Congenital adrenogenital disorders assoc w enzyme deficiency

This code represents a group of inherited conditions affecting the adrenal glands, specifically those where a defect in an enzyme involved in steroid hormone synthesis leads to an overproduction of androgens. The most common form is congenital adrenal hyperplasia (CAH), which can manifest with ambiguous genitalia in females, precocious puberty, or salt-wasting crises.

Use this code for patients diagnosed with congenital adrenogenital disorders directly resulting from an enzyme deficiency, such as 21-hydroxylase deficiency, 11-beta-hydroxylase deficiency, or 3-beta-hydroxysteroid dehydrogenase deficiency. Documentation should clearly state the congenital nature of the disorder and identify an underlying enzyme defect.

• Always specify the exact type of enzyme deficiency if known, using more specific codes when available (e.g., E25.00-E25.09).
• Do not use this code for acquired adrenal disorders or those not linked to an enzyme deficiency.
• Consider additional codes for associated manifestations or complications, such as ambiguous genitalia (Q56.x) or adrenal crisis (E27.2).