MacDonald A, Lilburn M, Cochrane B, Davies P, Daly A, Asplin D, Hall S K, Cousins A, Chakrapani A, Robinson P, Lee P
The Children's Hospital, Dietetic Department, Steelhouse Lane, Birmingham, UK.
J Inherit Metab Dis. 2004;27(2):127-35. doi: 10.1023/B:BOLI.0000028784.11080.9b.
Some older patients with phenylketonuria (PKU) fail to consume their protein substitute (with or without vitamin and mineral supplements) in prescribed amounts, which contributes to poor blood phenylalanine control. PKU Express (Vitaflo), is a new low-volume (amino acids 72 g/100 g), low-carbohydrate, phenylalanine-free protein substitute with added vitamins and minerals designed for people with PKU over 8 years of age. In an open intervention study, the aim was to investigate its acceptability and effectiveness in a group of teenagers and adults with PKU. Twenty-three subjects (15 female; 8 male) with PKU, who had a median age of 17 years (range 8-37 years) took the substitute for 8 weeks. A 3-day prospective diet diary, height, weight, plasma amino acids, biochemical and haematological nutritional analytes were measured at weeks 0 and 8. Skin-puncture bloods for plasma phenylalanine were collected every 2 weeks. The median weight of protein substitute (with or without vitamin and mineral supplements) consumed decreased by 33% from 150 g (range 140-180) daily to 100 g (range 100-125) daily ( p <0.001). Median change in energy intake decreased by a median of 10% (95% CI 2.0 to 18.0) when compared to intake on original protein substitute. On PKU Express, the intakes of all nutrients exceeded the dietary reference values but none was excessively high. Blood phenylalanine decreased by a mean of 37 micromol/L (95% CI-27 to 102) during the trial. Body mass index decreased in 40% of subjects. Changes in blood phenylalanine or body mass index were not statistically significant. Most of the nutritional, haematological and biochemical indices stayed within normal reference ranges for the analytes studied. Sixteen (70%) of the subjects had low plasma selenium at the start, but only 13 (57%) at the study end. Plasma vitamin B12 was high in 8 subjects at the start of the study and 9 at the end. Twenty-one subjects (96%) stated that the product was convenient and easy to prepare. However, 7 (32%) described the smell and 9 (46%) the texture as the same as or worse than those of previous protein substitutes. Because of the use of the premeasured sachets, some subjects were able to prepare their own protein substitute for the first time. PKU Express is a safe, efficacious, protein substitute that significantly reduces the daily volume of prescribed protein substitute.
一些老年苯丙酮尿症（PKU）患者未能按规定量食用其蛋白质替代品（无论是否添加维生素和矿物质补充剂），这导致血液中苯丙氨酸控制不佳。PKU Express（Vitaflo）是一种新型小剂量（每100克含72克氨基酸）、低碳水化合物、无苯丙氨酸的蛋白质替代品，添加了维生素和矿物质，专为8岁以上的PKU患者设计。在一项开放性干预研究中，目的是调查其在一组青少年和成年PKU患者中的可接受性和有效性。23名PKU患者（15名女性；8名男性），中位年龄为17岁（范围8 - 37岁），服用该替代品8周。在第0周和第8周测量了3天的前瞻性饮食日记、身高、体重、血浆氨基酸、生化和血液学营养分析物。每2周采集用于检测血浆苯丙氨酸的皮肤穿刺血样。食用的蛋白质替代品（无论是否添加维生素和矿物质补充剂）的中位重量从每天150克（范围140 - 180克）下降了33%，至每天100克（范围100 - 125克）（p<0.001）。与食用原来的蛋白质替代品相比，能量摄入的中位变化下降了10%（95%置信区间2.0至18.0）。服用PKU Express时，所有营养素的摄入量均超过膳食参考值，但均不过高。试验期间血液苯丙氨酸平均下降了37微摩尔/升（95%置信区间 - 27至102）。40%的受试者体重指数下降。血液苯丙氨酸或体重指数的变化无统计学意义。大多数营养、血液学和生化指标在所研究的分析物的正常参考范围内。16名（70%）受试者开始时血浆硒水平较低，但研究结束时只有13名（57%）。研究开始时8名受试者血浆维生素B12水平较高，结束时为9名。21名受试者（96%）表示该产品方便且易于制备。然而，7名（32%）受试者称其气味以及9名（46%）受试者称其质地与之前的蛋白质替代品相同或更差。由于使用了预先计量的小包装，一些受试者首次能够自行制备蛋白质替代品。PKU Express是一种安全、有效的蛋白质替代品，可显著减少规定蛋白质替代品的每日用量。
