title: Short stature and its treatment in Turner and Noonan syndromes.
abstract: OBJECTIVE
  We review recent developments in the approach to the treatment of short stature in patients with Turner and Noonan syndromes.
  
  
  RESULTS
  Turner syndrome and Noonan syndrome are clinically defined conditions associated with short stature. The Food and Drug Administration (FDA) approved treatment with recombinant human growth hormone (hGH) for patients with Turner syndrome in 1996 and for those with Noonan syndrome in 2007. Studies have shown that early appropriate use of hGH increases adult height in individuals with Turner syndrome. The combination of hGH and low-dose estrogen may also improve growth and adult height as well as possibly provide neurocognitive and behavioral benefits. Noonan syndrome is a genetically heterogeneous condition. In patients with Noonan syndrome phenotype, investigators have identified disease-associated genes (PTPN11, SOS1, RAF1, KRAS, and others). Treatment with hGH has been documented to result in short-term increases in growth velocity as well as modest gains in adult height.
  
  
  CONCLUSIONS
  Our understanding and management of short stature in children with Turner syndrome and Noonan syndrome has greatly advanced over the years. Recent developments with focus on these two common syndromes will be reviewed.
authors.affiliation: Mount Sinai School of Medicine, New York, USA.
authors.forename: Elizabeth M
authors.initials: EM
authors.lastname: Chacko
authors.forename: Robert
authors.initials: R
authors.lastname: Rapaport
mesh_terms: D000293:Adolescent; D000328:Adult; D045930:Anabolic Agents / Q000627:therapeutic use*; D001827:Body Height / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D004967:Estrogens / Q000627:therapeutic use*; D005260:Female; D013006:Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D009634:Noonan Syndrome / Q000235:genetics / Q000628:therapy*; D010641:Phenotype; D011994:Recombinant Proteins / Q000627:therapeutic use; D014424:Turner Syndrome / Q000235:genetics / Q000628:therapy*; D055815:Young Adult
chemical_list: D045930:Anabolic Agents; D004967:Estrogens; D011994:Recombinant Proteins; D013006:Growth Hormone