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Pathology- Congenital Adrenal Hyperplasias
Autosomal recessive defect in enzymes related to the production of cortical steroids.
Bilateral nodular hyperplasia of the adrenal gland with lipid-depleted cortical cells.
21-beta-hydroxylase deficiency disrupts the production of aldosterone and cortisol, leading to the redirection of precursor molecules to produce sex hormones.
11-beta-hydroxylase deficiency disrupts the production of aldosterone and cortisol, leading to the redirection of precursor molecules to produce sex hormones.
17 alpha-Hydroxylase deficiency disrupts the production of cortisol and sex hormones, leading to the redirection of precursor molecules to produce aldosterone.
21-beta Hydroxylase deficiency presents with masculinization, hypotension, hyperkalemia, and hyponatremia, which can result in hypovolemia due to salt wasting.
11-beta Hydroxylase deficiency presents with masculinization, premature puberty, hypertension (caused by the weak mineralocorticoid activity of 11 beta deoxycorticosterone), and no salt wasting.
17-alpha Hydroxylase deficiency presents with hypertension, hypokalemia, lack of sexual development, and low cortisol levels.
Administering hormones to correct deficiencies and providing treatment for symptoms.
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