The Clinical Characteristics of Type B Insulin Resistance

doi:10.12114/j.issn.1007-9572.2019.00.485

Abstract

Abstract: Objective　To summarize the clinical characteristics of Chinese patients with type B insulin resistance （B-IR） to improve their diagnosis and treatment.Methods　We searched the databases of CNKI，Wanfang Data Knowledge Service Platform and PubMed with the keyword “type B insulin resistance” from January 2000 to January 2019.Literatures reported Chinese patients were selected in PubMed database.According to the inclusion criteria，13 articles and 15 patients were included.The clinical manifestation，laboratory examination，treatment and follow-up were summarized.Results　Among 15 cases，the mean age of three male patients was （46.3±17.0） years，and the mean age of twelve female patients was （45.3±11.4） years；the age of all patients ranged from 24 to 63 years，and the mean age was （45.6±11.6） years.All patients （15/15） were accompanied with autoimmune diseases （AID），and 12 of them （12/15） with single AID，three of them （3/15） with more than two AIDs，nine cases（9/15） with systemic lupus erythematosus.Fourteen patients began with hyperglycemia and the mean value of HbA1c was （11.9±2.7）%，while one patient began with hypoglycemia with the value of HbA1c about 7.6%.There were 11 patients （11/15） whose fast serum insulin level were higher than 300 μU/ml and 5 patients（5/15） with hypertestosteronemia.Among the 15 patients，7 （7/15） were positive for anti-insulin receptor antibodies （AIRA），6 （6/15） were positive for 13C breath test，four of who were positive and two were negative.The combination of glucocorticoids and immunosuppressant drugs was the main treatment programs.There were 4 cases （4/15） starting with a program of pulse glucocorticoids therapy （methyprednisolone ≥250 mg/day for 3 days）．The remaining 11 patients （11/15） started with a program of methyprednisolone 20-40 mg/day or prednisone 40-60 mg/day.Twelve patients （12/15） were treated with low-dose glucocorticoid （methylprednisolone 4-8 mg/d，prednisone 2.5-10.0 mg/d） for maintenance therapy.As for the initial use of immunosuppressive drugs，8 patients （8/15） were treated with cyclophosphamide，0.4-0.6 g/week at the beginning，generally for 1-3 weeks.The time limit for blood glucose control was from 2 weeks to 1 year.One patient had no follow-up record，the others were followed up for 2 months to 7 years.Conclusion　B-IR is a rare disease，which has typical clinical characteristics including severe blood glucose disorder，hyperinsulinemia and autoimmune diseases.The treatment program mainly includes the combination of glucocorticoids and immunosuppressant drugs，and it is vital to have close follow-up in order to improve the prognosis.

Key words: Type B insulin resistance, Hyperinsulinism, Insulin antibodies, Glucocorticoids, Clinical characteristics, Treatment, Follow-up

摘要： 目的　了解我国B型胰岛素抵抗（B-IR）患者的临床特点，提高诊治水平。方法　以“B型胰岛素抵抗”为关键词，检索中国知网和万方数据知识服务平台；以“type B insulin resistance”为关键词，检索PubMed数据库，检索2000年1月—2019年1月公开发表的文献，其中PubMed数据库中所检索文献筛选出报道中国患者的文献。根据筛选标准，共纳入13篇文献，15例患者。对其临床表现、实验室检查、治疗及随访情况进行归纳总结。结果　15例患者中男3例，平均年龄（46.3±17.0）岁；女12例，平均年龄（45.3±11.4）岁；年龄24~63岁，平均年龄（45.6±11.6）岁。15例患者均合并自身免疫性疾病，其中12例（12/15）合并1种疾病，3例（3/15）合并两种及以上疾病；9例（9/15）合并系统性红斑狼疮。15例患者中14例以高血糖起病者糖化血红蛋白为（11.9±2.7）%，1例以低血糖起病者糖化血红蛋白为7.6%。11例（11/15）患者空腹血清胰岛素>300 μU/ml，5例（5/15）有高睾酮血症。15例患者中7例（7/15）进行胰岛素受体抗体（AIRA）检测，结果均为阳性；6例（6/15）行13C呼气试验，其中4例阳性，2例阴性。糖皮质激素联合免疫抑制剂作为治疗该病的主要方案，其中4例（4/15）采用起始大剂量糖皮质激素冲击治疗（甲泼尼龙≥250 mg/d，共3 d），其余11例（11/15）起始剂量为甲泼尼龙20~40 mg/d或醋酸泼尼松40~60 mg/d。12例（12/15）患者应用小剂量糖皮质激素（甲泼尼龙4~8 mg/d，醋酸泼尼松片2.5~10.0 mg/d）维持治疗。关于起始应用免疫抑制剂的方案，8例（8/15）患者起始选用环磷酰胺治疗，0.4~0.6 g/周起始应用，一般应用1~3周。血糖得以控制的时限为2周~1年。1例患者无随访记录，其余患者均进行随访，时间为2个月~7年。结论　B-IR临床罕见，有比较典型的血糖紊乱、高胰岛素血症和合并自身免疫性疾病，治疗方案主要包括糖皮质激素联合免疫抑制剂，需密切随访，改善预后。

关键词: B型胰岛素抵抗, 高胰岛素血症, 胰岛素抗体, 糖皮质激素类, 临床特征, 治疗, 随访

ZHAO Linlin,WANG Shoujun. The Clinical Characteristics of Type B Insulin Resistance　[J]. Chinese General Practice, 2020, 23(11): 1431-1435. DOI: 10.12114/j.issn.1007-9572.2019.00.485.
赵琳琳,王守俊. B型胰岛素抵抗的临床特点分析[J]. 中国全科医学, 2020, 23(11): 1431-1435. DOI: 10.12114/j.issn.1007-9572.2019.00.485.

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