Sodoyez-Goffaux F, Sodoyez J C
J Pediatr. 1977 Sep;91(3):395-9. doi: 10.1016/s0022-3476(77)81306-1.
This neonate developed marked hyperglycemia four days after birth and required insulin therapy for eight weeks. During the acute phase of the disease, immunoreactive insulin was undetectable in portal venous serum. Neither tolbutamide nor theophylline administration significantly triggered insulin secretion. Somatostatin infusion inhibited growth hormone release but had no effect on plasma glucagon or blood glucose concentrations. At 2 1/2 months, two weeks after insulin withdrawal, the infant was still intolerant to an oral glucose load, insulin response was markedly delayed, and growth hormone secretion was paradoxical. At five months, the insulin, glucagon, and growth hormone responses to glucose and to somatostatin were normalized. Thus, in this patient, insulin secretion was transiently deficient. Peculiarities of glucagon and growth hormone secretion were also present but are more characteristic of this age group than of diabetes. The hyperglycemic state was managed by intraportal infusion of 0.1 to 0.2 IU regular insulin/kg/hour. This mode of insulin administration proved efficient, secure, and easy to manage.
该新生儿出生四天后出现明显高血糖,需胰岛素治疗八周。在疾病急性期,门静脉血清中检测不到免疫反应性胰岛素。给予甲苯磺丁脲或茶碱均未显著激发胰岛素分泌。输注生长抑素可抑制生长激素释放,但对血浆胰高血糖素或血糖浓度无影响。在2个半月时,即停用胰岛素两周后,婴儿仍不能耐受口服葡萄糖负荷,胰岛素反应明显延迟,生长激素分泌异常。五个月时,胰岛素、胰高血糖素和生长激素对葡萄糖及生长抑素的反应恢复正常。因此,该患者的胰岛素分泌存在短暂性缺乏。胰高血糖素和生长激素分泌也有异常,但这更具该年龄组的特征,而非糖尿病的特征。高血糖状态通过门静脉内输注0.1至0.2 IU正规胰岛素/千克/小时进行处理。这种胰岛素给药方式证明有效、安全且易于管理。