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Short stature and primary gonadal failure are characteristic features of Turner Syndrome (TS). Both these aspects need to be addressed in the care of these patients for a satisfactory outcome. This is the first report from Sri Lanka documenting the short term outcome on linear growth and puberty following treatment with growth hormone (GH) and hormone replacement therapy (HRT). Method: A prospective analysis was done on 20 patients from a cohort of patients with TS followed up at the University Paediatric Unit at the Lady Ridgeway Hospital, Colombo. An interviewer-administered questionnaire was used to record the relevant information including details and outcome of treatment with GH and HRT. Ethics Review Committee of the Faculty of Medicine, University of Colombo approved the study. Results: Thirteen (65%) patients had a 45, X karyotype. Their mean (SD) age at presentation was 10.06 (2.83) years with a range of 3.16 to 13.58 years. Their heights at presentation were all th percentile on the TS specific growth chart and ranged from 84.0 to 129.8 cm with a mean (SD) of 113.6 (12.13) cm. Growth hormone (n=20) and HRT (n=14) were started at mean (SD) ages of 10.19 (2.72) and 13.41 (1.13) years respectively. Time to menarche after HRT in 9 patients ranged from 0.42 to 2 years with a mean (SD) of 1.26 (0.46) years. The gain in height with GH alone (n=12) was 3.5 to 26.4 cm and at the time of reporting at a mean (SD) age of 13.89 (2.86) years the heights of 7 patients were ≥75 th percentile on the TS specific growth chart. Conclusion: A satisfactory response to GH therapy was observed despite the mean (SD) age of presentation being 10.06 (2.83) years and thus initiating definitive therapy at a mean (SD) age of 10.19 (2.72) years. Sri Lanka Journal of Child Health , 2017; 46 : 55-58

sri lanka journal of child health

Outcome of linear growth and onset of menarche following therapy in children with Turner syndrome followed up at a tertiary care referral centre: An interim report from Sri Lanka