Price D E, Absalom S R, Davidson K, Bolia A, Bell P R, Howlett T A
Leicester Royal Infirmary, UK.
Clin Endocrinol (Oxf). 1992 Aug;37(2):187-8. doi: 10.1111/j.1365-2265.1992.tb02305.x.
A 42-year-old woman with a family history of multiple endocrine neoplasia type 1 (MEN 1) presented with symptomatic hypoglycaemia and peptic ulceration. Investigation revealed an insulinoma, hyperparathyroidism, hypercalcitoninaemia with a positive pentagastrin stimulation test, acromegaly due to a GRF-oma, hyperprolactinaemia and normal serum gastrin levels. Five pancreatic tumours were removed at laparotomy and immunostaining was positive for insulin, calcitonin, somatostatin and glucagon. Post-operatively she developed elevated serum gastrin levels and gross peptic ulceration, despite H2-blockers, and died of gastro-intestinal haemorrhage suggesting that removal of the somatostatinoma may have allowed increased gastrin secretion from a gastrinoma. This case emphasizes the importance of measuring a wide variety of tumour marker peptides in MEN 1 and suggests that caution is required in interpretation of the pentagastrin stimulation test in such cases. Patients with MEN 1 and known peptic ulceration may require perioperative omeprazole treatment even if serum gastrin levels are normal.
一名有1型多发性内分泌腺瘤病（MEN 1）家族史的42岁女性，出现症状性低血糖和消化性溃疡。检查发现患有胰岛素瘤、甲状旁腺功能亢进、五肽胃泌素刺激试验阳性的高降钙素血症、因生长激素释放因子瘤导致的肢端肥大症、高泌乳素血症以及血清胃泌素水平正常。剖腹手术切除了5个胰腺肿瘤，免疫染色显示胰岛素、降钙素、生长抑素和胰高血糖素呈阳性。术后，尽管使用了H2阻滞剂，她的血清胃泌素水平仍升高且出现严重消化性溃疡，最终死于胃肠道出血，提示切除生长抑素瘤可能使胃泌素瘤分泌的胃泌素增加。该病例强调了在MEN 1中检测多种肿瘤标志物肽的重要性，并表明在此类病例中解释五肽胃泌素刺激试验时需谨慎。即使血清胃泌素水平正常，患有MEN 1且已知有消化性溃疡的患者围手术期可能也需要使用奥美拉唑治疗。
