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KMID : 1022220210100030268
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Clinical Nutrition Research
2021 Volume.10 No. 3 p.268 ~ p.277
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Nutritional Management in a Patient with Citrullinemia Type 1
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Kang Hye-Jin
Kim Mi-Hyang
Lee Ji-Hyun
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Abstract
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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.
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KEYWORD
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Nutrition therapy, Citrullinemia, Hyperammonemia
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