Noninsulinoma pancreatogenous hypoglycemia syndrome: An update in 10 surgically treated patients
Noninsulinoma pancreatogenous hypoglycemia syndrome: An update in 10 surgically treated patients
复制标题
DOI：
10.1067/msy.2000.110243
复制
发表时间：
2000-12-01
期刊：
影响因子：
3.8
通讯作者：
Grant, CS
中科院分区：
文献类型：
--
作者：
Thompson, GB;Service, FJ;Grant, CS
背景内源性高胰岛素血症引起的神经性低血糖症通常由成人胰岛素瘤引起。我们最近报道了5例患者（患者1 ~ 5）发生的一种新型低血糖症，患者餐后神经性低血糖，72小时禁食阴性，围手术期影像学检查阴性，但钙刺激试验阳性，梯度引导下切除的胰腺中胰岛肥大和胰岛母细胞增多症。在这份报告中，我们比较了我们的经验，另外5例（患者6至10）与这种综合征的原始报告。患者6 ~ 10的临床特征与患者1 ~ 5相似。每例患者的钙离子刺激试验均为阳性，可指导远端胰腺切除术的范围和胰岛细胞肥大或胰岛母细胞增多症的组织学证据。10例患者术后均存活9 ~ 50个月，其中1例神经性低血糖无改善。3例患者发生轻微围手术期并发症。1例患者反复发作急性胰腺炎、假性囊肿形成和外分泌功能不全。我们已经发现患有弥漫性胰岛细胞病的严重餐后高胰岛素低血糖的成年患者，其中80%通过手术得到了良好的缓解。7名男性的结果优于3名女性，原因不明显。
Background. Neuroglycopenia from endogenous hyperinsulinism usually is caused by insulinomas in adults. We recently reported a novel hypoglycemic disorder in 5 patients (patients 1 to 5) with postprandial neuroglycopenia, negative 72-hour fasts, negative perioperative imaging studies, but positive calcium stimulation tests and islet hypertrophy and nesidioblastosis in the gradient-guided resected pancreata.Methods. In this report we compare our experience with 5 additional patients (patients 6 to 10) with this syndrome to that in the original report.Results. The clinical features of patients 6 to 10 were similar to those of patients 1 to 5. Each had positive calcium stimulation testing that guided the extent of the distal pancreatectomy and histologic evidence of islet cell hypertrophy or nesidioblastosis. All 10 patients are alive from 9 to 50 months after operation, 1 of whom had no amelioration of neuroglycopenia. Minor perioperative complications occurred in 3 patients. One patient has experienced repeated bouts of acute pancreatitis, pseudocyst formation, and exocrine insufficiency.Conclusions. We have identified adult patients with severe, postprandial hyperinsulinemic hypoglycemia from diffuse islet cell disease, 80% of whom have been well palliated with surgery. The results in 7 men have been better than those in the 3 women for reasons that are not obvious.