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Home/Diagnosis/ICD-10 Coding for Alpha-1 Antitrypsin Deficiency

ICD-10 Coding for Alpha-1 Antitrypsin Deficiency

E88.01

Complete ICD-10-CM coding and documentation guide for icd-10 coding for alpha-1 antitrypsin deficiency includes clinical validation requirements, medical necessity guidelines, and coding policies.

Also Known as:
AATD
Alpha-1 Proteinase Inhibitor Deficiency
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Key Information: ICD-10 Coding for Alpha-1 Antitrypsin Deficiency

Essential facts and insights about ICD-10 Coding for Alpha-1 Antitrypsin Deficiency

Use ICD-10 code E88.01 for alpha-1-antitrypsin deficiency, ensuring proper documentation in clinical notes.

Primary ICD-10-CM Codes

Alpha-1-antitrypsin deficiency

Billable Code
E88.01
Billable

Diagnostic Criteria

clinical:
  • • Serum AAT level <80 mg/dL
documentation:
  • • Phenotype PiZZ, PiSZ, or PiNull documented

Applicable To

  • • Genetic deficiency of alpha-1-antitrypsin

Important Notes

  • • Ensure phenotype and serum levels are documented to support the use of E88.01.
Ancillary Codes

Additional codes that may be used with this diagnosis

J43.9

Emphysema, unspecified

Use for emphysema related to AATD, confirmed by radiologic evidence.

K74.60

Unspecified cirrhosis of liver

Use for cirrhosis related to AATD, confirmed by liver biopsy.

Frequently Asked Questions

What is the ICD-10 code for alpha-1 antitrypsin deficiency?

The ICD-10 code for alpha-1 antitrypsin deficiency is E88.01. It is used when the deficiency is confirmed by lab tests and is the underlying cause of clinical manifestations.

How is alpha-1 antitrypsin deficiency documented?

Documentation should include phenotype confirmation, serum AAT levels, and any related clinical manifestations such as emphysema or cirrhosis.

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