B型胰岛素抵抗综合征三例临床分析并文献复习

Type B insulin resistance syndrome： 3 cases report and literature review

目的 通过回顾分析3例B型胰岛素抵抗综合征（B-IRS）病例及相关文献以提高临床医生对B-IRS的认识.方法 结合文献并详细分析3例B-IRS患者的临床特点、诊疗经过和随访结果.结果 3例患者均为中年女性,临床表现严重高血糖/反常性低血糖、严重高胰岛素血症.结合文献报告病例,总结B-IRS具有以下特点：（1）多见于中青年女性;（2）多合并自身免疫性疾病如系统性红斑狼疮、系统性硬化等;（3）代谢异常包括血糖异常、胰岛素样生长因子-1（IGF-1）下降、TG正常低限或降低、脂联素升高等;（4）自身免疫异常相关的表现,非特异的表现有白细胞和/或血小板减少,低白蛋白血症、补体下降、Ig G和/或Ig A水平升高;还可以有抗核抗体滴度升高、抗SSA抗体和抗SSB抗体阳性、抗双链DNA抗体阳性等特异性的免疫学指标异常.胰岛素受体抗体（INSRAb）阳性对B-IRS的诊断具有确诊价值;（5）主要的治疗为免疫抑制剂联合治疗,对于合并幽门螺杆菌感染者,根除幽门螺杆菌治疗对于严重胰岛素抵抗可能有益.结论 熟悉B-IRS临床特征,临床识别和诊断并不困难,治疗着眼于合并疾病的治疗.

Objective To understand type B insulin resistance syndrome （B-IRS） by reviewing 3 cases from our center and cases from literatures.Methods The clinical characteristics, diagnosis, treatment and follow-up data of the 3 patients with B-IRS were evaluated.Results All the 3 patients were middle-aged women with severe hyperglycemia or paradoxical hypoglycemia.The clinical findings were as follows.（1） B-IRS was associated with several autoimmune diseases such as systemic lupus erythematosus （SLE） and sclerosis.（2） The metabolic abnormalities of B-IRS include weight loss, severe hyperinsulinemia, high level of adiponectin, and low level of insulin-like growth factor type 1 （IGF-1） and TG.（3） B-IRS was characterized with nonspecific serological disorders （such as leukopenia, thrombocytopenia and hypoalbuminemia） and changes （decreased complements and elevated IgG and/or IgA）, and with specific immunological abnormalities [such as high titer of antinuclear antibody （ANA）, positive in anti-SSA, antiSSB and anti-dsDNA antibodies）.Positive in anti-insulin receptor antibody was of diagnostic value but not necessary.（4） Treatments include insulin in combination with immunosuppressive therapy.Patients with H.pylori （Hp） infection may be benefit with eradication therapy.Conclusions B-IRS is rare but not difficult to identify.Treatments include therapy of the underlying diseases and high dose of insulin.