Pubertal development was evaluated in nine males and 16 females with Willia
ms syndrome (WS). Our results indicate that puberty in WS occurred earlier
than in published population controls; specifically, 90% of menstruating fe
males reached menarche and 83% of pubertal males showed Tanner III pubic ha
ir development prior to the age of 12 years. The sequence of pubertal devel
opment was normal, bone age was always consistent with, or in excess of, ch
ronological age, and there was evidence of central (hypothalamic-pituitary
mediated) activation as the cause of early puberty in a subset of subjects.
Clin Dysmorphol 8: 117-121 (C) 1999 Lippincott Williams & Wilkins.