ESPE Abstracts

Objectives: Short stature is the main characteristics for Turner syndrome (TS) patients, and growth hormone (GH) therapy has been used as an essential treatment for developing final adult height. However, there are only a few studies on the difference responsiveness to GH therapy according to the karyotype of Turner syndrome in Korea. The aim of this study was to analyze the effect of different types of TS karyotype abnormality on the response of GH therapy.

Methods: The clinical parameters of 185 TS patients registered in the LG Growth Study (LGS) were retrospectively reviewed. Data for four group of subjects were obtained as follow: X-monosomy (n=52); X mosaicism without structural abnormality (n=40); X mosaicism with structural abnormality (n=68); Structural abnormality (n=25). Parameters of clinical characteristics and growth responses were compared during 3-year GH treatment.

Results: The baseline height standard deviation score (SDS), body mass index SDS, bone age (BA) – chronological age (CA) were significantly higher in the X-monosomy group than in other karyotypes. Height SDS and growth velocity (GV) were evaluated annually. The height increment in the first year was highest, and after that, it tended to decreased over time in all TS groups. The GV is highest in TS patients with X mosaicism without structural abnormality, followed by the structural abnormality, followed by the X mosaicism with structural abnormality and the lowest in the X-monosomy. The age at start GH treatment was inversely correlated with height SDS in third year in multiple regression analysis (P<0.05).

Conclusions: Our results indicate that there are difference in response to three years of growth hormone therapy according to karyotype of TS. X-monosomy determined a poorer response than in other karyotypes. On the other hand, the best response to the GH therapy was observed in TS patents with X mosaicism without structural abnormality. Moreover, early growth hormone administration with TS is helpful to improve height response to the treatment.