北大接受Sapropterin治疗两年后的蛋白质和微量营养素摄入
Protein and Micronutrient Intake After Two Years of Sapropterin Treatment in PKU.
摘要(中文译文)
沙丙蝶呤（sapropterin）可使有反应性的苯丙酮尿症（PKU）患者放宽饮食限制，增加天然蛋白质摄入并减少对蛋白质替代品（PSs）的依赖。由于蛋白质替代品提供必需微量营养素，放宽饮食限制可能增加营养不足的风险。关于接受沙丙蝶呤治疗的儿童详细微量营养素摄入的证据仍然有限。本二次分析评估了一项前瞻性纵向研究中21名有反应性儿童在接受沙丙蝶呤治疗24个月后的膳食蛋白质和微量营养素摄入情况。对照顾者填写的三日食物记录进行分析，评估蛋白质、钙、铁、锌、维生素D和维生素B的摄入量，并将微量营养素摄入量与英国膳食参考值（DRVs）进行比较。平均总蛋白质摄入量为75 ± 14 g/天，包括30 ± 16 g/天的天然蛋白质和45 ± 21 g/天的来自蛋白质替代品的蛋白质当量；天然蛋白质耐受量范围为8至66 g/天。蛋白质替代品提供了大部分微量营养素：钙80%、铁84%、锌87%、维生素D 96%、维生素B 78%。大多数儿童的微量营养素摄入量中位数超过膳食参考值；然而，有四名儿童的摄入量低于膳食参考值，几乎均发生在蛋白质替代品减少或省略时。一名未使用蛋白质替代品而每日摄入天然蛋白质>40 g的儿童铁（51%）、锌（90%）和维生素D（4%）摄入量偏低。一名依从性差的青少年钙（46%）、铁（64%）、锌（41%）和维生素D（60%）摄入量偏低。另一名每日从蛋白质替代品摄入60 g蛋白质当量的儿童因强化程度较低而维生素D摄入量略低（85%）。保持规律使用蛋白质替代品的儿童达到了微量营养素需求。经过两年沙丙蝶呤治疗，放宽饮食限制增加了天然蛋白质摄入，但并未持续确保充足的微量营养素摄入。微量营养素不足与蛋白质替代品使用减少相关，强调随着饮食变得更加灵活，需要在营养师指导下谨慎调整。
Abstract
Sapropterin allows dietary liberalisation in responsive individuals with phenylketonuria (PKU), increasing natural protein intake and reducing dependence on protein substitutes (PSs). As PSs provide essential micronutrients, dietary liberalisation may increase the risk of nutritional insufficiency. Evidence describing detailed micronutrient intake in sapropterin-treated children remains limited. This secondary analysis evaluated dietary protein and micronutrient intake after 24 months of sapropterin treatment in 21 responsive children from a prospective longitudinal study. Caregiver-completed three-day food records were analysed for protein, calcium, iron, zinc, vitamin D, and vitamin B, with micronutrient intakes compared with UK dietary reference values (DRVs). Mean total protein intake was 75 ± 14 g/day, comprising 30 ± 16 g/day natural protein and 45 ± 21 g/day protein equivalent from PSs; natural protein tolerance ranged from 8 to 66 g/day. PSs contributed most micronutrients: calcium 80%, iron 84%, zinc 87%, vitamin D 96%, and vitamin B 78%. Median micronutrient intakes exceeded DRVs for most children; however, four had intakes below DRVs, almost exclusively when PSs were reduced or omitted. One child consuming >40 g/day natural protein without PSs had low iron (51%), zinc (90%), and vitamin D (4%) intakes. A non-adherent adolescent had low intakes of calcium (46%), iron (64%), zinc (41%), and vitamin D (60%). Another child receiving 60 g/day protein equivalent from PSs had marginally low vitamin D intake (85%) due to lower fortification. Children maintaining regular PS use met micronutrient requirements. After two years of sapropterin treatment, dietary liberalisation increased natural protein intake but did not consistently ensure adequate micronutrient intake. Micronutrient shortfalls were associated with reduced PS use, emphasising the need for careful dietitian-guided adjustment as diets become more flexible.
如何引用
AMA
Ozlem Yilmaz Nas, Catherine Ashmore, Maria Ines Gama, Anne Daly, Sharon Evans, Alex Pinto, et al. Protein and Micronutrient Intake After Two Years of Sapropterin Treatment in PKU.. Nutrients. 2026; doi:10.3390/nu18101549.
APA
Ozlem Yilmaz Nas, Catherine Ashmore, Maria Ines Gama, Anne Daly, Sharon Evans, Alex Pinto, et al (2026). Protein and Micronutrient Intake After Two Years of Sapropterin Treatment in PKU.. Nutrients. https://doi.org/10.3390/nu18101549
GB/T 7714
Ozlem Yilmaz Nas, Catherine Ashmore, Maria Ines Gama, Anne Daly, Sharon Evans, Alex Pinto, et al. Protein and Micronutrient Intake After Two Years of Sapropterin Treatment in PKU.[J]. Nutrients, 2026 doi:10.3390/nu18101549.
在 PubMed增强版 阅读全文
翻译全文 · AI 文献问答 · PDF 原文下载 · 引用导出