ICD-10-CM Code E88.01

Alpha-1-antitrypsin deficiency

Version 2021 Billable Code

Valid for Submission

E88.01 is a billable code used to specify a medical diagnosis of alpha-1-antitrypsin deficiency. The code is valid for the fiscal year 2021 for the submission of HIPAA-covered transactions. The ICD-10-CM code E88.01 might also be used to specify conditions or terms like alpha-1-antitrypsin deficiency or alpha-1-antitrypsin hepatitis or panniculitis due to action of lipolytic enzymes or panniculitis due to alpha-1 anti-trypsin deficiency or pulmonary emphysema in alpha-1 pi deficiency.

ICD-10:E88.01
Short Description:Alpha-1-antitrypsin deficiency
Long Description:Alpha-1-antitrypsin deficiency

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code E88.01:

Inclusion Terms

Inclusion Terms
These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code.
  • AAT deficiency

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code E88.01 are found in the index:


Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Alpha-1-antitrypsin deficiency
  • Alpha-1-antitrypsin hepatitis
  • Panniculitis due to action of lipolytic enzymes
  • Panniculitis due to alpha-1 anti-trypsin deficiency
  • Pulmonary emphysema in alpha-1 PI deficiency

Clinical Information

  • ALPHA 1 ANTITRYPSIN DEFICIENCY-. deficiency of the protease inhibitor alpha 1 antitrypsin that manifests primarily as pulmonary emphysema and liver cirrhosis.

Convert E88.01 to ICD-9

  • 273.4 - Alpha-1-antitrypsin def

Code Classification

  • Endocrine, nutritional and metabolic diseases (E00–E90)
    • Metabolic disorders (E70-E88)
      • Other and unspecified metabolic disorders (E88)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021

Information for Patients


Alpha-1 Antitrypsin Deficiency

Also called: AATD, Alpha-1, Inherited emphysema

Alpha-1 antitrypsin deficiency (AAT deficiency) is an inherited condition that raises your risk for lung and liver disease. Alpha-1 antitrypsin (AAT) is a protein that protects the lungs. The liver makes it. If the AAT proteins aren't the right shape, they get stuck in the liver cells and can't reach the lungs.

Symptoms of AAT deficiency include

  • Shortness of breath and wheezing
  • Repeated lung infections
  • Tiredness
  • Rapid heartbeat upon standing
  • Vision problems
  • Weight loss

Some people have no symptoms and do not develop complications.

Blood tests and genetic tests can tell if you have it. If your lungs are affected, you may also have lung tests. Treatments include medicines, pulmonary rehab, and extra oxygen, if needed. Severe cases may need a lung transplant. Not smoking can prevent or delay lung symptoms.

NIH: National Heart, Lung, and Blood Institute

  • Alpha-1 antitrypsin deficiency (Medical Encyclopedia)
  • Alpha-1 antitrypsin test (Medical Encyclopedia)

[Learn More]

Alpha-1 antitrypsin deficiency Alpha-1 antitrypsin deficiency is an inherited disorder that may cause lung disease and liver disease. The signs and symptoms of the condition and the age at which they appear vary among individuals.People with alpha-1 antitrypsin deficiency usually develop the first signs and symptoms of lung disease between ages 20 and 50. The earliest symptoms are shortness of breath following mild activity, reduced ability to exercise, and wheezing. Other signs and symptoms can include unintentional weight loss, recurring respiratory infections, fatigue, and rapid heartbeat upon standing. Affected individuals often develop emphysema, which is a lung disease caused by damage to the small air sacs in the lungs (alveoli). Characteristic features of emphysema include difficulty breathing, a hacking cough, and a barrel-shaped chest. Smoking or exposure to tobacco smoke accelerates the appearance of emphysema symptoms and damage to the lungs.About 10 percent of infants with alpha-1 antitrypsin deficiency develop liver disease, which often causes yellowing of the skin and whites of the eyes (jaundice). Approximately 15 percent of adults with alpha-1 antitrypsin deficiency develop liver damage (cirrhosis) due to the formation of scar tissue in the liver. Signs of cirrhosis include a swollen abdomen, swollen feet or legs, and jaundice. Individuals with alpha-1 antitrypsin deficiency are also at risk of developing a type of liver cancer called hepatocellular carcinoma.In rare cases, people with alpha-1 antitrypsin deficiency develop a skin condition called panniculitis, which is characterized by hardened skin with painful lumps or patches. Panniculitis varies in severity and can occur at any age.
[Learn More]