Kodex

Code

E88.01

Alpha-1-antitrypsin deficiency

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Hierarchy

E00-E89 Endocrine, nutritional and metabolic diseases (E00-E89)
- E70-E88 Metabolic disorders (E70-E88)
  - 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

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.
- E07.0 Hypersecretion of calcitonin
- E34 Other endocrine disorders

Inclusion Terms

E88.01

AAT deficiency

Use Additional

codes for associated conditions

Excludes 1

D47.2 Monoclonal gammopathy
D89.0 Polyclonal hypergammaglobulinemia
C88.0 Waldenström macroglobulinemia

histiocytosis X (chronic) (C96.6)
- C96.6 Unifocal Langerhans-cell histiocytosis

E34.5 Androgen insensitivity syndrome
congenital adrenal hyperplasia (E25.0)
- E25.0 Congenital adrenogenital disorders associated with enzyme deficiency
hemolytic anemias attributable to enzyme disorders (D55.-)
- D55 Anemia due to enzyme disorders
Q87.4 Marfan's syndrome
5-alpha-reductase deficiency (E29.1)
- E29.1 Testicular hypofunction

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

Excludes 2

disorder of lipoprotein metabolism (E78.-)
- E78 Disorders of lipoprotein metabolism and other lipidemias

Q79.6 Ehlers-Danlos syndromes