title: [Cushing's disease associated with empty sella: a clinical case treated for years with ketoconazole].
abstract: Cushing's disease and empty sella without evidence of pituitary adenoma are rarely observed. To our knowledge, there is very little documentation on long-term therapeutic follow-up with the steroidogenesis inhibitor ketoconazole. A 48-year-old woman with uncontrolled insulin-dependent diabetes mellitus, severe hypertension, and clinical findings of hypercortisolism was referred to our hospital. Endocrine evaluation of adrenocortical function evidenced hypothalamic-pituitary-hypercortisolism, and excluded adrenal tumor or an ectopic corticotropin source. Magnetic resonance imaging disclosed an empty sella turcica but not pituitary adenoma. The patient was treated with a steroidogenesis inhibitor, ketoconazole (600 mg daily) which reduced urinary cortisol excretion to within the normal range. Serum cortisol levels also returned to normal in the morning but not in the evening. The patient has continued on ketoconazole therapy for the past 7 years, with neither side effects nor tachyphylaxis. The reduction of cortisol secretion brought about significantly improved control of diabetes mellitus and hypertension, although signs of hypercortisolism have persisted. Radiographic studies of the hypophysis during follow-up have not evidenced adenoma.
authors.affiliation: I Divisione di Medicina Interna, Ospedale Civile S. Chiara di I rento.
authors.forename: W
authors.initials: W
authors.lastname: Spagnolli
authors.forename: C
authors.initials: C
authors.lastname: Ramponi
authors.forename: M V
authors.initials: MV
authors.lastname: Davì
authors.forename: G
authors.initials: G
authors.lastname: Francia
mesh_terms: D002311:Cardiomyopathy, Dilated / Q000150:complications; D003480:Cushing Syndrome / Q000150:complications* / Q000188:drug therapy; D048909:Diabetes Complications; D004334:Drug Administration Schedule; D004652:Empty Sella Syndrome / Q000150:complications*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007654:Ketoconazole / Q000627:therapeutic use*; D008875:Middle Aged
chemical_list: D007654:Ketoconazole
vernacular_title: Malattia di Cushing associata a sella vuota: un caso clinico trattato per sette anni con ketoconazolo.