Long-Term Corticosteroid Replacement and Bone Mineral Density in Adult Women with Classical Congenital Adrenal Hyperplasia

King, Jeremy A.; Wisniewski, Amy B.; Bankowski, Brandon; Carson, Kathryn A.; Zacur, Howard A.; Migeon, Claude J.

doi:10.1210/jc.2005-0745

Cited by 94 publications

(87 citation statements)

References 16 publications

(25 reference statements)

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“…Ohmori et al did not find a direct correlation between BMD, cortisol, and androgens; however, they suggested a possible role of DHEAS increase in counterbalancing the negative bone effects of endogenous hypercortisolism (33). Greater bone loss was also found in women on replacement therapy for 21-hydroxylase deficit and lower androgen levels (34), supporting the hypothesis on the importance of cortisol-to-androgen ratio in BMD maintenance (8,32,35). Finally, a strong correlation was observed between DHEAS and lumbar/femoral BMD values in healthy women (36).…”

Section: Discussionmentioning

confidence: 97%

Effects of sex steroids on bone in women with subclinical or overt endogenous hypercortisolism

et al. 2007

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Objective: Glucocorticoid-induced osteoporosis is the most frequent cause of secondary osteoporosis. Nevertheless, limited data are available on bone status in patients with endogenous cortisol excess. This study is aimed at investigating the role of sex steroids and severity of hypercortisolism on bone mineral density (BMD) and prevalence of vertebral fractures in female patients. Design: Cross-sectional, case-control study. Patients: Seventy-one consecutive women were enrolled: 36 with overt hypercortisolism (26 with ACTH-secreting pituitary adenoma and 10 with cortisol-secreting adrenal tumor) and 35 with subclinical hypercortisolism due to adrenal incidentalomas. They were compared with 71 matched controls. Methods: At diagnosis, we measured serum cortisol, FSH, LH, estradiol, testosterone, androstenedione and DHEAS, and urinary cortisol excretion. BMD was determined by dual energy X-ray absorptiometry at the lumbar spine and femoral neck. Vertebral fractures were investigated by a semiquantitative scoring method. Results: Between women with overt and subclinical hypercortisolism BMD values and prevalence of any vertebral (69 vs 57%, PZ0.56), clinical (28 vs 11.4%, PZ0.22), and multiple vertebral fractures (36 vs 31%, PZ0.92) did not differ. Among patients with subclinical hypercortisolism, amenorrhoic women had a lower BMD (PZ0.035) and more frequent vertebral fractures (80 vs 40%; PZ0.043) when compared with the eumenorrhoic ones. Among women with overt hypercortisolism, there was no difference in lumbar BMD (PZ0.37) and prevalence of fractures (81 vs 60%; PZ0.26) between those amenorrhoic and eumenorrhoic. By logistic regression analysis, lumbar spine BMD values and cortisol-to-DHEAS ratio were the best predictors of vertebral fractures (P!0.01). Conclusions: Vertebral fractures are very common in women with endogenous cortisol excess, regardless of its severity. The deleterious effects of hypercortisolism on the spine may be partly counterbalanced by DHEAS increase at any degree of cortisol excess, and by preserved menstrual cycles in women with subclinical but not in those with overt hypercortisolism.

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Section: Discussionmentioning

confidence: 97%

Effects of sex steroids on bone in women with subclinical or overt endogenous hypercortisolism

et al. 2007

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“…Doses of the various glucocorticoids were converted to growth-retarding cortisol equivalents (1 mg of dexamethasoneZ16 mg of prednisoneZ80 mg of hydrocortisone) (15,16,17). Each year, we summed every daily dose of treatment and divided the total by the corresponding body area to obtain the annual cumulative doses of hydrocortisone (mg/m 2 per year).…”

Section: General Proceduresmentioning

confidence: 99%

Determining clinical and biological indicators for health outcomes in adult patients with childhood onset of congenital adrenal hyperplasia

