Aromatase Inhibitor Increases the Height of Patients with Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency.

OBJECTIVE

Patients with 21-hydroxylase deficiency (21OHD) typically suffer from short stature due to early exposure to adrenal-derived androgen. The aim of this study was to investigate whether adding aromatase inhibitor (AI) to gonadotropin-releasing hormone (GnRH) analogue (GnRHa) and recombinant human growth hormone (rhGH) therapy would increase the height of patients with 21OHD.

METHODS

This retrospective study included 15 patients with 21OHD. The AI/GnRHa/rhGH group consisted of 9 patients, who were treated with AI for at least 12 months in addition to GnRHa/rhGH therapy. The other 6 patients, who received GnRHa/rhGH therapy only, were defined as the GnRHa/rhGH group.

RESULTS

Patients were 6.3±1.7 years old, and 7/15 of patients were male. Among them, 12 patients exhibited simple virilization type, and 3 patients were salt-wasting type. In the AI/GnRHa/rhGH group, patients were 6.6±2.0 years old when AI therapy was initiated. Their bone age was 5.9±2.2 years ahead of their chronological age. They received the AI letrizole for an average of 25.1 months (range, 12 to 37 months). In the GnRHa/rhGH group, the patients were 5.9±0.9 years old when they started GnRHa/rhGH therapy, and their bone age was 6.2±1.7 years ahead of their chronological age. Patients received GnRHa/rhGH therapy for an average of 24.5 months (range, 12 to 41 months). The predicted final height increased from 145.9±7.9 to 158.0±8.4 cm in the AI/GnRHa/rhGH group (P = .001, compared with the baseline) and from 141.7±2.7 to 150.7±4.7 cm in the GnRHa/rhGH group (P = .001, compared with the baseline). Bone age progression was 0.15±0.05 per year versus 0.44±0.13 per year in the two groups, respectively (P = .032).

CONCLUSION

Addition of letrizole to GnRHa/rhGH therapy significantly delays bone maturation and may increase the final height.