“鼠尿儿”每年增两千 "Rat urine son"Increased each year two thousand

　　最馋米饭馒头 The most delicious rice steamed bread

　　多吃肉蛋奶会变痴呆 Much meat egg milk will become dementia

　　有这样一群孩子，他们的一生注定与美食无缘，他们不能吃任何含蛋白质的食物，面粉、大米、肉蛋、海鲜……统统都是他们的“健康杀手”，只要吃多了这些食物，他们就会变成痴呆患儿。这群可怜的孩子所患的是一种名叫苯丙酮尿症的罕见病。在昨天举行的“苯丙酮尿症患儿特殊奶粉补助项目”中期总结会上，专家表示，我国近年来每年大约新增2000例这样的病例。

Have such a group of children,Their life is bound to be for food,They can't eat any protein food,flour/rice/Meat and eggs/Seafood...They were all of them"Health killer",As long as to eat many these foods,They will become children with dementia.This group of poor children suffer from is a named phenylketonuria syndrome rare disease.At yesterday's"Propiophenone enuresis children special milk powder subsidy project"Summing-up meeting in the middle of the,Experts say,In recent years in China each year about 2000 new cases of such cases.

　　苯丙酮尿症，缩写为PKU，是一种罕见的遗传疾病。患者无法正常代谢一种名叫“苯丙氨酸”的氨基酸，一旦摄入过量便会导致大脑发育受损。然而，这种氨基酸广泛地存在于蛋白质当中。这意味着，我们生活中绝大多数的食物对苯丙酮尿症患者来说都是需要一生提防的神经毒药。

Phenylketonuria disease,Abbreviation for PKU,Is a rare genetic disease.Patients can't normal metabolism a name"phenylalanine"Amino acid,Once the excessive intake will lead to damaged brain development.however,This kind of amino acid widely lies of protein.This means that,We are living in the vast majority of food for phenylketonuria disease patients are for a lifetime to beware of nerve poison.

　　延庆县的小鹏就是一名苯丙酮尿症患儿。他出生时头发很黑，后来就变得又黄又稀，尿液也有一股特殊的味道，后来被确诊为苯丙酮尿症。小鹏的妈妈说，正常人所吃的鸡、鸭、肉等佳肴，母乳和普通粮食都可能伤害小鹏的大脑，他只能吃特殊配置的无(低)苯丙氨酸的奶粉、面粉、土豆和部分果蔬等。这种“病号餐”价格昂贵，色泽、味道也跟正常的饭菜相差甚远，顿顿吃这样的“病号餐”，小鹏早已味同嚼蜡，每每看到别人吃馒头、米饭，他就会馋得眼睛发亮。

Yanqing county of XiaoPeng is a propiophenone enuresis children.He was born of the hair when very black,Then becomes and yellow and thin,Urine also has a special taste,Later was diagnosed with phenylketonuria disorder.XiaoPeng mom said,Normal person eat chicken/duck/Meat delicacies such as,Breast milk and ordinary food could hurt XiaoPeng brain,He can only eat special configuration of no(low)Phenylalanine of milk powder/flour/Potatoes and some fruits and vegetables, etc.this"Patient eat"expensive,Colour and lustre/Taste with normal food differs very far,Repeatedly eating such"Patient eat",XiaoPeng already as dry as dust,Often see the other people to eat steamed bread/rice,He will chan that eyes shine.

　　专家介绍，苯丙酮尿症患儿通常皮肤色浅、头发细黄，不少家长会认为孩子只是长得像白种人娃娃，却不知道孩子可能已被PKU这个隐性“杀手”缠上。这些孩子出生时没有任何异常，可能会在3至6个月后出现比较频繁的呕吐，皮肤出现湿疹或奶癣，头发逐渐转黄，皮肤和虹膜色浅，尿液和汗有一种特殊的臭味，就像老鼠尿味。同时患儿智力发育落后的症状也开始有所体现，如不会抬头，无法集中注意力等；6个月后的患儿会有“软”的表现，例如不会爬、扶着站立时会突然“软趴”下去，近半数的患儿还合并有癫痫症发作。这些孩子如果得不到及时、持续的治疗，就会最终造成中度至极重度的智力低下，成为智障，无法生活自理，甚至可能死亡。

Expert introduction,Propiophenone enuresis children usually skin color is light/Hair fine yellow,Many parents think children just look like white doll,But he didn't know the child may have been PKU the recessive"killer"Tangle on.These children were born without any exception,May be in 3 to 6 months after appearance is quite frequent vomiting,The skin appear eczema or milk tinea,Hair gradually turn yellow,The skin and iris color is light,Urine and sweat have a kind of special odor,Be like a rat urine taste.At the same time the development of children intelligence behind the symptoms also beginning to reflect,If not looked up,Lack of concentration, etc;After six months of children will have"soft"performance,For example won't climb/To hold the stand when it"Soft grovel"down,Nearly half of the children were with epilepsy attack.These children if not promptly/Continuous treatment,Will eventually cause moderate extremely severe mental retardation,Become mentally disabled,Cannot independent living,May even death.

　　专家说，“鼠尿儿”并非无药可救，关键是保护大脑发育尽可能不受损。患儿在1个月内接受治疗，可不出现智力损害；半岁开始治疗，智商可接近正常；1岁以后开始治疗，智商常在50以下。然而，最让父母担忧的还不是医疗技术，而是特殊食品的成本。患儿所需的低苯丙氨酸食品价格远高于普通食品，很多不富裕的家庭无力承担。2009年，全国妇幼卫生监测办公室与美赞臣营养品(中国)有限公司合作发起了“苯丙酮尿症(PKU)患儿特殊奶粉补助项目”，这也是国内首个关注PKU患儿的项目，由美赞臣公司在5年内向中西部地区的PKU新生儿免费提供美赞臣无苯丙氨酸配方粉。目前，这一项目已为在中西部地区募集的509名贫困PKU患儿免费提供了美赞臣无苯丙氨酸配方粉补助，建立了规范的PKU治疗随访和管理体系。(记者代丽丽)

Experts say,"Rat urine son"Not hopeless,The key is to protect brain development as far as possible not damaged.Children treated in one month,Don't have mental damage;Half years old start treatment,Intelligence quotient (IQ) can be close to normal;1 years later start treatment,Intelligence quotient (IQ) often under 50.however,The most let parents concern is not the medical technology,But the cost of the special food.For children with low phenylalanine food prices far higher than ordinary food,Many don't wealthy family cannot afford.In 2009,,The maternal and child health monitoring office and MeiZanChen nourishment(China)Co., LTD., cooperation launched"Phenylketonuria disease(PKU)With special milk powder subsidy project",This is the first domestic PKU children pay attention to the project,By MeiZanChen company in five years the to the Midwest PKU neonatal provide free MeiZanChen no phenylalanine formula powder.At present,This project has been in the Midwest for raising of PKU 509 poor children free provides MeiZanChen no phenylalanine formula powder subsidies,Has established the standard PKU follow-up treatment and management system.(Reporter generation lily)