title: Height gains in response to growth hormone treatment to final height are similar in patients with SHOX deficiency and Turner syndrome.
abstract: BACKGROUND
  Patients with mutations or deletions of the Short Stature Homeobox-containing(SHOX) gene have variable degrees of growth impairment, with or without mesomelic skeletal dysplasia. If untreated, short patients with SHOX deficiency remain short in adulthood. Growth hormone (GH) treatment improves short-term linear growth; however, there are no data on GH treatment effects on final height.
  
  
  METHODS
  In a retrospective study, we assessed the relative effects of GH on final height gain in patients with SHOX deficiency (n = 14; 12 females) and Turner syndrome (TS) (n = 158). Patients were included if they fulfilled the following criteria: genetically-confirmed SHOX deficiency or TS, baseline height SDS <1.5, GH treatment started at Tanner stage < or =2, duration of GH treatment >2 years, and final height attained.
  
  
  RESULTS
  Both groups of patients were short at baseline (height SDS [mean +/- SD]: SHOX deficiency, -3.3 +/- 0.9; TS, -2.9 +/- 0.8). Height SDS gain from baseline to final height was significant for each patient group (SHOX deficiency, 1.1 +/- 0.7; TS, 1.2 +/- 0.8; p < 0.001); however, it was not significantly different between groups (p = 0.708).
  
  
  CONCLUSIONS
  Patients with SHOX deficiency receive similar final height benefit from GH treatment to those with TS.
authors.affiliation: Lilly Research Laboratories, Eli Lilly & Co., Bad Homburg, Germany. blum_werner@lilly.com
authors.forename: Werner F
authors.initials: WF
authors.lastname: Blum
authors.forename: Dachuang
authors.initials: D
authors.lastname: Cao
authors.forename: Volker
authors.initials: V
authors.lastname: Hesse
authors.forename: Susanne
authors.initials: S
authors.lastname: Fricke-Otto
authors.forename: Judith L
authors.initials: JL
authors.lastname: Ross
authors.forename: Christine
authors.initials: C
authors.lastname: Jones
authors.forename: Charmian A
authors.initials: CA
authors.lastname: Quigley
authors.forename: Gerhard
authors.initials: G
authors.lastname: Binder
mesh_terms: D000293:Adolescent; D041923:Adolescent Development / Q000187:drug effects; D000365:Age Determination by Skeleton; D001827:Body Height / Q000187:drug effects*; D002648:Child; D002657:Child Development / Q000187:drug effects; D005260:Female; D006130:Growth Disorders / Q000150:complications / Q000188:drug therapy* / Q000235:genetics* / Q000503:physiopathology; D018398:Homeodomain Proteins / Q000235:genetics*; D019382:Human Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D009634:Noonan Syndrome / Q000150:complications / Q000188:drug therapy / Q000235:genetics; D012189:Retrospective Studies; D000074122:Short Stature Homeobox Protein; D014424:Turner Syndrome / Q000150:complications* / Q000188:drug therapy / Q000235:genetics
chemical_list: D018398:Homeodomain Proteins; C106071:SHOX protein, human; D000074122:Short Stature Homeobox Protein; D019382:Human Growth Hormone