21-hydroxylase deficiency secondary prevention

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]

Overview

Continued monitoring of hormone balance and careful readjustment of glucocorticoid dose is helpful in controlling fertility and preventing adrenal crisis in patient with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Secondary Prevention

Preventing hyperandrogenism and optimizing fertility

Continued monitoring of hormone balance and careful readjustment of glucocorticoid dose is helpful in controlling fertility, but as a group, women with congenital adrenal hyperplasia have a lower fertility rate.

Preventing adrenal crisis

• To prevent adrenal crisis, all persons taking glucocorticoids replacement are taught to increase their doses in the event of illness, surgery, severe injury, or severe exhaustion.
• Vomiting warrants an injection (within hours) of hydrocortisone (e.g., SoluCortef) or other glucocorticoid. This recommendation applies to both children and adults.
• Because young children are more susceptible to illnesses and infections than adults, pediatric endocrinologists usually teach parents how to give hydrocortisone injections.