Prevalence of endocrine abnormalities in patients with the Zollinger-Ellison syndrome and in their families.

To evalute the frequency of associated and hereditary endocrinopathies in the Zollinger-Ellison syndrome, 10 patients with the syndrome were studied. In seven of them, coexisting endocrine disease was found. In six, the Zollinger-Ellison syndrome was probably a feature of multiple endocrine adenomatosis type I, whereas Cushing's syndrome in the remaining patient may have been caused by the production of an ACTH-like substance by a mixed pancreatic tumor. A total of 109 family members, including all living first degree relatives over 15 years of age, were screened for endocrine abnormalities. All six patients with evidence of multiple endocrine adenomatosis type I had relatives with endocrinopathies. In the families of the four other patients with the Zollinger-Ellison syndrome, no endocrine abnormalities were found. During this study four new cases of pituitary tumor, 17 of hyperparathyroidism, seven of the Zollinger-Ellison syndrome and one of insulinoma were detected. Although most of the disorders were asymptomatic, this clearly indicates that patients suffering from Zollinger-Ellison syndrome and also their families should undergo detailed endocrine studies.