Background: To find out the incidence of spontaneous pubertal developm
ent in girls with Turner syndrome we investigated patients with a bone
age of at least eleven years. Results: 6 out of 17 patients showed sp
ontaneous puberty. There exists no clear correlation between karyotype
and phenotype on the one hand and incidence of spontaneous puberty on
the other. Basal as well as stimulated gonadotropin levels in the lat
e childhood could better indicate spontaneous puberty in Turner syndro
me. Conclusion: In contrast to the generally held opinion spontaneous
pubertal development is not rare in girls with Turner syndrome. Theref
ore estrogen replacement therapy should be started only after it becam
e certain that there will be no spontaneous puberty.