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August 1963

Familial Endocrine Adenomatosis: A Family With Hyperinsulinism as the Predominant Manifestation

Author Affiliations

Louis E. Underwood, MD, Department of Pediatrics, Vanderbilt University School of Medicine, Nashville 5, Tenn.; From the Department of Pediatrics, Vanderbilt University School of Medicine.

Am J Dis Child. 1963;106(2):218-223. doi:10.1001/archpedi.1963.02080050220018

Introduction  In 1903, Erdheim 1 first described the occurrence of multiple endocrine tumors. Since then, the recognition of a syndrome has emerged. This syndrome, referred to as familial endocrine adenomatosis, or familial polyglandular adenomatosis, is characterized by the occurrence of a tumor or tumors, not necessarily simultaneously, of more than one endocrine gland, generally the parathyroid, pituitary, or islet cells of the pancreas, in more than one member of a family. This is the report of a family of 12 in whom three members—a father, son, and daughter—had one or more endocrine tumors. The presenting complaint in all three patients was related to hyperinsulinism.

Report of Cases  Case 1.—This patient was an 18-year-old white male who entered another hospital on Feb 3, 1961, with the chief complaint of "passing-out spells" for six to seven years. These episodes of unconsciousness, which were transient, were frequently preceded by nausea and perspiring and

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