Hutchinson-Gilford Progeria Syndrome

Hutchinson-Gilford Progeria syndrome, also known as HGPS, or Progeria, is a very rare genetic disease caused by a mutation in the cell. In 1886, Jonathan Hutchinson first reported case of a 3 ½ year old boy who had the appearance of an old man. In 1897 Hastings Gilford reported a second case with similar features. However, this mystery disease didn’t have a name until 1904, when it was named after the two men. People who have HGPS usually star showing symptoms by the age of 2, and only live to be a teen-mid-20s.

When thinking about the history of the world, one must always consider that merchants as well as trade have played an immerse role in shaping the world as it is today. They are responsible for many of the cross-cultural interactions that we have had in the past. Christianity and Islam, the two predominant religions of the world today, have both grown and spread through merchants and trade also. These two chief religions both have attitudes towards merchants and trade that have either developed or decreased overtime. According to these documents, from up to the year 1500, Christianity went from a negative view of merchants and trade to a positive view while Islam went from a positive view to a negative view, but both sides

Distortion of the facial features can take place in a newborn with this disorder due to the thick plates of skin

Lamin A helps sustain the normal structure of a cell’s nucleus, which contains our genetic information. In Progeria, this protein takes on an abnormal toxic form called “Progerin”. Many cells in the body make this progerin protein in small amounts, including normal healthy individuals. It’s thought that some characteristics of normal aging can be the result of this gradual buildup of progerin over a person’s lifetime. Progerin can’t be properly processed by the cell and will build up inside the cell’s nucleus, eventually causing the cell to become deformed or collapse (Figure 2).

Hutchinson-Gilford Progeria Syndrome (HGPS) affects approximately 1 in 4-8 million newborns. It is characterized by rapid aging, but no symptoms are seen at birth. Within a year, infected children start showing symptoms such as a receding jaw, pointy nose, partial to total hair loss (alopecia), fat loss, bone disfigurements, a short stature and skin problems (Pollex 2004). The disease progresses with time, and eventually leads to death at an average age of about 13 years. Death is usually caused by some form of cardiovascular disease, usually induced by atherosclerosis (Wuyts et al. 2005). Most cases of HGPS are due to de novo autosomal dominant point mutations in the lamin A/C gene (LMNA). There are some reported cases suggesting autosomal recessive inheritance, but further testing needs to be performed.

The show got multiple aspects of Korsakoff syndrome correctly. The woman confessed to the murder of her husband in spite of her inability to remember the act or why she did it. When confronted with questions about her murder by investigators she quickly confessed to the murder and signed confession papers. The doctor came in asked if she had picked up a cat on the side of the road named Millie. She agreed that the event happened and asked how he knew about it. The doctor says she has symptoms of Korsakoff syndrome and that she is extremely suggestible to altered memories and has anterograde amnesia. Anterograde amnesia is the inability to form new memories which seems to be the symptoms she is experiencing. The investigator that works with

Progeria, also known as Hutchinson Gilford Progeria Syndrome, and Progeria syndrome, is an extremely rare genetic disease wherein symptoms resembling aspects of aging are manifested at a very early age. The Progeria come from the Greek words “pro” meaning “before” and “gēras” meaning “old age”. The disorder has a very low incident rate, occurring in an estimated 1 per 8 million live births. Those born with Progeria typically live to their mid teens and early twenties. It is a genetic condition that occurs as a new mutation, and is rarely inherited. Although the term Progeria applies strictly to all diseases characterized by premature aging symptoms, and is often used as

The type of Progeria Sam had is called Hutchinson-Gilford Progeria Syndrome, “child Progeria” rather than Werner’s syndrome, also know as “adult Progeria”, that does not occur until late teens, resulting in longer lives into the 40’s-50’s (“Progeria 101/FAQ"). Progeria has a vast amount of symptoms that the majority of those suffering deal with as well as symptoms that are seen less often. Throughout early infancy, children with Progeria resemble normal infants’ physical appearance. Around age 1 or 2 they begin to display extreme growth delay causing them to be short, and have low weight. Their faces appear to be small compared to their head size; furthermore, their faces seem shrunken, wrinkled, and slender. Skulls will have visible veins along the forehead, nose-bridge, as well as the other areas across the head. Other symptoms include having a small jaw, delayed or failed tooth development, deformity of teeth with crowding, beaked nose, prominent eyes, brittle nails, dislocated hips, skeletal defects, and loss of hair, eyebrows, and eyelashes (Chandravanshi et al.). More damaging symptoms are atherosclerosis (hardening of the arteries), cardiovascular issues (strokes heart attacks), arthritis, and osteoporosis (“Progeria 101/FAQ"). The children who have Progeria are very similar in appearance with little effects from various ethnicities (“Progeria 101/FAQ"). Normally the complications of atherosclerosis lead to the deaths of the children around

