This Article Full Text (PDF) Submit a related Letter to the Editor Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Copyright Permission Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by FEUILLAN, P. P. Articles by CUTLER, G. B. Search for Related Content PubMed PubMed Citation Articles by FEUILLAN, P. P. Articles by CUTLER, G. B., Jr.

Luteinizing Hormone-Releasing Hormone (LHRH)-Independent Precocious Puberty Unresponsive to LHRH Agonist Therapy in Two Girls Lacking Features of the McCune-Albright Syndrome

Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health Bethesda, Maryland 20892

Address requests for reprints to: Penelope P. Feuillan, M.D., Building 10, Room 10N262, National Institutes of Health, Bethesda, Maryland 20892.

Two girls with precocious puberty (chronological age, 1 and 4 yr; bone age, 3 and 6 yr, respectively) were initially given the diagnosis of idiopathic, central precocious puberty and treated with the LHRH agonist deslorelin (D–Trp⁶– Pro⁹–NEt–LHRH) for 5 yr. Unlike other girls with central precocious puberty, both had persistently elevated rates of growth and bone maturation, and both menstruated during therapy. One girl had episodic ovarian enlargement and markedly elevated serum estradiol levels due to recurrent unilateral ovarian cysts. Although the bone and skin manifestations of McCune- Albright syndrome were absent, we hypothesize that the underlying defect of McCune-Albright syndrome was expressed in the ovaries, but not in the skin or bones, of these two girls. One of these girls appeared to benefit from the aromatase inhibitor testolactone, which is effective in suppressing precocious puberty in girls with the McCune-Albright syndrome.

Received January 14, 1991.

This article has been cited by other articles:

				S. J. Marx and W. F. Simonds Hereditary Hormone Excess: Genes, Molecular Pathways, and Syndromes Endocr. Rev., August 1, 2005; 26(5): 615 - 661. [Abstract] [Full Text] [PDF]

				E. Y. Adashi and J. D. Hennebold Single-Gene Mutations Resulting in Reproductive Dysfunction in Women N. Engl. J. Med., March 4, 1999; 340(9): 709 - 718. [Full Text] [PDF]

				E. A. Cummings, S. R. Salisbury, M. L. Givner, and R. S. Rittmaster Testolactone-Associated High Androgen Levels, a Pharmacologic Effect or a Laboratory Artifact? J. Clin. Endocrinol. Metab., March 1, 1998; 83(3): 784 - 787. [Abstract] [Full Text]

				J. A. Majzoub and R. E. Scully Case 7-1993- A Six-Year-Old Boy with Multiple Bone Lesions, Repeated Fractures, and Sexual Precocity N. Engl. J. Med., February 18, 1993; 328(7): 496 - 502. [Full Text]