Abstract
A rare cause of Cushing's syndrome is a pituitary-independant nodular dysplasia of the adrenal cortex. A 4 year old girl presented with advanced clinical features of Cushing's syndrome. Abdominal CT-scan and adrenal scintigraphy revealed bilateral adrenal hyperplasia whereas a cerebral CT-scan was normal. Serial determinations of plasma ACTH together with dynamic tests using dexamethasone, metyrapone and vasopressin indicated a primary, autonomous, adrenal dysfunction. Treatment with Trilostane - an inhibitor of 3β-hydroxysteroid-dehydrogenase - in a dose of 50 mg t.i.d. was effective, and cortisol excretion was instantly reduced to a low normal level with only a slight increase in ACTH. No side-effects of this treatment were seen and the clinical features of the syndrome regressed. The therapeutic index of Trilostane was small, however. Serum levels of adrenal androgens remain increased therefore a long-term treatment in this young girl was considered to be unfavourable. At laparotomy both adrenals were almost normal in size and a subtotal adrenalectomy was performed. Microscopically, the picture was typical for PAMD with normal structure of the cortex between the micronodules.
Trilostane seem to be effective in improving clinical and some of the biochemical parameters even in a severe case of PAMD but its use may be limited to a short-term preoperative treatment.
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Krabbe, S., Damkjaer, M. TRILOSTANE IN THE TREATMENT OF PRIMARY ADRENOCORTICAL MICRONODULAR DYSPLASIA (PAMD). Pediatr Res 20, 1203 (1986). https://doi.org/10.1203/00006450-198611000-00177
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DOI: https://doi.org/10.1203/00006450-198611000-00177
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