在阿尔卡普顿的尼提西治疗期间,有或没有营养干预的人类计量,身体组成和营养指标
L R Ranganath1,2, M Khedr1, A M Milan1
1Department of Clinical Biochemistry and Metabolic Medicine, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8YE, UK.
Nutrients
|August 29, 2024
概括
食干预在阿尔卡普托努里亚 (AKU) 患者在尼提西治疗保存瘦体质和减少角膜问题. 然而,蛋白质的限制可能会导致脂肪质量增加.
科学领域:
- 生物化学 生物化学
- 营养科学 营养科学
- 医学遗传学 医学遗传学
背景情况:
- 阿尔卡普顿 (AKU) 与蛋白质营养障碍有关,包括治疗前同质性酸 (HGA) 的升高和尼松治疗期间提洛辛 (TYR) 的增加.
- 饮食干预可能有助于在AKU患者中管理这些代谢障碍.
研究的目的:
- 为了前性地描述AKU患者的饮食及其影响,这些患者在接受尼提西诺治疗的国家阿尔卡普顿症中心 (NAC) 组内接受正式的饮食干预.
- 为了将这些结果与AKU患者进行比较,在没有尼提西的尼提西在Alkaptonuria 2 (SN2 N-) 和接受尼提西治疗的SN2 (SN2 N+) 组中,这些患者只接受了自我指导的饮食建议.
主要方法:
- 一项涉及63名 (NAC) 和69名 (SN2 N-, SN2 N+) AKU患者的四年研究.
- 评估人类学,身体组成 (肌肉质量,体脂,手握力) 和生化标志物 (血清TYR,尿液尿素/uUREA,尿液肌/uCREAT).
- 尼提西诺的剂量有所不同 (NAC中2毫克,SN2 N+中10毫克);营养学家管理NAC中的蛋白质摄入量,而其他组接受了关于自我指导的蛋白质限制的建议.
主要成果:
- 蛋白质限制在所有组的UREA下降证实.
- 在NAC和SN2N+组中,体重和BMI增加.
- 积极的饮食干预在NAC稳定了瘦身量和温和血清TYR水平,尽管蛋白质限制,与较少的角膜角质病变相比SN2 N+.
结论:
- 持续的饮食干预对于接受尼提西治疗的AKU患者至关重要,防止瘦体质量损失和减轻血清氨酸增加.
- 这种干预导致角膜角膜病变的发病率降低.
- 蛋白质限制虽然是必要的,但会带来脂肪质量增加的风险,强调需要均衡的营养管理.
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