A case of Cushing's disease in a ten year old girl with bilateral diffuse hyperplasia of the adrenal cortex and postoperative enlargment of the sella turcica is presented. The administration of dexamethasone elicited a paradoxical response with a clear elevation of the already high excretion of 17-hydroxycorticoids. The possible mechanisms for this previously described, but infrequent, response to dexamethasone are discussed. It is concluded that: 1) Interpretation of the classical dexamethasone suppression test can occasionally be misleading; and 2) Periodic hormonogenesis may account for this type of paradoxical response.
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