Rodman J S, Blackburn P, Williams J J, Brown A, Pospischil M A, Peterson C M
Clin Nephrol. 1984 Dec;22(6):273-8.
Seven patients with homozygous cystinuria were studied on a metabolic ward to determine the effect of dietary manipulation on urinary cystine excretion. Isocaloric diets were calculated based on body weight and activity. Balance studies were performed for 5 days on a low protein diet (9.4 +/- 1.0% total calories) with equal amounts of animal and plant protein sources and an additional 5 days on a high protein (27 +/- 3.0% of total calories) with 70% animal protein. A significant (p less than 0.01) decrease in the excretion of 1/2-cystine, histidine, 3-methylhistidine, 1-methylhistidine, lysine, and ornithine plus arginine occurred on the low protein diet. The mean excretion of 1/2-cystine on the high protein diet was 6.13 +/- 1.48 mMoles per 24 hours which decreased to 4.89 +/- 1.06 mMoles per 24 hours on the low protein diet (p less than 0.001). Thirty seven percent of urine collections during the study were less than 2 liters/24 hours despite the fact that patients were encouraged to drink enough fluid to make 3 liters of urine/24 hours. For patients whose customary diet is high in animal protein, a more vegetarian diet may significantly reduce urinary cystine excretion. Cystine excretion in patients with cystinuria may be significantly different from day to day if animal protein consumption is variable.
对7名纯合子胱氨酸尿症患者在代谢病房进行了研究，以确定饮食控制对尿胱氨酸排泄的影响。根据体重和活动量计算等热量饮食。在低蛋白饮食（占总热量的9.4±1.0%）且动植物蛋白来源等量的情况下进行了5天的平衡研究，其中动物蛋白占70%，然后在高蛋白饮食（占总热量的27±3.0%）下又进行了5天的平衡研究。低蛋白饮食时，半胱氨酸、组氨酸、3-甲基组氨酸、1-甲基组氨酸、赖氨酸以及鸟氨酸加精氨酸的排泄量显著减少（p<0.01）。高蛋白饮食时半胱氨酸的平均排泄量为每24小时6.13±1.48毫摩尔，低蛋白饮食时降至每24小时4.89±1.06毫摩尔（p<0.001）。尽管鼓励患者饮用足够的液体以使尿量达到每24小时3升，但研究期间37%的尿液收集量不足每24小时2升。对于习惯饮食中动物蛋白含量高的患者，更多的素食饮食可能会显著降低尿胱氨酸排泄量。如果动物蛋白摄入量不稳定，胱氨酸尿症患者的胱氨酸排泄量可能每天都有显著差异。
