Brief communicationThe Zollinger–Ellison Syndrome: Dangers and Consequences of Interrupting Antisecretory Treatment
Section snippets
Case Report: Patient 1
A 49-year-old man with diarrhea and a 10-kg weight loss over the past few months was admitted to the intensive care unit for exacerbated diarrhea and profuse vomiting with hypovolemic shock and acute renal failure. Computerized axial tomography showed large cystic hepatic lesions and important fluid accumulation in all intestinal loops. A nasogastric tube drained 3 L/d; IV pantoprazole 40 mg bid was started. The patient clinically improved, but symptoms quickly relapsed after withdrawal of
Case Report: Patient 2
A 51-year-old man treated for gastroesophageal reflux with esomeprazole 40 mg id was hospitalized for severe abdominal pain, vomiting, diarrhea, and dehydration. He was put on pantoprazole IV with rapid clinical improvement. Gastroscopy showed 3 ulcers in the distal duodenum. Computerized tomography scan revealed parietal thickness of the third duodenum with inflammatory changes around the duodenum and right psoas evoking microperforation.
Ultrasound endoscopy showed thickened gastric folds and
Discussion
PPIs revolutionized the treatment of ZES patients. Inadequate management of acid hypersecretion can result in rapid and life-threatening complications as shown here. Cessation of PPIs is recommended for investigation of ZES, but we disagree with this suggestion. The risk on the immediate health condition of these patients by stopping PPI far exceeds the potential benefit of interrupting it for an investigation that is elective and can rely on alternative strategies.
Conclusions
In ZES suspected patients, the risk of stopping PPI treatment on the health of these patients far exceeds the benefit of obtaining perfect diagnostic tests. In proven or suspected ZES: (1) never stop the PPI; (2) once patients are stabilized on a PPI and are out of danger for peptic complications, search for a differential diagnosis, and (3) if positive for ZES, look for the tumor to be removed surgically.
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Cited by (52)
Multiple endocrine neoplasia type 1 (MEN-1) and neuroendocrine neoplasms (NENs)
2022, Seminars in Cancer BiologyCitation Excerpt :In doubtful cases, provocative testing of fasting serum gastrin by intravenous secretin (2 U/kg) or calcium infusion (4 mg Ca2/kg/h for 3 h) can be helpful and should be performed, when possible, after 7 days withdrawal of proton pump inhibitors. However, discontinuation of proton pump inhibitors holds the risk of rebound acid secretion leading to acid-peptic complications [199,200]. Secretin inhibits the gastrin release in normal subjects but, on the contrary, stimulates gastrin secretion in Zollinger-Ellison syndrome patients and secretin provocation test has been reported to have high sensitivity (80–94 %) and specificity (81–100 %) for Zollinger-Ellison syndrome [201–203].
Multiple endocrine neoplasia type 1 (MEN1) presenting with renal stones: Case report and review
2020, Radiology Case ReportsGastrinomas
2018, Encyclopedia of Endocrine DiseasesPrimary lymph node gastrinoma: A single institution experience
2017, Surgery (United States)Citation Excerpt :We have found that many patients, especially those that present with advanced symptoms of visceral perforation or bleeding, are intolerant of withdrawal of antisecretory medications such as proton pump inhibitors, which are necessary for measurement of gastrin levels, gastric acid secretory studies and preferred for provocative tests. Rebound acid secretion off proton pump inhibitors in patients with ZES leading to visceral perforation has been described.26 The preoperative localization practices seen in our series are also atypical compared with the literature.
Assessing for Multiple Endocrine Neoplasia Type 1 in Patients Evaluated for Zollinger-Ellison Syndrome—Clues to a Safer Diagnostic Process
2017, American Journal of MedicineCitation Excerpt :A high level of suspicion for multiple endocrine neoplasia type 1 may have prevented such outcomes. These cases add to reports suggesting that abrupt discontinuation of PPI can lead to complications.1,5 PPI therapy can suppress defensive mechanisms against acid hypersecretion and lead to adverse events after discontinuation of therapy, due to continued hypersecretion of gastrin.5
Conflicts of interest The authors disclose no conflicts.