Objectives: To evaluate prognostic factors of the growth hormone (GH) treatment effectiveness in children with Turner syndrome (TS).
Methods: Retrospective observational study was conducted for 62 patients with TS (32 girls (52%) with 45,X karyotype, 8 (13%) with mosaic variant 45,X/46,XX, and 22 patients (35%) with structural abnormalities of X chromosome). All patients were treated with GH at a dose of 0.33 mg/kg per week continuously for a year or more. Anthropometrical and hormonal indicators of a growth axis (growth velocity (cm/year), height ΔSDS, bone age, insulin-like growth factor-1 (IGF-1) level) at GH therapy onset, after 1 year and at adult height achievement were estimated. The results were processed using SPSS.22.
Results: TS was diagnosed in patients with characteristic phenotypic signs according to the results of karyotyping at the age of 6.7±5.07 (0.1-17.3) years. The chronological age at the start of GH treatment was 9.1±3.5 years (2.6-15.2). All girls had significant growth retardation (height SDS -2.87±0.93) and a low growth velocity (4.7±1.3 cm/year). Observed growth velocity in the first year of treatment was 7.8±1.73 cm/year (P<0,001). Height ΔSDS for 1 year of therapy was 0.49±0.3 (P<0,001). No significant differences in height ΔSDS and growth velocity after 1 year GH treatment were found between patients with either X monosomy or X-chromosomal abnormalities or mosaic variant 45,X/46,XX. GH therapy was stopped in 20 girls when growth velocity decreased to <2 cm/year. Height ΔSDS was positively correlated with bone age retardation before treatment (r=0.5, P=0.025), first year height ΔSDS (r=0.499, P=0.025) and growth velocity (r=0.63, P=0.003). Correlation between height ΔSDS and mid-parental height (r=-0.15), mother height (r=-0.09), initial IGF-1 level (r=-0.23) was not revealed. No significant differences in reached height ΔSDS between girls with either spontaneous or inducted puberty (0.82 [0.62; 1.07] vs. 0.85 [0.74; 1.14]).
Conclusions: For TS patients, GH treatment significantly increases growth rate. It was revealed that bone age before therapy, dynamics of growth velocity and growth SDS on the first year of treatment are the important predictive factors of therapeutic efficacy.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology