Experience with growth hormone therapy in Turner syndrome in a single centre: Low total height gain, no further gains after puberty onset and unchanged body proportions

The experience gained since 1987, through observation of 85 girls with Turn er syndrome under growth hormone (GH) treatment, has enabled the analysis o f one of the largest cohorts. Our results show that age, karyotype a nd he ig ht reflect the heterogeneity of the patients examined at our growth cent re. in 47 girls, followed over 4 years on GH (median dose 0.72 IU/kg/week), the median age was 9.4 years and mean height SDS was -3.55 (Prader) and -0 .14 (Turner-specific), while height and other anthropometrical parameters [ weight body mass index, sitting height (SH), leg length (LL) SH/LL, head ci rcumference, arm span] were documented and compared to normative data as we ll as to Turner-specific references established on the basis of a larger(n = 165) untreated cohort from Tubingen. The latter data are also documented in this article. Although there was a trend towards normalization of these parameters during the observation period, no inherent alterations in the Tu rner-specific anthropometric pattern occurred. In 42 girls who started GH t reatment at a median age of 11.8 years, final height (bone age >15 years) w as achieved at 16.7 years. The overall gain in height SDS (Turner) from sta rt to end of GH therapy was 0.7 (+/- 0.8) SD, but 0.9 (+/- 0.6) SD from GH start to onset of puberty (spontaneous 12.2 years, induced 13.9 years) and -0.2 (+/- 0.8) from onset of puberty to end of growth. Height gain did not occur in 12 patients (29%) and a gain of > 5 cm was only observed in 16 pat ients (38%). Height gain correlated positively with age at puberty onset, d uration, and dose of GH, and negatively with height and bone age at the tim e GH treatment started. Final height correlated positively with height SDS at GH start and negatively with the ratio of SH/LL (SDS). We conclude that, in the future, GH should be given at higher doses, but oestrogen substitut ion should be done cautiously, owing to its potentially harmful effect on g rowth. LL appears to determine height variation in Turner syndrome and the potential to treat short statu re successfully with GH. Copyright (C) 2000 S. Karger AG,Basel.