Introduction:Treatment with aromatase inhibitors (AI) is a potential novel treatment in patients with congenital adrenal hyperplasia (CAH) and advanced bone age (BA), to increase near adult height (NAH). Not much is known about the efficacy of AI treatment in CAH and how AI treatment will influence the management of corticosteroid treatment. Case Presentation:At the age of 6 years and 3 months, a boy with salt-losing CAH presented with a BA 7 years in advance. Treatment with an AI (exemestane) was initiated to decelerate bone maturation. We continued the standard dosage of corticosteroid treatment. Precocious puberty was treated with 4 years of gonadotropin-releasing hormone agonist, while AI treatment was continued until attainment of NAH. His NAH 177.7 cm (-0.8 SDS) was considerably higher than his predicted adult height of 151.3 cm (-4.6 SDS) at the start of AI treatment. The higher serum androgen levels during AI treatment did not result in short adult stature. Discussion/Conclusion:This report shows that AI treatment can adequately decelerate bone maturation, causing predicted adult height to increase significantly in patients of CAH with accelerated bone maturation. We suggest continuing the same corticosteroid dosage during AI treatment and accepting higher serum androgen levels.

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VSNU Open Access deal
Hormone Research in Paediatrics
Department of Pediatrics

Goedegebuure, W.J. (Wesley J.), & Hokken-Koelega, A. (2019). Aromatase Inhibitor as Treatment for Severely Advanced Bone Age in Congenital Adrenal Hyperplasia: A Case Report. Hormone Research in Paediatrics. doi:10.1159/000501746