title: [Infantile cushing's disease with paradoxical response to dexamethasona due to a probable periodic hormogenesis (author's transl)].
abstract: 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.
mesh_terms: D015065:17-Hydroxycorticosteroids / Q000378:metabolism / Q000652:urine; D002648:Child; D003480:Cushing Syndrome / Q000175:diagnosis* / Q000652:urine; D003907:Dexamethasone; D005260:Female; D006801:Humans; D010912:Pituitary-Adrenal Function Tests; D010913:Pituitary-Adrenal System / Q000503:physiopathology
chemical_list: D015065:17-Hydroxycorticosteroids; D003907:Dexamethasone
vernacular_title: Enfermedad de Cushing infantil con respuesta paradójica a la dexametasona por probable hormogénesis periódica.