Wednesday, 17 November 2010

MRCP revision battle 52.1: Zollinger-Ellison Syndrome

Finally I have a topic to put under the 'z' catagory in my A to Z index!

Today we have:

MRCP revision battle 52.1: Zollinger-Ellison Syndrome
MRCP revision battle 52.2: Congential Hyperbilinrubinaemia
MRCP revision battle 52.3: Metformin
MRCP revision battle 52.4: Flecainide
MRCP revision battle 52.5: Arrhythmogenic right ventricular cardiomyopathy
MRCP revision battle 52.6: Pulmonary hypertension and cor pulmonale
MRCP revision battle 52.7: Scromboid poisoning

MRCP revision battle 52.1: Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome is a rare condition in which there are multiple gastric and duodenal ulcers in association with gastrin-secreting adenoma. 

The adenoma is usually pancreatic in origin but may be found in the stomach or duodenum.

50-60% of the adenomas in Zollinger-Ellison syndrome are malignant.
10-30% are associated with MEN-1.
(quick recap: MEN 1 = pancreatic tumours, parathyroid tumours and pituitary tumours)

  • epigastric pain from the ulcers
  • diarrhoea
  • malabsorption/steatorrhoea from inactivation of pancreatic enzymes

Diagnosis is by a raised fasting gastrin level.
Note gastrin levels will also be raised in achlorhydria - this differential can be eliminated by doing a secretin stimulation test which will cause a raised gastrin level in Zollinger-Ellison syndrome but no rise with achlorhydria.

Treatment is with high dose PPI (eg 60mg/day).
Ocreotide (somatostatin analogue) may help with symptom relief.

On to consider some congenital causes of jaundice...