Treatment of patients with Ullrich-Turner syndrome with conventional doses of growth hormone and the combination with testosterone or oxandrolone: effect on growth, IGF-I and IGFBP-3 concentrations.
Thirty-nine girls with Ullrich-Turner syndrome (UTS) (median age 9.5 years) were treated with growth hormone (GH) with either 12 or 18 IU/m2 per week for 12 months followed by combination therapy with either oxandrolone (Ox) (0.0625 mg/kg/day po) or low-dose testosterone (T) (5 mg in every 2 weeks). Growth velocity improved significantly after 12 IU/m2 per week (6.4 +/- 1.7 cm/year vs 4.0 +/- 1.3 cm/year, x +/- SD, P < 0.001) and 18 IU/m2 per week of GH (6.5 +/- 1.3 cm/year vs 4.5 +/- 1.4 cm/year, P < 0.001). Ox, but not T was effective in maintaining growth velocity during the 2nd year of therapy (6.9 +/- 1.3 vs 5.3 +/- 1.5 cm/year). Basal insulin-like growth factor-I (IGF-I) concentrations were in the lower normal range and increased significantly in patients treated with 18 IU/m2 per week (357 +/- 180 ng/ml vs 160 +/- 84 ng/ml) and 12 IU/m2 per week (273 +/- 121 ng/ml vs 140 +/- 77 ng/ml). IGF-I concentrations increased further after addition of Ox (533 +/- 124 ng/ml, P < 0.001) or T (458 +/- 158, P < 0.05). IGFBP-3 concentrations were in the upper normal range before therapy and increased only moderately in both GH dosage groups. However, IGF binding protein-3 (IGFBP-3) concentrations were not affected by additional Ox or T treatment.


CONCLUSIONS
1. Conventional GH doses are effective in increasing growth velocity in UTS, especially, when combined with Ox.(ABSTRACT TRUNCATED AT 250 WORDS)
Haeusler
G
G
Paediatric Department, University of Vienna, Austria.
Frisch
H
H
Schmitt
K
K
Blümel
P
P
Plöchl
E
E
Zachmann
M
M
Waldhör
T
T
D000365:Age Determination by Skeleton; D001827:Body Height; D001835:Body Weight; D002352:Carrier Proteins / Q000097:blood / Q000187:drug effects; D002648:Child; D004359:Drug Therapy, Combination; D005260:Female; D006128:Growth / Q000187:drug effects*; D013006:Growth Hormone / Q000008:administration & dosage*; D006801:Humans; D018969:Insulin-Like Growth Factor Binding Proteins; D007334:Insulin-Like Growth Factor I / Q000187:drug effects / Q000378:metabolism; D010074:Oxandrolone / Q000009:adverse effects / Q000627:therapeutic use*; D013739:Testosterone / Q000009:adverse effects / Q000627:therapeutic use*; D016896:Treatment Outcome; D014424:Turner Syndrome / Q000628:therapy*
D002352:Carrier Proteins; D018969:Insulin-Like Growth Factor Binding Proteins; D013739:Testosterone; D007334:Insulin-Like Growth Factor I; D010074:Oxandrolone; D013006:Growth Hormone