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)
Presentation:
- 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...
