Final height in congenital adrenal hyperplasia: the dilemma of hypercortisolism versus hyperandrogenism

Abstract

Objective: The purpose of this study was to identify factors that might interfere with reaching the final height in patients with 21-hydroxylase deficiency (21-OHD). Subjects and methods: Thirty-one patients with classical 21-OHD who reached their FH in our Institution were evaluated in order to compare the Z score for final height (FHZ) with: (1) the target height, (2) the standard height for the population, and (3) the hydrocortisone treatment schedule. Results: The FHZ of -2.13 ± 1.11 had a significant negative correlation with the hydrocortisone doses used throughout the period of study. Patients who reached FH within the normal population range were those who received lower doses of hydrocortisone, as compared to those whose FH remained below -2 SDS. Conclusion: We conclude that careful treatment adjustments have a major influence on growth of children with CAH, and that the dose range for hydrocortisone replacement that does not lead to side effects is relatively narrow. The better height outcome was achieved in 21-OHD patients who received lower doses of hydrocortisone. Arq Bras Endocrinol Metab. 2013;57(2):126-31 Keywords Congenital adrenal hyperplasia; steroid 21-hydroxylase; corticosteroids; hydrocortisone; body height RESUMO Objetivo: O objetivo do estudo foi a identificação de fatores que podem interferir na aquisição de altura final de pacientes com a deficiência de 21-hidroxilase (21OHD). Sujeitos e métodos: A altura final (escore Z: FHZ) de 31 pacientes com a forma clássica da 21OHD, acompanhados em nossa instituição, foi comparada com: (1) a altura alvo, (2) o padrão de referência para a população, e (3) a dose de hidrocortisona durante o acompanhamento. Resultados: Observou-se correlação negativa significativa entre o FHZ de -2,13 ± 1,11 e as doses de hidrocortisona utilizadas durante o período de estudo. Os pacientes que atingiram altura final dentro do padrão de referência para a população usaram doses mais baixas de hidrocortisona quando comparados àqueles que permaneceram abaixo de -2 DP. Conclusão: O cuidado nos ajustes das doses durante o tratamento da 21OHD tem grande influência sobre o crescimento das crianças. A faixa de variação da dose de reposição da hidrocortisona que não causa efeitos colaterais é relativamente estreita. O melhor resultado estatural foi observado nos pacientes com 21OHD tratados com doses mais baixas de hidrocortisona. Arq Bras Endocrinol Metab. 2013;57(2):126-31 Descritores

Cardiovascular risk factors and increased carotid intima-media thickness in young patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

“…We found glucocorticoid replacement in the studied group was uniform and appropriate, following the current recommendations and consistent with previous findings of therapeutic efficacy in the same group of children (21,22). Follow-up was performed systematically at the same medical facility, and patients had no clinical signs of glucocorticoid excess or suppressed levels of serum steroids.…”

The validity of the Bayley-Pinneau method in predicting final adult height at the onset of puberty in patients with classic congenital adrenal hyperplasia

“…Height outcome is related to the dose of corticosteroid, with some authors describing a negative correlation between hydrocortisone doses and FAH [32]. Although we noted that FAH in males on steroid doses ≤ 15 mg/m 2 /day was significantly higher than in those receiving higher doses, we found no correlation between the actual dose of steroids and FAH, suggesting that growth is maintained within this cut-off dose.…”

Congenital adrenal hyperplasia

“…A meta-analysis of 35 studies of classic 21OH deficiency showed an average final height of 1.38 standard deviations below the population norm. 72 Whereas studies reveal an association between higher doses of hydrocortisone and shorter final height, 92,93 earlier rather than later diagnosis and treatment has been associated with improved height outcomes, emphasizing the importance of prevention of hyperandrogenism.…”