Tyrosinemia type 1 phenylalanine supplementation tyrosine
Conditions
Interventions
Different doses of phenylalanine supplementation will be given during some days. The effect of the supplementation on metabolic control will be studied while receiving the different dosages.
Sponsors
University Medical Center Groningen
Eligibility
Inclusion criteria
Inclusion criteria: - Diagnosed with Tyrosinemia type 1 - Treated with NTBC - Adequate dietary control (tyrosine concentrations: 200-600 µmol/L)
Exclusion criteria
Exclusion criteria: - Tyrosinemia type 1 patients who received liver transplantation
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Blood phenylalanine concentrations Blood tyrosine concentrations | — |
Secondary
| Measure | Time frame |
|---|---|
| Blood NTBC concentrations Blood succinylacetone concentrations | — |
Contacts
Public ContactF.J. van Spronsen
Outcome results
None listed