Clinical and Genetic Investigation of a Large Kindred with Multiple Endocrine Adenomatosis

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Abstract
MULTIPLE endocrine adenomatosis is characterized by hyperplasia or tumors of one or more endocrine glands.1 There is usually increased function of the glands involved. The parathyroid glands, pancreatic islet cells and pituitary gland are most frequently affected. Of 85 patients with the syndrome reported on before 1964, 74 had evidence of hyperparathyroidism, and 69 had pancreatic islet-cell tumors, which were associated with demonstrable peptic ulcer in 42 and hypoglycemia in 25 patients; however, only 15 of the 55 patients with pituitary tumors had acromegaly.2 Less common findings resulting from these or other tumors attributed to the disease include pituitary insufficiency, . . .