Boden G, Owen O E
N Engl J Med. 1977 Mar 10;296(10):534-8. doi: 10.1056/NEJM197703102961003.
Basal immunoreactive glucagon was elevated in four of nine asymptomatic relatives of a patient with glucagonoma. Immunoreactive glucagon remained elevated throughout 22 to 25 hours of continuous observation. Glucagon responses to intravenous glucose and arginine or mixed meals (or both) were abnormal, whereas glucose and insulin responses were normal. Gel filtration of plasma revealed that over 85 per cent of the four relatives' immunoreactive glucagon had a molecular weight of greater than 9000 daltons whereas that of 70 per cent of the patients with glucagonoma had a molecular weight of 3500 daltons, with the remainder eluting in the area of 9000 daltons. Pancreatic angiograms and hepatic scintiscans were normal in all four relatives. The data suggest an autosomal dominant transmission of hyperglucagonemia in this family. Immunoreactive glucagon with a molecular weight of 3500 or 9000 daltons appears to be required for the development of the clinical glucagonoma syndrome.
在一名胰高血糖素瘤患者的9名无症状亲属中，有4人的基础免疫反应性胰高血糖素水平升高。在连续22至25小时的观察期间，免疫反应性胰高血糖素一直处于升高状态。胰高血糖素对静脉注射葡萄糖、精氨酸或混合餐（或两者）的反应异常，而葡萄糖和胰岛素反应正常。血浆凝胶过滤显示，这4名亲属中超过85%的免疫反应性胰高血糖素分子量大于9000道尔顿，而70%的胰高血糖素瘤患者的胰高血糖素分子量为3500道尔顿，其余在9000道尔顿区域洗脱。所有4名亲属的胰腺血管造影和肝脏闪烁扫描均正常。数据表明该家族中高胰高血糖素血症为常染色体显性遗传。临床胰高血糖素瘤综合征的发生似乎需要分子量为3500或9000道尔顿的免疫反应性胰高血糖素。
