Abstract
Turner syndrome is a chromosomal abnormality in which there is complete or partial absence of the X chromosome. Turner syndrome effects 1 in every 2000 live births. Short stature is a cardinal feature of Turner Syndrome and the standard treatment is recombinant human growth hormone. When growth hormone is started at an early age a normal adult height can be achieved. With delayed diagnosis young women with Turner Syndrome may not reach a normal height. Adjuvant therapy with oxandrolone is used but there is no consensus on the optimal timing of treatment, the duration of treatment and the long term adverse effects of treatment. The objective of this review and meta-analysis is to examine the effect of oxandrolone on adult height in growth hormone treated Turner syndrome patients. Eligible trials were identified by a literature search using the terms: Turner syndrome, oxandrolone. The search was limited to English language randomized-controlled trials after 1980. Twenty-six articles were reviewed and four were included in the meta-analysis. A random effects model was used to calculate an effect size and confidence interval. The pooled effect size of 2.0759 (95 % CI 0.0988 to 4.0529) indicates that oxandrolone has a positive effect on adult height in Turner syndrome when combined with growth hormone therapy. In conclusion, the addition of oxandrolone to growth hormone therapy for treatment of short stature in Turner syndrome improves adult height. Further studies are warranted to investigate if there is a subset of Turner syndrome patients that would benefit most from growth hormone plus oxandrolone therapy, and to determine the optimal timing and duration of such therapy.
Highlights
Turner syndrome (TS) is defined as the complete or partial absence of the second X chromosome in a phenotypic female
Upon initial review of the abstracts 9 studies were excluded because the outcome being investigated was not adult height [15,16,17,18,19,20,21,22,23]
Full-text articles were obtained for the remaining 17 articles. Of these 13 additional articles were excluded because they either involved studies on the same patient cohort reported at different time points [24,25,26,27,28,29,30], or the subjects were not all treated with growth hormone (GH) [7, 11], or adult height data was not available [12, 13, 31], or there was no control group for GH alone or GH plus placebo [32]
Summary
Turner syndrome (TS) is defined as the complete or partial absence of the second X chromosome in a phenotypic female. The most common features of TS are short stature and gonadal failure. Turner syndrome occurs in 1/2000 live female births. Short stature is one of the cardinal findings in TS, due to haploinsufficiency of the SHOX gene (short stature homeobox-containing gene on the X-chromosome). Growth failure can be seen in early childhood and is usually obvious by 4 years of age. Mean adult height of TS patients without growth hormone (GH) therapy is about 21 cm shorter compared to healthy female adults [1, 2]. Since 1997, the standard treatment for short stature in TS patients has been recombinant human GH.
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