Abnormal hypothalamic-pituitary function in polyostotic fibrous dysplasia.

Hypothalamic-pituitary dysfunction was found in two patients with polyostotic fibrous dysplasia and leontiasis ossea. Both patients probably had McCune--Albright syndrome with early sexual development, disfiguring craniofacial bone lesions, cutaneous hyperpigmentation and gigantism in childhood. Endocrinological studies revealed measurable plasma growth hormone throughout a 24-h sampling period with preservation of sleep augmented rises. The mean 24-h values were 329 and 7 ng/ml, respectively. Both their mean plasma prolactin concentrations, 385 and 45 ng/ml, and the 24-h secretory patterns of prolactin were abnormal. One patient had an elevated mean plasma cortisol concentration of 8.5 micrograms/dl but the 24-h pattern of cortisol secretion was normal. Basal plasma LH, FSH, TSH, T4 and T3 concentrations were normal but neither patient had a TSH response to TRH. Prolactin and growth hormone secretions were, however, increased after TRH. Oral glucose resulted in partial suppression of GH but an exaggerated insulin response in both patients. Post-mortem in one patient revealed a thick calvarium with bony encasement of the pituitary gland. The pituitary and hypothalamus appeared normal on gross, light microscopic and electronmicroscopic examination. These data strongly suggest the presence of hypothalamic-pituitary dysfunction in these two patients.