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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

  • Endocrinology
  • Original Paper
  • Published:
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Abstract

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 im 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. 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 independent of the GH status.

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Abbreviations

GH :

growth hormone

IGF-I :

insulin like growth factor-I

IGFBP :

IGF-binding protein-3

Ox :

oxandrolone

T :

testosterone

UTS :

Ullrich Turner syndrome

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Authors and Affiliations

  1. Paediatric Department, University of Vienna, Währinger Gürtel 18-20, A-1090, Vienna, Austria

    G. Haeusler & H. Frisch

  2. Landeskinderkrankenhaus, Linz, Austria

    K. Schmitt

  3. Gottfried von Preyer'sches Kinderspital, Vienna, Austria

    P. Blümel

  4. Landeskinderkrankenhaus, Salzburg, Austria

    E. Plöchl

  5. Paediatric Department, University of Zürich, Switzerland

    M. Zachmann

  6. Institute for Tumour Biology and Cancer Research, University of Vienna, Austria

    T. Waldhör

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Haeusler, G., Frisch, H., Schmitt, K. et al. 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. Eur J Pediatr 154, 437–444 (1995). https://doi.org/10.1007/BF02029351

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  • DOI: https://doi.org/10.1007/BF02029351

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