A review of the effects of therapy on growth and bone mineralization in children with congenital adrenal hyperplasia

Gallagher, Mary; Levine, Lenore S.; Oberfield, Sharon E.

doi:10.1016/j.ghir.2005.06.006

Cited by 13 publications

(11 citation statements)

References 63 publications

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“…Previous studies have attempted to address the potential impact of chronic glucocorticoid therapy in CAH patients on BMD but have been limited by the young age of study participants and have provided conflicting results [3,4,5,6,7,8,9,10,11,12,13]. Our study population has the advantage of including older subjects.…”

Section: Discussionmentioning

confidence: 99%

“…As such, there is a narrow therapeutic window through which the intended results can be achieved. In adulthood, the clinical management has received little attention, and may be complicated by iatrogenic Cushing’s syndrome with consequent obesity and reduced bone mineral density (BMD) [3], or by disturbed reproductive functions, especially in women with classic CAH [2, 4, 5]. In addition to hormonal causes, structural factors related to genital reconstructive surgery might reduce sexual activity and contribute to the infertility observed in 20–40% patients with classic CAH [4,5,6,7].…”

Section: Introductionmentioning

confidence: 99%

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Long-Term Outcome of Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Bachelot¹,

Plu‐Bureau²,

Thibaud³

et al. 2006

Horm Res Paediatr

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Aims: Conflicting results exist regarding bone mineral density (BMD), metabolism and reproductive function of adult patients with congenital adrenal hyperplasia (CAH). We evaluated the long-term outcome and the impact of chronic glucocorticoid replacement in these patients. Methods: Physical characteristics, serum hormone concentrations, BMD and metabolism were studied in 45 consecutive CAH adult patients. Results: Among the 36 women, only 14 (39%) had regular menses. Among the 27 women with classical CAH, the mean number of surgical reconstructions of virilized genitalia was 2.1 ± 0.2. Twenty of them (74%) were sexually active. Three men presented with testicular adrenal rest tumors. Twenty-five patients (55%) had decreased BMD at the femoral neck and/or at the lumbar spine. BMI was correlated with the BMD T-score at the femoral neck (p < 0.001) and at the lumbar spine (p < 0.01). Hydrocortisone dose was negatively correlated with the BMD T-score at the femoral neck (p = 0.04). Subjects with osteopenia had a significantly lower BMI and received higher hydrocortisone dose than those with normal BMD. Overweight was found in 21 patients (47%). There was a significantly positive correlation between HOMA and BMI (p < 0.001), and between HOMA and 17-OHP levels (p = 0.016). Conclusions: Adult patients with CAH treated with long-term glucocorticoids are at risk for decreased BMD, increased BMI, and disturbed reproductive function.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Long-Term Outcome of Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Bachelot¹,

Plu‐Bureau²,

Thibaud³

et al. 2006

Horm Res Paediatr

View full text Add to dashboard Cite

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“…Their participants had significantly lower lumbar and femoral neck BMD scores than controls. Studies conducted in subjects under glucocorticoid treatment have shown that long-term treatment may induce osteoporosis, with biochemical evidence of decreased bone turn-over and bone loss occurring principally within the first 6 months of treatment [21, 29]. It is also possible that 25-hydroxyvitamin D deficiency, observed in 44% of our patients, plays a role in the decreased BMD.…”

Section: Bone Mineral Densitymentioning

confidence: 93%

Classical Forms of Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency in Adults

Bachelot

Chakthoura²,

Rouxel³

et al. 2008

Horm Res Paediatr

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During childhood, the main aims of the medical treatment of congenital adrenal hyperplasia (CAH) secondary to 21-hydroxylase are to prevent salt loss and virilization and to attain normal stature and normal puberty. As such, there is a narrow therapeutic window through which the intended results can be achieved. In adulthood, the clinical management has received little attention, but recent studies have shown the relevance of long-term follow-up of these patients. The aims here are to review the multiple clinical, hormonal and metabolic abnormalities that could be found in adult CAH patients as such a decrease in bone mineral density, overweight and disturbed reproductive functions. In women with classic CAH, a low fertility rate is reported, and is probably the consequence of multiple factors including neuroendocrine and hormonal factors, feminizing surgery, and psychological factors. Men with CAH may present hypogonadism either through the effect of adrenal rests or from suppression of gonadotropins resulting in infertility. Therefore a multidisciplinary team with knowledge of CAH should carefully follow up these patients, from childhood through to adulthood, to avoid these complications and to ensure treatment compliance and tight control of the adrenal androgens.

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“…Estudos anteriores indicam que a baixa estatura final pode estar associada à altura média dos pais, à forma clínica da doença (perdedora de sal ou virilizante simples), à idade de início do tratamento, ao grau do controle hormonal, ao balanço de sal durante a infância e à dose de glicocorticoide durante a infância e a puberdade. 4 O tratamento da HAC consiste em administração de glicocorticoide na menor dose capaz de repor a necessidade diária do indivíduo e diminuir a hipersecreção de CRH-ACTH pelo eixo hipotálamo-hipófise. Esse procedimento tem como objetivo principal reduzir a hiperestimulação adrenal e, consequentemente, o hiperandrogenismo, visando a uma normalização do crescimento, virilização e fertilidade dos pacientes.…”

Section: Introductionunclassified

Novas perspectivas no tratamento da hiperplasia adrenal congênita

Faria¹,

Sampaio²,

Oliveira³

et al. 2010

cmbio

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A HAC é um distúrbio congênito, caracterizado pela deficiência na biossíntese do cortisol, associado ou não à deficiência de aldosterona e, consequentemente, à superprodução de andrógeno. Essa condição afeta igualmente homens e mulheres. No sexo feminino, acarreta virilização da genitália externa em graus variados. No sexo masculino, nenhuma anormalidade se apresenta fenotipicamente ao nascimento. Tradicionalmente, o tratamento dessa síndrome consiste na reposição diária de glicocorticoides e mineralocorticoides. Novas terapias têm sido propostas com intuito de se obterem resultados mais satisfatórios; dentre elas, existem: análogos do LHRH, GH, inibidores da aromatase, antiandrógenos e a adrenalectomia. Resultados preliminares apontam para uma melhora da altura final e do manejo clínico dos pacientes submetidos aos novos tratamentos. O objetivo deste estudo foi revisar a literatura acerca dessas novas modalidades terapêuticas, com o intuito de explorar o que há de mais novo na abordagem dessa síndrome.

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A review of the effects of therapy on growth and bone mineralization in children with congenital adrenal hyperplasia

Cited by 13 publications

References 63 publications

Long-Term Outcome of Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Long-Term Outcome of Patients with Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Classical Forms of Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency in Adults

Novas perspectivas no tratamento da hiperplasia adrenal congênita

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