Issue: August 2013
June 28, 2013
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Quality of life, steroid use assessed in adults with CAH

Issue: August 2013
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Adults with congenital adrenal hyperplasia had negative perceptions of their quality of life as it relates to their weight and type or dose of medication for the condition, research published in the European Journal of Endocrinology indicates.

Thang S. Han, MRCP, PhD, of the department of endocrinology at the University College London Hospitals, and colleagues conducted a cross-sectional analysis of adults (n=151) with congenital adrenal hyperplasia (CAH) aged 18 to 69 years.

According to data, quality-of-life (QOL) scores were associated with the type of glucocorticoid treatment the patient was using for vitality (P=.002) and mental health (P=.011). Greater z scores were related to improved QOL in patients on hydrocortisone monotherapy (P<.05), according to data.

However, QOL was not associated with prednisolone dose equivalent (PreDEq) or mutation severity, researchers wrote.

They identified three principal components (ie, disease control, blood pressure and mutations, adiposity and insulin resistance) accounting for 61% of the total variance among observed variables. Adiposity and insulin resistance were found to impair physical function, bodily pain, general health, the physical component summary score (P<.001) and vitality (P=.002), according to data.
“In this study, we found quality of life to be worse in adults taking stronger forms of steroids or combinations of steroids, and in those with markers of a worse metabolic profile such as weight gain around the middle and insulin resistance. Perhaps this is because they have a more severe form of the disease and so feel worse, but perhaps the stronger medication impacts on quality of life,” Richard Ross, MD, FRCP, chair of the Society for Endocrinology’s Congenital Adrenal Hyperplasia Adult Study Executive (CaHASE), said in a press release.

In a previous cross-sectional study measuring health outcomes of adult patients with CAH (n=196), Han and colleagues found that dexamethasone was linked to lower androgens but led to greater insulin resistance. Additionally, an increased dose of glucocorticoids was associated with increased BP, poor disease control and mutation severity.

According to data, age and sex adjustments for partial correlation analyses demonstrated PreDEq was poorly related with BP and androstenedione. However, increased PreDEq was associated with mutation severity (P<.01).

In the current study, researchers identified three principal components accounting for 62% of the total variance among observed variables. Although the second component demonstrated disease control, the third component showed that BP and mutations were positively linked to PreDEq (P<.001).

“We would recommend that doctors think before increasing the steroid dose, as this may only increase side effects. We also suggest that dexamethasone is only used in those patients that require strong androgen suppression. Finally, we would recommend that all adults with CAH are referred to a specialist endocrine center for specialist care,” Ross said.

For more information:

Han TS. Clin Endocrinol (Oxf). 2013;78:197-203.

Han TS. Eur J Endocrinol. 2013;168:887-893.

Disclosure: Ross is founding director and equity holder in Diurnal Ltd. that is developing new hydrocortisone preparations for patients with CAH. All other researchers report no relevant financial disclosures.