title: [Primary adrenocortical micronodular dysplasia].
abstract: Two girls (11 and 13 years old) with Cushing's syndrome due to primary adrenocortical micronodular dysplasia (PAMD) are presented. High plasma cortisol concentrations, elevated urinary free cortisol and 17-ketogenic steroids excretion, in addition to low or normal plasma adrenocorticotropic hormone (ACTH) levels pointed towards independent adrenal cortisol hypersecretion. In both girls bilateral adrenalectomy was performed, followed by replacement therapy with glucocorticoids and mineralocorticoids. Pathohistological findings of otherwise enlarged adrenal glands, showed characteristic small nodules measuring 1-2 mm, composed of cells resembling those of zona fasciculata, with abundant, clear cytoplasm. Our younger patient fulfilled the criteria of "Carney complex", because beside PAMD she has had the lentigines.
authors.affiliation: Klinika za pedijatriju, KBC Rebro, Zagreb.
authors.forename: M
authors.initials: M
authors.lastname: Dumić
authors.forename: J
authors.initials: J
authors.lastname: Ille
authors.forename: S
authors.initials: S
authors.lastname: Batinica
authors.forename: M
authors.initials: M
authors.lastname: Cacić
authors.forename: M
authors.initials: M
authors.lastname: Cvitanović
authors.forename: B
authors.initials: B
authors.lastname: Marinović
authors.forename: V
authors.initials: V
authors.lastname: Plavsić
authors.forename: M
authors.initials: M
authors.lastname: Lukenda
authors.forename: A
authors.initials: A
authors.lastname: Radica
mesh_terms: D000293:Adolescent; D000302:Adrenal Cortex / Q000473:pathology; D000303:Adrenal Cortex Diseases / Q000150:complications* / Q000473:pathology; D002648:Child; D003480:Cushing Syndrome / Q000209:etiology*; D005260:Female; D006801:Humans
vernacular_title: Primarna adrenokortikalna mikronodularna displazija.