Effect of Estrogen on Mental and Social Functioning in Girls With Turner's Syndrome

Controlled Study of Estrogen Effects on Cognitive and Social Function in Girls With Turner's Syndrome

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