新生儿糖尿病13例分析

Neonatal diabetes mellitus： a clinical analysis of 13 cases

目的 总结分析13例新生儿糖尿病（NDM）的临床特点,为临床诊断和治疗提供参考.方法 对2004年7月至2009年9月广州市妇女儿童医疗中心诊治的13例NDM的临床表现、实验室检杳及临床转归进行回顾性分析总结.结果 13例患儿的平均出生体重为2.30 kg,诊断年龄中位数为2个月,均在生后3个月内起病,初诊血糖均值为22.2 mmol/L.9例因感染而诱发症状加重来诊,有5例有典型＂三多一少＂糖尿病症状,主要临床表现有体重不增甚至消瘦、烦渴、轻中度脱水,其中3例发生酮症酸中毒（DKA）.1例同时伴有发育迟缓、癫痼,3例伴有高甘油三酯血症,1例伴有凝血功能异常,1例伴先天性心脏病（房间隔缺损）.有1例胰岛素抗体（IAA）阳性,有6例糖化血红蛋白＞6%（7.2%～14%）.所有患儿急性期均接受胰岛素治疗,胰岛素初始治疗剂量为0.56～1.00 U/（kg·d）,最大治疗剂量为1.35 U/（kg·d）,治疗24 h内血糖均明显下降,3例合并DKA患儿的酸中毒症状均在治疗48 h后缓解,血糖控制良好,急性期无一例死亡.随访至今（最短半年,最长6年）8例中4例符合暂时性NDM（TNDM）;3例符合永久性NDM（PNDM）;1例于2个月大时自行停用胰岛素治疗,3个月大时酮症酸中毒死亡.结论 NDM多无特异性临床表现,容易误诊误治,早期诊断和治疗预后良好,临床上确诊该病应注意与应激性高血糖、医源性或其他原因的高血糖症相鉴别;NDM可为暂时性和永久性,需密切动态观察.

Objective To study the clinical features of neonatal diabetes mellitus （NDM）.Method Thirteen cases with NDM were seen in our department between Jul. 2004 and Sept. 2009. Their clinical features were reviewed retrospectively. Results The average birth weight of the 13 cases was 2.30 kg. The median age at diagnosis was 2 months. The mean blood glucose at diagnosis was 22.2 mmol/L.Symptoms in 9 of 13 cases were exacerbated by infection and only 5 had typical symptoms of diabetes mellitus including polydipsia, polyuria, polyphagia and body weight loss. The common clinical findings included athrepsy, dipsesis, and moderate dehydration. Ketoacidosis attacked 3 cases and 3 children had hypertriglyceridemia, meanwhile, 2 children had complications of blood clotting dysfunction and congenital cardiopathy, respectively. Autoantibody to insulin （IAA） was tested in 11 cases, all but one case was negative. Glycosylated hemoglobin was increased in 6 cases. Insulin treatment was started in all the 13 on the variety of blood glucose. Blood glucose decreased significantly within 24 hours. Unfortunately, 1 case developed progressive blood glucose decline and recurrent hypoglycemia. Symptoms of the 3 cases who developed DKA were relieved 48 hours later, and their blood glucose was well under control. Among the 8 cases followed up, 4 had TNDM and 2 had PNDM. Unfortunately, 1 case died at the age of 3 months because insulin injection was stopped by the parents. Conclusion Early diagnosis and prompt management may lead to favorable prognosis. Blood glucose monitoring is a valuable method to avoid misdiagnosis and NDM should be differentiated from stress hyperglycemia, iatrogenic, or other causes of hyperglycemia.