Effects of short-course androgen therapy on the neurodevelopmental profile of infants and children with 49,XXXXY syndrome
Article first published online: 20 APR 2011
© 2011 The Author(s)/Acta Pædiatrica © 2011 Foundation Acta Pædiatrica
Volume 100, Issue 6, pages 861–865, June 2011
How to Cite
Samango-Sprouse, C. A., Gropman, A. L., Sadeghin, T., Kingery, M., Lutz-Armstrong, M. and Rogol, A. D. (2011), Effects of short-course androgen therapy on the neurodevelopmental profile of infants and children with 49,XXXXY syndrome. Acta Paediatrica, 100: 861–865. doi: 10.1111/j.1651-2227.2011.02252.x
- Issue published online: 27 APR 2011
- Article first published online: 20 APR 2011
- Accepted manuscript online: 1 MAR 2011 10:24AM EST
- Received 1 October 2010; revised 7 February 2011; accepted 25 February 2011.
- Klinefelter syndrome;
- Sex chromosome disorder variant forms;
Aim: The aim of this investigation was to ascertain whether an early course of androgen treatment (three injections testosterone enanthate, 25 mg) could have a positive impact on any domains of neurodevelopmental function in boys with 49,XXXXY.
Methods: A total of 22 boys with a karyotype of 49,XXXXY participated in a multidisciplinary assessment of neurocognition, speech and language, paediatric neurology and endocrinology evaluations. One group had received early androgen and another group did not receive any hormonal treatment prior to the evaluation. The mean age of treatment for Group 1 was 12 months with the mean age of first evaluation 74 months. The mean age of first evaluation for Group 2 was 87 months. Statistical analysis was completed to determine whether there was a positive treatment effect from androgen therapy.
Results: There was a significant positive treatment effect in speech and language domain, gestural communication and vocabulary development. No treatment effect was seen on nonverbal capacities.
Conclusion: Our findings revealed improved function in several areas of development which had been severely delayed in boys with 49,XXXXY. Continued research is underway to expand our understanding of the relationship of androgen, brain function and behavioural outcome in boys with 49,XXXXY.