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"Resources" on this report.
Copyright 1986, 1990, 1994, 1996, 1998, 1999, 2003, 2007, 2009
NORD is very grateful to Dru Leistritz, MS, Genetic Counselor, International Registry of Werner Syndrome, Department of Pathology, University of Washington, for assistance in the preparation of this report.
Synonyms of Werner Syndrome
- Atypical Werner Syndrome
- Progeria of Adulthood
- WNS
- WS
Disorder Subdivisions
General Discussion Werner Syndrome is a rare progressive disorder that is characterized by the appearance of unusually accelerated aging (progeria). Although the disorder is typically recognized by the third or fourth decades of life, certain characteristic findings are present beginning during adolescence and early adulthood.
Individuals with Werner Syndrome have an abnormally slow growth rate, and there is cessation of growth at puberty. As a result, affected individuals have short stature and low weight relative to height. By age 25, those with the disorder typically experience early graying (canities) and premature loss of scalp hair (alopecia). As the disease progresses, additional abnormalities include loss of the layer of fat beneath the skin (subcutaneous adipose tissue); severe wasting (atrophy) of muscle tissue in certain areas of the body; and degenerative skin changes, particularly in the facial area, the upper arms and hands, and the lower legs and feet (distal extremities). Due to degenerative changes affecting the facial area, individuals with Werner Syndrome may have unusually prominent eyes, a beaked or pinched nose, and/or other characteristic facial abnormalities.
Werner Syndrome may also be characterized by development of a distinctive high-pitched voice; eye abnormalities, including premature clouding of the lenses of the eyes (bilateral senile cataracts); and certain endocrine defects, such as impaired functioning of the ovaries in females or testes in males (hypogonadism) or abnormal production of the hormone insulin by the pancreas and resistance to the effects of insulin (non-insulin-dependent diabetes mellitus). In addition, individuals with Werner syndrome may develop progressive thickening and loss of elasticity of artery walls (arteriosclerosis). Affected blood vessels typically include the arteries that transport oxygen-rich (oxygenated) blood to heart muscle (coronary arteries). Some affected individuals may also be susceptible to developing certain benign (noncancerous) or malignant tumors. Progressive arteriosclerosis, malignancies, and/or associated abnormalities may result in potentially life-threatening complications by approximately the fourth or fifth decade of life. Werner syndrome is inherited as an autosomal recessive trait.
Organizations related to Werner Syndrome
- Cancer.Net
American Society of Clinical Oncology
2318 Mill Road Suite 800 Alexandria VA 22314
Phone #: 571-483-1780
800 #: 888-651-3038
e-mail: contactus@cancer.net
Home page: http://www.cancer.net/patient
- Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg MD 20898-8126
Phone #: 301-251-4925
800 #: 888-205-2311
e-mail: ordr@od.nih.gov
Home page: http://rarediseases.info.nih.gov/Default.aspx
- International Registry of Werner Syndrome
University of Washington Department of Pathology
Box 357470 Health Science Building Room K543 1959 NE Pacific Street Seattle WA 98195-7470
Phone #: 206-543-5088
800 #: --
e-mail: dru2@u.washington.edu
Home page: http://www.wernersyndrome.org/registry/registry.html
- March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains NY 10605
Phone #: 914-997-4488
800 #: 888-663-4637
e-mail: Askus@marchofdimes.com
Home page: http://www.marchofdimes.com
- NIH/National Institute of Allergy and Infectious Diseases
6610 Rockledge Drive
MSC 6612 Bethesda MD 20892-6612
Phone #: 301-496-5717
800 #: --
e-mail: N/A
Home page: http://www.niaid.nih.gov/
- NIH/National Institute on Aging
PO Box 8057
Gaithersburg MD 20892-8057
Phone #: 301-496-1752
800 #: 800-222-2225
e-mail: N/A
Home page: http://www.nih.gov/nia
- Progeria Research Foundation, Inc.
2 Bourbon Street
Suite 208 Peabody MA 01960
Phone #: 978-535-2594
800 #: --
e-mail: info@progeriaresearch.org
Home page: http://www.progeriaresearch.org
- Rare Cancer Alliance
1649 North Pacana Way
Green Valley AZ 85614
Phone #: 520-625-5495
800 #: --
e-mail: sharon.lane@rare-cancer.org
Home page: http://www.rare-cancer.org
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