Stevens-Johnson Syndrome, a form of Toxic Epidermal Necrolysis, is a rare life-threatening disorder of the skin and mucous membranes which arises from an intense autoimmune sensitivity to mild infections, cancers, and most commonly, medications.

Hutchinson-Gilford Progeria Syndrome is a condition in a child that has rapid growth at an early stage of childhood. The accelerating age starts in early infancy, where the child will grow slower than other children, but normal when first born. Usually the "failure to thrive" happens in the first year after being born. This condition does not stop the development of motor skills, like sitting, standing, and walking. The child will also have a normal immune system that helps with healing normally after being sick or getting scrapes/ cuts. This condition over time is life threatening to children who have been diagnosed with the progeria syndrome. Severe hardening of arteries occurs in the younger years of children, causing them to have heart attacks and strokes. Deaths have been between ages six and twenty years, the life span is usually 14.6 years after being diagnosed with the syndrome.

Hutchinson Gilford Syndrome or otherwise commonly known as Progeria; is a fatal disease. Sadly, death occurs in every case. This disease is a fast spreading disease in the body, it affects the body almost instantaneously.This disease is a rapid aging disorder caused by a LMNA anomaly. This anomaly release progerin, a mutant lamin. The lamin A/C is the official name of the gene more widely known as LMNA. The gene gives out a designated list of tasks to making different proteins called lamins. The infected cells show a decrease in heterochromatin, a increased amount a deoxyribonucleic acid, and cell cycle changes. Hutchinson-Gilford progeria syndrome (HGPS) is an extremely rare hereditary disease that affects the skin, musculoskeletal system, and vasculature. HGPS is characterized by signs of premature aging(Kara N Shah,HGPS, MedScape)

We’ve all heard this phrase from the kids we know and love, “I’m a big boy!” Well, progeria makes this true in a sense. According to The Mayo Clinic, Progeria, an exceptionally rare disorder, also known as Hutchinson-Gilford syndrome is defined as a progressive genetic disorder that causes children to age rapidly, beginning in their first two years of life. Now mentally these kids are as young as their own age, but their bodies are biologically much older than they should be. These kids’ bodies show symptoms like under-weight/height, large head for the face, hair loss, high pitched voice, visible veins, and wrinkled skin. Aside from this the children experience problems that normal only senior would have such as heart disease, and Arthritis.

As of 2012, Progeria Research Foundation provided over 3 million dollars for progeria related research projects performed in many states and in 6 other countries. As of right now, there are not any treatments to help cure progeria but there are some medications to help some of the problems such as arthritic, respiratory, and cardiovascular problems and to help make people living with progeria more comfortable. Doctors suggest that children with Progeria take aspirin to help with their heart health. Once the child has hypertension, strokes or seizures, the child will take medicines similar to what adults would take for these

rather than the conditions themselves. [5] This examination will often take the form of case

For ages, the United States has seemed to be the country where people seek to move to for a better life. The United States was built on immigrants. People have always migrated to the United States both legally and illegally. The main problem the country has face with immigrants is the amount that trespass the border illegally. Illegal immigration is the unlawful act of crossing a national border(Illegal Immigration Pros and Cons). The illegal immigrant population keeps growing at an annual average of about 300,000 people(Currie 10). In the recent year of 2014, there were about 11.3 million undocumented people living in the United States(Krogstad). Many have asked what harm they do to both the economy and the American citizen. Although there are many arguments as to how much harm they cause, there are as many arguments stating illegal immigration benefits the United States. Illegal immigration has a positive impact in the United States within the consumers, the working class, and the overall economy.