What is Hutchinson-Gilford progeria syndrome?
Hutchinson-Gilford progeria syndrome is a genetic condition characterized by the dramatic, rapid appearance of aging beginning in childhood. Affected children typically look normal at birth and in early infancy, but then grow more slowly than other children and do not gain weight at the expected rate (failure to thrive). They develop a characteristic facial appearance including prominent eyes, a thin nose with a beaked tip, thin lips, a small chin, and protruding ears.
Hutchinson-Gilford progeria syndrome also causes hair loss (alopecia), aged-looking skin, joint abnormalities, and a loss of fat under the skin (subcutaneous fat). This condition does not disrupt intellectual development or the development of motor skills such as sitting, standing, and walking.
People with Hutchinson-Gilford progeria syndrome experience severe hardening of the arteries (arteriosclerosis) beginning in childhood. This condition greatly increases the chances having a heart attack or stroke at a young age. These serious complications can worsen over time and are life-threatening for affected individuals.
How common is Hutchinson-Gilford progeria syndrome?'
This condition is very rare; it is reported to occur in 1 in 4 million newborns worldwide. More than 130 cases have been reported in the scientific literature since the condition was first described in 1886.
What genes are related to Hutchinson-Gilford progeria syndrome?
Mutations in the LMNA gene cause Hutchinson-Gilford progeria syndrome.
The LMNA gene provides instructions for making a protein called lamin A. This protein plays an important role in determining the shape of the nucleus within cells. It is an essential scaffolding (supporting) component of the nuclear envelope, which is the membrane that surrounds the nucleus. Mutations that cause Hutchinson-Gilford progeria syndrome result in the production of an abnormal version of the lamin A protein. The altered protein makes the nuclear envelope unstable and progressively damages the nucleus, making cells more likely to die prematurely. Researchers are working to determine how these changes lead to the characteristic features of Hutchinson-Gilford progeria syndrome.
Read more about the LMNA gene.
How do people inherit Hutchinson-Gilford progeria syndrome?
Hutchinson-Gilford progeria syndrome is considered an autosomal dominant condition, which means one copy of the altered gene in each cell is sufficient to cause the disorder. The condition results from new mutations in the LMNA gene, and almost always occurs in people with no history of the disorder in their family.
Friday, January 18, 2008
Hutchinson-Gilford progeria syndrome
Where can I find information about treatment for Hutchinson-Gilford progeria syndrome?
These resources address the management of Hutchinson-Gilford progeria syndrome and may include treatment providers.
• Gene review
• Progeria
You might also find information on treatment of Hutchinson-Gilford progeria syndrome in
How ca we I find additional information about Hutchinson-Gilford progeria syndrome?
You may find the following resources about Hutchinson-Gilford progeria syndrome helpful. These materials are written for the general public.
• NIH Publications - National Institutes of Health
• Health information (2 links)
• Information pages (4 links)
• For patients and families (4 links)
You may also be interested in these resources, which are designed for healthcare professionals and researchers.
• Clinical summary
• DNA tests ordered by healthcare professionals
• Linking patients to medical research
• Recent literature
• Genetic disorder catalog
These resources address the management of Hutchinson-Gilford progeria syndrome and may include treatment providers.
• Gene review
• Progeria
You might also find information on treatment of Hutchinson-Gilford progeria syndrome in
How ca we I find additional information about Hutchinson-Gilford progeria syndrome?
You may find the following resources about Hutchinson-Gilford progeria syndrome helpful. These materials are written for the general public.
• NIH Publications - National Institutes of Health
• Health information (2 links)
• Information pages (4 links)
• For patients and families (4 links)
You may also be interested in these resources, which are designed for healthcare professionals and researchers.
• Clinical summary
• DNA tests ordered by healthcare professionals
• Linking patients to medical research
• Recent literature
• Genetic disorder catalog
Overview
Overview
Because the "accelerated aging" diseases display different aspects of aging, but never every aspect, they are often called "segmental progerias" by biogerontologists Hutchinson-Gilford Progeria syndrome is an extremely rare genetic condition which causes physical changes that resemble greatly accelerated aging in sufferers. The disease affects between 1 in 4 million (estimated actual) and 1 in 8 million (reported) newborns.
