Gonadal DysgenesisPhase 2NCT00001253
The Effects of Estrogen on Cognition in Girls With Turner Syndrome
A Phase 2 study testing cognitive tests and scales in people with Gonadal Dysgenesis and Turner's Syndrome, run by Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) at 1 site in United States.
Trial summary
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. Read more about this study
- Who is it for?
- Any ageHealthy volunteers can take part
- Conditions the study is for
- Gonadal Dysgenesis, Turner's Syndrome
- What is being tested?
- cognitive tests and scales
- Type of treatment
- Lifestyle or therapy programme
- How many people
- 950 participants
- Where
- 1 siteUnited States
- Study dates
- May 1990to Mar 2004
- Phase
- Phase 2
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages Any age
- All sexes
- Healthy volunteers accepted
- Child, Adult, Older adult
Who may be able to join
- 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.
Who cannot join
- 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.
Trial locations and contacts
1 site in 1 country.
United States1 site
National Institute of Child Health and Human Development (NICHD)
Bethesda, Maryland, 20892
About this study
Official title: Estrogen Effects on Cognition in Girls With Turner Syndrome
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.
Read more
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.
Read the 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.
- Conditions
- Gonadal Dysgenesis, Turner's Syndrome
- Being tested
- cognitive tests and scales
- Keywords
- Turner's Syndrome, Estrogen, Ethinyl estradiol, Learning, Ovarian dysgenesis
- Medical subject headings
- Gonadal Dysgenesis, Turner Syndrome
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
cognitive tests and scalesBehavioural
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted4 Nov 1999 (estimated)
- Study startMay 1990
- Study completionMar 2004
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (3)Papers the sponsor has linked to this study.
- Other trials for Gonadal DysgenesisStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase 2
- Primary purpose
- Treatment
- Enrolment
- 950 participants
Sponsor and investigators
- Lead sponsor
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
- Sponsor type
- US National Institutes of Health
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT00001253
- Sponsor's protocol ID
- 900123
- Other IDs
- 90-CH-0123
- Status last verified
- Mar 2004
- First submitted
- 3 Nov 1999
- Last updated
- 4 Mar 2008
- Results posted
- No
Publications
Palmer CG, Reichmann A. Chromosomal and clinical findings in 110 females with Turner syndrome. Hum Genet. 1976 Dec 29;35(1):35-49. doi: 10.1007/BF00295617.
PubMed 1002163Singh RP, Carr DH. The anatomy and histology of XO human embryos and fetuses. Anat Rec. 1966 Jul;155(3):369-83. doi: 10.1002/ar.1091550309. No abstract available.
PubMed 5956901Park E, Bailey JD, Cowell CA. Growth and maturation of patients with Turner's syndrome. Pediatr Res. 1983 Jan;17(1):1-7. doi: 10.1203/00006450-198301000-00001. No abstract available.
PubMed 6835711
Study at a glance
From the registry record, last updated by the sponsor on 4 Mar 2008. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Estrogen Effects on Cognition in Girls With Turner Syndrome
- ClinicalTrials.gov ID
- NCT00001253
- Acronym / sponsor ID
- 900123
- Conditions
- Gonadal Dysgenesis, Turner's Syndrome
- Keywords
- Turner's Syndrome, Estrogen, Ethinyl estradiol, Learning, Ovarian dysgenesis
- Phase
- Phase 2
- Study type
- Interventional
- Recruitment status
- Completed
- Enrolment
- 950 participants
- Lead sponsor
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)US National Institutes of Health
- Study start
- May 1990
- Study completion
- Mar 2004
- First posted
- 4 Nov 1999 (estimated)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
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.
Study design
- Primary purpose
- Treatment
Who may qualify
- Sex
- All
- Healthy volunteers
- Accepted
- Age groups
- Child, Adult, Older adult
Key inclusion criteria (3 listed)
- 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.
Key exclusion criteria (2 listed)
- 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.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| cognitive tests and scales | Behavioural | – |
Primary outcome
Not reported in the source record.
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 4 Mar 2008.
- Background: PubMed 1002163Palmer CG, Reichmann A. Chromosomal and clinical findings in 110 females with Turner syndrome. Hum Genet. 1976 Dec 29;35(1):35-49. doi: 10.1007/BF00295617.
- Background: PubMed 5956901Singh RP, Carr DH. The anatomy and histology of XO human embryos and fetuses. Anat Rec. 1966 Jul;155(3):369-83. doi: 10.1002/ar.1091550309. No abstract available.
- Background: PubMed 6835711Park E, Bailey JD, Cowell CA. Growth and maturation of patients with Turner's syndrome. Pediatr Res. 1983 Jan;17(1):1-7. doi: 10.1203/00006450-198301000-00001. No abstract available.
Related trials for Gonadal Dysgenesis
RecruitingPhase 2/3
A Study in Pediatric Participants With Congenital Adrenal Hyperplasia (Balance-CAH)
Congenital Adrenal Hyperplasia, Classic Congenital Adrenal Hyperplasia
RecruitingObservational study
Natural History Study of Patients With Excess Androgen
Congenital Adrenal Hyperplasia (CAH), Familial Male-Limited Precocious Puberty (FMPP)
RecruitingPhase 3
A Study to Evaluate Atumelnant in Adults With Congenital Adrenal Hyperplasia
Congenital Adrenal Hyperplasia, Classic Congenital Adrenal Hyperplasia
RecruitingPhase 3
Trial Investigating the Efficacy and Safety of Weekly Lonapegsomatropin Compared to Daily Somatropin in Children and Adolescents With Short Stature or Growth Failure Due to Growth Hormone Sufficient Disorders
Turner Syndrome, Short Stature Homeobox Gene Mutation
RecruitingObservational study
Adrenal Diseases in the Population of Modena: A Clinical-Epidemiological Database of Patients Referred to the Adrenal Disorders Outpatient Clinic of the Clinical Unit of Endocrinology at the Modena University Hospital (AOU Modena)
Cushing s Syndrome, Cushing Disease
Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
The record lists no contact. Use the official record to reach the sponsor.
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
A Phase 2 study testing cognitive tests and scales in people with Gonadal Dysgenesis and Turner's Syndrome, run by Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 3 inclusion and 2 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 4 Mar 2008. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
What does this phase mean?
Phase 2 studies give the treatment to a larger group to see whether it has the intended effect and to keep checking safety.
Not every study follows the same path, and some studies (observational studies, device studies) have no phase.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT00001253, which also lists 3 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 4 Mar 2008.