Biotin and carnitine profiles in preterm infants in Japan
Biotin and carnitine profiles in preterm infants in Japan
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DOI：
10.1111/ped.12053
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发表时间：
2013-06-01
影响因子：
1.4
通讯作者：
Ohshima, Yusei
中科院分区：
文献类型：
--
作者：
Tokuriki, Shuko;Hayashi, Hisako;Ohshima, Yusei
背景生物素作为一种共价结合的辅酶在羧化酶中起着重要的作用。肉毒碱是必需的氧化运输长链脂肪酸通过线粒体内膜。本研究旨在评估接受母乳和/或日本生产的标准婴儿配方奶粉肠内喂养的早产儿中生物素和肉毒碱缺乏的风险。方法46例新生儿纳入研究。在1周龄和1月龄时收集尿液和血清样品以及干血斑。此外，在早产儿月经后40周和44周（PMA）时采集样本。游离肉毒碱和C5-OH酰基肉毒碱，其中包括3-羟基异戊酰肉毒碱作为一个主要的异构体，测定血清样品和干血斑使用串联质谱。采用气相色谱/质谱法测定尿3-羟基异戊酸（3-HIVA）。结果早产儿血清和干血斑中游离肉毒碱水平明显低于足月儿，但随着PMA的增加而升高。C5-OH酰基肉毒碱和尿3-HIVA水平，这是非常低的足月儿，增加与PMA早产儿。结论母乳和/或标准婴儿配方奶粉喂养的早产儿可能存在慢性生物素缺乏。分析干血斑和尿3-HIVA的肉毒碱谱是相对非侵入性的，并有助于早产儿生物素缺乏症的早期检测。
Background Biotin plays an important role as a covalently bound coenzyme for carboxylases. Carnitine is essential in -oxidation to transport long-chain fatty acids across the inner mitochondrial membrane. The present study was conducted to assess the risk of biotin and carnitine deficiencies in preterm infants who received enteral feeding with maternal milk and/or standard infant formula made in Japan. Methods Forty-six infants were enrolled in the study. Urine and serum samples and dried blood spots were collected at 1 week and 1 month of age. Additionally, samples were collected at 40 and 44 weeks post-menstrual age (PMA) in preterm infants. Free carnitine and C5-OH acylcarnitine, which consist of 3-hydroxyisovalerylcarnitine as a major isomer, were measured in serum samples and dried blood spots using tandem mass spectrometry. Urine 3-hydroxyisovaleric acid (3-HIVA) was measured using gas chromatography/mass spectrometry. Results The free carnitine levels in preterm infants were significantly lower than those in term infants, but increased with PMA in serum samples and dried blood spots. C5-OH acylcarnitine and urinary 3-HIVA levels, which were very low in term infants, were increased with PMA in preterm infants. Conclusion The present results may indicate chronic biotin deficiency in preterm infants fed maternal milk and/or standard infant formula. Analyses of carnitine profiles of dried blood spots and urine 3-HIVA are relatively non-invasive and useful for the early detection of biotin deficiency in preterm infants.