Currently, there are 51 known cases in the world. There is no known cure, but several discoveries have been made that have led to greater understanding and perhaps eventual treatment. Most people with progeria die at around 13 years of age. Progeria is of interest to scientists because the disease may reveal clues about the process of aging. Unlike most other "accelerated aging diseases" (such as Werner's syndrome, Cockayne's syndrome or xeroderma pigmentosum, progeria is not caused by defective DNA repair.
The condition was first identified in 1886 by Jonathan Hutchinson and Hastings Gilford. The condition was later named Hutchinson-Gilford Progeria syndrome (HGPS). Around 100 cases have been identified since then.
A 2003 report in Nature said progeria may be a de novo dominant trait. It develops during cell division in a newly conceived child or in the gametes of one of the parents. It is caused by mutations in a LMNA (Lamin A protein gene on chromosome.
Nuclear lamina is a protein scaffold around the edge of the nucleus that helps organize nuclear processes such as RNA and DNA synthesis.
Prelamin A contains a CAAX box at the C-terminus of the protein (where C is a cysteine and A is any aliphatic amino acids). This ensures that the cysteine is farnesylated, and this allows Prelamin A to bind membranes, specifically the nuclear membrane. After Prelamin A has been localized to the cell nuclear membrane the C-terminal amino acids, including the farnesylated cysteine, are cleaved off by a specific protease. The resulting protein is now Lamin A, is no longer membrane-bound and carries out functions inside the nucleus. In HGPS the recognition site that the enzyme requires for the cleavage of Prelamin A to Lamin A is mutated. Lamin A cannot be produced and Prelamin A builds up on the nuclear membrane, causing a characteristic nuclear blebbing.
This results in the premature aging symptoms of progeria, although the mechanism connecting the misshapen nucleus to the symptoms is not known. A mouse model of progeria exists, though in the mouse the LMNA prelamin A is not mutated, but instead the specific protease that is required to remove the C-terminus of Prelamin A is missing. Both cases result in the build up of farnesylated Prelamin A on the nuclear membrane and in the characteristic nuclear LMNA blebbing. Fong et al use a farnesyl transferase inhibitor (FTI) in this mouse model to inhibit protein farnesylation of Prelamin A. Treated mice had greater grip strength, lower likelihood of rib fracture and may live longer than untreated mice.
Note that this method does not directly 'cure' the underlying cause of progeria. This method prevents Prelamin A going to the nucleus in the first place so no Prelamin A can build up on the nuclear membrane, but equally there is no production of normal Lamin A in the nucleus. Luckily Lamin A does not appear to be essential, indeed mouse models in which the genes for Prelamin A and C are knocked out show no symptoms. This also shows that it is the build up of Prelamin A in the wrong place, rather than the loss of the normal function of Lamin A that causes the disease.
A study which compared HGPS patient cells with the skin cells from LMNA young and elderly human subjects found similar defects in the HGPS and elderly cells, including down-regulation of certain nuclear proteins, increased DNA damage and demethylation of histone leading to reduced heterochromatin. Nematodes over their lifespan show progressive lamin changes comparable to HGPS in all cells but neurons and gametes These studies suggest that lamin A defects contribute to normal aging.
Symptoms
The earliest symptoms include failure to thrive (FTT) and a localized scleroderma-like skin condition. As the child ages past infancy, additional conditions become apparent. Limited growth, alopecia, and a distinctive appearance with small face and jaw and pinched nose all are characteristic of progeria. Later the condition causes wrinkled skin, atherosclerosis and cardiovascular problems. Mental development is not affected. Individuals with the condition rarely live more than 17 years; the longest recorded life-span was 33 years. The development of symptoms is comparable to aging at a rate six to eight times faster than normal, although certain age-related conditions do not occur. Specifically, victims show no neurodegeneration or cancer predisposition. The people diagnosed with this disease usually have fragile elderly-like bodies.
