PRADER-WILLI综合征：原因，症状，治疗|学校努力

没有已知治愈;引起精神发育迟滞，身材矮小，低肌肉，不完全性发展，以及其主要特点，渴望吃一切和任何东西。Prader-Willi综合征首先被称为Prader-Labhart-Willi综合征，在1956年首次描述了该疾病的三名瑞士医生。医生描述了一小群肥胖，身材矮小和精神缺乏的小孩，新生儿肺炎（翻转）和一个渴望不断吃，因为他们总是饿了。由于描述了许多其他PW的特征，但与脂肪相关的极端肥胖和健康问题是最突出的特征。具有PWS的个人有一些但并非所有的特征和症状。

PWS是出生缺陷。怀疑丘脑中下丘脑的缺陷被怀疑是原因。下丘脑确定饥饿和饱腹感。他们不能脾气暴躁，所以他们总是有一个迫切兴奋。一些PWS病例如此失控，他们将吃瓶颈，玻璃，铅笔，垃圾，虫子，狗狗，以及他们嘴里的东西。“偷偷摸摸地获得奇迹的聪明才智和决心几乎是传说中的，并且掩盖了他们的认知缺陷。串行称重可能是发现这样的孩子是否是偷窃食物的唯一方法“（FINEY，1983）。PWS发生在10,000个诞生中。它平均地发生在雄性和女性中，并在所有种族和所有国家的人群中发现。它是遗传诊所中最常见的十种最常见的条件之一。带有PW的年轻人非常相似。大多数时候，他们看起来像兄弟姐妹。 Most of PWS people have almond shaped eyes, narrow foreheads, downturned mouth, thin upper lip and a small chin. Other common features are: obesity , they may be short; they have small hands and feet; have a skin picking habit, thick and sticky saliva, incomplete sexual development, a curved spine (scoliosis),and chronic sleepiness. PWS patients also have similar personalities: talkative, friendly, extreme attempts towards getting food, arguementivness, repetitive thoughts and behavior, stubbornness, frequent temper tantrums, and sometimes sudden acts of violence. Most people with PWS have some degree of mental deficiency. The average IQ of people with PWS is 65, and it ranges from 20 to 90. 41% of PWS people have IQs in the normal or borderline range. Specific academic weakness in math and writing are common, but reading and art are considered strengths. A delay in getting to early developmental milestones is common in PWS. The average IQ testing shows that people with PWS are mildly retarded, the range is from severely retarded to not retarded, with 40% having borderline retardation or just a low normal intelligence.

最受影响的儿童，除了他们的智商分数，将有许多严重的学习障碍，并将表现出贫困的学术无论他们的智商表现如何，表现都是表现。出生前有许多PWS的迹象和症状。有些人在80-90％中减少胎儿运动，并且由于具有真正的婴儿，20-30％的异常递送。PWS有两个不同的临床阶段。第1阶段与PWS的婴儿被称为“软盘婴儿”。那是因为他们有弱肌肉，正式肌肉被称为低氧症。这种几乎总是发生的过紊乱可能会轻度到严重。新生儿肺炎使吸吮难以使用，并且使用称为饲养的特殊饲养方法。通过口腔将管子放入胃中。他们在生命的第一天使用它很多..从特殊的喂养困难中减少热量摄入可能导致失败。 To keep the baby’s weight under control supervision by a professional nutritionist or a specialist who understands the syndrome might be necessary. Physical therapy is strongly recommended to improve muscle tone. When the muscle tone improves enough, an increased appetite and weight gain starts. The beginning of the second stage has begun. This hypotonia does not progress and begins to improve between 8 and 11 months of age in most cases. It improves, but it is never completely normal. Stage 2 stage 2 occurs between one and two years of age and is characterized by an appetite that cannot be satisfied which causes excessive weight gain. Speech problems, sleepiness, decreased pain sensitivity, skin picking habits and decreased growth are also characteristics of the second stage of PWS. The personality problems develop between ages 3 and 5 years also. Most parents who have a kid with PWS do not have another kid affected with PWS.

读：

坦博拉山的爆发：原因，事件，效果

PWS的案例被科学家思考，偶然发生了孤立的自然群体。但是，有一个有多个孩子的家庭报告，但这并不常见。曾曾曾毫不多于十几个家庭，曾曾报道过一个以上受影响的后代。对于高分辨率染色体分析的血液样本被绘制在被认为有PWS的任何人身上。这将检查染色体。染色体是我们身体细胞中发现的信息包。每个细胞具有一组46个染色体，其成对地分为1至23。父母从每对中贡献一条染色体。好的，现在Prader-Willi综合征是由于缺乏一些影响下丘脑功能的染色体的一些基因引起的。世界各地的许多实验室正在研究这一点。大约四分之三的PW人们有一个小块，这对一对染色体的一个成员缺失（父亲继承的人）。另一个四分之一被遗漏的所有人都错过了爸爸的贡献 the fathers chromosome fifteen and having two copies the mother’s chromosome fifteen. The genes in this region are not functional and no one understands why. As soon as the kid has improved muscle tone, and has increased its appetite, and is old enough to get move on the floor, than any food that can be easily gotten must be moved to a safer, out-of-reach place. To make inappropriate “food” unavailable to the kid with PWS, parents must learn special patterns of food storage and handling Sleepiness during the day and napping a lot are some of the common features of PWS. Recently, studies have shown that there is a strong link between this and sleep quality. Some of the types of sleep disorders that在PWS患者中被描述的有:睡眠-觉醒周期紊乱，阻塞性睡眠呼吸暂停，低通气综合征和嗜睡症。尽管PWS患者入睡非常快，但他们的睡眠周期明显受到频繁醒来和快速眼动睡眠(rem)异常模式的干扰。阻塞性睡眠呼吸暂停伴随着上呼吸道阻力的增加而发生，这可能是由于扁桃体增大、上呼吸道肌肉组织松弛或结构性气道异常造成的。有时在睡眠中会出现呼吸暂停。

读：

多个诞生：原因与怀孕

嗜睡症包括睡眠发作和偶尔肌肉张力丧失，矮小也是几乎所有PWS患者(80% -100%)的共同特征，但出生高度通常是正常的。成年女性的平均身高为59英寸，男性为61英寸。生长激素反应异常提示可能是下丘脑功能障碍，生长激素缺乏是导致身材矮小的一个因素。最近，一些生长激素缺乏的PWS儿童在接受生长激素治疗6个月后，生长速度得到改善，体重增加速度下降。已经报道的生长激素的其他重要作用是改善肌肉质量，肌肉力量，能量消耗，骨矿化，性发育，以及脂肪质量的减少，这导致了对PWS患者的进一步调查。患有PWS的儿童有明显的行为异常，因为所有与该综合征相关的挫折。这些行为可能早在两岁时就开始了。它们会有各种不同的进食行为，比如寻找食物，偷偷地吃大量的食物，以及其他继续进食的尝试。其他问题包括语言和身体上的攻击行为，如撒谎、偷窃、抓痒和抓皮肤。在患有PWS的儿童和青少年中，发脾气和无故爆发是很常见的。 People with mild cases of PWS can do many things their normal peers can do, such as go to school, get jobs, and sometimes even move away from home. However they need a lot of help. Kids going to school would need to be enrolled in special education programs (Otherwise they’d be eating their pencil and paper).They need to be constantly supervised.

引用本文：威廉安德森（Schoolworkhelper编辑组），“Prader-Willi综合征：原因，症状，治疗，”学校努力, 2019,//www.chadjarvis.com/prader-willi-syndrome-causes-symptoms-treatment/．

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