The Zollinger Ellison syndrome in children is confirmed by: (AIIMS Nov 2010)
|A||Larger gastric rugal folds|
|C||Edema of small bowel mucosa|
|D||Calcium infusion test|
a. The Zollinger-Ellison syndrome (ZES) is caused by the uncontrolled secretion of abnormal amounts of gastrin by a pancreatic or duodenal neuroendocrine tumor (i.e., gastrinoma).
b. Most cases (80%) are sporadic, but 20% are inherited. The inherited or familial form of gastrinoma is associated with multiple endocrine neoplasia type I (MEN I), which consists of parathyroid, pituitary, and pancreatic (or duodenal) tumors.
c. Gastrinoma is the most common pancreatic tumor in patients with MEN I.
d. Patients with MEN I usually have multiple gastrinoma tumors, and surgical cure is unusual.
e. Sporadic gastrinomas are more often solitary and amenable to surgical cure.
f. Currently, about 50% of gastrinomas are malignant, with lymph node, liver, or other distant metastases at presentation.
g. The diagnosis of ZES is confirmed by the secretin stimulation test.
h. An intravenous bolus of secretin (2 U/kg) is given and gastrin levels are checked before and after injection.
i. An increase in serum gastrin of 200 pg/mL or greater suggests the presence of gastrinoma.