Turner's syndrome

J Pediatr Endocrinol Metab. 2001 Jul:14 Suppl 2:959-65.

Abstract

Turner syndrome (TS) is the most common sex-chromosome abnormality in females. Short stature and hypogonadism are the classical clinical findings. The spontaneous final height (FH) ranges between 139 and 147 cm, representing a growth deficit of about 20 cm with respect to the unaffected population. GH therapy improves FH and should be started during childhood at a high dose of about 1 IU/kg/week (range 0.6-2 IU/kg/week). Some authors advocate combined therapy with an anabolic steroid at various doses (e.g. oxandrolone 0.05-0.1 mg/kg/day). This treatment results in a significantly increased FH, a large proportion of treated girls reaching a FH of more than 150 cm. Gonadal function is compromised during adolescence in about 80% of girls with TS, whilst in about 20% pubertal development occurs spontaneously. Oestrogen therapy should be started at the age of 13-14 years in hypogonadic patients; early onset of treatment (before 12 years) seems to compromise FH. Other concerns in these patients are fertility and osteopenia.

Publication types

  • Guideline
  • Review

MeSH terms

  • Adolescent
  • Child
  • Female
  • Gonadal Steroid Hormones / administration & dosage
  • Gonadal Steroid Hormones / therapeutic use
  • Growth Disorders / etiology
  • Growth Disorders / therapy*
  • Growth Hormone / administration & dosage
  • Growth Hormone / therapeutic use
  • Humans
  • Male
  • Puberty / drug effects
  • Puberty / physiology*
  • Turner Syndrome / drug therapy*

Substances

  • Gonadal Steroid Hormones
  • Growth Hormone