E25.0
BillableCongenital adrenogenital disorders associated with enzyme deficiency
HCC Category Mapping
What This Code Means
A group of inherited disorders where the adrenal glands produce abnormal hormone levels due to missing or defective enzymes, often causing virilization or salt-wasting problems.
Coding Tips
- •Specify the type of enzyme deficiency (21-hydroxylase, 11-beta-hydroxylase, etc.) if documented
- •Note whether the presentation is salt-wasting, simple virilizing, or non-classic form
Clinical Significance
Congenital adrenogenital disorders associated with enzyme deficiency, primarily congenital adrenal hyperplasia, are autosomal recessive conditions causing impaired cortisol synthesis with compensatory adrenocorticotropic hormone elevation and adrenal androgen excess. The most common form, 21-hydroxylase deficiency, can present with life-threatening salt-wasting crisis in neonates or virilization in females.
Documentation Requirements
- ✓Document the specific enzyme deficiency (21-hydroxylase, 11-beta-hydroxylase, 17-alpha-hydroxylase), clinical form (classic salt-wasting, simple virilizing, or non-classic/late-onset), 17-hydroxyprogesterone levels, adrenocorticotropic hormone stimulation test results, and hormone replacement regimen.