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Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome-Clues to a Safer Diagnostic Process.
Am J Med. 2017 May; 130(5):603-605.AJ

Abstract

BACKGROUND

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.

METHODS

We conducted a retrospective review of a cohort of patients with multiple endocrine neoplasia type 1.

RESULTS

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.

CONCLUSION

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.

Authors+Show Affiliations

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.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, Department of Internal Medicine, University Hospital "Jose E. Gonzalez", Autonomous University of Nuevo Leon, Monterrey, Mexico.Department of Surgery, Mayo Clinic, Rochester, Minn; Department of General Surgery, Cleveland Clinic, Ohio.Division of Endocrinology, Diabetes and Metabolism, Department of Medicine, Mayo Clinic, Rochester, Minn. Electronic address: wyoung@mayo.edu.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

28011308

Citation

Singh Ospina, Naykky, et al. "Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome-Clues to a Safer Diagnostic Process." The American Journal of Medicine, vol. 130, no. 5, 2017, pp. 603-605.
Singh Ospina N, Donegan D, Rodriguez-Gutierrez R, et al. Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome-Clues to a Safer Diagnostic Process. Am J Med. 2017;130(5):603-605.
Singh Ospina, N., Donegan, D., Rodriguez-Gutierrez, R., Al-Hilli, Z., & Young, W. F. (2017). Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome-Clues to a Safer Diagnostic Process. The American Journal of Medicine, 130(5), 603-605. https://doi.org/10.1016/j.amjmed.2016.11.035
Singh Ospina N, et al. Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome-Clues to a Safer Diagnostic Process. Am J Med. 2017;130(5):603-605. PubMed PMID: 28011308.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome-Clues to a Safer Diagnostic Process. AU - Singh Ospina,Naykky, AU - Donegan,Diane, AU - Rodriguez-Gutierrez,Rene, AU - Al-Hilli,Zahraa, AU - Young,William F,Jr Y1 - 2016/12/21/ PY - 2016/10/05/received PY - 2016/11/16/revised PY - 2016/11/21/accepted PY - 2016/12/25/pubmed PY - 2017/7/1/medline PY - 2016/12/25/entrez KW - Gastrin KW - Multiple endocrine neoplasia type 1 KW - Proton pump inhibitors KW - Zollinger-Ellison syndrome SP - 603 EP - 605 JF - The American journal of medicine JO - Am. J. Med. VL - 130 IS - 5 N2 - BACKGROUND: 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. METHODS: We conducted a retrospective review of a cohort of patients with multiple endocrine neoplasia type 1. RESULTS: 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. CONCLUSION: 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. SN - 1555-7162 UR - https://www.unboundmedicine.com/medline/citation/28011308/Assessing_for_Multiple_Endocrine_Neoplasia_Type_1_in_Patients_Evaluated_for_Zollinger_Ellison_Syndrome_Clues_to_a_Safer_Diagnostic_Process_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0002-9343(16)31249-9 DB - PRIME DP - Unbound Medicine ER -