Effect of Growth Hormone on Early Brain Development in Girls With Turner Syndrome
Condition: Turner Syndrome
MeSH terms: Turner Syndrome; Gonadal Dysgenesis; Syndrome
Study type: Interventional

Summary: 
      The purpose of this research study is to learn about brain development in very young girls
      with Turner syndrome (TS) and the effect that growth hormone (GH) therapy has on early brain
      development.
    

Detailed description: 
      Studies in older children and adults have found that IQ (intelligence quotient) in
      individuals with Turner syndrome (TS) and the general population are similar. However, many
      individuals with TS have a nonverbal learning disability which may cause problems with
      imagining and working with objects in space (for example: building blocks, working puzzles,
      copying designs, driving a car) as well as problems in planning, paying attention, and
      getting along with people their own age. Studies of brain structure have found changes in the
      size and function of specific areas of the brain that are involved in these nonverbal
      processes. However, there are no data published on brain structure or function in girls with
      Turner Syndrome in the first few years of life when brains are learning and growing most
      rapidly.

      In a recent study, very young, short girls with Turner Syndrome (TS)(averaging 2 years in
      age) were treated with Growth Hormone (GH) and almost reached an average height after 2
      years. The risks of GH for the young girls in that study appeared to be the same as those for
      older girls. Therefore, it is now recommended that GH therapy be considered as soon as a
      child with TS has growth failure. Growth failure often occurs during infancy; therefore, more
      children with TS are now receiving GH therapy as young as 12 months of age. In this study,
      the investigators wish to start understanding what effect GH has on learning and brain growth
      when given between the ages of 12 and 24 months. In this study, the girls with TS will have
      developmental studies, a physical examination, magnetic resonance imaging (MRI), and blood
      drawn at one and two years of age. Some of the girls in this study will receive GH from 12
      until 24 months of life, while others will not. Brain growth and development will be compared
      between those who have been treated with GH and those who have not. This is a pilot study in
      which the investigators will gather data to design a larger study that can answer these
      questions.
    

Intervention name(s): somatotropin
Intervention type(s): Drug
Intervention description(s): Subjects will receive 5 mg somatotropin (growth hormone) pens with cartridges. Subcutaneous injections are to be given every evening around bedtime. Dosing regimen: 50 mcg/kg/day to be adjusted at 4 month intervals to the closest 0.1 mg. Subjects will be given 12 months of treatment (from 12 to 24 months of life). Subjects will visit their pediatrician or pediatric endocrinologist at 4 and 8 months of life.
Other intervention name(s): Genotropin; Growth hormone
Intervention MeSH terms: Hormones
Allocation: Randomized
Intervention model: Parallel Assignment
Masking: None (Open Label)
Primary purpose: Basic Science
Keywords: Turner Syndrome; growth hormone; brain development