Nutritional management of patients with urea cycle disorders
RH Singh - Journal of inherited metabolic disease, 2007 - Springer
RH Singh
Journal of inherited metabolic disease, 2007•SpringerThe nutritional management of patients with urea cycle disorders (UCDs) involves restriction
of dietary protein along with provision of adequate protein-free energy, essential amino acid
supplements, and vitamins and minerals in combination with nitrogen-scavenging drugs.
The present paper discusses nutrition therapy for a range of circumstances: during an acute
hyperammonaemic episode and at hospital discharge; before, during, and after surgery; and
for lifelong chronic management of UCDs.
of dietary protein along with provision of adequate protein-free energy, essential amino acid
supplements, and vitamins and minerals in combination with nitrogen-scavenging drugs.
The present paper discusses nutrition therapy for a range of circumstances: during an acute
hyperammonaemic episode and at hospital discharge; before, during, and after surgery; and
for lifelong chronic management of UCDs.
Summary
The nutritional management of patients with urea cycle disorders (UCDs) involves restriction of dietary protein along with provision of adequate protein-free energy, essential amino acid supplements, and vitamins and minerals in combination with nitrogen-scavenging drugs. The present paper discusses nutrition therapy for a range of circumstances: during an acute hyperammonaemic episode and at hospital discharge; before, during, and after surgery; and for lifelong chronic management of UCDs.
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