Werner Syndrome

Werner syndrome, also known as progeria of the adult, is the most common of the premature aging disorders. Werner syndrome is inherited as an autosomal recessive disorder of chromosome 8, meaning that a defective gene is inherited from each parent. The syndrome is estimated to occur in 1 in 1 million individuals. Higher incidences of Werner syndrome have been reported in Japan and Sardinia. It affects both males and females.

Symptoms
Werner syndrome may begin in adolescence, but most often it begins in the mid-30s. Up until symptoms begin, the individual has healthy normal development. Once the disease process begins, the individual's body begins to age faster than normal. This produces symptoms such as:

Wrinkling and sagging of the face
Decreasing muscle mass
Thin skin and loss of fat under the skin
Graying hair and hair loss
A high-pitched voice

Along with the physical appearance of aging, body organs and systems also begin to age. This produces diseases such as:

Cataracts (cloudy spots on the lens of the eye)
Osteoporosis (weakened bones)
Diabetes (type 2)
Rare cancers such as thyroid cancer, lymphoma, and sarcoma
Heart and artery disease
Premature menopause in females.

Diagnosis and treatment
Diagnosis of Werner syndrome is based on physical examination. The most striking symptom is that the individual appears to be much older than he/she really is. There are no specific laboratory tests for Werner syndrome. However, since diabetes may occur, the blood sugar level should be checked, and since artery disease may develop, cholesterol levels should be monitored, and treated if necessary. There is no cure or specific treatment for Werner syndrome.

Future research
Scientists have developed a strain of mice which have all the symptoms of Werner syndrome. They plan to use the mice in research to look at the relationship between aging and cancer, as well as gaining insight into the mechanisms at work in Werner syndrome.


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