Randomised controlled trial of growth effect of hydrocortisone in congenital adrenal hyperplasia

Silva, Ivani Novato; Kater, Cláudio E.; Cunha, Cristiane de Freitas; Viana, Marcos Borato

doi:10.1136/adc.77.3.214

Cited by 56 publications

(31 citation statements)

References 25 publications

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“…The target range for serum 17-OHP level differs between review articles: 12 ng/mL [20] or 33.1 ng/mL [21]. The serum 17-OHP level also has a large diurnal variation and can change rapidly after administration of hydrocortisone [22][23][24]. Thus, a single measurement of serum 17-OHP concentration is not sufficient for evaluating the extent of disease control.…”

Section: Discussionmentioning

confidence: 96%

The prevalence of testicular adrenal rest tumors and associated factors in postpubertal patients with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency

et al. 2011

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Section: Discussionmentioning

confidence: 96%

The prevalence of testicular adrenal rest tumors and associated factors in postpubertal patients with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency

et al. 2011

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“…In a previous study, we have already reported in a group of CAH children that a short period of daily hydrocortisone doses of 15 mg/m² was associated to better growth velocity when compared to another short period using 25 mg/m² BSA, despite the limited inhibition of androgen production. At that time, we were concerned whether the high concentrations of androgens would cause advanced bone age, thus offsetting the benefit of the lower doses of GC upon FH (5). Others also observed a negative effect of a hydrocortisone dose of 17.64 ± 3.60 mg/m² BSA/ day on growth, preventing children from achieving their genetic potential (19).…”

Section: Discussionmentioning

confidence: 99%

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

Cordeiro

Silva

Goulart

et al. 2013

Arq Bras Endocrinol Metab

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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

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“…Firstly, hyperandrogenism during infancy due to undertreatment leads to growth velocity acceleration and a premature closure of epiphysial plates (31). Conversely, overtreated children suffer from growth retardation (32,33). One could wonder whether peak bone mass acquired during puberty might be affected by this therapy as total pubertal growth diminishes.…”

Section: Discussionmentioning

confidence: 99%

Impact of total cumulative glucocorticoid dose on bone mineral density in patients with 21-hydroxylase deficiency.

Chakhtoura

Bachelot²,

Samara-Boustani³

et al. 2008

European Journal of Endocrinology

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Objective: It remains controversial whether long-term glucocorticoids are charged of bone demineralization in patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. The aim of this study was to know whether cumulative glucocorticoid dose from the diagnosis in childhood to adulthood in patients with CAH had a negative impact on bone mineral density (BMD). Design: This was a retrospective study. Methods: Thirty-eight adult patients with classical and non-classical CAH were included. BMD was measured in the lumbar spine and femoral neck. Total cumulative glucocorticoid (TCG) and total average glucocorticoid (TAG) doses were calculated from pediatric and adult files. In the salt-wasting group, women were almost significantly endowed with a better BMD than men (PZ0.053). We found negative effects of TCG, TAG on lumbar (P!0.001, PZ0.002) and femoral T-scores (PZ0.006, P!0.001), predominantly during puberty. BMI was protective on BMD (PZ0.006). Conclusion: The TCG is an important factor especially during puberty for a bone demineralization in patients with 21-hydroxylase deficiency. The glucocorticoid treatment should be adapted particularly at this life period and preventive measures should be discussed in order to limit this effect.European Journal of Endocrinology 158 879-887

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Randomised controlled trial of growth effect of hydrocortisone in congenital adrenal hyperplasia

Cited by 56 publications

References 25 publications

The prevalence of testicular adrenal rest tumors and associated factors in postpubertal patients with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency

The prevalence of testicular adrenal rest tumors and associated factors in postpubertal patients with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency

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

Impact of total cumulative glucocorticoid dose on bone mineral density in patients with 21-hydroxylase deficiency.

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