Challenges in treatment of patients with non-classic congenital adrenal hyperplasia PMC

Challenges in treatment of patients with non-classic congenital adrenal hyperplasia PMC

Whether these treated women received glucocorticoid treatment before or during pregnancy did not influence the miscarriage rate. Moreover, also Eyal etal. (165) did not observe a difference in miscarriage rate in treated versus untreated women. On the contrary, studies by Feldman et al. (166) and Bidet et al. (167) reported normalized miscarriage rates in NCCAH women with glucocorticoid treatment. The other two studies (166, 173) did not report mutation analyses of the NCCAH women. If glucocorticoid treatment does not lead to conception, ovulation induction with clomiphene citrate might be successful (166, 167).

  • Chuck, Sarah, and Casey all face professional conflicts as they grow to respect each other.
  • During puberty, testicular androgen production greatly overshoots the adrenal androgens production in boys and enduring suppression of androgens seems unfavorable.
  • Bone age can be used as an additional clinical parameter besides monitoring growth velocity to evaluate the effect of adrenal androgens on growth (54, 68).
  • All content published within Cureus is intended only for educational, research and reference purposes.
  • Similarly, in 2006, 336 people in China were poisoned after eating pork containing the drug.
  • Non-hormonal causes should be considered, and adequate diagnostic measures taken to rule out malignancy or pregnancy in the event of breakthrough bleeding, as in the case of any abnormal vaginal bleeding.

Women who us e oral contraceptives should be strongly advised not to smoke. Another important risk factor for cardiovascular diseases is hypercholesterolemia. Hypercholesterolemia has been observed in 59% of the NCCAH females (92). This incidence was even higher compared to the classic CAH males and females (36% and 48% respectively) (92). Krysiak et al. (139) reported that atorvastatin, a statin that reduced the levels of cholesterol and androgens, decreased the cardiometabolic risk in untreated NCCAH women. If this decrease in risk also leads to fewer cardiovascular incidents in these patients, needs to be further elucidated.

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A small proportion of women will have persistent hypertriglyceridemia while on the pill. As discussed earlier (see Myocardial Infarction and Dose-Related Risk of Vascular Disease from Oral Contraceptives), changes in serum triglycerides and lipoprotein levels have been reported in oral contraceptive users. Oral contraceptive combinations containing ethinyl estradiol may inhibit the metabolism of other compounds. Increased plasma concentrations of cyclosporine, prednisolone, and theophylline have been reported with concomitant administration of oral contraceptives. In addition, oral contraceptives may induce the conjugation of other compounds.


For maximum effect from the agent, it can be used in combination with ketotifen, which prevents premature adaptation to the effects of the main substance. As the clinical picture of 11OHD patients is variable and glucocorticoid treatment has both short- and long-term side effects, it should be considered per patient whether administration of glucocorticoids is beneficial. An ACTH stimulation test, in which cortisol and other adrenal steroids are measured after ACTH administration, is currently the gold standard to assess cortisol production in a clinical setting. A suboptimal cortisol response is reported in about 21-60% of NCCAH patients (6–9, 26). Nandagopal et al. (7) and Stoupa et al. (8) reported no significant correlation between the cortisol response to synthetic ACTH and the genotype of the patients. NCCAH patients with 21OHD have impaired 21α-hydroxylase activity (CYP21A2) and NCCAH patients with 11OHD have impaired 11β-hydroxylase activity (CYP11B1).

In conclusion, NCCAH women may suffer from signs of hyperandrogenism such as hirsutism, acne vulgaris, or androgenetic alopecia. Glucocorticoid treatment is not recommended to reduce signs of hyperandrogenism in these patients. An individualized approach with topical and/or hormonal treatment is necessary for treating signs of hyperandrogenism, and consultation with a dermatologist can be of added value for these patients. Other off-label treatment options for hyperandrogenism are competitive antagonists of the androgen receptor, like flutamide, and 5α-reductase inhibitors, like finasteride and dutasteride (146). However, these should not be used in childhood and in women who try to conceive or are currently pregnant. These antiandrogens can have side effects like decreased libido, headache, dizziness, and nausea (155–157).

Therefore, yearly follow-up of growth and bone age is recommended. Glucocorticoid treatment should only be initiated in children with NCCAH after careful counseling of patients and parents and should be discontinued after reaching the final height. There may be times when you may not menstruate regularly after you have completed taking a cycle of pills. If you have taken your pills regularly and miss one menstrual period, continue taking your pills for the next cycle but be sure to inform your health care provider before doing so.

The effects of adrenal androgens on (increased) bone maturation become already relevant in the second year of life (48, 49). On the long term, these androgens lead to premature epiphyseal closure resulting in reduced final height. We do not recommend brands of cod liver oil that have low levels of vitamin A and/or low levels of vitamin D. Glucocorticoid treatment will restore the negative feedback on the pituitary gland similar to treatment in patients with 21OHD. This might be useful in patients with classic 21OHD but potentially harmful in classic 11OHD patients.

A new pack of tablets should be started no later than the eighth day after the last green tablet was taken. Besides the use of glucocorticoids, other risk factors like smoking, low intake of calcium and vitamin D, and low physical activity increase the risk of osteoporotic fractures (121). It is essential to inform NCCAH patients about these risk factors to preserve their bone mineral density and reduce the risk of osteoporotic fractures later in life.

In addition, oral contraceptives lower adrenal androgen production by inhibiting the enzymatic activities of 17-hydroxylase and 17,20-lyase which are necessary for androgen production (142). Furthermore, estrogens in oral contraceptives increase sex hormone binding globulin (SHBG) production and thereby reduce free testosterone levels (143, 144). Lastly, progestogens in oral contraceptives inhibit the function of 5α-reductase resulting in less conversion of testosterone into the more potent androgen dihydrotestosterone (145). Although oral contraceptives can contain different progestogens with either androgenic or antiandrogenic features, the net effect of combined oral contraceptives is always antiandrogenic (146).

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