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The Adrenogenital Syndrome

1963; Massachusetts Medical Society; Volume: 268; Issue: 23 Linguagem: Inglês

10.1056/nejm196306062682308

1533-4406

Alfred M. Bongiovanni, Allen W. Root,

Adrenal and Paraganglionic Tumors

THE adrenogenital syndrome due to congenital adrenocortical hyperplasia is a disorder of man, now properly recognized as a hereditable inborn error of metabolism. In the morphologic sense alone the involvement of the adrenal cortex and the genital changes that appear, sooner or later, in both sexes comprise the sense of the term "adrenogenital syndrome." The latter has often been interpreted generically, so that either tumor or hyperplasia of the adrenal cortex may constitute the basis of the disease. This review concerns only the form associated with adrenocortical hyperplasia and known to be related to an inherent defect of the normal . . .