Bachelot¹,

Golmard²,

Jérôme³

et al. 2015

European Journal of Endocrinology

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Aim: Adverse outcomes in adult congenital adrenal hyperplasia (CAH) patients are frequent. The determinants of them have not yet been established. Objective: To establish the prevalence of adverse outcomes and to find determining factors for each of them. Design, patients, and methods: Cross-sectional monocentric study of 104 patients with childhood onset of CAH (71 women, 33 men). Analysis established first the determinants of clinical, hormonal, genetic variables and second a composite criterion for some of the outcomes and determinants. Results: BMI was above 25 kg/m 2 in 44% of the cohort, adrenal hyperplasia and/or nodules were present in 45% of the patients, and irregular menstrual cycles and hyperandrogenism were found in 50 and 35% of the women respectively. In univariate analysis, the determinants of these outcomes were all linked to disease control, especially 17-hydroxyprogesterone (17OHP) and androstenedione concentrations. Low weight was a determinant of abnormal bone mineral density (BMD) (60% of the cohort). Multivariate analysis confirmed these data. A classic form (CF) of CAH was a determinant of testicular adrenal rest tumors (TARTs) (36% of the men). Total cumulative glucocorticoid dose was a determinant of BMI and TART, whereas fludrocortisone dose was a determinant of TART (PZ0.03). In men, the composite criterion was associated with androstenedione concentration and CF. In women, the composite criterion was associated with total testosterone concentration. Conclusion:The present study confirms the high prevalence of adverse outcomes in CAH patients. These are, most often, related to disease control. The impaired health status of adults with CAH could therefore be improved through the modification of treatment.

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“…Some studies reported reduced BMD in all PAI patients (2,3), some only in postmenopausal women (4,5) or only in men (6,7), whereas other studies found no significant differences in BMD in either gender (8,9,10). In CAH patients some studies reported no significant differences in BMD between patients with CAH and controls (10,11,12,13,14,15,16), whereas others found reduced BMD in all or some subpopulations of CAH patients (17,18,19,20,21,22,23,24). Until now all studies in this area have had a crosssectional design.…”

Section: Introductionmentioning

confidence: 98%

Reduction in daily hydrocortisone dose improves bone health in primary adrenal insufficiency

Schulz

Frey

Cooper

et al. 2016

European Journal of Endocrinology

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Objective: Individuals with primary adrenal insufficiency (PAI) or congenital adrenal hyperplasia (CAH) receive life-long glucocorticoid (GC) replacement therapy. Current daily GC doses are still higher than the reported adrenal cortisol production rate. This GC excess could result in long-term morbidities such as osteoporosis. No prospective trials have investigated the long-term effect of GC dose changes in PAI and CAH patients. Methods: This is a prospective and longitudinal study including 57 subjects with PAI (42 women) and 33 with CAH (21 women). Bone mineral density (BMD) was measured by dual energy X-ray absorptiometry at baseline and after 2 years. Subjects were divided into three groups (similar baseline characteristics) depending on changes in daily hydrocortisone equivalent dose (group 1: unchanged 25.2G8.2 mg (meanGS.D., nZ50); group 2: increased 18.7G10.3 to 25.9G12.0 mg (nZ13); group 3: decreased 30.8G8.5 to 21.4G7.2 mg (nZ27)). Results: Subjects in group 1 showed normal lumbar and femoral Z-scores which were unchanged over time. Group 2 subjects showed a significant decrease in femoral neck Z-scores over time (K0.15G1.1 to K0.37G1.0 (P!0.05)), whereas group 3 subjects showed a significant increase in lumbar spine and hip Z-scores (L1-L4: K0.93G1.2 to -0.65G1.5 (P!0.05); total hip: K0.40G1.0 to K0.28G1.0 (P!0.05)). No changes in BMI over time were seen within any group. Reduction in GC dose did not increase the risk of adrenal crisis. Conclusion: This study demonstrates for the first time that cautious reduction in hydrocortisone equivalent doses leads to increases in BMD, whereas dose increments reduced BMD. These data emphasize the need for the lowest possible GC replacement dose in AI patients to maintain health and avoid long-term adverse effects.

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Long-Term Corticosteroid Replacement and Bone Mineral Density in Adult Women with Classical Congenital Adrenal Hyperplasia

Cited by 94 publications

References 16 publications

Effects of sex steroids on bone in women with subclinical or overt endogenous hypercortisolism

Effects of sex steroids on bone in women with subclinical or overt endogenous hypercortisolism

Determining clinical and biological indicators for health outcomes in adult patients with childhood onset of congenital adrenal hyperplasia

Reduction in daily hydrocortisone dose improves bone health in primary adrenal insufficiency

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