Werner syndrome is a disease where the person ages rapidly starting in early adolescence or adulthood. Progeria is a disorder of fast aging that happens in childhood.

What is Werner syndrome?

Definition:

Werner syndrome is an inherited condition of fast aging and high cancer risk, beginning in adolescence or early adulthood. This syndrome is sometimes also known as adult progeria.

Causes and prevalence:

Werner syndrome is a genetic condition that is due to a mutation of the WRN gene. This is the gene that fixes DNA errors. The disease is rare and follows autosomal recessive inheritance rules. This means that people who carry the mutated gene for the disease can pass it to their children. The prevalence of Werner syndrome is about 1 in every 100,000 births but is more common in Japan.

Symptoms and complications:

Symptoms of the condition include being short, having gray hair that thins. The skin of these patients demonstrates signs of aging, including discolorations and wrinkles. By age 30, patients also show cataracts, diabetes, osteoporosis; the nose often has a beaked shape. Patients usually die in their 40s or 50s from age-related problems. Patients have an elevated risk of cancer such as thyroid cancer or melanoma.

Diagnosis:

The diagnosis of Werner syndrome is by the physical changes that are seen in the body, the age of the person, and the presence of the mutated WRN gene.

Treatment:

The treatment varies depending on the symptoms. Treatment may be aimed at alleviating symptoms and fixing problems related to the aging process. In the case of cancer, chemotherapy, surgery, or radiation therapy may be done. Cataracts can be corrected with surgery and diabetes managed with medication. 

What is Progeria?

Definition:

Progeria is a condition of rapid aging that begins in the first two years of life. 

Causes and prevalence:

Progeria is caused by a random genetic mutation of the Lamin A (LMNA) gene. This is a gene that makes certain types of proteins known as lamins. Progeria is a rare disease with only 1 in about 4 million children having the condition.

Symptoms and complications:

The signs of the disorder first appear in very young children. The signs include specific physical traits that include an unusually large head, small eyes, small chin, and a narrow nose. The nose also often looks beaked. The condition always leads to death due to typical conditions we associate with old age such as heart problems like coronary artery disease.

Diagnosis:

Diagnosis is made based on physical appearance of the child and genetic testing where variants of the LMNA gene are detected.

Treatment:

There is a medication called Lonafarnib that helps to extend life by interfering with a protein that builds up in patients with progeria.

Difference between Werner syndrome and Progeria?

Definition

Werner syndrome is a disorder of rapid aging and increased risk of cancer that begins in early adulthood or adolescence. Progeria is a disorder of rapid aging that starts at a very young age.

Age of onset

The age that Werner syndrome begins is in adolescence or in early adulthood. The age that progeria begins is in early childhood, often by age 2.

Average life span

The average life span of a person with Werner syndrome is 40 or 50 years. The average life span of a person with progeria is 14 years.

Physical appearance

Thinning and gray hair, beaked nose, and discolored and wrinkled skin before age 30 are features of Werner syndrome. Large eyes and head; narrow nose, and a smaller than expected chin and mouth are features seen in children with progeria.

Treatment

The treatment of Werner syndrome varies but may include medication for conditions like diabetes; cancer treatment methods such as chemotherapy and radiation therapy may also be required. The treatment of progeria relies on the medicine called Lonafarnib.

Most common cause of death

Most people with Werner syndrome die from cancer or heart disease. Most people with progeria die from atherosclerosis or coronary artery disease.

Table comparing Werner syndrome and Progeria

Summary of Werner syndrome and Progeria

• Werner syndrome is like the Hutchinson-Gillford progeria in that it also causes aging.
• Werner syndrome affects young adults while progeria affects children.
• Progeria is a rare childhood condition with distinctive features.
• People with Werner syndrome show rapid aging but they also have a high cancer risk.

FAQ

What are similar syndromes to progeria?

Cockayne syndrome and Werner are similar to Hutchinson-Gillford progeria, in that these are all aging syndromes.

What are the two types of progeria?

Werner syndrome and Hutchinson-Gillford progeria are both progeroid disorders.

What is the life expectancy of someone with Werner syndrome?

An individual with Werner syndrome can expect to live about 40 years to 50 years.

What disease is opposite of progeria?

There is no disease that causes reverse aging.

What age does Werner syndrome start?

Werner syndrome begins sometime in adolescence of early adulthood.

What is the progression of Werner syndrome?

The person with this syndrome shows signs of aging such as gray and thinning hair and later develops cataracts, diabetes, and cancer.

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