Journal of Clinical Endocrinology & Metabolism, Vol 58, 1161-1165, Copyright © 1984 by Endocrine Society

ARTICLES

Absence of pubertal gonadotropin secretion in girls with McCune- Albright syndrome

CM Foster, JL Ross, T Shawker, OH Pescovitz, DL Loriaux, GB Cutler Jr and F Comite

Precocious puberty in girls with McCune-Albright syndrome has been attributed in some cases to early activation of the hypothalamic- pituitary-gonadal axis and in other cases to sex steroid secretion by apparently autonomous ovarian cysts. We evaluated serum gonadotropins and sex steroids in six girls (aged 1-9 yr) with McCune-Albright syndrome. The children had Tanner stage II-IV pubertal development. In five patients, nocturnal gonadotropin concentrations and the gonadotropin response to LHRH were within the normal range for prepubertal children. Thus, the precocious puberty in these patients could not be explained by activation of the hypothalamic-pituitary- ovarian axis. One child had high amplitude nocturnal pulses of serum LH and a LH-predominant response to LHRH. She was the oldest of the six girls and had a bone age of 13.5 yr which is within the range in which hypothalamic-pituitary-ovarian activation normally occurs. The children all had ovarian enlargement and ovarian cysts determined by ultrasound. It appears that precocious puberty in McCune-Albright syndrome may result from ovarian estrogen secretion in the absence of normal pubertal activation of the hypothalamic-pituitary-ovarian axis.

This article has been cited by other articles:

				N. Mendoza, F. J. Moron, F. Quereda, F. Vazquez, M. C. Rivero, T. Martinez-Astorquiza, L. M. Real, R. Sanchez-Borrego, A. Gonzalez-Perez, and A. Ruiz A Digenic Combination of Polymorphisms Within ESR1 and ESR2 Genes Are Associated With Age at Menarche in the Spanish Population Reproductive Sciences, March 1, 2008; 15(3): 305 - 311. [Abstract] [PDF]

				D. D. Elsholz, V. Padmanabhan, R. L. Rosenfield, P. R. Olton, D. J. Phillips, and C. M. Foster GnRH agonist stimulation of the pituitary-gonadal axis in children: age and sex differences in circulating inhibin-B and activin-A Hum. Reprod., December 1, 2004; 19(12): 2748 - 2758. [Abstract] [Full Text] [PDF]

				S. B. Nunez, K. Calis, G. B. Cutler Jr., J. Jones, and P. P. Feuillan Lack of Efficacy of Fadrozole in Treating Precocious Puberty in Girls with the McCune-Albright Syndrome J. Clin. Endocrinol. Metab., December 1, 2003; 88(12): 5730 - 5733. [Abstract] [Full Text] [PDF]

				L. S. WEINSTEIN, M. CHEN, and J. LIU Gs{alpha} Mutations and Imprinting Defects in Human Disease Ann. N.Y. Acad. Sci., June 1, 2002; 968(1): 173 - 197. [Abstract] [Full Text] [PDF]

				L. S. Weinstein, S. Yu, D. R. Warner, and J. Liu Endocrine Manifestations of Stimulatory G Protein {alpha}-Subunit Mutations and the Role of Genomic Imprinting Endocr. Rev., October 1, 2001; 22(5): 675 - 705. [Abstract] [Full Text] [PDF]