Because the "accelerated aging" diseases display different aspects of aging, but never every aspect, they are often called "segmental progerias" by biogerontologists Hutchinson-Gilford Progeria syndrome is an extremely rare genetic condition which causes physical changes that resemble greatly accelerated aging in sufferers. The disease affects between 1 in 4 million (estimated actual) and 1 in 8 million (reported) newborns.
Currently, there are 51 known cases in the world. There is no known cure, but several discoveries have been made that have led to greater understanding and perhaps eventual treatment. Most people with progeria die at around 13 years of age. Progeria is of interest to scientists because the disease may reveal clues about the process of aging. Unlike most other "accelerated aging diseases" (such as Werner's syndrome, Cockayne's syndrome or xeroderma pigmentosum, progeria is not caused by defective DNA repair.
The condition was first identified in 1886 by Jonathan Hutchinson and Hastings Gilford. The condition was later named Hutchinson-Gilford Progeria syndrome (HGPS). Around 100 cases have been identified since then.
A 2003 report in Nature said progeria may be a de novo dominant trait. It develops during cell division in a newly conceived child or in the gametes of one of the parents. It is caused by mutations in a LMNA (Lamin A protein gene on chromosome.
Nuclear lamina is a protein scaffold around the edge of the nucleus that helps organize nuclear processes such as RNA and DNA synthesis.
Prelamin A contains a CAAX box at the C-terminus of the protein (where C is a cysteine and A is any aliphatic amino acids). This ensures that the cysteine is farnesylated, and this allows Prelamin A to bind membranes, specifically the nuclear membrane. After Prelamin A has been localized to the cell nuclear membrane the C-terminal amino acids, including the farnesylated cysteine, are cleaved off by a specific protease. The resulting protein is now Lamin A, is no longer membrane-bound and carries out functions inside the nucleus. In HGPS the recognition site that the enzyme requires for the cleavage of Prelamin A to Lamin A is mutated. Lamin A cannot be produced and Prelamin A builds up on the nuclear membrane, causing a characteristic nuclear blebbing.
This results in the premature aging symptoms of progeria, although the mechanism connecting the misshapen nucleus to the symptoms is not known. A mouse model of progeria exists, though in the mouse the LMNA prelamin A is not mutated, but instead the specific protease that is required to remove the C-terminus of Prelamin A is missing. Both cases result in the build up of farnesylated Prelamin A on the nuclear membrane and in the characteristic nuclear LMNA blebbing. Fong et al use a farnesyl transferase inhibitor (FTI) in this mouse model to inhibit protein farnesylation of Prelamin A. Treated mice had greater grip strength, lower likelihood of rib fracture and may live longer than untreated mice.
Note that this method does not directly 'cure' the underlying cause of progeria. This method prevents Prelamin A going to the nucleus in the first place so no Prelamin A can build up on the nuclear membrane, but equally there is no production of normal Lamin A in the nucleus. Luckily Lamin A does not appear to be essential, indeed mouse models in which the genes for Prelamin A and C are knocked out show no symptoms. This also shows that it is the build up of Prelamin A in the wrong place, rather than the loss of the normal function of Lamin A that causes the disease.
A study which compared HGPS patient cells with the skin cells from LMNA young and elderly human subjects found similar defects in the HGPS and elderly cells, including down-regulation of certain nuclear proteins, increased DNA damage and demethylation of histone leading to reduced heterochromatin. Nematodes over their lifespan show progressive lamin changes comparable to HGPS in all cells but neurons and gametes These studies suggest that lamin A defects contribute to normal aging.
Symptoms
The earliest symptoms include failure to thrive (FTT) and a localized scleroderma-like skin condition. As the child ages past infancy, additional conditions become apparent. Limited growth, alopecia, and a distinctive appearance with small face and jaw and pinched nose all are characteristic of progeria. Later the condition causes wrinkled skin, atherosclerosis and cardiovascular problems. Mental development is not affected. Individuals with the condition rarely live more than 17 years; the longest recorded life-span was 33 years. The development of symptoms is comparable to aging at a rate six to eight times faster than normal, although certain age-related conditions do not occur. Specifically, victims show no neurodegeneration or cancer predisposition. The people diagnosed with this disease usually have fragile elderly-like bodies.
