E88.01 Alpha-1-antitrypsin deficiency

Instructional Notes:

E88.01
Description: Alpha-1-antitrypsin deficiency
Inclusion Term:
- AAT deficiency
↓
E88.0
Description: Disorders of plasma-protein metabolism, not elsewhere classified
Exclude1:
- monoclonal gammopathy (of undetermined significance) (D47.2)
- polyclonal hypergammaglobulinemia (D89.0)
- Waldenström macroglobulinemia (C88.00)
Exclude2:
- disorder of lipoprotein metabolism (E78.-)
↓
E88
Description: Other and unspecified metabolic disorders
Exclude1:
- histiocytosis X (chronic) (C96.6)
Use Additional Code:
- codes for associated conditions
↓
E70-E88
Description: Metabolic disorders (E70-E88)
Exclude1:
- 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)
Exclude2:
- Ehlers-Danlos syndromes (Q79.6-)
↓
E00-E89
Description: Endocrine, nutritional and metabolic diseases
Exclude1:
- transitory endocrine and metabolic disorders specific to newborn (P70-P74)
Notes:
- 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.