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The Zollinger–Ellison Syndrome: Dangers and Consequences of Interrupting Antisecretory Treatment

https://doi.org/10.1016/j.cgh.2011.08.012Get rights and content

Background & Aims

Patients with Zollinger–Ellison syndrome (ZES) are treated by proton pump inhibitors (PPIs) to protect them from severe and potentially lethal peptic complications related to massive gastric acid hypersecretion. We report here on the dangerous consequences of interrupting PPI therapy.

Methods

We describe 2 ZES patients in whom interruption of PPI treatment for diagnostic evaluation generated severe health complications.

Results

Less than 48 hours after stopping PPIs, patient 1 developed multiple strictures of the esophagus caused by massive vomiting of gastric acid, and patient 2 presented with severe abdominal pain with intestinal microperforation from duodenal ulcers. Because of persistent gastrin stimulatory drive and because of the abolition of reflex protective defense mechanisms against gastric acid hypersecretion during PPI treatment, patients with ZES under PPI therapy are exposed to severe peptic complications when facing rebound acid secretion at the interruption of their antisecretory treatment.

Conclusions

PPI treatment interruption has dangerous consequences, and PPI therapy always should be maintained in patients known or suspected of ZES. Diagnostic evaluations should be performed under PPI protection.

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.

References (17)

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Conflicts of interest The authors disclose no conflicts.

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