Stevens-Johnson Syndrome
Stevens-Johnson Syndrome is a potentially deadly skin disease that usually results from a drug reaction. Another form of the disease is called Toxic Epidermal Necrolysis, and again this usually results from a drug-related reaction. Both forms of the disease can be deadly as well as very painful and distressing. In most cases, these disorders are caused by a reaction to a drug, and one drug that has come under fire lately is the cox-2 inhibitor Bextra, which is already linked to these disorders.
There are other drugs that have been linked to Stevens-Johnson Syndrome, and these include some other NSAIDS (non-steroid anti-inflammatory drugs), Allopurinol, Phenytoin, Carbamazepine, barbiturates, anticonvulsants, and sulfa antibiotics. The condition can sometimes – although not very often – be attributed to a bacterial infection, and in some cases there is no known cause for the onset of Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis. However, the most common cause is through drug related reaction.
Stevens-Johnson Syndrome can affect any age group. However, it occurs most commonly in older people, and this could be because older people tend to use more of the drugs associated with the disease and are therefore collectively more at risk from the disease. People that have AIDS are also at an increased risk of contracting Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis. Those in the higher risk groups are urged to remain vigilant for any signs of these skin diseases, and are also advised to remain well informed about the symptoms that could indicate the presence or onset of Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis.
There are other drugs that have been linked to Stevens-Johnson Syndrome, and these include some other NSAIDS (non-steroid anti-inflammatory drugs), Allopurinol, Phenytoin, Carbamazepine, barbiturates, anticonvulsants, and sulfa antibiotics. The condition can sometimes – although not very often – be attributed to a bacterial infection, and in some cases there is no known cause for the onset of Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis. However, the most common cause is through drug related reaction.
Stevens-Johnson Syndrome can affect any age group. However, it occurs most commonly in older people, and this could be because older people tend to use more of the drugs associated with the disease and are therefore collectively more at risk from the disease. People that have AIDS are also at an increased risk of contracting Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis. Those in the higher risk groups are urged to remain vigilant for any signs of these skin diseases, and are also advised to remain well informed about the symptoms that could indicate the presence or onset of Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis.
The symptoms :
The symptoms :
Both Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis can start with non-specific symptoms such as cough, aching, headaches, and feverishness. This may be followed by a red rash across the face and the trunk of the body, which can continue to spread to other parts of the body. The rash can form into blisters, and these blisters can form in areas such as the eyes, mouth and vaginal area. The mucous membranes can become inflamed, and with Toxic Epidermal Necrolysis layers of the skin can also come away with ease and often the skin peels away in sheets. The hair and nails can also come away in some cases, and sufferers can become cold and feverish.
With Toxic Epidermal Necrolysis the most common cause of death is infection, which can enter through the exposed areas. This disease can leave the skin looking as though it has been burned, and areas where skin has flayed away can seep copiously and quickly become infected.
Treating these diseases:
Those suffering from SJS or TEN are treated in hospital, and if the cause of the problem is drug related then the drugs are stopped with immediate effect. Surviving patients are treated intravenously to replace any lost fluids, and the skin is left to re-grow on its own. However, the chances of survival can be hit and miss depending on the level of damage and the degree of infection incurred by the patient.
It is vital that those taking drugs that could result in these skin diseases are vigilant and can identify the danger signs associated with these problems. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis can be deadly, and the earlier the symptoms are recognised the faster treatment can be initiated.
Of course, those affected by drugs in this way – or the families of those that have passed away from these skin problems – have every right to file for compensation against the manufacturer of the drug in question. Lawyers now specialize in this type of litigation, and those that feel as though they have grounds for compensation are advised to go through an experienced drugs litigation lawyer.
Both Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis can start with non-specific symptoms such as cough, aching, headaches, and feverishness. This may be followed by a red rash across the face and the trunk of the body, which can continue to spread to other parts of the body. The rash can form into blisters, and these blisters can form in areas such as the eyes, mouth and vaginal area. The mucous membranes can become inflamed, and with Toxic Epidermal Necrolysis layers of the skin can also come away with ease and often the skin peels away in sheets. The hair and nails can also come away in some cases, and sufferers can become cold and feverish.
With Toxic Epidermal Necrolysis the most common cause of death is infection, which can enter through the exposed areas. This disease can leave the skin looking as though it has been burned, and areas where skin has flayed away can seep copiously and quickly become infected.
Treating these diseases:
Those suffering from SJS or TEN are treated in hospital, and if the cause of the problem is drug related then the drugs are stopped with immediate effect. Surviving patients are treated intravenously to replace any lost fluids, and the skin is left to re-grow on its own. However, the chances of survival can be hit and miss depending on the level of damage and the degree of infection incurred by the patient.
It is vital that those taking drugs that could result in these skin diseases are vigilant and can identify the danger signs associated with these problems. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis can be deadly, and the earlier the symptoms are recognised the faster treatment can be initiated.
Of course, those affected by drugs in this way – or the families of those that have passed away from these skin problems – have every right to file for compensation against the manufacturer of the drug in question. Lawyers now specialize in this type of litigation, and those that feel as though they have grounds for compensation are advised to go through an experienced drugs litigation lawyer.
SJ and children
SJ and children
Stevens Johnson Syndrome is a serious and potentially fatal skin condition that can be caused in a number of ways, most commonly through the use of some medications. This skin disease most commonly affects children and young adults, and the symptoms can cause pain, discomfort and even death. Stevens Johnson Syndrome can start with non-specific symptoms such as cough, aching, headaches, and feverishness. This may be followed by a red rash across the face and the trunk of the body, which can continue to spread to other parts of the body. The rash can form into blisters, and these blisters can form in areas such as the eyes, mouth and vaginal area. The mucous membranes can become inflamed, and the hair and nails can also come away in some cases, and sufferers can become cold and feverish.
The causes of SJS in children can vary, but medication can play a big part. Amongst the children’s medication associated with Stevens Johnson Syndrome are ibuprofen based medications such as Advil and children’s Motrin. At the moment, children’s Motrin is under fire following the effects that this medication has had on one young girl, which has results in legal action. The seven-year old girl was given children’s Motrin for feverishness, and began to develop symptoms the very next day. After being admitted to hospital, she was rendered blind within two days as a result of SJS caused by taking children’s Motrin.
There are other medications that have been linked to SJS, including sulfonamide antibiotics, penicillin, phenytoin (Dilantin) and valdecoxib (Bextra). Viral and bacterial infections can also result in Stevens Johnson Syndrome, as can radiation therapy. And in some cases, there is no known cause for the onset of Stevens Johnson Syndrome. However, should any child display the symptoms of this disease it is vital that medical attention is sought immediately.
Pharmaceutical companies, such as the manufacturers of children’s Motrin, are now being urged to add warnings to their medication packaging, warning parents about the possible side effects of giving their children this medication. The Food and Drugs Administration is also being asked to look into this matter further, with many concerned parents now expressing concerns over what they once classed as a safe, effective painkiller.
Treating a child with SJS is an involved process, and every effort is first made to identify the cause. If a regular medication is the cause of SJS, then it is immediately stopped. Care is given in the burns unit of a hospital due to the severe skin problems sustained by the child and the risk of infection being contracted through the areas where skin has come away and left exposed flesh. Depending on whether another infection has occurred, antibiotics may also be administered.
Oral examinations and treatment, eye examinations, and fluid replacement can all form part of the treatment for SJS, and in some cases topical and oral corticosteroids may also be administered by the doctor.
Parents that have been administering medication such as children’s Motrin and Advil to their children may understandably be concerned, and these parents are strongly advised to cease the medication and seek immediate advice from their doctor. Hopefully, the manufacturers of these children’s medications will now be forced to add adequate warnings to their packaging, but in the meantime it is always advisable to check with a doctor with regards to any potential side effects of medicine that you are giving to your child.
Although awareness about SJS leaves a lot to be desired, there are now support groups available that can help both children and their parents. These support groups can provide valuable therapy, advice and general support to affected SJS children and their parents.
