Background: Turner Syndrome (TS) is associated with short stature and skeletal disproportion. The impact of treatment with recombinant human growth hormone (rhGH) and oestrogen therapy on body proportions is unclear.
Objectives: To evaluate sitting height (SH) and leg length (LL) in TS before growth promoting therapies and at adult height.
Method: Retrospective study of height (Ht), SH and LL standard deviation score (SDS) in TS. Body proportion was also evaluated using SH SDS-LL SDS and SH:Ht ratio SDS. Eligible girls were d at least 4 years at baseline, had not yet started rhGH and had no other chronic disease. Thirty girls had serial measurements until adult height.
Results: In 59 prepubertal girls prior to rhGH (6.6±0.3 years), Ht SDS was −2.6±0.1 with disproportionately shorter legs, LL SDS −3.4±0.2 compared to spine, SH SDS −1.2±0.1 [P<0.001]. SH SDS-LL SDS was 2.2±0.1 (>2 SD in 34/59 [58%]) and SH:Ht ratio SDS was 2.3±0.1 (>+2.0 SD in 37/59 [63%]). Disproportion did not differ between girls with 45,X, (n=19) and those with other karyotypes (n=38). Therapy with rhGH and oestrogen led to stabilisation of SH SDS but continued improvement in LL SDS. LL SDS pre-rhGH, pre-oestrogen and at adult height were −3.6±0.2, −3.0±0.2 (P< 0.0001 vs pre-rhGH) and −2.1±0.2 (P<0.0001 vs pre-rhGH and pre-oestrogen), respectively. SH:Ht ratio SDS did not change with rhGH therapy prior to pubertal induction: 2.5±0.2 to 2.3±0.2 (P=0.21 vs baseline) but was 1.8±0.1 at adult height (P<0.0001 vs baseline), suggesting a lesser degree of disproportion.
Conclusion: Prepubertal girls with TS have significantly shorter legs compared to their spine. Our study is the first to evaluate the impact of rhGH and oestrogen on body proportions in TS. At adult height, disproportion was still present but less pronounced.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology