Congenital_Adrenal_Hyperplasia_Flashcards
Why might affected females with congenital adrenal hyperplasia need surgery?
Affected females sometimes need corrective surgery for external genitalia. They have a uterus and ovaries so they are reared as girls.
When is definitive surgical correction usually performed for congenital adrenal hyperplasia?
Definitive surgical correction is usually delayed until puberty.
What is the management for an acute (salt losing) crisis in congenital adrenal hyperplasia?
IV saline (0.9% sodium chloride), IV hydrocortisone 200mg, IV dextrose.
What is the long-term management for congenital adrenal hyperplasia?
Life-long glucocorticoids (hydrocortisone) to suppress ACTH levels (and hence testosterone).
What additional medication is needed if there is salt loss in congenital adrenal hyperplasia?
Mineralocorticoids (fludrocortisone) if there is salt loss.
What parameters should be monitored in congenital adrenal hyperplasia?
Monitoring growth, skeletal maturity, plasma androgens and 17α-hydroxyprogesterone levels.
When is additional hormone replacement needed in congenital adrenal hyperplasia?
Additional hormone replacement at times of illness or surgery.
