E71.310 Long chain/very long chain acyl CoA dehydrogenase deficiency

Instructional Notes:

E71.310
Description: Long chain/very long chain acyl CoA dehydrogenase deficiency
Inclusion Term:
- LCAD deficiency
- VLCAD deficiency
↓
E71.31
Description: Disorders of fatty-acid oxidation
↓
E71.3
Description: Disorders of fatty-acid metabolism
Exclude1:
- peroxisomal disorders (E71.5)
- Refsum's disease (G60.1)
- Schilder's disease (G37.0)
Exclude2:
- carnitine deficiency due to inborn error of metabolism (E71.42)
↓
E71
Description: Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
↓
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.