Pituitary-dependent Cushing disease and primary adrenocortical nodular dysplasia in childhood. Presentation of 4 cases.
Four patients with Cushing syndrome were followed for 1.5-12 years. The main clinical symptoms were marked growth retardation and truncal obesity. Two patients had pituitary-dependent Cushing disease, while the other two had primary adrenocortical nodular dysplasia. The treatment of choice of pituitary-dependent Cushing disease is transsphenoidal resection of the microadenoma while in primary adrenocortical nodular dysplasia bilateral adrenalectomy with subsequent steroid replacement is recommended. In our pre- or early pubertal patients catch-up growth occurred following surgical therapy and in all cases pubertal development proceeded normally.
D000236:Adenoma / Q000150:complications / Q000601:surgery; D000293:Adolescent; D000311:Adrenal Glands / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003480:Cushing Syndrome / Q000209:etiology*; D005260:Female; D006801:Humans; D006965:Hyperplasia; D008297:Male; D010911:Pituitary Neoplasms / Q000150:complications / Q000601:surgery