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在PKU和其它氨基酸代谢障碍中的母乳养 - - 一个单一的体验中心

Agnieszka Kowalik¹, Sylwia Gudej-Rosa¹, Marta Nogalska¹

¹Institute of Mother and Child, Department of Inborn Errors of Metabolism and Paediatrics, Kasprzaka 17A, 01-211 Warsaw, Poland.

|August 10, 2024

概括

哺乳婴儿与氨基酸代谢 (IEM) 的先天性错误是可能的,只有仔细管理. 早期饮食治疗和按需母乳养,以及定期监测,支持IEM婴儿持续摄入母乳.

科学领域:

代谢障碍代谢障碍代谢障碍
儿科儿科儿科
人类哺乳期哺乳期

背景情况:

母乳提供了显著的好处,但需要小心管理婴儿与氨基酸代谢 (IEM) 的先天性错误.
扩大新生儿查 (NBS) 的引入影响了IEM的诊断和管理.
了解患有IEM的婴儿的母乳养实践对于优化营养支持至关重要.

研究的目的:

调查婴儿IEM母乳养的作用和持续时间.
为了比较扩展NBS实施前后的母乳养模式.
确定影响该人口维持哺乳期的因素.

主要方法:

对127名通过NBS或选择性查 (SS) 诊断出IEM (基尿和其他IEM) 的婴儿进行了回顾性研究.
分析饮食治疗的开始情况和产后1,3个月和6个月母乳 (挤出或直接) 的比例.
在NBS和SS组之间比较母乳养结果.

主要成果:

通过NBS (PKU和非PKU) 诊断的婴儿比通过SS诊断的婴儿更早开始饮食治疗.
与SS组 (38%) 相比,NBS组 (74-80%) 的婴儿在第一个月接受母乳的比例更高.
母乳摄入量随着时间的推移而下降,特别是在奶瓶养的婴儿中,这表明奶可能会影响哺乳期的维持.

结论:

维持哺乳期和母乳养长达6个月的婴儿可能患有IEM.
按需母乳养,加上定期的生化监测 (每周一次的PKU,每两周一次的其他IEM),支持持续的哺乳.
通过奶瓶奶可能会对哺乳时间产生负面影响,这凸显了尽可能直接母乳养的重要性.

关键词:

母乳养哺乳期母乳养氨基酸代谢中的先天性错误婴儿婴儿婴儿婴儿婴儿基尿症的现象基尿症的现象

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