Dospělá výška dívek s Turnerovým syndromem: rozhoduje dobazahájení estrogenní substituce?

Adult Height of Girls with Turner’s Syndrome: is the Time of Initiation of Oestrogen Substitu-tion Decisive?

In 21 girls with Turner’s syndrome (TS) without spontaneous puberty treated with growth hormone (GH) theauthors evaluated factors which influenced their final height. Special attention was focused on the time of onset ofoestrogen substitution.GH treatment was started at the age of 13.1 ± 1.7 years (mean ± SD) and lasted 3.7 ± 1.4 years. The GH dosewas 1 IU/kg/week and was divided into 7 injections per week. The period of treatment with growth hormone only(„oestrogen free“ interval) was between -3.9 and +4.2 years in relation to the onset of oestrogen substitution.The patients achieved a height of 151.2 ± 6.8 cm. The difference between the attained height and the foreseenheight in adult age (calculated by the projection method from Ranke’s standard for TS) was 2.8 ± 5.5 cm. Thisheight increment correlated with the period of GH administration (r = 0.45, p = 0.0008), with the period of GHtreatment without concurrent oestrogen substitution (r = 0.28, p = 0.01). The group of girls who were givenoestrogens before the onset of GH treatment („oestrogen free“ interval < 0) (n = 8) achieved a final height of 145.1± 5.8 cm and their height gain (and loss resp.) was -1.8 ± 3.7 cm, while girls with an „oestrogen free“ interval > 0(n = 13) had an adult height of 155.0 ± 4.2 cm with a gain of 5.7 ± 4.3 cm.The period of GH treatment and its „oestrogen free“ interval influence by 84% the height gain of girls with TSand are the main predictors of their adult height. Our first group of patients with TS was negatively affected bythe late onset of GH treatment and early onset of oestrogen substitution.

Key words:
Turner’s syndrome, adult height, growth hormone treatment, oestrogen substitution