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

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

Condition	Intervention	Phase
Turner Syndrome	Drug: estradiol	Phase IV

Further Study Details: 

Primary Outcomes: The size of the uterus evaluated by MR-scan; Bones evaluated by DEXA-scan
Secondary Outcomes: The diameter of the aortic root evaluated by MR-scan; Development of biochemical markers; Body composition evaluated by DEXA-scan; Emotional wellbeing and self-esteem evaluated by questionnaires; The size of the uterus evaluated by ultrasound
Expected Total Enrollment:  60

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 is also features of the syndrome. Treatment with growth hormone and female sex-hormones are well-established treatments.

Girls and young women with Turner Syndrome age 10 til 21 years will participate. The girls age 15 to 21 years will randomly and double blinded receive treatment with either 2 mg or 4 mg estrogen for 5 years. Yearly examinations with blood tests, psychical 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

Ages Eligible for Study:  10 Years   -   21 Years,  Genders Eligible for Study:  Female

Criteria

Inclusion Criteria:

• Verified Turner Syndrome
• Age 10-21 years

Exclusion Criteria:

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

Please refer to this study by ClinicalTrials.gov identifier  NCT00134745

Line Cleemann, doctor      +45 48294356    licle@fa.dk
Kirsten Holm, doctor, ph.d.      +45 48294829    kihol@fa.dk

Denmark, Frederiksborg County
      Pediatric Unit, Hillerod Hospital, Hillerod,  Frederiksborg County,  4300,  Denmark; Recruiting

Study chairs or principal investigators

Line Cleemann, doctor,  Principal Investigator,  Pediatric Unit, Hillerod Hospital   

Study ID Numbers:  120895
Last Updated:  August 24, 2005
Record first received:  August 24, 2005
ClinicalTrials.gov Identifier:  NCT00134745
Health Authority: Denmark: The Danish National Committee on Biomedical Research Ethics
ClinicalTrials.gov processed this record on 2005-08-30