AMMONAPS 500 MG CPR
(Phenulbutyrate sodique) - Maladie rare sans désignation orpheline
Adjunctive therapy in the chronic management of urea cycle disorders, involving deficiencies of carbamyl phosphate synthetase, ornithine transcarbamylase, or argininosuccinate synthetase. It is indicated in all patients with neonatal-onset presentation (complete enzyme deficiencies, presenting within the first 28 days of life). It is also indicated in patients with late-onset disease (partial enzyme deficiencies, presenting after the first month of life) who have a history of hyperammonaemic encephalopathy.