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ICD-10-CM Code E88.01
Alpha-1-antitrypsin deficiency

Billable Code
Billable codes are sufficient justification for admission to an acute care hospital when used a principal diagnosis.
| ICD-10 from 2011 - 2016

E88.01 is a billable ICD code used to specify a diagnosis of alpha-1-antitrypsin deficiency. A 'billable code' is detailed enough to be used to specify a medical diagnosis.

The ICD code E880 is used to code Alpha 1-antitrypsin deficiency

Alpha 1-antitrypsin deficiency (α1-antitrypsin deficiency, A1AD) is a genetic disorder that causes defective production of alpha 1-antitrypsin (A1AT), leading to decreased A1AT activity in the blood and lungs, and deposition of excessive abnormal A1AT protein in liver cells. There are several forms and degrees of deficiency; the form and degree depend on whether the sufferer has one or two copies of the defective allele because it is a co-dominant trait. Severe A1AT deficiency causes panacinar emphysema or COPD in adult life in many people with the condition (especially if they are exposed to cigarette smoke). The disorder can lead to various liver diseases in a minority of children and adults, and occasionally more unusual problems. It is treated through avoidance of damaging inhalants and, in severe cases, by intravenous infusions of the A1AT protein or by transplantation of the liver or lungs. It usually produces some degree of disability and reduces life expectancy.

Specialty: Pulmonology, Medical Genetics
MeSH Code: D019896
ICD 9 Code: 273.4

Structure of Alpha 1-antitrypsin

Source: Wikipedia

Coding Notes for E88.01 Info for medical coders on how to properly use this ICD-10 code

Inclusion Terms:
Inclusion Terms
Inclusion Terms are a list of concepts for which a specific code is used. The list of Inclusion Terms is useful for determining the correct code in some cases, but the list is not necessarily exhaustive.
AAT deficiency

MS-DRG Mapping

  • DRG Group #642 - Inborn and other disorders of metabolism.

Coding Advice SNOMET-CT

  • Consider additional code to identify specific condition or disease

ICD-10-CM Alphabetical Index References for 'E88.01 - Alpha-1-antitrypsin deficiency'

The ICD-10-CM Alphabetical Index links the below-listed medical terms to the ICD code E88.01. Click on any term below to browse the alphabetical index.


Alpha-1-antitrypsin deficiency (exact match)

This is the official exact match mapping between ICD9 and ICD10, as provided by the General Equivalency mapping crosswalk. This means that in all cases where the ICD9 code 273.4 was previously used, E88.01 is the appropriate modern ICD10 code.

Parent Code: E88.0 - Disorders of plasma-protein metabolism, not elsewhere classified

** This Document Provided By ICD.Codes **
Source: http://icd.codes/icd10cm/E8801