SFEBES2018 Poster Presentations Reproduction (23 abstracts)
A prospective study of testicular development and function in boys with Duchenne Muscular Dystrophy
M Denker 1, , S Joseph 1, , M DiMarco 3 , J Dunne 3 , I Horrocks 3 , SF Ahmed 1 & SC Wong 1
1Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK; 2School of Medicine, University of Glasgow, Glasgow, UK; 3Paediatric Neurosciences Research Group, Royal Hospital for Children, Glasgow, UK.
Introduction: There is a need to understand testicular development in adolescents with Duchenne Muscular Dystrophy (DMD).
Objective: To evaluate testicular development and function in DMD over 12 months.
Methods: All data are presented as median(range). 23 boys aged 12.4 years (10.0, 16.8) with a bone age delay of 0.9 years (−2.4, 7.1) had physical and biochemical assessment of puberty at Month 0(M0) and Month 12(M12) and divided into groups depending on pubertal progress: A-remained prepubertal (n,11); B-treated with testosterone (n,5); C- spontaneous virilisation (n,7).
Results: Testes Z-scores adjusted for bone age at M0 and M12 were −1.3 (−3.6, 1.5), and −2.5 (−3.6, 1.1)(p=0.08).
Group A: Aged 11.2 years (10.0, 12.4), all were on glucocorticoids by M12. 8/11 (73%) and 9/11 (82%) had undetectable LH at M0 and M12; 9/11 (82%) had undetectable testosterone at M0 and M12. Median inhibin B Z-scores at M0 and M12 were −0.4 (−3.2, 1.3) and −1.7 (−3.1, 0.7) (P=0.02).
Group B: Aged 15.9 years (13.6, 16.8), all were on glucocorticoids and although they virilised with testosterone therapy, testes remained less than 4 ml in all 5 (100%). 3/11 (60%) had undetectable LH at M0 and M12. Median inhibin B Z-scores at M0 and M12 were −1.1 (−2.7, −0.0) and −2.1 (−2.4, 0.0) (P=0.89).
Group C: Aged 15.1 years (10.9, 16.6), 4 (57%) were on glucocorticoids by M12. Median LH at M0 and M12 was 1.7 U/l (0.6, 8.2) and 1.7 U/l (0.3, 5.6) (P=0.89). Median testosterone at M0 and M12 was 4.8 nmol/l (0.7, 16.2) and 9.1 nmol/L (1.2, 13.1)(P=0.04). Median inhibin B Z-scores at M0 and M12 were −2.2 (−2.8, −1.3) and −2.1 (−2.9, −1.4) (P=0.27).
Conclusion: Boys with DMD have relatively small testes. This may be associated with Leydig and Sertoli cell dysfunction as a result of functional hypogonadotrophic hypogonadism.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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