Hardy J D, Doolittle P D
Ann Surg. 1977 Jun;185(6):661-71. doi: 10.1097/00000658-197706000-00008.
Twelve patients with Zollinger-Ellison syndrome and one patient with WDHA syndrome are reviewed. Three of the Z-E patients exhibited MEA, two having hyperinsulinism and one hyperparathyroidism. Ages ranged from nine to 71 years. Diagnosis of Z-E syndrome was established from history, gastric acid secretion, radiologic studies, serum gastrin measurements and from actual tissue biopsy in 10 of the 12 patients. Total gastrectomy was performed in 8 of the 12 Z-E patients, with abolition of the ulcer diathesis in all. However, in none of our patients was there objective evidence of subsequent tumor regression. Three patients remain alive. Four died of tumor, one from post-total gastrectomy complications, one from post-subtotal gastrectomy in another hospital, two from ulcer hemorrhage, and one from electrolyte imbalance with autopsy diagnosis of Z-E tumor. A patient is recorded in detail who exhibited both hyperinsulinemia and hypergastrinemia from a malignant islet cell tumor, had the tumor "debulked" four times over a 14 year period and whose hepatic metastases were temporarily abolished by streptozotocin infusion. The question is raised regarding relationships between chronic organic hyperinsulinism and subsequent hypergastrinemia.
对12例卓-艾综合征患者和1例WDHA综合征患者进行了回顾性研究。12例卓-艾综合征患者中有3例出现多发性内分泌腺瘤，其中2例为高胰岛素血症，1例为甲状旁腺功能亢进。年龄范围为9岁至71岁。12例患者中，10例根据病史、胃酸分泌、放射学检查、血清胃泌素测定及实际组织活检确诊为卓-艾综合征。12例卓-艾综合征患者中有8例行全胃切除术，术后所有患者的溃疡素质均消失。然而，我们的患者中均无肿瘤消退的客观证据。3例患者存活。4例死于肿瘤，1例死于全胃切除术后并发症，1例死于另一家医院的胃次全切除术后，2例死于溃疡出血，1例死于电解质失衡，尸检诊断为卓-艾肿瘤。详细记录了1例患者，该患者因恶性胰岛细胞瘤出现高胰岛素血症和高胃泌素血症，在14年期间肿瘤“减瘤”4次，链脲佐菌素输注暂时消除了其肝转移。文中提出了关于慢性器质性高胰岛素血症与随后的高胃泌素血症之间关系的问题。
