Steroid hormones are derivatives of cholesterol. They include the progestogens, androgens, oestrogens, corticosteroids and mineralocorticoids. Their targets, and hence their physiological properties, are determined by the nature of the side chains.
Inherited defects can occur at a number of steps in the steroid biosynthetic pathway, but the most common is 21ɑ-hydroxylase deficiency (see: CAH below). Since corticosteroids and mineralocorticoids are hydroxylated at C21, the syndrome results in deficiencies of both these types of hormone. ACTH levels are raised because the normal feedback control is impaired, and enhanced synthesis of progestogens and overflow down the androgen pathway causes virilisation.
Deoxycorticosterone can act like aldosterone !
Syndrome of Adrenal androgen excess
excess production of dehydroepiandrosterone (DHEA), and androstenedione, which are converted to testosterone in extraglandular tissues.
Etiology includes CAH, rarely adrenal adenoma and adrenal carcinomas.
Two conditions to differentiate:
Precocious puberty is caused by premature activation of hypothalamus-pituitary-gonad (HPG) axis, while precocious pseudo-puberty is caused by a gonadotropin-independent process, typically an excess of sex steroids.
the most common adrenal disorder of infancy and childhood