Stevens Johnson Syndrome is a serious and potentially fatal skin condition that can be caused in a number of ways, most commonly through the use of some medications. This skin disease most commonly affects children and young adults, and the symptoms can cause pain, discomfort and even death. Stevens Johnson Syndrome can start with non-specific symptoms such as cough, aching, headaches, and feverishness. This may be followed by a red rash across the face and the trunk of the body, which can continue to spread to other parts of the body. The rash can form into blisters, and these blisters can form in areas such as the eyes, mouth and vaginal area. The mucous membranes can become inflamed, and the hair and nails can also come away in some cases, and sufferers can become cold and feverish.
The causes of SJS in children can vary, but medication can play a big part. Amongst the children’s medication associated with Stevens Johnson Syndrome are ibuprofen based medications such as Advil and children’s Motrin. At the moment, children’s Motrin is under fire following the effects that this medication has had on one young girl, which has results in legal action. The seven-year old girl was given children’s Motrin for feverishness, and began to develop symptoms the very next day. After being admitted to hospital, she was rendered blind within two days as a result of SJS caused by taking children’s Motrin.
There are other medications that have been linked to SJS, including sulfonamide antibiotics, penicillin, phenytoin (Dilantin) and valdecoxib (Bextra). Viral and bacterial infections can also result in Stevens Johnson Syndrome, as can radiation therapy. And in some cases, there is no known cause for the onset of Stevens Johnson Syndrome. However, should any child display the symptoms of this disease it is vital that medical attention is sought immediately.
Pharmaceutical companies, such as the manufacturers of children’s Motrin, are now being urged to add warnings to their medication packaging, warning parents about the possible side effects of giving their children this medication. The Food and Drugs Administration is also being asked to look into this matter further, with many concerned parents now expressing concerns over what they once classed as a safe, effective painkiller.
Treating a child with SJS is an involved process, and every effort is first made to identify the cause. If a regular medication is the cause of SJS, then it is immediately stopped. Care is given in the burns unit of a hospital due to the severe skin problems sustained by the child and the risk of infection being contracted through the areas where skin has come away and left exposed flesh. Depending on whether another infection has occurred, antibiotics may also be administered.
Oral examinations and treatment, eye examinations, and fluid replacement can all form part of the treatment for SJS, and in some cases topical and oral corticosteroids may also be administered by the doctor.
Parents that have been administering medication such as children’s Motrin and Advil to their children may understandably be concerned, and these parents are strongly advised to cease the medication and seek immediate advice from their doctor. Hopefully, the manufacturers of these children’s medications will now be forced to add adequate warnings to their packaging, but in the meantime it is always advisable to check with a doctor with regards to any potential side effects of medicine that you are giving to your child.
Although awareness about SJS leaves a lot to be desired, there are now support groups available that can help both children and their parents. These support groups can provide valuable therapy, advice and general support to affected SJS children and their parents.
TEN
TEN
Toxic Epidermal Necrolysis, also know as TENS, is a potentially deadly skin disease that usually results from a drug reaction. Another form of the disease is called Stevens-Johnson Syndrome, and again this usually results from a drug-related reaction. Both forms of the disease can be deadly as well as very painful and distressing. In most cases, these disorders are caused by a reaction to drugs, such as the cox-2 inhibitor Bextra, which has already been linked to SJS and TENS. Another drug commonly linked to Toxic Epidermal Necrolysis is the antibiotic, penicillin.
In many cases there is no known cause for TENS, although drugs are the main cause of this skin disease. This condition can be extremely serious, and can lead to massive discomfort and pain, and in some cases the infection that can be contracted through this disease can result in the death of the patient.
Any age group can find themselves affected by Toxic Epidermal Necrolysis. However, it is normally seen in older patients rather than younger ones, and this is because older patients tend to take more medication, and are therefore more likely to come across a drug to which they have a reaction. There are also other groups that are more vulnerable when it comes to contracting Toxic Epidermal Necrolysis.
This includes those with AIDS, who have lower immunity to infections and diseases. The elderly and all other age groups are advised to look out for any signs of TENS if they are on medication.
Toxic Epidermal Necrolysis, also know as TENS, is a potentially deadly skin disease that usually results from a drug reaction. Another form of the disease is called Stevens-Johnson Syndrome, and again this usually results from a drug-related reaction. Both forms of the disease can be deadly as well as very painful and distressing. In most cases, these disorders are caused by a reaction to drugs, such as the cox-2 inhibitor Bextra, which has already been linked to SJS and TENS. Another drug commonly linked to Toxic Epidermal Necrolysis is the antibiotic, penicillin.
