2020 ICD-10-CM Diagnosis Code E88.01

Alpha-1-antitrypsin deficiency

    2016 2017 2018 2019 2020 Billable/Specific Code
  • E88.01 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM E88.01 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E88.01 - other international versions of ICD-10 E88.01 may differ.
Applicable To
  • AAT deficiency
The following code(s) above E88.01 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E88.01:
  • E00-E89
    2020 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    Note
    • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2020 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndrome (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
  • E88
    ICD-10-CM Diagnosis Code E88

    Other and unspecified metabolic disorders

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • histiocytosis X (chronic) (C96.6)
    Use Additional
    • codes for associated conditions
    Other and unspecified metabolic disorders
  • E88.0
    ICD-10-CM Diagnosis Code E88.0

    Disorders of plasma-protein metabolism, not elsewhere classified

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • disorder of lipoprotein metabolism (E78.-)
    • monoclonal gammopathy (of undetermined significance) (D47.2)
    • polyclonal hypergammaglobulinemia (D89.0)
    • Waldenström macroglobulinemia (C88.0)
    Disorders of plasma-protein metabolism, not elsewhere classified
Approximate Synonyms
  • Alpha 1 antitrypsin deficiency
Clinical Information
  • A genetic disorder characterized by decreased alpha-1 antitrypsin activity in the lungs and blood and deposition of alpha-1 antitrypsin protein in the hepatocytes. These abnormalities result from defective production of alpha-1 antitrypsin and lead to the development of emphysema, cirrhosis, and liver failure.
  • Alpha-1 antitrypsin deficiency is an inherited disorder that can cause lung disease in adults and liver disease in adults and children. Alpha-1 antitrypsin (aat) is a protein that protects the lungs. The liver usually makes the protein, and releases it into the bloodstream. Because of a gene problem, some people have little or none of it. Not having enough aat puts you at risk of emphysema or liver problems. If you smoke, you increase your risk. Children with aat deficiency can develop liver problems that last their whole lives. Aat deficiency can be treated but not cured. One treatment involves adding to or replacing the missing protein. A lung transplant may be an option if you are seriously ill. Staying away from cigarette smoke is crucial.a blood test can tell if you have the deficiency. If you have it, your family members should also take the blood test.
  • Autosomal recessive trait leading to destruction of lung tissue by neutrophil elastase and eventual emphysema; second only to cystic fibrosis as most common lethal genetic disorder among caucasians of northern european ancestry.
  • Deficiency of the protease inhibitor alpha 1-antitrypsin that manifests primarily as pulmonary emphysema and liver cirrhosis.
ICD-10-CM E88.01 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 642 Inborn and other disorders of metabolism

Convert E88.01 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change

Diagnosis Index entries containing back-references to E88.01:

ICD-10-CM Codes Adjacent To E88.01
E87.4 Mixed disorder of acid-base balance
E87.5 Hyperkalemia
E87.6 Hypokalemia
E87.7 Fluid overload
E87.70 …… unspecified
E87.71 Transfusion associated circulatory overload
E87.79 Other fluid overload
E87.8 Other disorders of electrolyte and fluid balance, not elsewhere classified
E88 Other and unspecified metabolic disorders
E88.0 Disorders of plasma-protein metabolism, not elsewhere classified
E88.01 Alpha-1-antitrypsin deficiency
E88.02 Plasminogen deficiency
E88.09 Other disorders of plasma-protein metabolism, not elsewhere classified
E88.1 Lipodystrophy, not elsewhere classified
E88.2 Lipomatosis, not elsewhere classified
E88.3 Tumor lysis syndrome
E88.4 Mitochondrial metabolism disorders
E88.40 Mitochondrial metabolism disorder, unspecified
E88.41 MELAS syndrome
E88.42 MERRF syndrome
E88.49 Other mitochondrial metabolism disorders

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.