CN107751998A - 一种用于苯丙酮尿症患者的食用配方粉 - Google Patents
一种用于苯丙酮尿症患者的食用配方粉 Download PDFInfo
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Abstract
本发明涉及一种用于苯丙酮尿症患者的食用配方粉，按重量计包含以下组分：30‑85份植物油脂肪粉，15‑60份复合氨基酸，0.01‑1份核苷酸，2‑5份复合维生素和2‑5份复合矿物质，所述食用配方粉中不包含苯丙氨酸。本发明的食用配方粉以脂肪、碳水化合物、氨基酸、维生素、矿物质等为基本的处方组成，配比科学，能满足婴幼儿的生长发育的需要。所述配方粉为机体提供能量，补充生长发育过程中必需的氨基酸，并且不添加苯丙氨酸，一方面不产生苯丙氨酸及其酮酸的蓄积，另一方面保证患者的氨基酸摄入，维持机体的氨基酸正常代谢。所述食用配方粉还可包含0.01‑1重量份的复合不饱和脂肪酸以满足0‑10岁的患儿的营养需求，促进大脑的发育，提高认知能力。
Description
技术领域
本发明涉及苯丙酮尿症治疗领域，更多地，涉及一种可用于苯丙酮尿症患者的食用配方粉。
背景技术
苯丙酮尿症(phenylketonuria，PKU)是一种常见的氨基酸代谢障碍疾病，是由于苯丙氨酸(PA)代谢途径中的酶缺陷，使得苯丙氨酸不能转变成为酪氨酸，导致苯丙氨酸及其酮酸蓄积，并从尿中大量排出。本病在遗传性氨基酸代谢缺陷疾病中比较常见，发病率为1/10000。其遗传方式为常染色体隐性遗传。临床表现不均一，主要临床特征为智力低下、精神神经症状、湿疹、皮肤抓痕征及色素脱失和鼠气味等、脑电图异常。如果能得到早期诊断和早期治疗，则前述临床表现可不发生，智力正常，脑电图异常也可得到恢复。
由于一般的食物、奶粉和母乳中都包含有苯丙氨酸，为了应对苯丙酮尿症，需要对患者进行饮食控制，限制苯丙氨酸的摄入。
发明内容
为解决以上问题，本发明提供了一种用于苯丙酮尿症患者的食用配方粉，其特征在于，按重量计包含以下组分：30-85份植物油脂肪粉，15-60份复合氨基酸，0.01-1份核苷酸，2-5份复合维生素和2-5份复合矿物质，所述食用配方粉中不包含苯丙氨酸。
本发明的食用配方粉以脂肪、碳水化合物、氨基酸、维生素、矿物质等为基本的处方组成，配比科学，能满足婴幼儿的生长发育的需要。其中植物油脂肪粉中含有脂肪和碳水化合物，为机体提供能量，复合氨基酸为人体补充生长发育过程中必需的氨基酸，并且不添加苯丙氨酸，一方面不产生苯丙氨酸及其酮酸的蓄积，另一方面保证患者的氨基酸摄入，维持机体的氨基酸正常代谢。
优选地，所述复合氨基酸由以下氨基酸组成：L－丙氨酸；L－精氨酸和/或L－盐酸精氨酸、L－天冬氨酸和/或L－天冬氨酸镁；L－胱氨酸、L－半胱氨酸、L－盐酸半胱氨酸中的一种或多种组合；L－谷氨酸、L－谷氨酸钾、L－谷氨酰胺中的一种或多种组合；甘氨酸；L－组氨酸和/或L－盐酸组氨酸；L－异亮氨酸、L－亮氨酸；L－盐酸赖氨酸和/或L－赖氨酸醋酸盐；L－甲硫氨酸和/或L－乙酰－甲硫氨酸；L－脯氨酸；L－丝氨酸；L－苏氨酸；L－色氨酸；L－酪氨酸；L－缬氨酸，并且L－酪氨酸占所述复合氨基酸重量的6-15％。
优选地，所述复合氨基酸按重量计由以下组分组成：14份L－丙氨酸、24份L－精氨酸、35份L－天冬氨酸、9份L－胱氨酸、28份L－谷氨酰胺、36份甘氨酸、14份L－组氨酸、24份L－异亮氨酸、37份L－亮氨酸、25份L－盐酸赖氨酸、7份L－甲硫氨酸、26份L－脯氨酸、16份L－丝氨酸、25份L－苏氨酸、L－色氨酸8份、36份L－酪氨酸和28份L－缬氨酸。
