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Nutritional Management in a Patient with Citrullinemia Type 1

For patients with citrullinemia type 1, nutritional management is essential to prevent the occurrence of complications associated with hyperammonemia. This report describes a patient who had been receiving nutrition intervention for more than 3 years. A newborn diagnosed with hyperammonemia due to c...

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Detalles Bibliográficos
Autores principales: Kang, Hyejin, Kim, Mihyang, Lee, Ji Hyun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Korean Society of Clinical Nutrition 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8331288/
https://www.ncbi.nlm.nih.gov/pubmed/34386445
http://dx.doi.org/10.7762/cnr.2021.10.3.268
Descripción
Sumario:For patients with citrullinemia type 1, nutritional management is essential to prevent the occurrence of complications associated with hyperammonemia. This report describes a patient who had been receiving nutrition intervention for more than 3 years. A newborn diagnosed with hyperammonemia due to citrullinemia visited Ajou University Hospital and was referred to the nutrition team. After receiving acute treatment, the infant was regularly fed with specialized formula. A protein-restricted diet is recommended for maintaining normal development and achieving long-term survival. Through continuous provision of nutritional intervention, the child showed normal growth and development, and the energy-protein supply was maintained appropriately. This case clearly shows the importance of medical nutrition therapy for patients with citrullinemia.