）FCPD的经典临床症状为腹痛、脂肪泻和糖尿病，但症状往往不典型而易漏诊。（2）对于体形消瘦、血糖高、胰岛储备功能差、无酮症倾向者要想到FCPD的可能，x线、B超、CT或磁共振成像发现胰管结石和胰腺纤维化为本病诊断的重要依据。

Objective To enhance the understanding of fibrocalculous pancreatic diabetes （FCPD） by reviewing its clinical features and auxiliary examination results. Methods Three patients in our hospital were reported in detail. Combined with the clinical data of 50 cases of FCPD had been reported in our country, the sex ratio of patients, age of onset, clinical features, laboratory examination, imaging characteristics and treatment were discussed through retrospective analysis. Results In total 53 cases, there were 37 male and 16 famale, the diagnose age was 27 -60 years old. These patients were mainly distributed in Sichuan （ 18 cases） , Guangxi （ 18 cases） , and Guangdong （ 12 cases） province. Some scattered in Hainan （1 case）, Guizbou （1 case）, Hunan （1 case）, Zhejiang （1 case） and Inner Mongolia （1 case）. Edible cassava was in 11 cases, and drinking history in 4 cases. One patient was misdiagnosed as type 1 diabetes mellitus （T1DM） for 5 years, and the other 10 patients were diagnosed as type 2 diabetes mellitus （T2DM） for 2 - 6 years respectively. 33 cases had abdominal pain, 19 patients had steatorrhea. The calculus and fibrosis were confirmed by X-ray （ 11 case） , B ultrasound （ 30 cases ） , computed tomography （35 cases）, magnatic resonance imaging（MRI） and surgical pathology（ 1 case）. Most of the patients （n = 52 ） had low body weight. Admission random blood glucose levels were 14. 0 - 30. 8 retooL/L, plasm insulin and C-peptide were significantly decreased, but only 2 cases had ketosis. 51 cases were treated with insulin, only 2 cases were treated with sulfonylurea oral glucose-lower drugs to control blood sugar. Conclusions （1） The classic clinical symptoms of FCPD are abdominal pain, steatorrhea and diabetes but almost atypical. （2）Some findings, including low weight, high blood sugar, poor pancreas islet function and no ketosis, are helpful for making an early and accurate diagnosis. Calculus and fibrosis of pancreas through X-ray,B ultrasound, CT or MRI are useful for the final diagnosis.