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
|
Disorders of urea cycle metabolism |
French
|
Anomalies du cycle de l'uréogenèse |
| Level | Code | Title | |
|---|---|---|---|
| 1 | IV | Endocrine, nutritional and metabolic diseases | |
| 2 | E70-E90 | Metabolic disorders | |
| 3 | E72 | Other disorders of amino-acid metabolism | |
| 4 | E72.2 | Disorders of urea cycle metabolism |
| Active Ingredient | Description | |
|---|---|---|
| Glycerol phenylbutyrate |
Glycerol phenylbutyrate is a nitrogen-binding medicinal product. Urea cycle disorders (UCDs) are inherited deficiencies of enzymes or transporters necessary for the synthesis of urea from ammonia (NH3, NH4+). Absence of these enzymes or transporters results in the accumulation of toxic levels of ammonia in the blood and brain of affected patients. Glycerol phenylbutyrate is indicated for use as adjunctive therapy for chronic management of patients with urea cycle disorders (UCDs). |
|
| Sodium phenylbutyrate |
Sodium phenylbutyrate is a pro-drug and is rapidly metabolised to phenylacetate. Phenylacetate is a metabolically active compound that conjugates with glutamine via acetylation to form phenylacetylglutamine which is then excreted by the kidneys. On a molar basis, phenylacetylglutamine is comparable to urea (each containing 2 moles of nitrogen) and therefore provides an alternate vehicle for waste nitrogen excretion. |
