The Albright syndrome associated with acromegaly: report of a case and review of the literature.
The association of the Albright syndrome (polyostotic fibrous dysplasia of bone, hyperpigmented skin macules, and endocrine disorders) with acromegaly has been infrequently substantiated. The case of an 18-year-old girl with the classic Albright syndrome and acromegaly is described. The patient had a history of coarsening of acral and facial features, an insulin-resistant form of diabetes mellitus and elevated fasting growth hormone values. Neuro-endocrine studies demonstrated failure of growth hormone to suppress to less than 5 ng/ml during an oral glucose tolerance test, and the abnormal release of growth hormone upon injection of thyrotropin-releasing hormone. Although L-dopa failed to decrease growth hormone levels, bromocriptine produced a modest decline in growth hormone within two hours of ingestion. The patient had also experienced secondary amenorrhea with sub-normal follicle-stimulating-hormone (FSH) and luteinizing hormone (LH) levels, both of which demonstrated a prolonged sluggish response to an injection of gonadotropin-releasing hormone (GnRH); this response suggested hypogonadotropic hypogonadism, possibly on the basis of a tumor involving both pituitary and hypothalamus. Sellar polytomography demonstrated an enlarged sella with dorsal erosion and an asymmetric floor. Computerized tomography of the brain visualized a suprasellar mass extending into the hypothalamus. These findings suggest a hypersecretion of hypothalamic releasing factors, pituitary hormones, or both as an etiology for the endocrinopathy in this patient, and lend support to the theory that the endocrinopathies associated with the Albright syndrome result from over-production of hypothalamic-releasing hormones or autonomous secretion of pituitary hormones from an adenoma.
D000172:Acromegaly / Q000150:complications*; D000293:Adolescent; D005260:Female; D005357:Fibrous Dysplasia of Bone / Q000150:complications*; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications* / Q000503:physiopathology; D013006:Growth Hormone / Q000378:metabolism; D006801:Humans; D010906:Pituitary Hormone-Releasing Hormones / Q000378:metabolism; D010908:Pituitary Hormones, Anterior / Q000378:metabolism
D010906:Pituitary Hormone-Releasing Hormones; D010908:Pituitary Hormones, Anterior; D013006:Growth Hormone