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• W2957507977 abstract "Johanson AJ, Brasel JA, Blizzard RM. J Pediatr 1969;75:1015-21. Short stature, the most common feature in Turner syndrome, is caused by haploinsufficiency of the SHOX gene located on the distal short arm of the X chromosome. In this retrospective study, the authors describe a cohort of 26 female patients, mean age 13.3 years, with gonadal dysgenesis (sex chromosomes consistent with Turner syndrome) who received fluoxymesterone, a nonaromatizable androgen, for 7-32 months. This group demonstrated a significant increase in growth rate and adult height in comparison with 21 patients receiving estrogen. Interestingly, at the time growth hormone was not believed to be effective at increasing height in girls with Turner syndrome. Fifty years later, what is different? Growth hormone is now the mainstay of treatment for short stature, with early initiation around 4-6 years of age allowing for an increased adult height into the lower normal range for adult women.1Gravholt C.H. Andersen N.H. Conway G.S. Dekkers O.M. Geffner M.E. Klein K.O. et al.Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting.Eur J Endocrinol. 2017; 177: G1-G70PubMed Google Scholar Oxandrolone, another nonaromatizable androgen, has fewer virilizing side effects than fluoxymesterone2Urban M.D. Lee P.A. Dorst J.P. Plotnick L.P. Migeon C.J. Oxandrolone therapy in patients with Turner syndrome.J Pediatr. 1979; 94: 823-827Abstract Full Text PDF PubMed Scopus (49) Google Scholar and is currently used as adjunctive therapy with growth hormone for increasing adult height, particularly in cases of delayed diagnosis with severe short stature.3Sheanon N.M. Backeljauw P.F. Effect of oxandrolone therapy on adult height in Turner syndrome patients treated with growth hormone: a meta-analysis.Int J Pediatr Endocrinol. 2015; 2015: 18Crossref PubMed Google Scholar It is important to realize that quality of life in adults with Turner syndrome is unaffected by previous growth hormone treatment, but hearing impairment and delayed pubertal induction after age 15 years are associated with lower quality of life scores.4Carel J.-C. Ecosse E. Bastie-Sigeac I. Cabrol S. Tauber Mt Léger J. et al.Quality of life determinants in young women with Turner's syndrome after growth hormone treatment: results of the StaTur population-based cohort study.J Clin Endocrinol Metab. 2005; 90: 1992-1997Crossref PubMed Scopus (92) Google Scholar Promoting early growth permits starting estrogen treatment at a similar age to peers. Therefore, although attention to growth is important, it is essential to consider other aspects of care for these girls." @default.
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• W2957507977 title "50 Years Ago in" @default.
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• W2957507977 doi "https://doi.org/10.1016/j.jpeds.2019.05.073" @default.
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