【摘要】 回顾性分析2002年我院收治的1例行近胰腺次全切除术治疗的先天性高胰岛素血症（CHI）性低血糖患者随访至今的临床资料,并复习相关文献。患儿系ABCC8基因突变所致的三磷酸腺苷敏感性钾通道型先天性高胰岛素血病(K_ATP-HI),内科治疗无效,出生后12d行胰腺次全切除术治疗,术后24h血糖、胰岛素恢复正常,随访生长发育未受影响,学习成绩中等。术后第5年起反复低血糖发作;术后第7年出现糖耐量受损;术后第10年诊断为糖尿病,胰岛素治疗不能维持血糖平稳;术后第13年予维格列汀联合二甲双胍口服治疗,血糖平稳,无不良反应。对于CHI性低血糖患儿,根据基因突变类型选择合适治疗方案,维持血糖平稳,避免脑损害。针对内科药物治疗无效的ABCC8基因突变所致K_ATP-HI,可考虑行手术治疗。胰腺次全切除术后存在合并低血糖、糖尿病的可能。应监测胰腺内分泌功能,采取个体化综合治疗方案,维持血糖平稳。更多还原

【Abstract】 Analyze the clinical data of a patients after subtotal pancreatectomy because of congenital hyperinsulinism hypoglycemia in 2002 retrospectively,and review the relevant literatures.The patient was diagnosed as KATP-HI caused by ABCC8 gene mutations.Subtotal pancreatectomy was performed at 12 days after birth because of invalid medical therapy.24 hours after surgery,his blood glucose and insulin level returned to normal.Then the patient was followed up,his growth and development were unaffected,and he was an average student in school.Recurrent hypoglycemia appeared 5 years after surgery,and impaired glucose tolerance appeared 5 years after surgery.At 10 years postoperation,he was diagnosed as diabetes mellitus.Insulin therapy was unable to maintain his blood glucose,and 13 years after surgery,he was treated with vildagliptin and Metformin.After that,his blood glucose was stable and he had no side effect.For congenital hyperinsulinemia hypoglycemia,appropriate treatment should be selected according to the type of gene mutation to maintain stable blood glucose,and prevent brain damage.For medical therapy ineffective patients with KATP-HI caused by ABCC8 genetic mutations,surgery should be considered.Hypoglycemia and diabetes may appear after subtotal pancreatectomy,so the pancreatic endocrine function must be monitored,and individualized comprehensive treatment should be given to the patients to control blood glucose.更多还原