E88.01

Alpha-1-antitrypsin deficiency

Risk-adjusting (HCC)Billable code

HCC risk adjustment mapping

CMS-HCC V28

HCC50

Amyloidosis, Porphyria, and Other Specified Metabolic Disorders

CMS-HCC ESRD V24

HCC23

Other Significant Endocrine and Metabolic Disorders

RxHCC V08

RXHCC40

Alpha-1-Antitrypsin Deficiency

Coding notes

Excludes 1 never coded together · Excludes 2 may co-exist

This code · E88.01
Synonyms

AAT deficiency

Inherited from E88.0Disorders of plasma-protein metabolism, NEC
Excludes 1

monoclonal gammopathy (of undetermined significance) (D47.2) polyclonal hypergammaglobulinemia (D89.0) Waldenström macroglobulinemia (C88.00)

Excludes 2

disorder of lipoprotein metabolism (E78.-)

Inherited from E88Other and unspecified metabolic disorders
Excludes 1

histiocytosis X (chronic) (C96.6)

Use additional

codes for associated conditions

Inherited from E70-E88Metabolic disorders
Excludes 1

androgen insensitivity syndrome (E34.5-) congenital adrenal hyperplasia (E25.0) hemolytic anemias attributable to enzyme disorders (D55.-) Marfan syndrome (Q87.4-) 5-alpha-reductase deficiency (E29.1)

Excludes 2

Ehlers-Danlos syndromes (Q79.6-)

Inherited from chapter 4Endocrine, nutritional and metabolic diseases
Excludes 1

transitory endocrine and metabolic disorders specific to newborn (P70-P74)

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.

Official Guidelines for E88.01

FY 2026 ›
I.C.4Chapter-specific guidanceChapter 4: Endocrine, Nutritional, and Metabolic Diseases (E00-E89)

Subsections

Sections that reference E88.01

Sections whose FY 2026 text names E88.01, its E88 category, or a range containing it — the chapter guidance above is the authoritative source.

Find E88.01 in the alphabetic index

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