The Prader-Willi syndrome

The Prader-Willi syndrome Prader-Willi Syndrome is a serious genetic disorder that begins at birth with no known cure ; causing mental retardation,short stature,low muscle tone,incomplete sexual development,and its main charecteristic,the desire to eat everything and anything in sight. Prader-Willi syndrome was first known as Prader-Labhart-Willi Syndrome after three Swiss doctors who first described the disorder in 1956. The doctors described a small group of kids with obesity, short stature and mental deficiency , neonatal hypotonia (floppiness) and a desire to constantly eat because they are always hungry. Many other features of PWS have since been described, but extreme obesity and the health problems associated with being fat are the most prominent features. Individuals with PWS have some but not all of the same features and symptoms. PWS is a birth defect. A defect in the hypothalamus, a region of the brain, is suspected to be the cause.The hypothalamus determines hunger and satiety.They cant fell satiety,so they always have a urge to eat.Some PWS cases are so out of control thay will eat bottlecaps,glass,pencils,garbage,bugs,dogfood, and anything else they can stuff in their mouths. The ingenuity and determination of PWS children in surreptitiously obtaining edibles is almost legendary and belies their cognitive defects. Serial weighing may be the only way to discover whether such a child is, in fact, stealing food(Finey,1983). PWS occurs in about l in 10,000 births. It occurs in both males and females equally and is found in people of all races and all nations.It is one of the ten most common conditions seen in genetics clinics. Young people with PWS resemble each other very much.Most of the time, they look like brother and sister. 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 saliiva,incomplete sexual development, a curved spine (scoliosis),and chronic sleepiness. PWS patients also have similar personalities: talkative, friendly,extreme attempts towards getting food,arguementivness,repetitve 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. Most affected children, besides their IQ scores, will have many, severe learning disabilities,and will show poor academic performance no matter what their IQ shows to their mental abilities. There are many signs and symptoms of PWS that show up before birth.some are decreased fetal movement in 80-90% and having an abnormal delivery in 20-30% due to having a really floppy baby. There are two distinct clinical stages of PWS. Stage 1 Babys with PWS are called floppy babies a lot. Thats because they have weak muscles, officially it is known as hypotonia. This hypotonia,which almost always occurs, could be mild to severe. Neonatal hypotonia makes sucking difficult, and a special feeding method called a gavage is used.A gavage the placing of a tube into the stomach through the mouth.They use it during the first days of life a lot.. Decreased caloric intake from the special feeding difficulties may lead to failure to gain weight. To keep the babys 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 completly normal. Stage 2 Stage 2 occurs between one and two years of age and is characterized by an appetite that can not be satisfied whic 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. The cases of PWS are thought by scientist to have occured by chance in isolated flukes of nature. But, there have been reports of families with more than one kid with PWS, but it is not common. Fewer than a dozen families with more than one affected offspring have ever been reported. A blood sample for high resolution chromosome analysis is drawn on anyone who is though to have PWS.This will check out the chromosones. Chromosomes are packages of information found in the cells of our bodies. Each cell has a set of 46 chromosomes, which come in pairs numbered from 1 to 23. Parents contribute with one chromosome from each pair.Okay,now Prader-Willi Syndrome is caused by the absence of some genes on one of the chromosones that affect the functionimg of the hypothalamus.Many laboratories around the world are researching this. About three-fourths of people with PWS have a tiny piece missing from one member of the pair of chromosone fifteens (the one inherited by the father).The other one fourth are missing the dads contribution to this part of the chromosone by missing all of the fathers chromosone fifteen and having two copies the mothers chromosone fifteen.The genes in this region are not functional and noone understands why. As soon an 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 show that there is a strong link between this and sleep quality. Some of the types of sleep disorders that have been described in PWS affected people are: disturbance to the sleep wake cycle, obstructive sleep apnea, hypoventilation syndromes and narcolepsy. Although patients with PWS fall asleep very quickly, their sleep period is significantly disrupted with frequent awakenings and abnormal patterns of rapid eye movements sleep (rems). Obstructive sleep apnea occurs with increased upper airways resistance, either from enlarged tonsils , relaxation of the upper airway musculature, or from structural airway anomalies. Sometimes actual pauses in breathing during sleep can occur.. Narcolepsy, which involves sleep attacks and occasional loss of muscle tone, Short stature is also a common feature of almost all PWS affected people (80- 100%), but birth height is usually normal. The average adult height is 59 inches in women and 61 inches in men. Abnormal growth hormone response suggests a possible dysfunction of the hypothalamus and, growth hormone deficiency as a contributing factor in short stature. Improvement in growth rate and decreased rate of weight gain have recently been demonstrated in several growth hormone- deficient children with PWS after six months of growth hormone treatment Other significant actions of growth hormone that have been reported is an improvement of muscle mass, muscle strength, energy expenditure, bone mineralization ,sexual development ,and also a decrease in fat mass ,have led to further investigations in people with PWS. Children with PWS have distinct behavioral abnormalities because of all the frustrations associated with the syndrome. These behaviors may begin as early as two years of age. They will get a variety of different eating behaviors like foraging for food, secretly eating large amounts of food, and other attempts to continue eating. Other problems include verbally and physically aggressive behaviors such as lying, stealing, scratching and skin picking. Tantrums and unprovoked outbursts are common among children and youths with PWS. 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 theyd be eating their pencil and paper).They need to be constantly supervised.

