Acosta P B, Yannicelli S, Marriage B, Mantia C, Gaffield B, Porterfield M, Hunt M, McMaster N, Bernstein L, Parton P, Kuehn M, Lewis V
Medical Department, Ross Products Division/Abbott Laboratories, Columbus, Ohio 43215, USA.
J Pediatr Gastroenterol Nutr. 1998 Sep;27(3):287-91. doi: 10.1097/00005176-199809000-00003.
Because of reports of poor growth, a study was conducted for 6 months in 35 infants with classic phenylketonuria diagnosed during the neonatal period who were fed Phenex-1 Amino Acid Modified Medical Food With Iron (Ross Products Division, Columbus, OH, U.S.A.).as their primary protein source.
Diet diaries and anthropometric measures were obtained monthly as part of a larger study in which nutrition status was evaluated.
In 6-month-old infants, mean percentiles for crown-heel length (59.14+/-4.31 SEM), head circumference (63.88+/-4.50) and weight (71.51+/-4.25) were normal. Mean (+/- SEM) daily intake of medical food was 79+/-4 g; protein and energy intakes were 17.3+/-0.6 g and 2772+/-75.6 kJ (660+/-18 kcal). Mean daily phenylalanine and tyrosine intakes per kilogram of body weight were 40+/-1 mg and 219+/-9 mg. Intakes of protein, energy, and tyrosine were positively correlated with crown-heel length, head circumference, and weight at 3 months of study. Overall plasma phenylalanine and tyrosine concentrations during the 6-month study were 297+/-41 micromol/l and 58+/-5 micromol/l, respectively. Neither plasma phenylalanine nor tyrosine concentration was correlated with growth.
Phenex-1 supports normal growth when fed in adequate amounts. These data support those of the Medical Research Council Working Party on Phenylketonuria for 3 g/kg per day of amino acids from medical food.
由于有生长发育不良的报告，对35名新生儿期诊断为经典型苯丙酮尿症的婴儿进行了为期6个月的研究，这些婴儿以含铁血苯丙氨酸-1氨基酸改良医用食品（美国俄亥俄州哥伦布市罗斯产品部）作为主要蛋白质来源。
作为一项评估营养状况的更大规模研究的一部分，每月获取饮食日记和人体测量数据。
6个月大的婴儿，顶臀长（59.14±4.31标准误）、头围（63.88±4.50）和体重（71.51±4.25）的平均百分位数正常。医用食品的平均每日摄入量为79±4克；蛋白质和能量摄入量分别为17.3±0.6克和2772±75.6千焦（660±18千卡）。每千克体重的平均每日苯丙氨酸和酪氨酸摄入量分别为40±1毫克和219±9毫克。在研究3个月时，蛋白质、能量和酪氨酸的摄入量与顶臀长、头围和体重呈正相关。在为期6个月的研究中，血浆苯丙氨酸和酪氨酸的总体浓度分别为297±41微摩尔/升和58±5微摩尔/升。血浆苯丙氨酸和酪氨酸浓度均与生长无关。
适量喂食时，苯丙氨酸-1支持正常生长。这些数据支持医学研究委员会苯丙酮尿症工作组关于每天从医用食品中摄入3克/千克氨基酸的数据。
