Cushing syndrome, sexual precocity, and polyostotic fibrous dysplasia (Albright syndrome) in infancy.
The sexual precocity of polyostotic fibrous dysplasia is occasionally accompanied by other endocrine disorders, but in only two previous instances has Cushing syndrome been reported. The history of a 6-month-old girl is presented, in whom this syndrome was complicated by congenital Cushings syndrome. Although endocrinopathies of polyostotic fibrous dysplasia have usually been ascribed to a central (hypothalamic) origin, the findings in this patient suggest autonomous hyperfunction of the peripheral endocrine glands, with the Cushing syndrome caused by hyperplastic nodules in the adrenal glands and the precocity by luteinized follicular cysts of the ovary.
D000311:Adrenal Glands / Q000473:pathology; D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000096:biosynthesis; D003480:Cushing Syndrome / Q000150:complications*; D004958:Estradiol / Q000097:blood; D004970:Estrone / Q000097:blood; D005260:Female; D005357:Fibrous Dysplasia of Bone / Q000150:complications*; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications*; D006130:Growth Disorders / Q000209:etiology; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007223:Infant; D010053:Ovary / Q000473:pathology; D012741:Sexual Maturation; D013739:Testosterone / Q000097:blood
D004970:Estrone; D013739:Testosterone; D004958:Estradiol; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone