The Effects of Estrogen on Cognition in Girls With Turner Syndrome

This study has been completed.
Sponsor:
Information provided by:
National Institutes of Health Clinical Center (CC)
ClinicalTrials.gov Identifier:
NCT00001253
First received: November 3, 1999
Last updated: March 3, 2008
Last verified: March 2004
  Purpose

The development of the brain in females is a result of a combination of factors. During puberty estrogen plays a role in influencing brain development. Cultural and environmental factors also play a role in the development of the brain.

Female patients with Turner syndrome lack the ability to produce estrogen due to undeveloped ovaries. Therefore, Turner syndrome is the perfect condition to study how estrogen (or the lack of estrogen) influences a person's behavior and thinking.

This study will compare cognitive differences (visual motor skills, visual-spatial, psychosocial behavior, and visual memory) of patients with Turner syndrome to normal patient controls. Researchers will use the Weschler Intelligence Scale for Children-Revised (WISC-R) along with other tests and scales to measure different aspects of the patient's cognitive ability. In addition the study will review patients with Turner syndrome who previously received estrogen replacement as infants and children in a related research study.

Researchers hope to demonstrate that estrogen replacement will improve cognition and behavior in girls with Turner syndrome.


Condition Intervention Phase
Gonadal Dysgenesis
Turner's Syndrome
Behavioral: cognitive tests and scales
Phase 2

Study Type: Interventional
Study Design: Endpoint Classification: Safety/Efficacy Study
Primary Purpose: Treatment
Official Title: Estrogen Effects on Cognition in Girls With Turner Syndrome

Resource links provided by NLM:


Further study details as provided by National Institutes of Health Clinical Center (CC):

Estimated Enrollment: 950
Study Start Date: May 1990
Estimated Study Completion Date: March 2004
Detailed Description:

Estrogen influences brain development in females at puberty. Environmental and cultural factors interact with the biological effects of estrogen on the brain and consequently on cognition and behavior. Turner syndrome females lack endogenous estrogen as a result of dysgenetic ovaries. Turner syndrome therefore represents a unique, estrogen-deficient model in which to study the biological effects of estrogen on cognition and behavior. The specific aims of this project are to: 1) document further, the cognitive differences between girls with Turner syndrome at ages 5 to adult (less than or equal to age 50) versus age-matched, female controls. 2) to examine the differential effects of continuous estrogen replacement in infancy and in early childhood on cognitive and social function in a unique, previously approved, randomized, double-blind, placebo-controlled, treatment trial (87-CH-0152). Specifically, we hypothesize that estrogen replacement in early childhood will reduce the cognitive deficits of girls with Turner syndrome. In addition, we hypothesize that the degree of socialization ability in these girls will correlate with social-behavioral and social recognition ability. Finally, we hypothesize that earlier (infancy to 8 years) and longer estrogen replacement will result in less impairment of visual-motor ability, visual-spatial ability, socialization ability, and affective competence compared to later (9 to 12 years) estrogen replacement in girls with Turner syndrome.

Children with Turner syndrome and controls will be tested in the Outpatient Departments at the two approved sites of protocol 87-CH-0152; the NIH and Thomas Jefferson University.

  Eligibility

Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   Yes
Criteria

INCLUSION CRITERIA:

Patients will include girls and women ages 5-50 yr with the diagnosis of Turner syndrome based on absence of all or part of one of the X chromosomes.

Control subjects must be within +/- 2SD for height and weight and have normal intelligence and educational achievement.

Biological parents (both male and female) of TS subjects may be included in this study, but only to have blood drawn for genetic testing in order to determine the origin of the X-chromosome of their daughters.

EXCLUSION CRITERIA:

Those with severe physical or neurocognitive impairment, preventing accurate completion of the cognitive tasks, will be excluded.

Normal subjects who have qualified for or participated in gifted and talented or remedial education programs.

  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT00001253

Locations
United States, Maryland
National Institute of Child Health and Human Development (NICHD)
Bethesda, Maryland, United States, 20892
Sponsors and Collaborators
  More Information

Publications:
ClinicalTrials.gov Identifier: NCT00001253     History of Changes
Other Study ID Numbers: 900123, 90-CH-0123
Study First Received: November 3, 1999
Last Updated: March 3, 2008
Health Authority: United States: Federal Government

Keywords provided by National Institutes of Health Clinical Center (CC):
Turner's Syndrome
Estrogen
Ethinyl estradiol
Learning
Ovarian dysgenesis

Additional relevant MeSH terms:
Syndrome
Turner Syndrome
Gonadal Dysgenesis
Primary Ovarian Insufficiency
Congenital Abnormalities
Disease
Pathologic Processes
Disorders of Sex Development
Urogenital Abnormalities
Sex Chromosome Disorders of Sex Development
Heart Defects, Congenital
Cardiovascular Abnormalities
Cardiovascular Diseases
Heart Diseases
Sex Chromosome Disorders
Chromosome Disorders
Genetic Diseases, Inborn
Gonadal Disorders
Endocrine System Diseases
Ovarian Diseases
Adnexal Diseases
Genital Diseases, Female
Estrogens
Hormones
Hormones, Hormone Substitutes, and Hormone Antagonists
Physiological Effects of Drugs
Pharmacologic Actions

ClinicalTrials.gov processed this record on October 16, 2014