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Minerva Endocrinologica 2016 March;41(1):105-21

Copyright © 2016 EDIZIONI MINERVA MEDICA

lingua: Inglese

The current management of Turner Syndrome

Ruta KRIKSCIUNIENE ¹, Birute ZILAITIENE ², Rasa VERKAUSKIENE ² ✉

¹ Lithuanian University of Health sciences, Kaunas, Lithuania; ² Institute of Endocrinology, Lithuanian University of Health sciences, Kaunas, Lithuania

Turner Syndrome (TS) is a rare disease, with the incidence of 1 of 2500 life born females. Characteristic features are: growth retardation, gonadal dysgenesis and impairment, congenital and acquired cardiovascular disorders. New management possibilities in Turner Syndrome are coming along with the new scientific evidence on the pathogenesis of TS developmental, metabolic, cardiovascular and reproductive issues. Attitude to the growth retardation treatment and hormone replacement therapy is changing. The effectiveness of additional androgen doses for growth improvement and low estrogen doses in the early childhood for better puberty induction and metabolic outcomes has been demonstrated recently. There are some new concerns about pregnancy induced progression of cardiovascular pathology in TS. Inadequate follow-up despite strict and clear guidelines of TS patients is still an issue in the health care system in many countries. This rare disorder requires multidiscipline approach of experienced professionals. The aim of this review is to overview recent studies evaluating TS, to focus on the possibilities to avoid crucial outcomes of this disorder and to improve management and follow-up.