Singh Ospina Naykky, Donegan Diane, Rodriguez-Gutierrez Rene, Al-Hilli Zahraa, Young William F
Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, University of Florida, Gainesville; Knowledge and Evaluation Research Unit in Endocrinology (KER-Endo), Division of Endocrinology, Diabetes, Metabolism and Nutrition, Department of Medicine, Mayo Clinic, Rochester, Minn.
Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, Mayo Clinic, Rochester, Minn.
Am J Med. 2017 May;130(5):603-605. doi: 10.1016/j.amjmed.2016.11.035. Epub 2016 Dec 21.
Zollinger-Ellison syndrome is a rare cause of tumoral hypergastrinemia; 1 of 5 patients with this syndrome also has multiple endocrine neoplasia type 1. The diagnosis of this disease is complicated by the widespread use of proton pump inhibitors that can elevate serum gastrin levels, the cornerstone for biochemical diagnosis. Abrupt discontinuation of proton pump inhibitors could lead to adverse outcomes. Clinician awareness of the relationship between Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1 could lead to a safer diagnostic pathway.
We conducted a retrospective review of a cohort of patients with multiple endocrine neoplasia type 1.
There were 287 patients with multiple endocrine neoplasia type 1 (73 with gastrinoma) evaluated between 1997 and 2014. Two patients experienced adverse events after proton pump inhibitor therapy was discontinued to re-measure serum gastrin level during the evaluation of severe peptic ulcer disease. In both cases, the diagnosis of multiple endocrine neoplasia type 1 was made after proton pump therapy was discontinued.
Abrupt discontinuation of proton pump therapy can lead to adverse outcomes in patients with Zollinger-Ellison syndrome. Clinical assessment for features of multiple endocrine neoplasia type 1 (eg, serum calcium levels, personal and family history of hypercalcemia, pituitary or pancreatic tumors) could identify patients with higher risk for a tumoral source of hypergastrinemia where imaging studies can help support the diagnosis without the potential side effects of abrupt discontinuation of proton pump inhibitor therapy.
卓-艾综合征是肿瘤性高胃泌素血症的罕见病因；该综合征患者中五分之一还患有1型多发性内分泌腺瘤病。由于广泛使用可升高血清胃泌素水平（生化诊断的基石）的质子泵抑制剂，该疾病的诊断变得复杂。突然停用质子泵抑制剂可能会导致不良后果。临床医生对卓-艾综合征与1型多发性内分泌腺瘤病之间关系的认识有助于建立更安全的诊断途径。
我们对一组1型多发性内分泌腺瘤病患者进行了回顾性研究。
1997年至2014年间，共评估了287例1型多发性内分泌腺瘤病患者（73例患有胃泌素瘤）。在评估严重消化性溃疡疾病期间，为重新测量血清胃泌素水平而停用质子泵抑制剂治疗后，有2例患者出现了不良事件。在这两例中，均在停用质子泵治疗后确诊为1型多发性内分泌腺瘤病。
突然停用质子泵治疗可导致卓-艾综合征患者出现不良后果。对1型多发性内分泌腺瘤病特征进行临床评估（如血清钙水平、高钙血症的个人及家族史、垂体或胰腺肿瘤），可识别出高胃泌素血症肿瘤来源风险较高的患者，在此情况下，影像学检查有助于支持诊断，而无需承担突然停用质子泵抑制剂治疗的潜在副作用。
