胰腺纤维钙化性糖尿病16例临床特点分析并文献复习

Analysis of clinical characteristics of 16 cases with fibrocalculous pancreatic diabetes and literature review

目的分析胰腺纤维钙化性糖尿病(fibrocalculous pancreatic diabetes,FCPD)的临床特点,提高临床对FCPD的认识。方法采用回顾性分析,对2015至2019年间我院收治的16例FCPD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,并对其中1例典型患者进行详细病例报告。结果我院诊断的16例FCPD患者中男8例,女8例,确诊年龄23~67岁,平均确诊年龄女性38岁,男性51岁,男性的平均确诊年龄高于女性,差异有统计学意义。8例患者有腹痛症状。3例患者有饮酒史,所有患者均无长期木薯进食史。分别通过CT、MRI或手术病理(1例)证实存在胰管结石和/或胰腺萎缩。绝大部分患者(12例)均体形中等不瘦、胰腺萎缩;所有患者C肽均有不同程度降低;仅有4例患者出现酮症,其中1例为酮症酸中毒;11例患者接受胰岛素治疗,5例患者应用口服降糖药物可维持血糖稳定。结论 1)FCPD的经典三联征是腹痛、胰腺炎和糖尿病,但症状常常不典型而漏诊。2)对于胰岛功能差但无酮症倾向、无糖尿病家族史的糖尿病患者应注意筛查FCPD,CT或MR可助诊,确诊后尽早手术治疗有利于胰岛细胞功能修复。

Objective To analyze the clinical characteristics of fibrocalculous pancreatic diabetes(FCPD), and to improve clinical understanding of FCPD.Methods Retrospective analysis was used to comprehensively analyze the 16 FCPD cases admitted in our hospital from 2015 to 2019, age of onset, clinical characteristics, laboratory examinations, imaging characteristics and treatment methods were discussed, and one of the typical cases was reported in detail.Results Among the sixteen cases, there were eight male and eight female.The diagnose age was 23-67 years old.The average diagnose age in men(51 years old) was significantly higher than that in women(38 years old).A total of eight cases had symptoms of abdominal pain,three cases had a history of alcohol consumption, and none cases had long-term cassava eating history.The pancreatic calculus and(or) pancreatic atrophy were confirmed by CT, MRI, or surgical pathology(one case).The majority patients(12 cases) were with moderately lean and pancreatic atrophy, and all patients had C-peptide decreased.Only four patients developed ketosis, including one with diabetic ketoacidosis.A total of eleven cases were treated with insulin and five cases were treated with oral antidiabetic drug to control blood sugar.Conclusion 1) The classic clinical symptoms of FCPD is abdominal pain, pancreatitis and diabetes, but often atypical and misdiagnosed.2) For diabetic patients with poor pancreas islet function but no ketosis and no family history of diabetes,attention should be paid to screening FCPD.CT or MR scan are useful for final diagnosis of FCPD and early surgical treatment is helpful for insulin cell function repair.