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产品名称:能全特®XP-2特殊配方营养粉

通用名称:苯丙酮尿症系列1岁以上至成人期专用

产品特性:

- 无苯丙氨酸配方, 营养要素全面均衡
- 高浓度蛋白质,适用广泛
- 100%英国原装进口,质检严格,安全双重保障


产品网站链接:www.china-pku.com

 

关于PKU更多治疗和产品信息,可咨询各大城市纽迪希亚儿科营养顾问。


  • 处方资料摘要
  • 产品文献
  • FAQ
  • 科室应用
能全特®XP-2的使用方法:
1、根据中国营养学会推荐《中国居民膳食营养素参考摄入量》,计算每日蛋白质摄入总量,平均分成3-4次;
2. 冲调:将10g能全特® XP-2溶解在100-150ml 液体里到达渗透压;可添加至低苯丙氨酸的果汁、菜汤或粥中,或添加至固体饭菜中,同时配以适量饮水。 添加番茄汁或葡萄汁味道最佳。

In this paper, we review neuropsychological test results of early arid continuously treated Phenylketonuria (PKU) patients. To increase insight into the neuropsychological profile of this population, we have attempted to place the results within attentional network model, which proposes interacting but dissociable attentional networks for orienting, vigilance, and executive control of attention. Executive control of attention is discussed against the background of the process-specific theory of working memory (WM) | Handbook of neuropsychology, 1994|, which postulates a distinction between the maintenance-function of WM and the manipulation and monitoring-function. Neuropsychological results are presented for 67 early and continuously treated PKU patients and 73 controls aged 7-14 years. Four neuropsychological tasks were employed to measure orienting, mnemonic processing,interference suppression, and top-down control in visual search. No differences were found in orienting and the maintenance-function of WM. In addition to previously reported impairments in sustained attention/vigilance and inhibition of prepotent responding, FKU patients exhibited deficits when top-down control was required in a visual search task, but showed no impairment when interference suppression was required. It is discussed how the specific neuropsychological impairments in PKU may be a consequence of mid-dorsolateral prefrontal cortex (DLPFC) dysfunctioning due lo deficiencies in catecholamine modulation.

The neuropsychological profile of early and continuously treated phenylketonuria: orienting, vigilance, and maintenance versus manipulation-functions of working memory

Huijbregts SC, de Sonneville LM .Neurosci Biobehav Rev. 2002 Oct;26(6):697-712.

Results: Data from 1921 patients on dietary management were included. Blood Phe concentrations were well controlled and comparable across centers in the early years of life. The percentages of blood Phe concentrations meeting each centre's local and national target ranges were 88% in children aged up to 1 year, 74% for 1-10 years, 89% for 11-16 years and 65% for adults (>16 years). The frequency of home blood sampling, compared with local and national recommendations for monitoring Phe concentrations, appeared to decline with age (from approximately 100% in infancy to 83% in teenagers and 55% in adults). Conclusions: Although blood Phe control generally deteriorated with age, some improvement was observed in adolescent years across the 10 European centres. The blood Phe control achieved seemed comparable in many of the European centres irrespective of different dietary treatments or national policies.

Blood phenylalanine control in phenylketonuria: a survey of 10 European centres

Ahring K, Bélanger-Quintana A. Eur J Clin Nutr. 2011 Feb;65(2):275-8. Epub 2010 Dec 1.

Conclusion Due to the lack of internationally accepted guidelines, management of phenylketonuria still varies between different countries. Our efforts should focus on the formulation of internationally acceptable and accepted recommendations for the treatment of patients with phenylketonuria at different ages.

Survey of national guidelines for the treatment of phenylketonuria

Schweitzer-Krantz S, Burgard P. Eur J Pediatr. 2000 Oct;159 Suppl 2:S70-3.

Q:经典型PKU饮食治疗原则是什么?
A:“80+20”原则,即身体每日所需80%的蛋白质来源于无特殊配方营养粉(如能全特® XP系列),20%的蛋白质来源于天然食物(低苯丙氨酸的水果、蔬菜等)。
坚持称量食物、计算每日蛋白质和苯丙氨酸的摄入量。定期监测血尿中苯丙氨酸浓度
Q:为什么1岁以上PKU孩子建议食用能全特® XP-2?
A:能全特® XP-2是无苯丙氨酸配方,高浓度蛋白质含量,每100克含有75克蛋白质,最大程度增加对天然食物的摄取,满足饮食的多样性。同时足够的蛋白质促进钙的吸收,增强骨密度和利于牙齿的发育。能全特® XP-2每克优质蛋白质性价比高,且适用年龄广泛,无需为频繁适应口味而烦恼。
Q:当孩子1岁以后,能否长久用能全特®XP-1代替能全特®XP-2?
A:不同年龄的PKU应使用针对该年龄的配方。
孩子进入儿童期后,需要更多蛋白质保证机体和智力发育,更多充足的营养从天然食物中获取。只有每日机体所需80%-85%的蛋白质由无苯丙氨酸的高浓度蛋白质配方的能全特®XP-2提供,才有更多的机会摄取更多的天然食物。
  • 遗传代谢

    低苯丙氨酸治疗法是经典型苯丙酮尿症国际公认的唯一治疗有效手段。

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