贪得无厌的饥饿和低代谢
Although it is rare, Prader Willi Syndrome (PWS) is the most common genetic cause of potentially life-threatening childhood obesity. The Prader-Willi Syndrome Association explains, "A chronic feeling of hunger that, coupled with a metabolism that utilizes drastically fewer calories than normal, can lead to过量进食和威胁生命的肥胖“。
国际普拉德 - 威利综合征组织（IPWSO）指出，普拉德 - 威利综合征的两个“标志性”是非常低的代谢和渴求永无止境。IPWSO报价汉斯Zellwegger博士关于这个强迫进食的程度，“PWS儿童经常出现的困扰一个无情的饥饿感，主宰自己的生活。The presence of this insatiable urge to eat, which is beyond the control of the patient, his family, or physician, becomes the primary focus for the child and inhibits all other activities and interest."
用PWS儿童的父母往往有限制儿童的食物进入并锁定橱柜和冰箱。
普瑞德威利症候群的症状
Infants with Prader Willi Syndrome present with weak muscle tone and reduced bone mineral density, have difficulty feeding, poor growth, and delayed development. At first, they will have trouble growing and gaining weight. Then in early childhood, usually between 1 and 6 years of age, the appetite becomes exaggerated to an extreme that leads to chronic consumption and obesity.
Infants with Prader Willi Syndrome may exhibit symptoms出生时，如小和软盘的外观。相较于身体的其他部分时，其他的物理外观是杏仁状的眼睛，一个狭窄的双额颅骨，身材矮小，和非常小的手和脚。男性新生儿可能有隐睾症。儿童可能推迟或不完整的性发育。
谁拥有普瑞德威利症候群孩子有一定的认知障碍和学习障碍，以及较差的运动技能。发脾气，固执，和强迫行为和睡眠障碍也很常见。
Most patients with PWS are incapable of having children.
普瑞德威利症候群的原因
Discovered in 1956, doctors at first had no idea about what was causing the features of Prader-Willi Syndrome. In 1981, it was discovered that many afflicted with PWS had the same gene segment missing from one of their chromosomes. It also was found that the missing gene segment on chromosome 15 was responsible for about seventy percent of Prader Willi Syndrome cases.
在情况的另一个25％，而普拉德威利综合征折磨的人将继承从母亲15号染色体的两个副本instead of a single copy from each parent.
All children of all races are susceptible and the possibility of a child having it is about one in fifteen thousand.
治疗普瑞德威利症候群
有没有治疗的鲜红斑痣。生长激素已获得美国食品和药物管理局治疗普瑞德威利症候群。这种激素有助于提高力量和敏捷，身高，肌肉质量，重量，体力分配和骨矿物质密度。有人担心，服用生长激素可能导致睡眠呼吸暂停，让儿童接受生长激素治疗需要每年睡眠研究。
与综合症的儿童，最好由多学科方法，其中包括生长激素替代，物理治疗，职业治疗，言语和语言支持，学习障碍服务行为管理帮助，家人的支持，以及饮食和锻炼服务。
普瑞德威利症候群的饮食
普拉德 - 威利综合征协会笔记儿童PWS需要保持在一个低热量饮食。标准的食物指南金字塔由农业部美国农业部设计需要修改，以解决一个普拉德威利饮食，因为广义的部分太多了病痛的孩子，对于他们的新陈代谢。
推荐1600至每天2800卡路里的热量，应减少到约800至1200推荐份量煮熟或生蔬菜的半杯，面包1片，米半杯，麦片1盎司，½水果或可以的½果汁杯，1杯脱脂牛奶，奶酪1盎司，半无脂无糖酸奶2盎司瘦的肉，鱼，家禽，鸡蛋1杯，花生酱1汤匙。
Fats, oils, and sweets such as butter, sugar, sweets, gravy, and fried foods should be limited to one time per month because they are nutrient deficient and add unwanted calories to Prader Willi diet.
普拉德 - 威利综合征协会报告PWS患者可能需要持续不断的监督来限制整个成年生活他们获得食物。没有食欲抑制剂已经发现，将始终如一地缓解饥饿感在谁拥有普拉德 - 威利综合征的人。
References:
New York Times -http://health.nytimes.com/health/guides/disease/prader-willi-syndrome/overview.html- 访问12年8月11日
普瑞德威利症候群协会 -http://www.pwsausa.org/syndrome/Genetics_of_PWS.htm- 访问12年8月11日
考研健康 -http://www.medicinenet.com/prader-willi_syndrome/page2.htm#what_are_the_signs_symptoms_and_features_of_prader-willi_syndrome- 访问
WebMD表示 -http://www.medicinenet.com/prader-willi_syndrome/page2.htm#what_are_the_signs_symptoms_and_features_of_prader-willi_syndrome- 访问12年8月11日
International Prader Willi Syndrome Organisationhttp://www.ipwso.org/- 访问12年8月14日
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