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-S CONCENTRATIONS

Thirty-nine girls with Ullrich-Turner syndrome (UTS) (median age 9.5 y ears) were treated with growth hormone (GH) with either 12 or 18 IU/m( 2) 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 im every 2 weeks). Growth velocity improved significantly after 12 IU/m(2) per week (6.4 +/- 1.7 cm/year vs 4.0 +/- 1.3 cm/year, x +/- S D, P < 0.001) and 18 IU/m(2) 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 maintain ing growth velocity during the 2nd year of therapy (6.9 +/- 1.3 vs 5.3 +/- 1.5 cm/year). Basal in insulin-like growth factor-I (IGF-I) conce ntrations were in the lower normal range and increased significantly i n patients treated with 18 IU/m(2) per week (357 +/- 180 ng/ml vs 160 +/- 84 ng/ml) and 12 IU/m(2) 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 co ncentrations were in the upper normal range before therapy and increas ed only moderately in both GH dosage groups. However, IGF binding prot ein-3 (IGFBP-3) concentrations were not affected by additional Ox or T treatment. Conclusions 1. Conventional GH doses are effective in incr easing growth velocity in UTS, especially, when combined with Ox. This additive effect is not evident when GH is combined with low dose T. 2 . Changes in growth velocity are accompanied by an increase of the IGF -I/IGFBP-3 ratio. 3. Ox obviously acts by increasing IGF-I levels inde pendent of the GH status.