Background: Short stature is the most common presenting symptom in Turner Syndrome (TS). GH treatment helps alleviating short stature in TS, although response to treatment varies significantly.
Objective and hypotheses: We aimed to evaluate the response to GH treatment and factors affecting this response in children with TS.
Method: Forty-nine patients with TS diagnosed by cytogenetic analysis and who had been followed minimum of 1-year with GH therapy were included in the study. Clinical and anthropometric parameters were obtained retrospectively from patients files. Height SDS changes >0.5 SD in first year of treatment was considered as good response.
Results: The mean age at diagnosis was 9.9±3.8 years of age, the mean height standard deviation score (SDS) was −3.2±1.3. The most common karyotype was 45X (40.8%) followed by 45X/46XX (14.3%) and 46X,i(X)(q10) (12.2%). 40% of patients had intrauterine growth retardation (birth weight<2500 gr). The initial GH dosage was 41±1 μg/kg per day. The average height SDS at the beginning, first year and second year of therapy were −3.0±1.1, −2.5±1.0 (n;49) and −2.3±1.1 (n:40), respectively. 27 patients had good response to treatment. Target height (TH), presenting age, birth weight, chronological age-bone age difference and change in IGFI SDS after treatment were not different between good and poor responders. However, good responders had lower height SDS at presentation (−3.2±1.3 vs. −2.6±1.0, P<0.05) and lower peak GH response to stimulation (6.5±4 vs. 8.9±5 mg/dL, P<0.05) and lower IGF-I SDS (−2.1±1.1 vs. −1.7±1.3) although the latter was nonsignificant. The final height was available in 15 patients and was 150±4.9 cm (−2.0±0.8 SDS). Good responders reached final height of 152.5±2.3 (n:7) which was higher than poor responders [147.1±5.6 cm (n:8), P<0.05]. Final heights in poor responders were significantly lower than their target height [147.1±5.6 vs. 158±7 cm (P<0.01)], whereas, this was not significant in good responders (152.5±2.3 vs. 156±6 cm, P:0.07).
Conclusion: Only significant factor affecting first year response to GH treatment was GH levels on provocative testing in children with TS. In those, who reached final height, height gain was also better in good responders.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology