17例成人暴发性1型糖尿病临床分析

Clinical Analysis of 17 Adult Patients With Fulminant Type 1 Diabetes Mellitus

目的探讨1型糖尿病的一种特殊亚型——暴发性1型糖尿病(fulminant type 1 diabetes mellitus,FT1DM)患者的临床特征。方法收集2010–2019年在四川大学华西医院住院、以糖尿病酮症酸中毒(diabetic ketoacidosis,DKA)起病的1型糖尿病(type 1 diabetes mellitus,T1DM)和FT1DM患者的临床资料,并对FT1DM患者进行随访。结果以DKA起病的住院T1DM患者共70例,其中FT1DM17例(24.3%),非FT1DM患者53例。两组患者(FT1DM组和非FT1DM组)平均年龄分别为(33.2±12.8)岁和(27.5±11.2)岁,平均体质量指数分别为(22.6±2.9)kg/m2和(19.2±2.9)kg/m2。14例FT1DM患者起病前有上呼吸道感染或急性胃肠炎症状,4例与妊娠相关,FT1DM组患者起病到初诊DKA时间〔中位数(P25~P75):2(1~4)d〕,P<0.001)短于T1DM组患者〔30(17~78)d〕。FT1DM组患者初诊时平均最高血糖〔(39.9±11.4)mmol/L〕高于非FT1DM患者〔(28.9±9.2)mmol/L,P<0.001〕,但糖化血红蛋白(6.6%±0.6%,P<0.001)和糖化血清白蛋白(21.4%±3.0%,P=0.001)低于非FT1DM组患者(糖化血红蛋白:12.8%±2.7%;糖化血清白蛋白:44.8%±15.0%)。FT1DM组患者血清淀粉酶高于非FT1DM组患者〔101(54~336)IU/L vs.54(42~166)IU/L,P=0.045〕,血清脂肪酶有高于T1DM组患者的趋势〔81(57~154)IU/L vs.46(28~195)IU/L,P=0.051〕。8.7%的非FT1DM患者GAD-Ab阳性,而FT1DM患者均阴性。出院时FT1DM患者日均胰岛素剂量(0.67±0.22)IU/kg,与非FT1DM组患者〔(0.74±0.29)IU/kg〕相比差异无统计学意义(P=0.349)。对FT1DM组患者随访约6.5年后,其日均胰岛素剂量为(0.73±0.19)IU/kg,与出院时差异无统计学意义(P=0.409)。结论相比DKA起病的非FT1DM患者,FT1DM患者“三多一少”症状更少,空腹C-肽水平更低,血淀粉酶水平更高,呕吐等胃肠道感染症状发生率增加,在临床上更容易误诊。因此,临床医生早期识别FT1DM患者并予早期且长期胰岛素替代治疗至关重要。

Objective To explore the clinical characteristics of adult patients with fulminant type 1 diabetes mellitus(FT1DM),a specific subtype of type 1 diabetes mellitus(T1DM).Methods We collected the clinical data of patients who were admitted to West China Hospital,Sichuan University in 2010-2019 for FT1DM and type 1 diabetes mellitus(T1DM)presenting with diabetic ketoacidosis(DKA)at the onset.In addition,all the FT1DM patients were followed up.Results A total of 70 patients presenting with DKA at the onset of T1DM were admitted to and received treatment at West China Hospital in 2010–2019.Among them,17(24.3%)had FT1DM and 53 did not.The mean ages of the FT1DM patients and the non-FT1DM patients were(33.2±12.8)years and(27.5±11.2)years,and the mean body mass indices were(22.6±2.9)kg/m2 and(19.2±2.9)kg/m2,respectively.A total of 14 FT1DM cases had symptoms of upper respiratory tract infection or acute gastroenteritis before the onset of the disease and 4 cases were related to pregnancy.The median time from the onset of the disease to the first diagnosis of DKA of the FT1DM group(median[P25-P75]:2[1-4]days,P<0.001)was significantly shorter than that of the non-FT1DM group(median[P25-P75]:30[17-78]days).The mean maximum blood glucose levels at the time of the first visit to the doctor of the FT1DM patients([39.9±11.4]mmol/L,P<0.001)were significantly higher than that of the non-FT1DM patients([28.9±9.2]mmol/L),but the HbA1c(6.6%±0.6%,P<0.001)and glycosylated serum albumin(GA)(21.4%±3.0%,P=0.001)levels of the FT1DM patients were significantly lower than those of the non-FT1DM group(HbA1c:12.8%±2.7%;GA:44.8%±15.0%).The median serum amylase in the FT1DM group was significantly higher than that in the non-FT1DM group(101[54-336]IU/L vs.54[42-166]IU/L,P=0.045)and the median serum lipase in the FT1DM group showed a trend of being higher than that in the T1DM group(81[57-154]IU/L vs.46[28-195]IU/L,P=0.051).8.7%of the non-FT1DM patients tested positive for anti-glutamic acid decarboxylase antibody(GAD-Ab),while the FT1DM patients all tested negative.At the time of discharge,the mean daily insulin dose of the FT1DM patients was(0.67±0.22)IU/kg,which was not significantly different from that of the non-FT1DM group([0.74±0.29]IU/kg,P=0.349).After about 6.5 years of follow-up,the mean daily insulin dose of the FT1DM patients was(0.73±0.19)IU/kg,which was similar to the insulin dosage on discharge(P=0.409).Conclusion Compared with the non-FT1DM patients presenting with DKA at the onset,FT1DM patients have fewer typical diabetic symptoms,lower fasting C-peptide levels,higher serum amylase levels,and increased incidence of vomiting or other symptoms of gastrointestinal infections,and are more likely to be misdiagnosed.Therefore,it is very important for clinicians to correctly identify FT1DM as early as possible and administer early and long-term insulin replacement therapy.