这是第一项评估苯丙酮尿症（PKU）患者维生素K状态与饮食摄入量和苯丙氨酸饮食依从性的关系的研究。计算了34例PKU患者的膳食和PKU配方维生素K摄入量，通过测量维生素K缺失诱导的凝血酶原（PIVKA-II）确定维生素K状态。考虑了前12个月的血液苯丙氨酸浓度。PIVKA-II浓度正常的患者比PIVKA-II水平异常的患者的苯丙氨酸结果超过6mg/dL（p=0.035）。同样，在PIVKA-II水平正常的患者中观察到更高的维生素K总摄入量和膳食维生素摄入量,以μg/天(两者p=0.033)和%RDA(分别为p=0.0002和p=0.003)表示.异常的PIVKA-II浓度与较低的OR(0.1607;95CI:0.0273-0.9445,p=0.043)相关,所述OR具有高于6mg/dL的中值苯丙氨酸浓度。总之,维生素K缺乏症在苯丙酮尿症中并不少见，维生素K摄入充足的患者也可能发生。饮食依从性较好的PKU患者维生素K缺乏的风险较高。本研究结果强调需要进一步研究，以重新评估有关维生素K摄入量的饮食建议。都涉及天然产品的配方和饮食消费。
This is the first
study to evaluate vitamin K status in relation to dietary intake and phenylalanine dietary compliance in patients with phenylketonuria (PKU). The dietary and PKU formula intake of vitamin K was calculated in 34 PKU patients, with vitamin K status determined by the measurement of prothrombin induced by vitamin K absence (PIVKA-II). Blood phenylalanine concentrations in the preceding 12 months were considered. There were significantly more phenylalanine results exceeding 6 mg/dL in patients with normal PIVKA-II concentrations than in those with abnormal PIVKA-II levels (p = 0.035). Similarly, a higher total intake of vitamin K and dietary vitamin intake expressed as μg/day (p = 0.033 for both) and %RDA (p = 0.0002 and p = 0.003, respectively) was observed in patients with normal PIVKA-II levels. Abnormal PIVKA-II concentrations were associated with a lower OR (0.1607; 95%CI: 0.0273-0.9445, p = 0.043) of having a median phenylalanine concentration higher than 6 mg/dL. In conclusion, vitamin K deficiency is not uncommon in phenylketonuria and may also occur in patients with adequate vitamin K intake. PKU patients with better dietary compliance have a higher risk of vitamin K deficiency. The present findings highlight the need for further studies to re-evaluate dietary recommendations regarding vitamin K intake, both concerning formula-based and dietary consumption of natural products.
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