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Study on the Development of the Therapeutic Formula for Inborn Errors of Amino Acid Metabolism Consisting of Synthetic Peptide as a Nitrogen Source

以合成肽为氮源的先天性氨基酸代谢缺陷治疗配方的研制

基本信息

批准号：
62870040
负责人：
KITAGAWA Teruo
金额：
$ 7.74万
依托单位：
Nihon University
依托单位国家：
日本
项目类别：
Grant-in-Aid for Developmental Scientific Research
财政年份：
1987
资助国家：
日本
起止时间：
1987 至 1989
项目状态：
已结题

项目摘要

During the 3 year period, 260 kg of low-phenylalanine peptides (LPP) were obtained by enzymic hydrolysis of whey protein and 1350 kg of therapeutic formula for PKU consisting of LPP as a nitrogen source were prepared. In animal experiments we confirmed that LPPs were more rapidly absorbed than amino acids. Because the palatability as well as nutritional quality seventeen infants and young children with PKU were treated with LPP formula for more than one year. Five of them have been on LPP formula for more than 4 years. In four of 17 cases who have been on LPP formula serum phenylalanine (Phe) levels were difficult to maintain below 8 mg/dl because lOOg of LPP formula contain 69 mg of Phe. In those cases, a mixture of 19 kinds of amino acids except Phe were supplemented. Normal growth was observed in all these infants and children. DQ or IQ were all within the normal range. The serum ami no acid levels in these cases were compatible to those during treatment with conventional therapeutic formula consisting of amino acids as a nitrogen source. Previously, we succeeded to have the first case of maternal PKU where a normal baby was born following dietary treatment using the LPP formula started in the preconceptional period. This woman expected to have a second baby and she received LPP formula and solid food cooked with LPP powder prior to and during pregnancy. Her blood Phe levels maintained below less than 10 mg/dl. The 2nd baby was born without any complication related to the maternal PKU. Development of LPP powder and formula resulted not only a better prognosis of infants and children with PKU but also successful prevention of maternal PKU in Japan.

在3年的时间里，通过酶解乳清蛋白获得260 kg低苯丙氨酸多肽(LPP)，并制备了以LPP为氮源的1350 kg PKU治疗配方。在动物实验中，我们证实了LPPs比氨基酸吸收更快。由于适口性和营养质量的原因，17例婴幼儿PKU患者使用LPP配方奶粉治疗一年以上。其中五人服用LPP配方奶粉超过4年。在17例服用LPP配方奶粉的患者中，有4例的血清苯丙氨酸(Phe)水平很难维持在8 mg/dl以下，因为LPP配方奶粉中含有69 mg Phe。在这些情况下，除了苯丙氨酸之外，还添加了19种氨基酸的混合物。所有这些婴儿和儿童都观察到了正常的生长。DQ或IQ均在正常范围内。这些病例的血清氨基酸值与传统的以氨基酸为氮源的治疗方案相容。在此之前，我们成功地进行了首例母体型PKU，其中正常婴儿出生于妊娠期前开始使用LPP配方的饮食治疗。这名妇女希望生第二个孩子，她在怀孕前和怀孕期间接受了LPP配方奶粉和用LPP粉末烹调的固体食物。她的血液苯丙氨酸水平维持在10毫克/分升以下。第二个婴儿出生时没有任何与母体PKU相关的并发症。在日本，LPP粉剂和配方奶粉的开发不仅改善了婴幼儿PKU的预后，而且成功地预防了母体PKU。