Introduction: Testotoxicosis and other causes of precocious puberty can result in compromised final adult height and various treatments have been used in an attempt to address this. We report final height data in children with testotoxicosis who were treated with a variety of regimens and who have attained/are predicted to attain, a final height in excess of the mid-parental target.
Patients and methods: Growth data from four patients with activating mutations of the LH receptor (4 Met398Thr; 1 Ala572Val) were analysed. An additional boy carried the Met398Thr mutation but didnt develop precocious puberty. Final height data in those with precocious puberty (n=3) as well as final and predicted final height data (n=4) were compared with UK population norms and mid-parental height.
Results: The mean age at diagnosis was 5.5 years (range 4.37.2 years). Three children received treatment that included an aromatase inhibitor (testolactone or letrozole) and one child received the anti-androgen cyproterone acetate with GH. Other agents used included spironolactone and ketoconazole. One boy developed gonadotrophin responses typical of true precocious puberty and was also treated with a GnRH analogue. Mean final height (n=3) was 182.3 cm (+0.68 S.D.), a mean of 7.7 cm (range 510 cm) above the mid-parental target. Mean final height/predicted final height (n=4) was 184.5 cm (+1 S.D.), 9 cm (range 513 cm) above the mid-parental target. All patients achieved final heights above their mid-parental centile but aromatase inhibitors were more successful at normalising height velocity. There were no significant adverse events and adult patients have normal testicular ultrasounds with afternoon testosterone values of 12.220.2 nmol/l
Conclusions: Patients with testotoxicosis respond favourably to a treatment regimen that includes aromatase inhibitors and anti-androgens. Only aromatase inhibition was associated with normalisation of growth velocity.
09 - 11 Nov 2011
British Society for Paediatric Endocrinology and Diabetes