Dorchy H, Ooms H, Loeb H
Z Kinderheilkd. 1975;118(4):271-81. doi: 10.1007/BF00492333.
The 11th case of permanent neonatal diabetes mellitus appearing during the first month of life is reported. A critical review of the literature is also presented. The permanence of diabetes is demonstrated by the duration of insulin therapy still necessary after 30 months. Insulin-stimulation tests have been performed some for the first time in such a young diabetic. They have shown a nearly total failure in beta-cell response, only very high doses of glucagon provoking a moderate insulin secretion. The absence of acetonuria is discussed. It can perhaps be explained by the hyperglycemia which, by a mass effect, brings about cellular glucose penetration and this stops liberation of Nefa's from adipose tissue.
报告了第11例在出生后第一个月出现的永久性新生儿糖尿病病例。同时还对文献进行了批判性综述。糖尿病的持续性通过30个月后仍需胰岛素治疗的时间得以证明。首次对如此年幼的糖尿病患者进行了胰岛素刺激试验。试验显示β细胞反应几乎完全失败,只有非常高剂量的胰高血糖素能引起适度的胰岛素分泌。讨论了无丙酮尿症的情况。这或许可以通过高血糖来解释,高血糖通过质量效应导致细胞葡萄糖渗透,从而阻止脂肪组织中脂肪酸的释放。