ABNORMAL HYPOTHALAMIC-PITUITARY FUNCTION IN POLYOSTOTIC FIBROUS DYSPLASIA

Publisher Website
Google Scholar
Abstract
Hypothalamic-pituitary dysfunction was found in 2 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 [GH] 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 [PRL] concentrations, 385 and 45 ng/ml, and the 24-h secretory patterns of PRL were abnormal. One patient had an elevated mean plasma cortisol concentration of 8.5 .mu.g/dl but the 24-h pattern of cortisol secretion was normal. Basal plasma luteinizing hormone, follicle stimulating hormone, TSH [thyrotropin], thyroxine and triiodothyronine concentrations were normal but neither patient had a TSH response to TRH [thyrotropin releasing hormone]. PRL and GH secretions were, however, increased after TRH. Oral glucose resulted in partial suppression of GH but an exaggerated insulin response in both patients. Postmortem in 1 patient revealed a thick calvarium with bony encasement of the pituitary gland. The pituitary and hypothalamus appeared normal on gross, light microscopic and EM examination. The presence of hypothalamic-pituitary dysfunction in these 2 patients is suggested.