PMID: 7019531Jul 1, 1981Paper

The Albright syndrome associated with acromegaly: report of a case and review of the literature

The Johns Hopkins Medical Journal
A Lipson, T H Hsu

Abstract

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 en...Continue Reading

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