Intelligence Customers :: essays research papers

INTELLIGENCE CUSTOMERS The collapse of the Soviet Union has finally brought the cold war to a conclusion in the 1980’s. This collapse brought about a transfer in the global power structure. Threats such as terrorism, nuclear proliferation, narcotics trafficking and organized crime e became more prominent. The United States’ national security policies were revolutionized to be successful against the new risks towards the Nation. It may be assumed that the best group to handle the new pressures of the world would be the U.S. Intelligence Community, but the unique threats do not harbor the traditional roles that were designed for the U.S. Intelligence Community. Biological hazards such as Anthrax and Small pox, which are both remarkable threats to global and national security, have been collected, analyzed and dealt with by civilian government agencies and academic circles. The Intelligence Community should only use its technology to collect non-traditional intelligence that may infringe on only national and global security issues. There are hundreds of intelligence customers, but there are 14 primaries that come together for national security purposes.   Ã‚  Ã‚  Ã‚  Ã‚  To gain a better understanding of intelligence, the term must be defined. Intelligence is when data is collected, organized and becomes useful information. The information is then analyzed and becomes intelligence. The U.S. Intelligence Community supports the President of the United States, the cabinet, Congress, and down to the tactical level of deployed military forces. Each customer is equally as important as the other. The members of the Intelligence Community, also known as intelligence customers are comprised of 14 members: The Defense Intelligence Agency is a major producer and manager of foreign military intelligence. They provide military intelligence to warfighters, defense policymakers and force planners, the Department of Defense and the Intelligence Community in order to support U.S. military planning, operations, and weapon systems acquisition. The National Security Agency is the Nation's cryptologic organization. They coordinate, direct, and perform highly specialized activities to protect U.S. information systems and produce foreign intelligence information. The NSA is one of the most important centers of foreign language analysis and research within the Government. The Federal Bureau of Investigation is the principal investigative arm of the United States Department of Justice. The FBI is to uphold the law through the investigation of violations of federal criminal law; to protect the United States from foreign intelligence and terrorist activities; to provide leadership and law enforcement assistance to federal, state, local, and international agencies; and to perform these responsibilities in a manner that is responsive to the needs of the public and is faithful to the Constitution of the United States.