优选地，所述植物油脂肪粉由固体葡萄糖浆、预糊化淀粉、脂肪酸、乳化剂通过喷雾干燥而制成。植物脂肪粉中包含脂肪和碳水化合物，为机体提供能量。
优选地，所述食用配方粉还包含0.01-1重量份的复合不饱和脂肪酸，所述复合不饱和脂肪酸由DHA和ARA组成，DHA与ARA的重量比为1:1-6:1。含有复合不饱和脂肪酸的食用配方粉可用于0-10岁的患儿，长链不饱和脂肪酸，促进大脑的发育，提高认知能力；促进身体发育，保证健康体质；保证视力发育，降低视力缺陷；呵护早产婴儿，提高健康程度；支持免疫发育，提高呼吸系统健康。10岁以上的患者的食用配方粉可不需要包含所述复合不饱和脂肪酸。
优选地，所述核苷酸由腺苷酸、尿苷酸、鸟苷酸、胞苷酸、肌苷酸组成，重量比为5:5:2:7:3。
优选地，所述复合维生素按重量计由以下组分组成：64份维生素A、740份维生素C、29份维生素D、350份维生素E、10份维生素K1、16份维生素B、32份维生素B2、10份维生素B6、16份维生素B12、27份烟酸、26份叶酸、58份泛酸、16份生物素、350份肌醇、1495份胆碱和340份牛磺酸。复合维生素为患者提供身体所需的维生素，参与身体的各种生化反应。
优选地，所述复合矿物质按重量计由以下成分组成：3000份钠、5185份氯、4000份钾、8400份钙、4000份磷、760份铁、35份锌、19份锰、40份铜、1975份镁、30份硒和50份碘。矿物质是构成机体组织的重要成份；维持机体的酸碱平衡及组织细胞渗透压；维持神经肌肉兴奋性和细胞膜的通透性。
优选地，所述食用配方粉还包含0-1重量份的香精。所述香精为巧克力、香蕉、草莓、橙子、西柚及其他水果味中的一种。香精的添加可为本发明的食用配方粉提供特有的风味，以适合不同的患者。
具体实施方式
以下结合实例对本发明的原理和特征进行描述，所举实例只用于解释本发明，并非用于限定本发明的范围。
实施例
本发明的实施例中通过以下方法制备食用配方粉。
1)将复合不饱和脂肪酸、核苷酸、复合维生素、复合矿物质、香精分别过80目筛；
2)将复合不饱和脂肪酸、核苷酸、复合维生素、复合矿物质、香精投入小型混料器中初步混合，得到混合料；
3)称取氨基酸混合，得到复合氨基酸；
4)称取植物油脂肪粉，投入混料机中，加入混合料和复合氨基酸，混合15-30分钟，得到食用配方粉。
在以上步骤中，所加的原料的重量分别如表1所示。
表1 各实施例中所加的组分的量
其中，复合氨基酸按重量计由14份L－丙氨酸、24份L－精氨酸、35份L－天冬氨酸、9份L－胱氨酸、28份L－谷氨酰胺、36份甘氨酸、14份L－组氨酸、24份L－异亮氨酸、37份L－亮氨酸、25份L－盐酸赖氨酸、7份L－甲硫氨酸、26份L－脯氨酸、16份L－丝氨酸、25份L－苏氨酸、L－色氨酸8份、36份L－酪氨酸和28份L－缬氨酸组成；
植物油脂肪粉由固体葡萄糖浆、预糊化淀粉、脂肪酸、乳化剂通过喷雾干燥而制成；
核苷酸由腺苷酸、尿苷酸、鸟苷酸、胞苷酸、肌苷酸，重量比为5:5:2:7:3；
复合不饱和脂肪酸由DHA和ARA组成，DHA与ARA的重量比为1：1-6:1，；
复合维生素按重量计由以下组分组成：64份维生素A、740份维生素C、29份维生素D、350份维生素E、10份维生素K1、16份维生素B、32份维生素B2、10份维生素B6、16份维生素B12、27份烟酸、26份叶酸、58份泛酸、16份生物素、350份肌醇、1495份胆碱和340份牛磺酸；
复合矿物质按重量计由以下成分组成：3000份钠、5185份氯、4000份钾、8400份钙、4000份磷、760份铁、35份锌、19份锰、40份铜、1975份镁、30份硒和50份碘；
