Precocious puberty associated with neurofibromatosis and optic gliomas. Treatment with luteinizing hormone releasing hormone analogue
L. Laue, F. Comite, K. Hench, D. L. Loriaux, G. B. Cutler Jr and O. H. Pescovitz
Seven children with central precocious puberty and either neurofibromatosis
and/or optic gliomas were referred to the National Institutes of Health,
Bethesda, Md, for evaluation and treatment with the long-acting luteinizing
hormone releasing hormone analogue (LHRHa) D-Trp6-Pro9-NEt-LHRH. Only six
of the seven children chose to receive treatment. Four children presented
with neurofibromatosis, three of whom also had optic gliomas; the remaining
three children had isolated optic gliomas, without other neurocutaneous
stigmas. All had central precocious puberty mediated by activation of the
hypothalamic-pituitary-gonadal axis. Six months of LHRHa therapy caused
suppression of gonadotropin and sex steroid levels, stabilization or
regression of secondary sexual characteristics, and decreases in growth
velocity and the rate of bone age maturation. We conclude that LHRHa
therapy is effective in the treatment of central precocious puberty
secondary to neurofibromatosis and/or optic gliomas.
