Kang Hyejin, Kim Mihyang, Lee Ji Hyun
Food Services and Clinical Nutrition Team, Ajou University Hospital, Suwon 16499, Korea.
Department of Medical Nutrition, Graduate School of East-West Medical Science, Kyung Hee University, Yongin 17104, Korea.
Clin Nutr Res. 2021 Jul 20;10(3):268-277. doi: 10.7762/cnr.2021.10.3.268. eCollection 2021 Jul.
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.
对于1型瓜氨酸血症患者，营养管理对于预防与高氨血症相关并发症的发生至关重要。本报告描述了一名接受营养干预超过3年的患者。一名因瓜氨酸血症被诊断为高氨血症的新生儿前往峨山大学医院就诊，并被转诊至营养团队。在接受急性治疗后，该婴儿定期喂食特殊配方奶粉。建议采用蛋白质限制饮食以维持正常发育并实现长期生存。通过持续提供营养干预，该儿童表现出正常的生长发育，能量 - 蛋白质供应得到适当维持。本病例清楚地表明了医学营养治疗对瓜氨酸血症患者的重要性。
