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接受治疗的苯丙酮尿症患儿的营养摄入情况。

Nutrient intake of treated infants with phenylketonuria.

作者信息

Acosta P B, Wenz E, Williamson M

出版信息

Am J Clin Nutr. 1977 Feb;30(2):198-208. doi: 10.1093/ajcn/30.2.198.

Abstract

Growth, energy, and nutrient intake of 88 treated infants in the Collaborative Study of Children Treated for Phenylketonuria were evaluated longitudinally and compared to normative data and by treatment group. Growth parameters (height and weight) did not differ according to treatment group assignment, nor did they differ from normative data. Subjects in treatment group 2 had a significantly higher intake of phenylalanine than did subjects in treatment group 1. Differences in intake of other nutrients disappeared when intake was compared on an energy or body weight basis. Differences in intake by males and females also disappeared when compared on an energy and a body weight basis. From none to 10% of the subjects had energy intakes below two-thirds of the 1968 Recommended Dietary Allowances (RDA). Low individual energy intakes were more commonly found in the first and fourth quarters, and only during the first quarter of infancy did mean energy intake meet the RDA. From 48 to 80% of subjects had intakes of preformed niacin below two-thirds of the 1968 RDA. Few subjects had low intakes of protein, iron, or vitamin A, and intakes of all subjects were greater than 67% of the RDA for calcium, phosphorus, thiamin, riboflavin, and ascorbic acid. It is suggested from the data presented that a supplemental casein hydrolysate supplies adequate essential amino acids and nitrogen to support normal growth in infants when protein and energy intakes are fed at the levels described.

摘要

在苯丙酮尿症患儿协作研究中,对88名接受治疗的婴儿的生长、能量和营养摄入情况进行了纵向评估,并与标准数据进行比较,同时按治疗组进行了比较。生长参数(身高和体重)在不同治疗组之间没有差异,与标准数据也没有差异。治疗组2的受试者苯丙氨酸摄入量显著高于治疗组1的受试者。当按能量或体重进行比较时,其他营养素摄入量的差异消失。按能量和体重进行比较时,男性和女性的摄入量差异也消失。从0%到10%的受试者能量摄入量低于1968年推荐膳食摄入量(RDA)的三分之二。个体能量摄入量低在第一季度和第四季度更为常见,且仅在婴儿期的第一季度平均能量摄入量达到RDA。48%至80%的受试者预形成烟酸摄入量低于1968年RDA的三分之二。很少有受试者蛋白质、铁或维生素A摄入量低,所有受试者钙、磷、硫胺素、核黄素和抗坏血酸的摄入量均大于RDA的67%。根据所呈现的数据表明,当蛋白质和能量摄入量按所述水平供给时,补充酪蛋白水解物可为婴儿提供足够的必需氨基酸和氮以支持正常生长。

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