RATIONALE: Turner's syndrome is a disease in which females are missing all or part of one X
chromosome and do not produce estrogen. Giving estrogen is standard treatment for girls who
have Turner's syndrome. Estrogen may be effective treatment for mental and social
functioning problems experienced by girls with Turner's syndrome.
PURPOSE: Clinical trial to study the effectiveness of long term estrogen therapy on mental
and social functioning in girls who have Turner's syndrome.
PROTOCOL OUTLINE: Participants are evaluated for cognitive and social function at entry and
4 years following entry. Assessments include the Wechsler Intelligence Scale for
Children-Revised, a child behavior checklist, the Children's Self-Concept Scale, and
visual-spatial, visual-motor, attention, memory, language, and facial recognition tasks.
Patients (and parents) undergo X-chromosome analysis; brain magnetic imaging is optional.
Controls are matched using school selection and telephone interviews.
A study duration of 12 years is estimated.
PROTOCOL ENTRY CRITERIA:
- Girls aged 8 and 12 years with Turner's syndrome-compatible karyotype
- Age-matched girls without Turner's syndrome entered as controls
- Concurrent registration on Thomas Jefferson University growth study required of
subjects with Turner's syndrome