Lestradet H, Deschamps I, Tichet J, Lestradet M O
Département de Diabétologie Pédiatrique, Hôpital Robert-Debré, Paris.
Arch Fr Pediatr. 1989 Jan;46(1):19-23.
We report the clinical records of 45 children with abnormalities regarding glycemic regulation characterized by a non-insulin deficient hyperglycemia (NIDH), known under the different names of chemical diabetes, sub-clinical diabetes and more recently MODY. These 45 children belong to 31 families with 532 relatives comprising 137 cases of NIDH which could have been studied. The symptoms of this biochemical abnormality, the pathophysiology of which is not yet clearly understood, are the following: lack of clinical manifestations, except for a variable and intermittent glycosuria; constant abnormal glucose tolerance tests, above 97 percentiles of the reference value with some variations over time; normal immunoreactive insulin levels; percentage of glycosylated hemoglobin at the upper range of normal; dominant autosomal genetic transmission and no association with HLA markers like in insulin-dependent diabetes; lack of degenerative complications of the micro-angiopathic type, at least on these cases even after more than 30 years of follow-up; finally, no tendency towards insulin-dependent diabetes. The NIDH should not be confused with the slow and progressive beginning of insulin-dependent diabetes for which prolonged delay is needed to affirm the diagnosis. The frequency of the biochemical phenomena is about 1.8% of the cases of authentic diabetes mellitus occurring before the age of 15.
我们报告了45例血糖调节异常儿童的临床记录，这些儿童的特点是存在非胰岛素缺乏性高血糖（NIDH），它曾有化学性糖尿病、亚临床糖尿病等不同名称，最近又称为青少年发病的成年型糖尿病（MODY）。这45名儿童分属31个家庭，共有532名亲属，其中137例可能患有NIDH，本可对其进行研究。这种生化异常的症状如下：除了可变的间歇性糖尿外，缺乏临床表现；葡萄糖耐量试验持续异常，高于参考值的第97百分位数，且随时间有所变化；免疫反应性胰岛素水平正常；糖化血红蛋白百分比处于正常范围上限；呈常染色体显性遗传，且不像胰岛素依赖型糖尿病那样与HLA标记相关；至少在这些病例中，即使经过30多年的随访，也没有微血管病变类型的退行性并发症；最后，没有发展为胰岛素依赖型糖尿病的趋势。NIDH不应与胰岛素依赖型糖尿病缓慢进展的初期相混淆，后者需要较长时间才能确诊。这种生化现象的发生率约占15岁前真正糖尿病病例的1.8%。