In many cases there is no known cause for TENS, although drugs are the main cause of this skin disease. This condition can be extremely serious, and can lead to massive discomfort and pain, and in some cases the infection that can be contracted through this disease can result in the death of the patient.
Any age group can find themselves affected by Toxic Epidermal Necrolysis. However, it is normally seen in older patients rather than younger ones, and this is because older patients tend to take more medication, and are therefore more likely to come across a drug to which they have a reaction. There are also other groups that are more vulnerable when it comes to contracting Toxic Epidermal Necrolysis.
This includes those with AIDS, who have lower immunity to infections and diseases. The elderly and all other age groups are advised to look out for any signs of TENS if they are on medication.
The symptoms & treatment of TENS
The symptoms of TENS
Both Toxic Epidermal Necrolysis and SJS can start with non-specific symptoms such as cough, aching, headaches, and feverishness. This can be followed by more specific symptoms such as a red rash across the face and the trunk of the body, which can continue to spread to other parts of the body. The rash can form into blisters, and these blisters can form in areas such as the eyes, mouth and vaginal areas. The mucous membranes can become inflamed, and with Toxic Epidermal Necrolysis layers of the skin can also come away with ease and often the skin peels away in sheets. The hair and nails can also come away in some cases, and sufferers can become cold and feverish.
The most common cause of death in the case of TENS is infection, which can be contracted through the exposed areas where the skin has come away from the flesh. The skin on patients that have this disease can resemble badly burned skin because of the extent to which it can flay, and exposed areas can seep fluids and become quickly infected.
Treatment for TENS
Those suffering from TENS are treated in hospital, and if the cause of the problem is drug related then the drugs are stopped immediately. Surviving patients are treated intravenously to replace any lost fluids, and the skin is left to re-grow on its own. However, the chances of survival can vary dramatically depending on the level of damage and the degree of infection incurred by the patient.
It is strongly advised that those taking drugs that could result in these TENS are vigilant and can identify the danger signs associated with this skin disease. Toxic Epidermal Necrolysis is potentially deadly, and the earlier the symptoms are recognised the faster treatment can be initiated.
People that have been affected by any drug in this way – or the families of those that have passed away because of problems and diseases such as TENS – may be eligible to claim compensation, and are advised to seek legal assistance as soon as possible following diagnosis of the disease
Both Toxic Epidermal Necrolysis and SJS can start with non-specific symptoms such as cough, aching, headaches, and feverishness. This can be followed by more specific symptoms such as a red rash across the face and the trunk of the body, which can continue to spread to other parts of the body. The rash can form into blisters, and these blisters can form in areas such as the eyes, mouth and vaginal areas. The mucous membranes can become inflamed, and with Toxic Epidermal Necrolysis layers of the skin can also come away with ease and often the skin peels away in sheets. The hair and nails can also come away in some cases, and sufferers can become cold and feverish.
The most common cause of death in the case of TENS is infection, which can be contracted through the exposed areas where the skin has come away from the flesh. The skin on patients that have this disease can resemble badly burned skin because of the extent to which it can flay, and exposed areas can seep fluids and become quickly infected.
Treatment for TENS
Those suffering from TENS are treated in hospital, and if the cause of the problem is drug related then the drugs are stopped immediately. Surviving patients are treated intravenously to replace any lost fluids, and the skin is left to re-grow on its own. However, the chances of survival can vary dramatically depending on the level of damage and the degree of infection incurred by the patient.
It is strongly advised that those taking drugs that could result in these TENS are vigilant and can identify the danger signs associated with this skin disease. Toxic Epidermal Necrolysis is potentially deadly, and the earlier the symptoms are recognised the faster treatment can be initiated.
People that have been affected by any drug in this way – or the families of those that have passed away because of problems and diseases such as TENS – may be eligible to claim compensation, and are advised to seek legal assistance as soon as possible following diagnosis of the disease
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