Scarlet Letter Chapter 20-24 Questions and Answers

Chapter 20 â€Å"The Minister in a Maze†1. Where have Hester and Dimmesdale decided to go when they leave Boston ? Why do they choose to go to Europe rather than remain in the New World. He and Hester have decided to go to Europe, since it offers more anonymity and a better environment for Dimmesdale’s fragile health. 2. Why does Dimmesdale consider it fortunate that the ship is not to sail for 4 days? He wants to give his last final and most emotionally powerful sermon on Election Day. 3. Dimmesdale considers the timing of the ship's departure to be â€Å"most fortunate! Why? He was to preach the Election Sermon and he could not have chanced upon a more suitable mode and time of terminating his professional career. 4. How was Dimmesdale's trip return from the forest different that his departing trip through the forest? He has much more energy than when he left only two days earlier, and everything looks different to him. Three times in a row he is approached by various people, and he struggles not to utter blasphemy. He is even tempted to teach dirty words to a group of small Puritan children. 5.Dimmesdale suddenly perceives that everything in the town is now different as he returns to it. What has happened? He has a new perception of life and feels like a new man after his meeting with Hester. 6. Dimmesdale, as he walks through town to reach his dwelling, is suddenly struck by urges to do terrible things. What were the four instances in which he was tempted to do evil? He encounters an elderly woman to whom he nearly blurts out a devastating â€Å"unanswerable argument against the immortality of the human soul,† but something stops him, and the widow totters away satisfied. He next ignores a young woman whom he has recently converted to the church because he fears that his strange state of mind will lead him to plant some corrupting germ in her innocent heart. Passing one of the sailors from the ship on which he plans to escape, Dimmesdale has the impulse to engage with him in a round of oaths. This comes only shortly after an encounter with a group of children, whom the minister nearly teaches some â€Å"wicked words. †7. What is Dimmesdale's voyage through the town like in this chapter?He's full of temptation to corrupt everyone he passes by in town.8. Why did Dimmesdale and Hester decide to leave for England instead of any place else?They had decided that Europe, with its crowds and cities, offered them a better home and hiding pla ce than anywhere in America, with its choice between an Indian dwelling and a few settlements along the coast. Also, with the minister’s gifts, refinement, and his education meant he needed to live in a civilized place, the more civilized, the better.9. What did Mistress Hibbins accuse Dimmesdale of?What was Dimmesdale's response? She accused him of making a visit to the forest. He denies it. 10. Why did Hester and Dimmesdale decide to leave for England instead of somewhere else?~SAME AS QUESTION 8~ Chapter 21 & Chapter 22 â€Å"The New England Holiday † & â€Å" The Procession†1. What have the crowds of people gathered in the market-place to witness?The people are celebrating Election Day and gathered to witness the inauguration of the Governor.2. What piece of unwelcome news does the master of the ship on which, she, Pearl , and Dimmesdale are to sail have for Hester?The piece of unwelcome news, the master of the ship on which Pearl and Dimmesdale are to sail tells Hester is that Chillingworth will be joining them on their passage because the ship needs a doctor and Chillingworth has told the captain that he is a member of Hester's party.3. Where does Hester stand during the procession and during Dimmesdale’s sermon in the church?Hester takes Pearl and goes to stand near the foot of the scaffold in order to listen to Dimmesdale's speech.4. Dimmesdale must cross a swamp to return to the scaffold. What happens in the swamp?He falls inside and almost drowns.6. Why does Pearl say that Dimmesdale is a â€Å"strange, sad man?† Because of the demeanor he possesses and his profile.7. What is the New England holiday?Holiday celebrated with a procession involving the Governor and other very good people and soldiers.8. What did Pearl want from Dimmesdale that Hester would not permit?Pearl questions Dimmesdale's love for Hester and her by asking if he loves them enough to walk back to town with them hand in hand.9. Hester's â€Å"spir it sank with the idea that all must have been a delusion.† What's happening at this point? Chillingworth has ruined her plans with Dimmesdale that she had so been looking forward to. It was obvious that he was aware of their plans and decided to travel with them and follow.10. What news does the shipmaster ask Pearl to tell Hester? Same answer as number 2.11. What did Pearl want from Dimmesdale that Hester would not permit? ~SAME AS QUESTION 8~12. Hester's â€Å"spirit sank with the idea that all must have been a delusion.What's happening at this point?~SAME AS QUESTION 9~ 13. What news does the shipmaster ask Pearl to tell Hester?~SAME AS QUESTION 10 and 2~ Chapter 23 â€Å"The Revelation of the Scarlet Letter†1. Who empowered Dimmesdale to stand on the scaffold? Hester and Perl carry Dimmsdale to go up on the scaffold and he leans heavily upon their shoulders.2. Describe the most significant thing Dimmesdale does in this chapter. He stands on the scaffold with Hester and his daughter, showing everyone his badge of sin .3.What did Pearl do when standing next to her father on the scaffold? Why? Pearl kisses his lips, his confession has created sympathy in her heart, not just for him, but for her mother, as well.4. Describe what happens to Pearl during this final scaffold moment. As her tears fell upon her father's cheek, they were the pledge that she would change from the cold and distant child that she was, transforming the trouble child she once was.5. What does Chillingworth do in response to Dimmesdale's actions at the scaffold? Why?He tries to talk him out of confessing because once Dimmesdale escapes him, he will have nothing to live forward to.Chapter 24 â€Å"Conclusion†1. What does Hawthorne say about what happened at the scaffold at the beginning of this chapter? That there are many versions, according to people, about what happened during Dimmesdale's â€Å"confession. † Some say his sin was eating him from his heart unti l it appeared on his chest. Some blame it on Roger's drugs. Others claim it was as bright as the one on Hester Prynne's chest. While other claim that there was nothing there, and that his death in a sinful woman's arms was just an act of human righteousness.2. What important lesson has Dimmesdale taught the people of the town? Find a quote that supports this. His death was a parable to teach people that we are all sinners in the eyes of God. After exhausting life in his efforts for mankind’s spiritual good, he had made the manner of his death a parable, in order to impress on his admirers the mighty and mournful lesson, that, in the view of Infinite Purity, we are sinners all alike†¦ nd especially a clergyman’s—will sometimes uphold his character; when proofs, clear as the mid-day sunshine on the scarlet letter, establish him a false and sin-stained creature of the dust(pg. 231). †3. Who did Chillingworth leave his property to? He left everything to P earl.4. What do people think the scarlet letter stood for Dimmesdale’s breast?5. What details do we know about Chillingworth after reading this chapter? That he dies a year after Dimmesdale and leaves all his fortune in Pearl's hands.

