| 王淑琴,高怡青,朱磊,等.新生儿糖尿病 6例病例特点及临床分析[J].安徽医药,2023,27(2):280-284. |
| 新生儿糖尿病 6例病例特点及临床分析 |
| Characteristics and clinical analysis of 6 cases of neonatal diabetes mellitus |
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| DOI：10.3969/j.issn.1009-6469.2023.02.015 |
| 中文关键词: 新生儿糖尿病 格列本脲 临床特征 KCNJ11基因 |
| 英文关键词: Neonatal diabetes mellitus Glibenclamide Clinical features KCNJ11 |
| 基金项目:徐州市卫生健康委科技项目面上项目( XWKYHT20200033) |
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| 中文摘要: |
| 目的总结 6例新生儿糖尿病( neonatal diabetes mellitus，NDM)的临床特点及随访资料，为临床诊疗提供参考。方法对 2013年 2月至 2021年 6月就诊于徐州医科大学附属徐州儿童医院内分泌科 6例 NDM病儿的临床特征、诊疗经过等进行回顾性分析。结果病儿诊断年龄范围为 3～256 d，随访时间范围为 4.5～102.2个月。反应差起病 3例，发热感染起病 3例。初诊时 4例合并糖尿病酮症酸中毒， 1例合并糖尿病酮症。急性期胰岛素治疗血糖控制良好。 5例行格列本脲经验性治疗， 4例有效， 1例无效停药，经基因检测证实格列本脲治疗有效为 KCNJ11基因突变，其中 1例基因突变为首次报道。 4例使用格列本脲病儿随访至今血糖均控制良好， 1例随访至 6月龄停格列本脲， 8岁复发后予胰岛素治疗。结论格列本脲对 KCNJ11基因突变 NDM治疗有效，长期随访安全。 TNDM缓解后可出现复发，复发后可予胰岛素治疗。 |
| 英文摘要: |
| Objective To summarize the clinical characteristics and follow-up data of 6 cases of neonatal diabetes mellitus (NDM), so as to provide reference for clinical diagnosis and treatment.Methods The clinical characteristics, diagnosis and treatment of 6 chil-dren with NDM in the Department of Endocrinology, Xuzhou Children's Hospital Affiliated to Xuzhou Medical University from Febru-ary 2013 to June 2021 were analyzed retrospectively.Results The age of diagnosis was 3-256 days and the follow-up was 4.5-102.2 months. There were 3 cases of poor response and 3 cases of fever infection. At the initial diagnosis, 4 cases had diabetic ketoacidosisand 1 cases had diabetic ketosis. Blood glucose was well controlled by insulin treatment in acute stage. Five cases were treated withglibenclamide empirically, 4 cases were effective and 1 case was ineffective. Gene test confirmed that the effective treatment of gliben-clamide was KCNJ11 gene mutation, of which 1 gene mutation was reported for the first time. The blood glucose of 4 children treatedwith glibenclamide was well controlled since follow-up. One case stopped glibenclamide after follow-up to 6 months, relapsed at the age of 8 years and was treated with insulin.Conclusion Glibenclamide is effective in the treatment of NDM with KCNJ11 gene mutation.TNDM relapses after remission, and insulin treatment can be given after recurrence. |
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