Bone mineral density and body composition in children with congenital adrenal hyperplasia

Halper, Alyssa; Sánchez, Benigno; Hodges, James S.; Kelly, Aaron S.; Dengel, Donald R.; Nathan, Brandon M.; Petryk, Anna; Sarafoglou, Kyriakie

doi:10.1111/cen.13580

Cited by 19 publications

(12 citation statements)

References 41 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Most studies on adults and children with CAH have reported an increased BMI (6, 89, 124, 125, 128, 129, 130, , 131, 132, 133, 134, 135, 136, 137, 138), but not all (4,73,94,139,140,141,142). The frequency of obesity did not differ between patients with NCAH and classic CAH in some studies (6,94,122,124,125,143), while others reported a lower BMI in patients with NCAH (89).…”

Section: Obesitymentioning

confidence: 93%

MANAGEMENT OF ENDOCRINE DISEASE: Diagnosis and management of the patient with non-classic CAH due to 21-hydroxylase deficiency

Nordenström

Falhammar

2019

European Journal of Endocrinology

124

129

View full text Add to dashboard Cite

Non-classic congenital adrenal hyperplasia (NCAH) is a relatively common disorder regardless of ethnicity, but most cases are never diagnosed, especially in males. A baseline 17-hydroxyprogesterone measurement may be used for screening, but 17-hydroxyprogesterone measurement after ACTH stimulation is the gold standard. We advocate a CYP21A2 mutation analysis to verify the diagnosis, for genetic counselling and for better prognostic and treatment guidance. Most patients are diagnosed in adolescence and adult life with hirsutism, acne, a PCOS-like picture and fertility issues. Many men with NCAH never seek medical attention and escape diagnosis. Although treatment is somewhat controversial, an early diagnosis and start of treatment may have positive implications on growth and be relevant for preventing and ameliorating the symptoms and consequences of androgen excess that develop over time, including fertility issues. Long-term treatment with glucocorticoids will improve the androgen symptoms but may result in long-term complications, such as obesity, insulin resistance, hypertension, osteoporosis and fractures. The glucocorticoid doses should be kept low. However, complications seen in NCAH, assumed to be caused by the glucocorticoid treatment, may also be associated with long-term androgen exposure. Oral contraceptive pills are a common treatment option for young females with NCAH. Regular clinical monitoring to improve the clinical outcome is recommended. It is important to acknowledge that glucocorticoid treatment will lead to secondary cortisol insufficiency and the need for stress dosing. Studies focusing on the specific difficulties patients with NCAH face, both those with a late clinical diagnosis and those with a neonatal diagnosis obtained by screening, are warranted.

show abstract

Section: Obesitymentioning

confidence: 93%

MANAGEMENT OF ENDOCRINE DISEASE: Diagnosis and management of the patient with non-classic CAH due to 21-hydroxylase deficiency

Nordenström

Falhammar

2019

European Journal of Endocrinology

124

129

View full text Add to dashboard Cite

show abstract

“…Whether the increased prevalence of obesity results from an increased lean body mass (as a possible consequence of excess androgens) or total/regional fat mass has been explored through bioimpedance analysis (11, 26, 27), dual x-ray absorptiometry (DEXA) (12, 15, 28–32), and computer tomography (CT) imaging (33) (Table 1). Several studies relying on DEXA documented that either males or females with classic CAH exhibit higher total fat mass (12, 15, 28–30) and reduced lean body mass (29) than controls.…”

Section: Traditional CV Risk Factorsmentioning

confidence: 99%

“…Only a few data are available regarding NC CAH (27, 28, 32), documenting no significant differences between classic and NC forms. Interestingly, one study reported a higher lean body mass in NC CAH in comparison to matched controls, suggesting an effect of longer exposure to excess androgens (28).…”

Section: Traditional CV Risk Factorsmentioning

confidence: 99%

Cardiovascular Health in Children and Adolescents With Congenital Adrenal Hyperplasia Due to 21-Hydroxilase Deficiency

Improda¹,

Barbieri²,

Ciccarelli³

et al. 2019

Front. Endocrinol.

View full text Add to dashboard Cite

Increasing evidence indicates that adults with Congenital Adrenal Hyperplasia (CAH) may have a cluster of cardiovascular (CV) risk factors. In addition, ongoing research has highlighted that children and adolescents with CAH are also prone to developing unfavorable metabolic changes, such as obesity, hypertension, insulin resistance, and increased intima-media thickness, which places them at a higher risk of developing CV disease in adulthood. Moreover, CAH adolescents may exhibit subclinical left ventricular diastolic dysfunction and impaired exercise performance, with possible negative consequences on their quality of life. The therapeutic management of patients with CAH remains a challenge and current treatment regimens do not always allow optimal biochemical control. Indeed, overexposure to glucocorticoids and mineralocorticoids, as well as to androgen excess, may contribute to the development of unfavorable metabolic and CV abnormalities. Long-term prospective studies on large cohorts of patients will help to clarify the pathophysiology of metabolic alterations associated with CAH. Meanwhile, further efforts should be made to optimize treatment and identify new therapeutic approaches to prevent metabolic derangement and improve long-term health outcomes of CAH patients.

show abstract

“…Moreover, a higher prevalence of obesity, impaired glucose tolerance, and dyslipidemia has been demonstrated in patients with Addison's disease [ 39 ], with increased use of antihypertensive drugs and lipid-lowering agents, as well as an increased risk for cardiovascular morbidity compared to general population [ 40 ]. Children with CAH, on supraphysiological glucocorticoid doses that cause an altered diurnal cortisol profile, have high rate of obesity, hypertension, growth suppression, and low bone density [ 41 – 43 ]. Increased evening cortisol levels have been shown to reduce glucose tolerance, insulin secretion, and insulin sensitivity in healthy young adults [ 44 ].…”

Section: Treatment Of Aimentioning

confidence: 99%

Pediatric Adrenal Insufficiency: Diagnosis, Management, and New Therapies

Bowden

Henry

2018

International Journal of Pediatrics

View full text Add to dashboard Cite

Adrenal insufficiency may result from a wide variety of congenital or acquired disorders of hypothalamus, pituitary, or adrenal cortex. Destruction or dysfunction of the adrenal cortex is the cause of primary adrenal insufficiency, while secondary adrenal insufficiency is a result of pituitary or hypothalamic disease. Timely diagnosis and clinical management of adrenal insufficiency are critical to prevent morbidity and mortality. This review summarizes the etiologies, presentation, and diagnosis of adrenal insufficiency utilizing different dynamic hormone testing and describes current treatment recommendations and new therapies.

show abstract

Bone mineral density and body composition in children with congenital adrenal hyperplasia

Abstract: Lower BMD was observed in CAH cases compared with controls although no differences in body composition were identified. Among CAH cases, increased chronic androgen exposure, as measured by bone age Z-scores, was associated with higher BMD but was not associated with VAT.

Cited by 19 publications

References 41 publications

MANAGEMENT OF ENDOCRINE DISEASE: Diagnosis and management of the patient with non-classic CAH due to 21-hydroxylase deficiency

MANAGEMENT OF ENDOCRINE DISEASE: Diagnosis and management of the patient with non-classic CAH due to 21-hydroxylase deficiency

Cardiovascular Health in Children and Adolescents With Congenital Adrenal Hyperplasia Due to 21-Hydroxilase Deficiency

Pediatric Adrenal Insufficiency: Diagnosis, Management, and New Therapies

Contact Info

Product

Resources

About