Defining the Optimal Hormonal Replacement Therapy in Turner Syndrome - Full Text View

Sponsors and Collaborators:	Hillerod Hospital, Denmark Novo Nordisk The County of Frederiksborg The foundation of Kaptajnløjtnant Harald Jensen and Wife The foundation of Queen Louise childrens hospital The foundation of Mrs. Olga Bryde The foundation of Mr. Ivan Nielsen
Information provided by:	Hillerod Hospital, Denmark
ClinicalTrials.gov Identifier:	NCT00134745

Purpose

The purpose of this study is to examine whether a larger dosage of estrogen than the one used today will secure the development of a normal sized uterus and increase the strength of the bones in girls and young women with Turner syndrome.

The purpose is also to evaluate whether aortic dilatation is present in this group of patients, and if the estrogen dosage will influence the emotional well-being and self-esteem of the patients.

Condition	Intervention	Phase
Turner Syndrome	Drug: estradiol	Phase IV

Genetics Home Reference related topics: Turner syndrome

MedlinePlus related topics: Nuclear Scans Turner Syndrome

Drug Information available for: Depogen Estradiol Estradiol 3-benzoate Estradiol acetate Estradiol cypionate Estradiol dipropionate Estradiol valerate Polyestradiol phosphate

U.S. FDA Resources

Study Type:	Interventional
Study Design:	Treatment, Randomized, Double Blind (Subject, Caregiver, Investigator, Outcomes Assessor), Placebo Control, Single Group Assignment, Pharmacodynamics Study
Official Title:	The Growth of Genitalia Interna and the Bone Mineralization Under Hormonal Replacement Therapy and the Presence of Aortic Root Dilatation in Girls With Turner Syndrome

Further study details as provided by Hillerod Hospital, Denmark:

Primary Outcome Measures:

The size of the uterus evaluated by magnetic resonance (MR)-scan [ Time Frame: 5 years ] [ Designated as safety issue: Yes ]
Bones evaluated by dual-energy X-ray absorptiometry (DEXA)-scan [ Time Frame: 5 years ] [ Designated as safety issue: Yes ]

Secondary Outcome Measures:

The diameter of the aortic root evaluated by MR-scan [ Time Frame: 5 years ] [ Designated as safety issue: Yes ]
Development of biochemical markers [ Time Frame: 5 years ] [ Designated as safety issue: Yes ]
Body composition evaluated by DEXA-scan [ Time Frame: 5 years ] [ Designated as safety issue: Yes ]
Emotional wellbeing and self-esteem evaluated by questionnaires [ Time Frame: 5 years ] [ Designated as safety issue: Yes ]
The size of the uterus evaluated by ultrasound [ Time Frame: 5 years ] [ Designated as safety issue: Yes ]

Estimated Enrollment:	60
Study Start Date:	June 2005
Estimated Primary Completion Date:	March 2013 (Final data collection date for primary outcome measure)

Intervention Details:

tablets, 2 mg day 1 through day 22 of the menstrual cycle for 5 years

Detailed Description:

Turner syndrome is a common chromosomal disorder with only one X-chromosome or partial deletions in one X-chromosome in all or some of the cell-lines in the body. Appr. 18 girls/year are being born with the syndrome in Denmark. The syndrome is mostly known for reduced final height and the lack of pubertal development with infertility, but it is also known for diseases influencing other parts of the body like the heart with the risk of development of aortic dilatation and subsequently dissection with the risk of rupture, profuse bleeding and sudden death. Reduced bone strength and increased risk of bone fracture are also features of the syndrome. Treatment with growth hormone and female sex-hormones are well-established treatments.

Girls and young women with Turner syndrome ages 10 til 21 years will participate. The girls ages 15 to 21 years will randomly and double blindly receive treatment with either 2 mg or 4 mg estrogen for 5 years. Yearly examinations with blood tests, physical examinations, questionnaires, ultrasound and MR-scan of the internal female genitalia, DEXA-scan of the bones and MR-scan of the heart will be performed.

Eligibility

Ages Eligible for Study:	10 Years to 25 Years
Genders Eligible for Study:	Female
Accepts Healthy Volunteers:	No

Criteria

Inclusion Criteria:

Verified Turner syndrome
Ages 10-25 years

Exclusion Criteria:

Contraindications to the MR-scan
Contraindications to the trial medication
Severe or chronic sickness with impact on the parameters of the study or incompatibility with the trial medication
Intake of medications with interactions with trial medication

Contacts and Locations

Please refer to this study by its ClinicalTrials.gov identifier: NCT00134745

Contacts

Contact: Line Cleemann, Doctor	+45 48294356	licle@noh.regionh.dk
Contact: Kirsten Holm, Doctor, Ph.D.	+45 48294829	kihol@noh.regionh.dk

Locations

Denmark, Frederiksborg County
Pediatric Unit, Hillerod Hospital	Recruiting
Hillerod, Frederiksborg County, Denmark, 4300
Contact: Line Cleemann, Doctor +45 48294356 licle@noh.regionh.dk
Contact: Kirsten Holm, Doctor, Ph.D. +45 49294829 kihol@noh.regionh.dk

Sponsors and Collaborators

Hillerod Hospital, Denmark

Novo Nordisk

The County of Frederiksborg

The foundation of Kaptajnløjtnant Harald Jensen and Wife

The foundation of Queen Louise childrens hospital

The foundation of Mrs. Olga Bryde

The foundation of Mr. Ivan Nielsen

Investigators

Principal Investigator:

Line Cleemann, Doctor

Pediatric Unit, Hillerod Hospital

More Information

Responsible Party:	Hillerod Hospital ( Dr. Line Cleemann )
Study ID Numbers:	120895
Study First Received:	August 24, 2005
Last Updated:	May 6, 2008
ClinicalTrials.gov Identifier:	NCT00134745
Health Authority:	Denmark: The Danish National Committee on Biomedical Research Ethics

Keywords provided by Hillerod Hospital, Denmark:

Turner Syndrome
Hormonal replacement therapy
Bone mineralization
Uterine size
aortic root dilatation

Study placed in the following topic categories:

Chromosomal abnormalities
Genital dwarfism
Dilatation, Pathologic
Gonadal Disorders
Estradiol valerate
Chromosome Disorders
Endocrine System Diseases
Estradiol 17 beta-cypionate
Sex Differentiation Disorders
Estradiol
Monosomy X

Turner Syndrome
Gonadal dysgenesis
Urogenital Abnormalities
Genetic Diseases, Inborn
Estradiol 3-benzoate
Turner syndrome
Ovarian dwarfism
Endocrinopathy
Polyestradiol phosphate
Congenital Abnormalities
Gonadal Dysgenesis

Additional relevant MeSH terms:

Pathologic Processes
Disease
Syndrome
Sex Chromosome Disorders

ClinicalTrials.gov processed this record on January 30, 2009