Abstract
A 6 1/2 yr old hoy shows a combination of polyostotic fibrous dysplasia, sclerosis of the base of the skull, “café-au-lait” spots and enlarged testes (12/15 mis). Histological examination, at biopsy, displays a pattern of mature testicular tissue with spermatogenesis. No presence of secondary sex characteristics. No advanced bone age. Height: 90th percentile. Plasma testosterone at upper limits of prepubertal range. After Gn-RH stimulation FSH fails to rise, whereas LH response may still be considered within normal. GH and cortisol after insuline tolerance test normal. No rise of TSH elicited after TRH, while prolactin increases normally. No uptake of 131-I in a large area of the right lobe of thyroid (scanning) neither before nor after TSH stimulation. Normal plasma T4 and T3 (RIA). The gathered data substantiate the presence of pluriglandular involvement, the pathogenesis of which still appears uncertain.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Giovannelli, G., Bernasconi, S. & Banchini, G. McCUNE–ALBRICHT's SYN. IN A MALE CHILD: A CLINICAL-ENDOCRINOLOGICAL PUZZLE. Pediatr Res 9, 669 (1975). https://doi.org/10.1203/00006450-197508000-00028
Issue Date:
DOI: https://doi.org/10.1203/00006450-197508000-00028