ESPE2014 Poster Category 2 Turner Syndrome (11 abstracts)
aBashkir State Medical University, Ufa, Russia; bEndocrinological Research Center, Moscow, Russia
Background: Patients with Turners syndrome (TS) develop osteoporosis, resulting from chromosomal deficiency and estrogen deficiency by gonadal dysgenesis.
Objective and hypotheses: The aim of this study was to assess bone mineral density (BMD) and parameters of bone remodeling during somatropin therapy in prepubertal girls with TS.
Population and/or methods: We examined 22 girls with TS of the age of 1115 years (the mean age of 13.1±0.9 years), treating by somatropin Rastan (Pharmstandart, Russia) daily at a dose of 0.05 mg/kg subcutaneously (group 1). The comparison group was composed of 11 girls of the same age, not receiving somatropin (group 2). All patients were diagnosed with TS at chromosomal examination of peripheral blood. 19 (57.6%) patients had 45, XO and 14 (42.4%) patients had mosaicism. All girls with TS underwent measurement of areal BMD using dual-energy X-ray absorptiometry (DXA) to obtain anteroposterior lumbar spine values at L1L4 before treatment and 12 months after beginning of therapy with recombinant GH. We defined osteoporosis as a Z-score <−2.5 and osteopenia as <−1 and ≥−2.5. Girls with hypothyroidism were not included in the study. Statistical significance of parameters before and after treatment was evaluated by Wilcoxons single-rank test.
Results: In groups 1 and 2, the median Z-score was −1.9 and −1.8, respectively; osteoporosis was revealed in 2 (9.1%) and 1 (9.1%) girls respectively, osteopenia in 5 (22.7%) and 3 (27.3%) girls, respectively. The median Z-score rose from −1.9 to −1.7 after 12 months (P<0.019). In group 2, change of Z-score at dynamic follow-up were statistically non-significant.
Conclusions: Prepubertal TS patients are very likely to develop osteopenia as they reach pubertal age. GH treated prepubertal girls with TS showed improvements in BMD.