Birmingham Women's and Children's Hospital, Birmingham B4 6NH, UK.
School of Health Professions, Faculty of Health, University of Plymouth, Plymouth PL4 8AA, UK.
Nutrients. 2023 Jul 6;15(13):3046. doi: 10.3390/nu15133046.
In phenylketonuria (PKU), a previous intervention study assessing the patients ability to tolerate fruits and vegetables containing phenylalanine 76-100 mg/100 g without limit or measurement, found that an extra 50 mg/day phenylalanine, but not 100 mg/day, was tolerated from these fruits and vegetables. In a further 6-month extension study, we examined the effect of the 'free' use of this group of fruits and vegetables on blood phenylalanine control. For 6 months, the patients ate fruits and vegetables containing phenylalanine 76-100 mg/100 g without limit or measurement. Three-day diet diaries and the patients' weights were collected monthly. Blood phenylalanine spots were collected weekly aiming for blood phenylalanine levels <360 μmol/L. Retrospective blood phenylalanine was collected 6 months pre-trial. All 16 patients (69% females) from the intervention study took part in the extension study. Most of the patients ( = 14/16) had classical PKU with a median age of 10.5 years (range: 6-13). There was no statistically significant difference in the median blood phenylalanine pre-study (270, range: 50-760 μmol/L) compared to the 6-month extension study (250, range: 20-750 μmol/L) (= 0.4867). The patients had a median of 21 and 22 bloodspots, pre- and post-trial, respectively. In the extension study, the patients had an actual mean intake of 11 g/day (4-37) natural protein and 65 g/day (60-80) protein equivalent from a protein substitute. The mean phenylalanine intake was 563 mg/day (200-1850) with only 19 mg/day (0-146) phenylalanine from fruits and vegetables containing phenylalanine 76-100 mg/100 g. The weight z-scores remained unchanged (1.52 vs. 1.60, = 0.4715). There was no adverse impact on blood phenylalanine control when fruits and vegetables containing phenylalanine 76-100 mg/100 g were eaten without limit or measurement. However, the fruits and vegetable portion sizes eaten were small (60 g/week). Further longitudinal work is necessary to examine the 'free' use of fruits and vegetables containing phenylalanine 76-100 mg/100 g on metabolic control in patients with PKU.
在苯丙酮尿症（PKU）中，之前的一项干预研究评估了患者在不限制或测量的情况下耐受含有 76-100mg/100g 苯丙氨酸的水果和蔬菜的能力，发现每天额外摄入 50mg 苯丙氨酸是可以耐受的，但 100mg/天则不能耐受。在进一步的 6 个月扩展研究中，我们研究了这些水果和蔬菜的“自由”使用对血液苯丙氨酸控制的影响。在 6 个月的时间里，患者不限制或测量地食用含有 76-100mg/100g 苯丙氨酸的水果和蔬菜。每月收集三天的饮食日记和患者的体重。每周采集血斑以将血液苯丙氨酸水平控制在<360μmol/L 以下。回顾性采集试验前 6 个月的血液苯丙氨酸。干预研究中的所有 16 名患者（69%为女性）均参加了扩展研究。大多数患者（=14/16）患有经典型 PKU，中位年龄为 10.5 岁（范围：6-13 岁）。与研究前（270μmol/L，范围：50-760μmol/L）相比，扩展研究中（250μmol/L，范围：20-750μmol/L）的中位血液苯丙氨酸无统计学差异（=0.4867）。在试验前和试验后，患者分别有中位数为 21 和 22 个血斑。在扩展研究中，患者实际平均摄入 11g/天（4-37）天然蛋白质和 65g/天（60-80）来自蛋白质替代物的蛋白质当量。平均苯丙氨酸摄入量为 563mg/天（200-1850），其中只有 19mg/天（0-146）来自含有 76-100mg/100g 苯丙氨酸的水果和蔬菜。体重 Z 评分保持不变（1.52 与 1.60，=0.4715）。当不限制或测量地食用含有 76-100mg/100g 苯丙氨酸的水果和蔬菜时，不会对血液苯丙氨酸控制产生不良影响。然而，患者食用的水果和蔬菜量较小（每周 60g）。需要进一步的纵向研究来检查 PKU 患者在不限制或测量的情况下自由使用含有 76-100mg/100g 苯丙氨酸的水果和蔬菜对代谢控制的影响。
