Hyperparathyroidism: a prerequisite for Zollinger-Ellison syndrome in multiple endocrine adenomatosis Type 1—report of a further family and a review of the literature

Abstract

A family with Multiple Endocrine Adenomatosis Type 1 (MEA 1) is described. Of the 59 members, 15 were affected. Zollinger-Ellison syndrome was present in six individuals of whom the five available for study had hyperparathyroidism. The youngest of these patients with Zollinger-Ellison Syndrome was 25 years old. Hyperparathyroidism alone was present in nine individuals of whom the eldest was 30 years old. Three other members had raised serum gastrin levels but lacked symptoms suggestive of Zollinger-Ellison syndrome and in these individuals, the serum calcium levels were normal. A review of the literature confirms the finding that, in MEA 1, Zollinger-Ellison syndrome has not been shown to occur in the absence of hypercalcaemia. Screening of affected members of such families can therefore be restricted to the measurements of serum calcium levels.