All of the following are true about Zollinger Ellison Syndrome except -
Ans. is 'd' i.e., They may be associated with insulinomas Zollinger Ellison syndromeo ZES is caused by gastrin secreting gut neuroendocrine tumors (gastrinomas), which result in hypergastrinemia and acid hypersecretion.o Clinical situations that create suspicion for ZES are -# ulcers in unusual locations# ulcers in second part of duodenum & beyond# ulcers refractory to standard medical therapy# ulcer recurrance after acid reducing surgery# ulcers presenting with frank complications (bleeding, perforation and obstruction)# ulcers in the absence of H. pylori or NS AID ingestion. - giant ulcers (> 2 cm)# multiple duodenal ulcers# ulcers associated with diarrhoea# ulcer patient with hypercalcemia or family h/o ulcers# ulcers with severe esophagitis.# ulcers associated with prominent gastric folds# ulcers with findings s/o MEN-1 (endocrinopathy, family h/o ulcers or endocrinopathy, nephrolithiasis)Diagnosis of ZES (gastrinoma)o The first step in evaluation of a patient suspected of having ZES or gastrinoma is to obtain as fasting gastrin level. All gastrinoma patients have a fasting gastrin level > 150 pg/mL.o The next step is to assess acid secretion by measuring basal acid output (BAO) which should be 15 meq/h (normal < 4 meq/h) A gastric pH of > 3.0 implies hypochlorhydria and excludes gastrinomao Gastrin Provocative tests# In a pt. with pH < 2.0 or BAO 15 meq/hr and fasting gastrin level > 1000 pg/mL, the diagnosis of ZES is confirmed.# With pH < 2.0 or BAO 15 meq/L and lower gastrin levels (150-1000 pg/mL), a secretin stimulation test is perforfmed to distinguish ZES from other causes of hypergastrinemia.# A rise of 200 pg/mL of serum gastrin level within 15 min of secretin injection, confirms ZES.
It is due to the presence of a gastrinoma
Gastrin provocation tests are a part of diagnostic work up
It might be associated with ulcers in unusual locations in stomach
They may be associated with insulinomas
Pathophysiology - Pancreatic Endocrine Tumors