Zollinger–Ellison Syndrome :

 

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Zollinger–Ellison Syndrome (ZES)

Definition

Zollinger–Ellison Syndrome is a rare disorder characterized by one or more gastrin-secreting tumors (gastrinomas), usually located in the pancreas or duodenum, leading to excessive gastric acid secretion. This results in recurrent, severe peptic ulcers, gastroesophageal reflux disease (GERD), and diarrhea.

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Causes

• Primary cause: Gastrinoma (neuroendocrine tumor secreting gastrin).

• Locations of gastrinomas:

  • Duodenum (most common)

  • Pancreas

  • Lymph nodes (rare)

• Associated conditions:

  • Multiple Endocrine Neoplasia type 1 (MEN1) – about 25% of cases

• Risk factors: Family history of MEN1, genetic mutations.

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Signs and Symptoms

• Gastrointestinal manifestations:

  • Recurrent peptic ulcers (often resistant to standard therapy)

  • Epigastric pain (burning or gnawing)

  • Gastroesophageal reflux (heartburn, regurgitation)

  • Diarrhea and steatorrhea (due to acid inactivation of pancreatic enzymes)

• Systemic findings:

  • Weight loss

  • GI bleeding (melena, hematemesis)

  • Nausea, vomiting

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Investigations

1. Blood Tests:

   • Fasting serum gastrin level (elevated > 1000 pg/mL strongly suggestive)

   • Secretin stimulation test (rise in gastrin confirms diagnosis)

   • Serum calcium and parathyroid hormone (to rule out MEN1)

2. Imaging Studies:

   • Endoscopic ultrasound (EUS)

   • Somatostatin receptor scintigraphy (Octreoscan)

   • CT/MRI of pancreas and duodenum

3. Endoscopy:

   • Visualization of multiple/refractory ulcers

   • Gastric pH measurement (acid hypersecretion)

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Medical Treatment

• Acid suppression:

  • High-dose Proton Pump Inhibitors (e.g., omeprazole, pantoprazole)

  • H2-receptor antagonists (less effective, adjunct)

• Somatostatin analogs (e.g., octreotide): Inhibit gastrin release and tumor growth.

• Chemotherapy (for metastatic disease): Streptozocin, 5-FU, doxorubicin.

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Surgical Treatment

• Curative approach: Surgical resection of localized gastrinoma if feasible.

• In MEN1-associated cases: Surgery often limited due to multiple tumors.

• Advanced/metastatic disease:

  • Debulking surgery

  • Liver resection or radiofrequency ablation (for liver metastases)

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Nursing Care Plan

Nursing Diagnosis:

1. Acute pain related to ulcer disease and gastric hypersecretion.

2. Imbalanced nutrition: less than body requirements related to malabsorption and diarrhea.

3. Risk for gastrointestinal bleeding related to ulceration.

4. Knowledge deficit related to disease process and treatment regimen.

Goals/Expected Outcomes:

• Patient will verbalize pain relief after treatment.

• Patient will maintain adequate nutritional status.

• Patient will remain free from active GI bleeding.

• Patient will demonstrate understanding of disease and self-care.

Nursing Interventions:

• Monitor pain level and administer prescribed acid-suppressive therapy.

• Assess stool for blood and monitor hemoglobin/hematocrit.

• Encourage small, frequent, non-irritating meals.

• Monitor weight and nutritional intake.

• Educate patient about medication adherence, follow-up tests, and signs of complications (bleeding, obstruction).

• Provide emotional support due to chronic nature of illness.

Evaluation:

• Pain controlled and patient reports relief.

• Stable hemoglobin and no signs of GI bleeding.

• Improved weight and nutritional status.

• Patient demonstrates knowledge of treatment and lifestyle modifications.

Nursing Diagnosis	Goals/Expected Outcomes	Nursing Interventions	Rationale	Evaluation
Acute pain related to peptic ulceration and gastric hypersecretion	- Patient reports pain relief - Improved comfort and rest	- Assess pain level regularly - Administer PPIs/H2 blockers as prescribed - Provide comfort measures (positioning, relaxation)	Acid suppression reduces gastric irritation; comfort promotes coping	- Pain score decreased - Patient verbalizes comfort
Imbalanced nutrition: less than body requirements related to malabsorption & diarrhea	- Maintain/achieve adequate nutrition - Stable weight	- Provide small, frequent, non-irritating meals - Monitor daily weight & intake - Collaborate with dietitian	Reduces gastric stimulation, ensures adequate calories	- Stable/gradual weight gain - Adequate intake maintained
Risk for gastrointestinal bleeding related to ulcer disease	- No evidence of GI bleeding - Stable hemoglobin/hematocrit	- Monitor stool for occult blood - Monitor H/H and vitals - Educate patient on signs of bleeding (melena, hematemesis)	Early detection prevents complications; patient self-monitoring enhances safety	- No bleeding episodes - Lab values stable
Knowledge deficit related to disease process & management	- Patient verbalizes understanding of disease, treatment & self-care	- Provide education on medication adherence - Teach importance of follow-up tests - Educate on avoiding NSAIDs, alcohol, smoking	Knowledge promotes adherence and reduces risk of complications	- Patient correctly explains disease & treatment - Demonstrates correct self-care behaviors