Zollinger- Ellison Syndrome

ZOLLINGER- ELLISON SYNDROME


ZOLLINGER- ELLISON SYNDROME

ZES is a rare disorder which is characterised by the triad-

  1. Severe peptic ulceration
  2. Gastric acid hyper secretion
  3. Non β- cell islet tumour of pancreas (gastrinoma)
  • Males are more commonly affected.
  • Most common between 30 & 50 years.

Pathophysiology

  • Hypergastrinemia originates from autonomus neoplasm.
  • Gastrin stimulates acid secretion through gastrin receptor on parietal cells.
  • It is caused by gastrin secreting tumour. (G cells)
  • It is an exocrine tumour.

Clinical features-

  • Peptic ulcer is most common clinical features.
  • Diarrhoea & steatorrhea
  • MEN type 1 is present.
  • Bleeding & perforation
  • Duodenal tumors are commonly occurring.
  • Postbulbar ulcers
  • Recurrent or refractory ulcers

Investigations-

  • Serum gastrin elevated
  • Radiolabelled somatostatin receptor scintigraphy.
  • Gastrinoma patients have fasting gastrin level >150- 200 pg/ml
  • BAO >15 meq/h in the presence of hypergastrinemia is pathognomonic of ZES.
  • BAO/ MAO ratio >0.6 being highly suggestive of ZES.
  • The most sensitive and specific gastrin provocative test for the diagnosis of gastrinoma is the secretin study. (An increase in gastrin of ≥120 pg within 15 min of secretin injection has a sensitivity and specificity of >90% for ZES.)

Treatment-

  • PPIs are the treatment of choice (Omeprazole)
  • Recurrence may occur after surgery.

Exam Important

  • ZES is a rare disorder which is characterised by the triad-
  1. Severe peptic ulceration
  2. Gastric acid hyper secretion
  3. Non β- cell islet tumour of pancreas (gastrinoma)

Pathophysiology-

  • Hypergastrinemia originates from autonomus neoplasm.
  • Gastrin stimulates acid secretion through gastrin receptor on parietal cells.
  • It is caused by gastrin secreting tumour. (G cells)
  • It is an exocrine tumour.

Clinical features-

  • Peptic ulcer is most common clinical features.
  • Diarrhoea & steatorrhea
  • MEN type 1 is present.
  • Bleeding & perforation
  • Duodenal tumors are commonly occurring.
  • Postbulbar ulcers
  • Recurrent or refractory ulcers

Investigations-

  • Serum gastrin elevated
  • Radiolabelled somatostatin receptor scintigraphy.
  • Gastrinoma patients have fasting gastrin level >150- 200 pg/ml
  • BAO >15 meq/h in the presence of hypergastrinemia is pathognomonic of ZES.
  • BAO/ MAO ratio >0.6 being highly suggestive of ZES.
  • The most sensitive and specific gastrin provocative test for the diagnosis of gastrinoma is the secretin study. (An increase in gastrin of ≥120 pg within 15 min of secretin injection has a sensitivity and specificity of >90% for ZES.)

Treatment-

  • PPIs are the treatment of choice (Omeprazole)
  • Recurrence may occur after surgery.
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