Triad of Severe PUD, Gastric acid hypersecretion & Gastrinoma

Pathology:

• Around 90% of tumors occur in the pancreatic head or proximal duodenal wall
• At least half are multiple, and tumor size can vary from 1 mm to 20 cm.
• Approximately one-half to two-thirds are malignant but are often slow-growing. 
• Adenomas of the parathyroid and pituitary glands (MEN type 1) 

Clinical features

• PUD: 

• Severe, multiple, at unusual sites →the postbulbar duodenum, jejunum, esophagus

• H. Pylori and NSAIDs negative ulcer

• Poor response to standard ulcer therapy. 

• Diarrhea: 

• Abdominal mass

• Pancreatic primary or hepatic metastasis

SAQ. A6-year-old female presented to medical emergency with 2 episodes of hematemesis in the last 24 hours. On query, she complains about recurrent loose stool with occasional severe upper abdominal pain in the last 6 months. No drug history, except PPI. BMI- 18, Hb 10, Na- 130, K- 4.0, HCO3 21, Ca- 3.2, Creatinine 1.3 mg/dl, Urea breath is negative. Upper GI endoscopy showed two separate ulcers at duodenal cap and near the ampul.

1. Explain the pathophysiology of diarrhea in this patient

Multiple factory may be repole for diarrhea in ZES

The acid output large

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Reaches the upper small intestine,

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Reduce the luminal pH to 2 or less.

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Pancreatic lipase is inactivated and bile acids are precipitated.

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                                                                              Diarrhea, steatorrhea 

• Epithelial damage by acid leads to mild degree of maldigestion and malabsorption

1. Name 5 investigations to reach the diagnosis precisely

1. Mention 3 investigations to look for common association with this disease 

• Serum PTH
• MRI Brain, Serum Prolactin
• Pancreatic polypeptide
• Adrenal CT

Management

• Unifocal tumor → resection
• Multifocal/metastatic disease → surgery not appropriate 
  • Continuous high dose PPI therapy
  • Somatostatin analogue therapy

Prognosis:

• 5 year survival is 60-75%

Special Advice: 

• Genetic screening for MEN 1