When should a patient with Zollinger-Ellison Syndrome be referred to a specialist for further management?

Patients with Zollinger-Ellison Syndrome should be referred to a specialist for further management when there is diagnostic uncertainty, failure to respond adequately to initial treatment, or when complex management such as surgical intervention or advanced medical therapy is required.

Although the provided UK guidelines do not explicitly mention Zollinger-Ellison Syndrome, the general principles for referral in complex acid-related disorders apply. Referral to a gastroenterologist or specialist service is recommended if symptoms are refractory to treatment or unexplained after initial management in primary care, which would include cases of Zollinger-Ellison Syndrome given its complexity and need for specialist investigation and management.

Specifically, patients with peptic ulcer disease that is refractory to standard treatment, or those with complications or suspicion of gastrinoma (the cause of Zollinger-Ellison Syndrome), should be referred urgently to a specialist for further assessment, including biochemical testing, imaging, and consideration of surgical options.

Therefore, referral should occur when:

• There is suspicion or confirmation of Zollinger-Ellison Syndrome based on clinical features such as severe or refractory peptic ulcer disease.
• Symptoms do not respond to proton pump inhibitors or other acid suppression therapies.
• There is a need for specialist investigations such as gastrin level measurement, secretin stimulation test, or imaging for tumour localization.
• Consideration of surgical management or advanced therapies is required.

This approach aligns with the NICE guidance on referral for complex or refractory acid-related disorders and specialist management of peptic ulcer disease and dyspepsia.

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