E83.09
BillableOther disorders of copper metabolism
HCC Category Mapping
V28HCC 50 — Glycogen/Amino-Acid/Other Metabolic Disorders
0.289RxHCCHCC 42 — Endocrine Disorders and Metabolic Conditions
0.000What This Code Means
Rare inherited or acquired conditions affecting copper metabolism in the body, other than Wilson's disease.
Coding Tips
- •Document the specific type of copper metabolism disorder when possible to support medical necessity
- •Distinguish from Wilson's disease and unspecified copper metabolism disorders
Clinical Significance
Other disorders of copper metabolism captures copper metabolism conditions other than Wilson disease, including Menkes disease (kinky hair syndrome), occipital horn syndrome, and copper-associated conditions not elsewhere classified. Menkes disease, caused by ATP7A mutations, results in copper deficiency in tissues and is a severe X-linked neurodegenerative disorder.
Documentation Requirements
- ✓Specific copper metabolism disorder documented (e.g., Menkes disease, occipital horn syndrome)
- ✓Confirmatory testing: serum copper and ceruloplasmin (both LOW in Menkes), genetic testing (ATP7A mutations)
- ✓Clinical manifestations: kinky hair, seizures, failure to thrive, connective tissue abnormalities (Menkes)
- ✓Differentiation from Wilson disease (copper excess) vs. Menkes (copper deficiency in tissues)
- ✓Treatment plan: subcutaneous copper histidine injections (Menkes), supportive care
Commonly Confused Codes
E83.01 — Wilson's disease: copper EXCESS disorder; Menkes is copper DEFICIENCY in tissuesE83.00 — Disorder of copper metabolism, unspecified: use only when the specific disorder is unknownE61.0 — Copper deficiency: nutritional copper deficiency, not genetic copper metabolism disorderQ84.1 — Congenital morphological disturbances of hair, not elsewhere classified: kinky hair in Menkes requires the metabolic code
Code Hierarchy
└E83Disorders of mineral metabolism└E83.0Disorders of copper metabolism└E83.09Other disorders of copper metabolism
└E83.09Other disorders of copper metabolism