Pws Sentence Examples

It is certainly derived, through Rossiya, from Slavonic Rus or Ros (Byzantine `Pws or `Pc o-oc), a name first given to the Scandinavians who founded a principality on the Dnieper in the 9th century; and afterwards extended to the collection of Russian states of which this principality formed the nucleus.

Prader-Willi syndrome (PWS) is a genetic condition caused by the absence of chromosomal material from chromosome 15.

Insatiable appetite is a classic feature of PWS.

The first patients with features of PWS were described by Dr. Prader, Dr. Willi, and Dr. Lambert in 1956.

Since that time, the complex genetic basis of PWS has begun to be understood.

Initially, scientists found that individuals with PWS have a portion of genetic material deleted (erased) from chromosome 15.

In order to have PWS, the genetic material must be deleted from the chromosome 15 received from one's father.

Over time, scientists realized that some individuals with PWS do not have genetic material deleted from chromosome 15.

Scientists are still discovering other causes of PWS.

A small number of patients with PWS have a change (mutation) in the genetic material on the chromosome 15 inherited from their father.

Newborns with PWS generally have poor muscle tone, (hypotonia) and do not feed well.

Genitalia can be smaller than normal, and a male with PWS may have undescended testicles.

Some patients with PWS have unique and subtle facial characteristics that are detectable only by physicians.

As children with PWS age, development is typically slower than normal.

Developmental delay continues into adulthood for approximately 50 percent of individuals with PWS.

At about one to two years of age, children with PWS develop an uncontrollable, insatiable appetite.

The symptoms and features of PWS require lifelong support and care.

If food intake is strictly monitored and various therapies provided, individuals with PWS have a normal life expectancy.

If these genes are deleted, not inherited, or incorrectly imprinted, PWS develops.

Seventy percent of the cases of PWS are caused when a piece of material is deleted, or erased, from the paternal chromosome 15.

This is what causes the symptoms associated with PWS.

In 99 percent of the cases of PWS, the deletion is sporadic.

If a child has PWS due to a sporadic deletion in the paternal chromosome 15, the chance the parents could have another child with PWS is less than 1 percent.

In fewer than 1 percent of the cases of PWS there is a chromosomal rearrangement in the family that causes the deletion.

If a parent has a translocation the risk of having a child with PWS is higher than 1 percent.

This is seen in approximately 25 percent of the cases of PWS.

Maternal uniparental disomy for chromosome 15 leads to PWS because the genes on chromosome 15 that should have been inherited from the father are missing, and the genes on both the chromosome 15s inherited from the mother are imprinted.

If a child has PWS due to maternal uniparental disomy, the chance the parents could have another child with PWS is less than 1 percent.

Approximately 2-5 percent of patients with PWS have a change (mutation) in a gene located on the q arm of chromosome 15.

If a child has PWS due to a mutation that changes imprinting, the chance the parents could have another child with PWS is approximately 5 percent.

Infants with PWS have weak muscle tone (hypotonia).

This hypotonia causes problems with sucking and eating so that infants with PWS may initially have problems gaining weight.

Consequently, some infants with PWS may be diagnosed with failure to thrive due to slow growth and development.

Hypotonia may also During infancy, babies with PWS may also sleep more than normal and have problems controlling their temperature.

Some of the unique physical features associated with PWS can be seen during infancy.

This may be more evident in males with PWS.

The unique facial features seen in some patients with PWS may be difficult to detect in infancy.

This uncontrollable appetite is a classic feature of PWS.

Individuals with PWS lack the ability to feel full or satiated because of a flaw in the hypothalamus part of their brain, which normally registers feelings of hunger and satiety.

Some individuals with PWS may also have a reduced ability to vomit.

Behavior problems are a common feature of PWS.

Individuals with PWS may also pick their own skin (skin picking).

Parents should call a doctor if they notice symptoms that are characteristic of PWS.

During infancy the diagnosis of PWS may be suspected if poor muscle tone, feeding problems, small genitalia, or the unique facial features are present.

If an infant has these features, testing for PWS should be performed.

This testing should also be offered to children and adults who display features commonly seen in PWS (developmental delays, uncontrollable appetite, small genitalia, etc.). There are several different genetic tests that can detect PWS.

Methylation testing detects 99 percent of the cases of PWS.

Although methylation testing can accurately diagnose PWS, it cannot determine if the PWS is caused by a deletion, maternal uniparental disomy, or a mutation that disrupts imprinting.

Chromosome analysis can determine if the PWS is the result of a deletion in the q arm of chromosome 15.

The unique DNA patterns are then compared with the DNA from the child with PWS.

This testing is only recommended if the mother or father is known to have a chromosome rearrangement, or if they already have a child with PWS syndrome.

Some infants with PWS also require special nipples or tube feeding to improve weight gain.

Growth hormone therapy has been shown to improve the poor muscle tone and reduced height typically associated with PWS.

Individuals with PWS typically excel in highly structured environments.

Verbal ability often becomes an area of strength for children with PWS.

Treatment and management of PWS during childhood, adolescence, and adulthood is typically focused on weight control.

Unfortunately, diet medications nor surgery have not been shown to significantly prevent obesity in PWS or eliminate the need for strict dieting and supervision around food.

Although in the past many people with PWS died in adolescence or young adulthood, prevention of obesity can enable those with the syndrome to live a normal lifespan.

New medications, including psychotropic drugs and synthetic growth hormone, are already improving the quality of life for some people with PWS.

Genetic counseling is recommended for parents who may be at risk for having a child with PWS.

Additionally, a diagnosis of PWS opens the doors to a network of information and support from professionals and other families who are dealing with the syndrome.

Adolescents and adults with PWS can function well in group and supported living programs, if the necessary diet control and structured environment are provided.

However, residential and vocational providers must be fully informed regarding management of PWS.