The Effects of Estrogen on Cognition in Girls With Turner Syndrome
Gonadal DysgenesisTurner's SyndromeThe 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.
Evaluating Acceptance of New Liquid Somatropin Formulation in Children With Growth Hormone Deficiency...
Growth Hormone DisorderGrowth Hormone Deficiency in Children2 moreThis trial is conducted in Asia. The aim of this trial is to evaluate the new liquid somatropin formulation in children with growth hormone deficiency.
Quantitative Liver Functions in Turner Syndrome With and Without Hormone Replacement Therapy
Turner SyndromeSeveral studies have demonstrated that Turner Syndrome patients have elevated liver enzymes readily suppressible by a short course of HRT. We wanted to estimated quantitative liver functions in a young group of Turner syndrome patients compared to a healthy control group.
Long-Term Growth and Skeletal Effects of Early Growth Hormone Treatment in Turner Syndrome
Turner SyndromeThis is an extension study that will gather long-term data on the effect of early growth hormone (GH) treatment on adult height and other aspects of health and development in girls with Turner syndrome. The main purpose is to determine whether girls who received 2 years of GH treatment before 6 years of age achieve taller adult height than girls who were untreated during this time. The study will also look at middle ear and hearing function, and cognitive and behavioral development. Protocol completion is defined as attainment of height velocity less than or equal to 1.0 cm/year, or bone age greater than or equal to 15 years.
Use of Somatropin in Turner Syndrome
Genetic DisorderTurner SyndromeThis trial is conducted in Europe. The aim of this trial is to study the dose-response relationship and effect of somatropin (Norditropin®) on final height in girls with Turner Syndrome.
Safety of Somatropin and Induction of Puberty With 17-beta-oestradiol in Girls With Turner Syndrome...
Genetic DisorderTurner SyndromeThis trial is conducted in Europe. The aim of this trial is to assess whether increasing doses of somatropin (Norditropin®) can maintain the initial increase in height velocity and improve final height. This trial has two trial periods, a main period of 4 years and an extension period until final height is reached.
Effect of Estrogen on Mental and Social Functioning in Girls With Turner's Syndrome
Turner's SyndromeRATIONALE: Turner's syndrome is a disease in which females are missing all or part of one X chromosome and do not produce estrogen. Giving estrogen is standard treatment for girls who have Turner's syndrome. Estrogen may be effective treatment for mental and social functioning problems experienced by girls with Turner's syndrome. PURPOSE: Clinical trial to study the effectiveness of long term estrogen therapy on mental and social functioning in girls who have Turner's syndrome.
Hormone Replacement Therapy to Treat Turner Syndrome
OsteoporosisTurner's SyndromeThis study will evaluate the effects of hormone replacement therapy on patients with Turner syndrome (TS)-a genetic disorder in females in which part or all of one X chromosome is missing. Most girls and women with TS have underdeveloped ovaries-the female reproductive organs that produce the female sex hormones estrogen and progesterone, and smaller amounts of the male sex hormone, testosterone. These hormones affect muscle and bone strength, sex drive, energy, and an overall sense of well being. Estrogen may also play a role in memory and mood and have a protective effect against heart disease. Women with TS have a much higher risk of developing osteoporosis (loss of bone density), high blood pressure, high cholesterol and diabetes than women without this disorder. Girls and women with Turner syndrome between the ages of 14 and 50 years may be eligible for this 2-year study. Three months before beginning treatment, all patients will wear an estrogen patch and take a progesterone tablet daily for 10 days each month. They will then be randomly assigned to one of two treatment groups to compare the effects of estrogen alone with estrogen plus testosterone on bone strength, muscle and fat mass and psychosocial well being. Both groups will wear an estrogen patch and take oral progesterone. One group will also wear a testosterone patch while the other group will wear a placebo patch (a patch that does not contain any testosterone). Neither study participants nor the doctors will know who is getting the testosterone until the study is complete. Patients will undergo the following procedures before beginning treatment and at 6, 12 and 24 months after starting treatment: Physical examination. DEXA scans (dual energy X-ray absorptiometry) to measure body composition and bone thickness. Low radiation X-rays scan the whole body to measure fat, muscle and bone mineral content.. Magnetic resonance imaging (MRI) scan of the abdomen to measure the amount of fat around the internal organs. The patient lies on a stretcher in a large tube surrounded by a magnetic field during the scanning. The procedure uses a strong magnet and radio waves to produce the images. Heel ultrasound to measure bone thickness. The heel is placed in a chamber and sound waves pass through it to produce images. Oral glucose tolerance test (OGTT) for diabetes and problems with carbohydrate metabolism. The patient drinks a sugary substance. A small amount of blood is drawn before taking the drink and four times afterwards. Blood and urine tests to measure blood counts, liver and kidney function, ovarian hormones, growth factors, thyroid function, blood lipids, bone strength markers, and to test for pregnancy. Blood pressure measurements. Psychological testing for the effect of treatment on mood, self-esteem, quality of life, social shyness, anxiety and sexual function. Neurocognitive tests (at first inpatient visit and 1 and 2 years after starting treatment) to measure nonverbal memory and visual-perceptual abilities. During the hospital admissions, patients will be given a "metabolic diet" that contains specific amounts of salt and carbohydrates to ensure accurate blood pressure and sugar metabolism measurements. Patients will keep a record of their menstrual periods and physical activity throughout the treatment period.
Defining the Optimal Hormonal Replacement Therapy in Turner Syndrome
Turner SyndromeThe 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.
Estrogen Treatment (Oral vs. Patches) in Turner Syndrome
Turner SyndromeHypogonadism1 moreThe study attempts to evaluate if the way of administering estrogen, the principal female hormone, via patches or orally, affects the way estrogen works in girls with Turner Syndrome. These are girls who are very short and whose ovaries do not work. We will examine changes bone, protein and fat metabolism under the influence of estrogen delivered by a patch trough the skin vs estrogen taken orally. These studies are conducted while the girls are taking GH therapy.