Specific codes in ICD-10 are unique alphanumeric designations used to identify and categorize diseases, disorders, and conditions. They consist of 3-5 characters, including both letters and numbers, that provide a high level of detail and specificity.
| Language | Translation |
|---|---|
English
|
Classical phenylketonuria |
French
|
Phénylcétonurie classique |
| Level | Code | Title | |
|---|---|---|---|
| 1 | IV | Endocrine, nutritional and metabolic diseases | |
| 2 | E70-E90 | Metabolic disorders | |
| 3 | E70 | Disorders of aromatic amino-acid metabolism | |
| 4 | E70.0 | Classical phenylketonuria |
| Active Ingredient | Description | |
|---|---|---|
| Sapropterin |
Sapropterin is a synthetic version of the naturally occurring 6R-BH4, which is a cofactor of the hydroxylases for phenylalanine, tyrosine and tryptophan. The rationale for administration of sapropterin in patients with BH4-responsive PKU is to enhance the activity of the defective phenylalanine hydroxylase and thereby increase or restore the oxidative metabolism of phenylalanine. The rationale for administration of sapropterin in patients with BH4 Deficiency is to replace the deficient levels of BH4, thereby restoring the activity of phenylalanine hydroxylase. |
