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首页> 美国卫生研究院文献>Nutrients >Large Neutral Amino Acids (LNAAs) Supplementation Improves Neuropsychological Performances in Adult Patients with Phenylketonuria
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Large Neutral Amino Acids (LNAAs) Supplementation Improves Neuropsychological Performances in Adult Patients with Phenylketonuria

机译：大型中性氨基酸（LNAAs）补充改善了成人苯丙酮尿症患者的神经心理表现

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摘要

Phenylketonuria is an inborn error of phenylalanine (Phe) metabolism diagnosed by newborn screening and treated early with diet. Although diet prevents intellectual disability, patients often show impairment of executive functions, working memory, sustained attention, and cognitive flexibility. Large neutral amino acids (LNAAs) have been proposed as a dietary supplement for PKU adults. Few studies show that LNAAs may help in improving metabolic control as well as cognitive functions. In this study, 10 adult PKU patients with poor metabolic control were treated for 12 months with LNAAs (MovisCom, 0.8–1 g/kg/day) and underwent Phe and Tyrosine (Tyr) monitoring monthly. Neuropsychological assessment was performed at T0, T+3, and T+12 months by using the American Psychological General Well-Being Index, the Wisconsin Card Sorting Test, the Test of Attentional Performance, and the 9-Hole Peg Test. No change in plasma Phe levels was observed during LNAAs supplementation, while Tyr levels significantly improved during LNAAs supplementation ( = 0.03). Psychometric tests showed an improvement of distress and well-being rates, of executive functions, attention, and vigilance, whereas no difference was noted regarding hand dexterity. This study adds evidence of the advantage of LNAAs supplementation in improving cognitive functions and well-being in patients with PKU with poor metabolic control.
机译：苯丙酮尿症是苯丙氨酸（Phe）代谢的先天性错误，可通过新生儿筛查诊断出来，并尽早进行饮食治疗。尽管饮食可以预防智力障碍，但患者通常会表现出执行功能，工作记忆，持续注意力和认知灵活性受损。大中性氨基酸（LNAAs）已被提议作为北大成人的膳食补充剂。很少有研究表明，LNAA可能有助于改善代谢控制以及认知功能。在这项研究中，对10名代谢控制不佳的成人PKU患者进行LNAA治疗（MovisCom，0.8-1 g / kg /天），治疗12个月，并每月进行苯丙氨酸和酪氨酸（Tyr）监测。使用美国心理一般健康指数，威斯康星卡片分类测试，注意力表现测试和9孔钉测试，在T0，T + 3和T + 12个月进行神经心理学评估。在补充LNAA期间未观察到血浆Phe水平的变化，而在补充LNAA期间Tyr水平显着提高（= 0.03）。心理测验显示苦恼和幸福感，执行功能，注意力和警觉性得到改善，而手部敏捷度则没有差异。这项研究增加了补充LNAAs改善代谢控制不良的PKU患者的认知功能和幸福感的证据。

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