E71.510 Zellweger syndrome

Instructional Notes:

E71.510
Description: Zellweger syndrome
↓
E71.51
Description: Disorders of peroxisome biogenesis
Exclude1:
- Refsum's disease (G60.1)
Inclusion Term:
- Group 1 peroxisomal disorders
↓
E71.5
Description: Peroxisomal disorders
Exclude1:
- Schilder's disease (G37.0)
↓
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.