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Journal of Clinical Endocrinology & Metabolism Vol. 73, No. 6 1370-1373
doi:10.1210/jcem-73-6-1370
Copyright © 1991 by the Endocrine Society.
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Luteinizing Hormone-Releasing Hormone (LHRH)-Independent Precocious Puberty Unresponsive to LHRH Agonist Therapy in Two Girls Lacking Features of the McCune-Albright Syndrome

PENELOPE P. FEUILLAN, JANET JONES, KAREN E. OERTER, PENELOPE K. MANASCO and GORDON B. CUTLER, Jr.

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.




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Copyright © 1991 by The Endocrine Society