Growth during pubertal induction in girls with Turner Syndrome: a retrospective cohort analysis.

ABSTRACT

CONTEXT Patients with Turner Syndrome often present with short stature and ovarian insufficiency. The optimal method of pubertal induction to maximize adult height (AH) is unknown. OBJECTIVE: To identify variables related to pubertal induction that are associated with growth and AH. DESIGN & SETTING: Retrospective cohort analysis of patients attending a specialized Turner Syndrome clinic at a quaternary children's hospital. PATIENTS Patients with Turner Syndrome (n=107) who attended the clinic between 2015 and 2021. Of these, 51 received estradiol for pubertal induction. MAIN OUTCOME MEASURES: Change in height standard deviation score (ΔHeightSDS) during pubertal induction, and AH. METHODS: Age at pubertal induction, bone age delay, midparental height (MPH), growth hormone treatment, and karyotype were assessed as predictors of AH and ΔHeightSDS. Associations between karyotype and comorbidities were also assessed. RESULTS: AH was predicted by MPH (0.8cm/cm, P=0.0001) and bone age delay (-1.84 cm/year, P= 0.006). ΔHeightSDS was predicted by growth hormone dose (0.09 SDS/mg/m2/week; P=0.017), bone age delay (-1.37 SDS/year; P=0.003), and age at pubertal induction (0.44 SDS/year; P=0.001). There was an interaction between bone age delay and pubertal induction age (P=0.013), with the combination of younger age at pubertal induction and a less-delayed bone age associated with a lower ΔHeightSDS. Karyotype did not influence AH or ΔHeightSDS, but did affect rates of other comorbidities. CONCLUSIONS: Decisions around timing of pubertal induction in patients with Turner Syndrome should be tailored to the individual. The current approach to estrogen supplementation needs to be refined in order to facilitate pubertal induction in a physiological manner without compromising height.