E70.0
BillableClassical phenylketonuria
E70.0 is the ICD-10-CM code for Classical phenylketonuria. It is a billable, specific code valid for reimbursement.
E00-E89 Endocrine, nutritional and metabolic diseases
What this means — in plain language
MedlinePlus · NIHPhenylketonuria (PKU) is a type of amino acid metabolism disorder . It is inherited. If you have it, your body can't process phenylalanine (Phe). Phe is an amino acid, a building block of proteins. It is in almost all foods. If your Phe level gets too high, it can damage your brain and cause severe intellectual disability. All babies born in U.S. hospitals must now have a screening test for PKU. This makes it easier…
Read more about Phenylketonuria at MedlinePlus ↗Source: U.S. National Library of Medicine (MedlinePlus). Informational only — not medical advice.
Medications indicated for this condition
FDA labeling- Sapropterin›
- Nitisinone4-Hydroxyphenyl-Pyruvate Dioxygenase Inhibitor [EPC]›
- TioproninReducing and Complexing Thiol [EPC]›
- Glycerol Phenylbutyrate›
- PenicillamineAntirheumatic Agent [EPC]›
- Phenylbutyrate›
- Cholic AcidBile Acid [EPC]›
- PegvaliasePhenylalanine Metabolizing Enzyme [EPC]›
- Sepiapterin›
Drugs whose FDA labeling lists this condition's category among its indications. Informational only — not a treatment recommendation or medical advice.
Conditions mapped to this code
MedlinePlus · NIHPlain-language health-topic references grounded in MedlinePlus (NIH/NLM).
Frequently asked questions
Source: CMS ICD-10-CM FY2026. For informational purposes only — not medical advice. See our medical disclaimer.
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