Norton J A, Kahn C R, Schiebinger R, Gorschboth C, Brennan M F
Ann Intern Med. 1979 Aug;91(2):213-5. doi: 10.7326/0003-4819-91-2-213.
A 47-year-old white man had a malignant glucagonoma and severe necrolytic migratory erythema. His plasma glucagon levels were markedly elevated at 50 ng/mL and plasma amino acids diminished to 45% of normal. To test the hypothesis that the skin rash associated with a glucagonoma is secondary to an amino acid deficiency, we obtained 2 d of fasting baseline laboratory data from the patient while he consumed his usual diet. He was then given 3 L/d of supplemental intravenous amino acids for 3 d. His plasma amino acid levels increased slightly, and there was some improvement in his skin rash. Immediately thereafter, total parenteral nutrition was administered for 3 d without added zinc or fatty acids. During total parenteral nutrition, 14 of 17 plasma amino acids became normal, and the patient's skin rash rapidly disappeared. These findings suggest that the skin rash associated with a glucagonoma is most likely due to an amino acid deficiency and can be reversed by parenteral nutrition.
一名47岁的白人男性患有恶性胰高血糖素瘤及严重的坏死性游走性红斑。其血浆胰高血糖素水平显著升高至50 ng/mL,血浆氨基酸水平降至正常的45%。为了验证与胰高血糖素瘤相关的皮疹是由氨基酸缺乏继发引起这一假说,我们在患者食用平常饮食时获取了其2天的空腹基线实验室数据。随后,给他连续3天每天静脉补充3升氨基酸。其血浆氨基酸水平略有升高,皮疹也有一定改善。此后紧接着,在未添加锌或脂肪酸的情况下给予全胃肠外营养3天。在全胃肠外营养期间,17种血浆氨基酸中有14种恢复正常,患者的皮疹迅速消失。这些发现表明,与胰高血糖素瘤相关的皮疹很可能是由氨基酸缺乏所致,且可通过胃肠外营养得到逆转。