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Phase 4 N=41 Randomized Treatment

Estrogen Dosing in Turner Syndrome: Pharmacology and Metabolism

Turner Syndrome · Hypogonadism · Premature Ovarian Failure

Enrolled (actual)
41
Serious AEs
0.0%
Results posted
Feb 2014
Primary outcome: Primary: Change in Weight From Baseline at 12 Months — 1.1; 1.9 kilograms

Study Design & Population

Study type
Interventional
Phase
Phase 4
Interventions
17 B estradiol orally (Drug); 17 B estradiol (Drug)
Age
Pediatric, Adult · 13+ yrs
Sex
Female
Sponsor
Nemours Children's Clinic
Primary completion
Jun 2012

Outcome Measures

OutcomeResultp-value
PRIMARY
Change in Weight From Baseline at 12 Months
1.1; 1.9
PRIMARY
Change in Body Mass Index From Baseline at 12 Months
0.075; 0.65
PRIMARY
Change in Percent Fat Mass From Baseline in 12 Months
-0.14; -0.64
PRIMARY
Change in Fat Free Mass From Baseline at 12 Months
1.03; 1.67
SECONDARY
Changes in Insulin Growth Factor-I From Baseline at 12 Months
-16; 28
SECONDARY
Lipids Concentrations After Using Oral Versus Transdermal 17B Estradiol Replacement for 12 Months
168; 153; 93; 88; 56; 50
SECONDARY
Rates of Lipid Oxidation After Using Oral Versus Transdermal 17B Estradiol Replacement for 12 Months
10; 7.3
SECONDARY
Serum 17B Estradiol Concentrations After Using Oral Versus Transdermal 17B Estradiol Replacement for 12 Months
124; 74
SECONDARY
Serum Estrone Concentrations After Using Oral Versus Transdermal 17B Estradiol Replacement for 12 Months
504; 43
SECONDARY
Serum Estrone Sulfate Concentrations After Using Oral Versus Transdermal 17B Estradiol Replacement for 12 Months
63638; 1875

Summary

Estrogen is necessary for feminization during puberty and to decrease bone resorption, the latter critical for the achievement of peak bone mass and normal bone health in the female. The practicing pediatric endocrinologist often faces the dilemma of how to best feminize girls with hypogonadism (lack of estrogen), manifested as delayed or arrested puberty, due to disorders of the brain or the ovaries. We propose a series of studies to address which type, dose, and route of delivery of estrogen are suitable choices in feminizing and sustaining estrogen concentrations in adolescent girls with Turner syndrome. To accomplish this we will study girls/young woman between the ages of 13 to 20 with Turner Syndrome in 2 protocols. In Protocol # 1 we will study 24 girls with TS, they will receive 3 different estrogen preparations, either by mouth or via a patch for a total of 6 weeks. They will come to the clinical research center for blood draws after 2 wks of taking the estrogen. With this study, we hope to learn how the body responds to estrogen differently, depending on the form estrogen is given and how high, estrogen levels gets in the blood in these girls with Turner Syndrome. We will be comparing these patients estrogen levels to girls that menstruate normally and do not have Turner Syndrome. In Protocol #2, 40 patients with TS will be recruited; these patients will take estrogen for 1 year, either by mouth or via a patch. Patients will come to the lab for blood drawn in 7 occasions and we will measure estrogen levels as well as other hormones and lipid levels. We will also perform a Dual-energy X-ray absorptiometry (DXA) study (like an X ray) to assess body composition and bone mineralization. We will adjust doses based on the estrogen levels we find. With this study we hope to learn how estrogen affects body composition, i.e., the amount of fat vs. muscle, and how different forms of estrogen affect blood cholesterol and other hormones. This study will allow us to understand better how to best replace young woman with Turner Syndrome with estrogen.

Eligibility Criteria

Inclusion Criteria

  • Girls with Turner Syndrome (45X, or related karyotypes) diagnosed clinically and cytogenetically
  • Female subjects with Y material will be allowed providing gonadectomies have been performed previously
  • Age: 13-20 years
  • Subjects have completed or nearly completed their linear growth
  • Previous growth hormone (GH) therapy discontinued at least 6 months prior to study participation
  • Stable thyroid replacement therapy will be allowed
  • Celiac disease on stable diets will be allowed
  • Any previous hormone replacement therapy (HRT) will be allowed

Exclusion Criteria

  • Diabetes Mellitus on insulin therapy, insulin sensitizers or oral hypoglycemics
  • Inflammatory Bowel Disease (ulcerative colitis or Crohn's disease), celiac disease
  • Cigarette smoking
  • Any other chronic conditions, that, in the opinion of investigators could impair the metabolism of nutrients
  • Severe obesity, i.e., Body Mass Index (BMI) >95th centile
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT00837616). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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