所述香精为巧克力、香蕉、草莓、橙子、西柚及其他水果味中的一种。
将实施例1-5得到的食用配方粉用于苯丙酮尿症患儿食用，患儿生长发育正常，智力发展与常人无异。可见本发明的食用配方粉可为苯丙酮尿症患儿提供良好的营养，满足了患儿的生长发育需要。
以上所述仅为本发明的较佳实施例，并不用以限制本发明，凡在本发明的精神和原则之内，所作的任何修改、等同替换、改进等，均应包含在本发明的保护范围之内。
Claims (9)
1.一种用于苯丙酮尿症患者的食用配方粉，其特征在于，按重量计包含以下组分：30-85份植物油脂肪粉，15-60份复合氨基酸，0.01-1份核苷酸，2-5份复合维生素和2-5份复合矿物质，所述食用配方粉中不包含苯丙氨酸。
2.根据权利要求1所述的食用配方粉，其特征在于，所述复合氨基酸由以下氨基酸组成：L－丙氨酸；L－精氨酸和/或L－盐酸精氨酸、L－天冬氨酸和/或L－天冬氨酸镁；L－胱氨酸、L－半胱氨酸、L－盐酸半胱氨酸中的一种或多种组合；L－谷氨酸、L－谷氨酸钾、L－谷氨酰胺中的一种或多种组合；甘氨酸；L－组氨酸和/或L－盐酸组氨酸；L－异亮氨酸、L－亮氨酸；L－盐酸赖氨酸和/或L－赖氨酸醋酸盐；L－甲硫氨酸和/或L－乙酰－甲硫氨酸；L－脯氨酸；L－丝氨酸；L－苏氨酸；L－色氨酸；L－酪氨酸；L－缬氨酸，并且L－酪氨酸占所述复合氨基酸重量的6-15％。
3.根据权利要求2所述的食用配方粉，其特征在于，所述复合氨基酸按重量计由以下组分组成：14份L－丙氨酸、24份L－精氨酸、35份L－天冬氨酸、9份L－胱氨酸、28份L－谷氨酰胺、36份甘氨酸、14份L－组氨酸、24份L－异亮氨酸、37份L－亮氨酸、25份L－盐酸赖氨酸、7份L－甲硫氨酸、26份L－脯氨酸、16份L－丝氨酸、25份L－苏氨酸、L－色氨酸8份、36份L－酪氨酸和28份L－缬氨酸。
4.根据权利要求1所述的食用配方粉，其特征在于，所述植物油脂肪粉由固体葡萄糖浆、预糊化淀粉、脂肪酸、乳化剂通过喷雾干燥而制成。
5.根据权利要求1所述的食用配方粉，其特征在于，还包含0.01-1重量份的复合不饱和脂肪酸，所述复合不饱和脂肪酸由DHA和ARA组成，DHA与ARA的重量比为1:1-6:1。
6.根据权利要求1所述的食用配方粉，其特征在于，所述核苷酸由腺苷酸、尿苷酸、鸟苷酸、胞苷酸、肌苷酸，重量比为5:5:2:7:3。
7.根据权利要求1所述的食用配方粉，其特征在于，所述复合维生素按重量计由以下组分组成：64份维生素A、740份维生素C、29份维生素D、350份维生素E、10份维生素K1、16份维生素B、32份维生素B2、10份维生素B6、16份维生素B12、27份烟酸、26份叶酸、58份泛酸、16份生物素、350份肌醇、1495份胆碱和340份牛磺酸。
8.根据权利要求1所述的食用配方粉，其特征在于，所述复合矿物质按重量计由以下成分组成：3000份钠、5185份氯、4000份钾、8400份钙、4000份磷、760份铁、35份锌、19份锰、40份铜、1975份镁、30份硒和50份碘。
9.根据权利要求1-8中任一项所述的食用配方粉，其特征在于，还包含0-1重量份的香精。
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| WO2020010896A1 (zh) * | 2018-07-10 | 2020-01-16 | 宁波特壹食品有限公司 | 一种用于苯丙酮尿症患者的配方粉及其制备方法 |
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