Movie Review Breaking Bad - 862 Words

Breaking Bad was one of the original shows I started watching on Netflix’s. From there I was hooked on almost all cop, drug, and violence related shows and movies due to the fact I’m a Criminal Justice Major. One thing I can say about Breaking Bad is that as the season progresses it just gets stranger and stranger. At this point you can visually see a lot of the character changing for either the good or bad. This show I believe gives a great representation of what would happen in the real world if you were to sell drugs. This show doesn’t mask the bad or the good you see all parts, and that helps whoever is watching the show see the true struggles that many individuals face every single day while fulfilling the life of a drug dealer. The in class video we watched on a man selling drugs in a well-known city and what he said helped me greatly put what he dealt with and what Walt deals with every single day into perspective. In Season three of Breaking Bad I believed we learned a lot about each individual character and how they truly feel. The first two seasons slowly introduce you to the show, and all the characters and I now feel like the true colors of these individuals are coming out. Throughout this season if you take out all the violence, and drug selling I focused greatly on the marriage between Walt and Skyler. In this season Skyler finds out about Walt’s drug dealing and threatens to rat him out to the cops. In most cases of family members with drugShow MoreRelatedThe Breakfast Club Essay1198 Words   |  5 Pageswho others believe they are or should be. This becomes a main theme in the movie The Breakfast Club. John Hughes, the director, succeeds at breaking down stereotypes in The Breakfast Club by finding a common denominator between the five main characters which makes them realize they share many feelings and problems. 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