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首页> 外文期刊>Journal of human nutrition and dietetics >Long-term efficacy of 'ready-to-drink' protein substitute in phenylketonuria.
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Long-term efficacy of 'ready-to-drink' protein substitute in phenylketonuria.

机译：“即饮型”蛋白替代品在苯丙酮尿症中的长期疗效。

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BACKGROUND: In phenylketonuria (PKU), protein substitute is an essential part of dietary treatment. Short-term studies have demonstrated that liquid protein substitutes (LPS) are efficacious, and improve compliance in teenagers and adults with PKU, although there are no data available to demonstrate that their effectiveness is sustained over time. The present retrospective study aimed to evaluate the long-term efficacy of ready-to-drink protein substitute in a group of people with PKU. METHODS: Thirty-four patients (17 females and 17 males, median age 14.9 years, range 7.2-53.8 years) with PKU on dietary management were recruited from Birmingham Children's Hospital. All patients who were taking a LPS for a median of 2.4 years (range 6 months to 4.1 years), had their plasma phenylalanine concentrations, anthropometric and nutritional biochemical markers reviewed, both before and when taking the LPS. RESULTS: There was a significant improvement in median plasma phenylalanine (P < 0.05), vitamin B(12) (P < 0.01), calcium (P < 0.05) and albumin (P < 0.05) concentrations in subjects (n = 13) aged >18 years when taking the LPS. In the children aged 7-18 years (n = 21), median plasma phenylalanine concentrations were maintained on LPS. Their plasma selenium concentrations (P < 0.05) deteriorated, but calcium (P < 0.05), albumin (P < 0.01), haemoglobin (P < 0.01) and haematocrit (P < 0.01) significantly improved. CONCLUSIONS: This retrospective review suggested that, in adult patients, the long-term use of LPS is associated with better compliance by lowering blood phenylalanine and improving nutritional biochemical markers.
机译：背景：在苯丙酮尿症（PKU）中，蛋白质替代品是饮食治疗的重要组成部分。短期研究表明，液态蛋白替代品（LPS）有效，可以改善青少年和成人的PKU依从性，尽管尚无可用数据证明其有效性随着时间的推移而持续。本回顾性研究旨在评估即饮型蛋白质替代品在一组PKU患者中的长期疗效。方法：从伯明翰儿童医院招募了34名进行饮食管理的PKU患者（男17例，男17例，中位年龄14.9岁，范围7.2-53.8岁）。所有接受LPS的中位数为2.4年（6个月至4.1年）的患者，在服用LPS之前和之后均进行了血浆苯丙氨酸浓度，人体测量学和营养生化标志物检查。结果：年龄（n = 13）的受试者的血浆苯丙氨酸中位数（P <0.05），维生素B（12）（P <0.01），钙（P <0.05）和白蛋白（P <0.05）浓度显着改善。服用LPS时> 18年。在7-18岁的儿童（n = 21）中，LPS维持血浆苯丙氨酸的中值浓度。他们的血浆硒浓度（P <0.05）恶化，但钙（P <0.05），白蛋白（P <0.01），血红蛋白（P <0.01）和血细胞比容（P <0.01）明显改善。结论：这项回顾性研究表明，在成年患者中，长期使用LPS与降低血液中的苯丙氨酸和改善营养生化标志物具有更好的依从性有关。

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