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E88.01

E88.01Alpha-1-antitrypsin deficiency

Billable / specific codeICD-10-CM

E88.01 is a billable, specific ICD-10-CM code for alpha-1-antitrypsin deficiency. It is valid for submission on a claim.

Inclusion terms

Alternative wordings a physician may use in the chart for this same code.

  • AAT deficiency

Risk adjustment

E88.01 risk-adjusts. Under the CMS-HCC V28 model it maps to this category:

  • HCC 50 Amyloidosis, Porphyria, and Other Specified Metabolic Disorders0.648 RAF

Coefficient shown for the Community Non-dual Aged segment. A higher HCC in the same hierarchy will suppress this one, so capturing it does not always add score.

Medicare coverage

E88.01 is named in 4 Medicare coverage policies 4 listing it as supporting medical necessity. Which of them applies to you depends on your Medicare contractor, and coverage genuinely differs by state.

  • A56199Billing and Coding: Molecular Pathology Procedures
  • A56717Billing and Coding: Respiratory Therapy (Respiratory Care)
  • A57224Billing and Coding: Respiratory Care
  • A60409Billing and Coding: Molecular Pathology Procedures

How E88.01 is indexed

Coders do not find codes by browsing the tabular list — they look them up in the alphabetic index, under the word the physician wrote. These are the index entries that lead here, so you can see which chart wordings map to E88.01.

  • Deficiency, deficientalpha-1-antitrypsin
  • Deficiency, deficientserum antitrypsin, familial

Related codes at this level

Codes that share E88.0. If E88.01 is not quite right, the correct code is usually one of these.

Questions about E88.01

Is E88.01 a billable ICD-10-CM code?
Yes. E88.01 is a billable, specific ICD-10-CM code and is valid for submission on a claim.
Where does E88.01 sit in the tabular list?
Endocrine, nutritional and metabolic diseases (E00-E89), in the block Metabolic disorders (E70-E88).
ICD-10-CMFY2026-Apr· effective April 1, 2026

Loaded directly from the CMS/NCHS ICD-10-CM public-domain release — see data sources and our editorial policy. If this page disagrees with the CMS tabular list, this page is wrong. Reference information for professional coders; not medical or billing advice.