Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome-Clues to a Safer Diagnostic Process

Naykky Singh Ospina, Diane Donegan, Rene Rodriguez-Gutierrez, Zahraa Al-Hilli, William Francis Young

Research output: Contribution to journalArticle

3 Citations (Scopus)

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.

Original languageEnglish (US)
JournalAmerican Journal of Medicine
DOIs
StateAccepted/In press - 2016

Fingerprint

Zollinger-Ellison Syndrome
Multiple Endocrine Neoplasia Type 1
Proton Pump Inhibitors
Proton Therapy
Proton Pumps
Gastrins
Serum
Gastrinoma
Hypercalcemia
Peptic Ulcer
Calcium
Therapeutics
Neoplasms

Keywords

  • Gastrin
  • Multiple endocrine neoplasia type 1
  • Proton pump inhibitors
  • Zollinger-Ellison syndrome

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome-Clues to a Safer Diagnostic Process. / Singh Ospina, Naykky; Donegan, Diane; Rodriguez-Gutierrez, Rene; Al-Hilli, Zahraa; Young, William Francis.

In: American Journal of Medicine, 2016.

Research output: Contribution to journalArticle

@article{98e2d112afdf426c855fef957d39ba82,
title = "Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome-Clues to a Safer Diagnostic Process",
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.",
keywords = "Gastrin, Multiple endocrine neoplasia type 1, Proton pump inhibitors, Zollinger-Ellison syndrome",
author = "{Singh Ospina}, Naykky and Diane Donegan and Rene Rodriguez-Gutierrez and Zahraa Al-Hilli and Young, {William Francis}",
year = "2016",
doi = "10.1016/j.amjmed.2016.11.035",
language = "English (US)",
journal = "American Journal of Medicine",
issn = "0002-9343",
publisher = "Elsevier Inc.",

}

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 Francis

PY - 2016

Y1 - 2016

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.

AB - 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.

KW - Gastrin

KW - Multiple endocrine neoplasia type 1

KW - Proton pump inhibitors

KW - Zollinger-Ellison syndrome

UR - http://www.scopus.com/inward/record.url?scp=85011017827&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85011017827&partnerID=8YFLogxK

U2 - 10.1016/j.amjmed.2016.11.035

DO - 10.1016/j.amjmed.2016.11.035

M3 - Article

C2 - 28011308

AN - SCOPUS:85011017827

JO - American Journal of Medicine

JF - American Journal of Medicine

SN - 0002-9343

ER -