Disease Information for Progeria/adult (Werners syndrome)

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Clinical Manifestations
Signs & Symptoms
Facies particular
Baldness/Alopecia
Coarsening skin
Gray Hair
Gray hair, premature
Hair loss
Increased wrinkling/skin
Localized Scleroderma Skin area
Non-scarring Alopecia/balding
White Hair
High-pitched voice
Premature Aging
Short stature
Short stature Child
Blue Sclera
Typical Clinical Presentation
Presentation/Coronary Heart Disease Young Age
Clinical Presentation & Variations
Presentation/Arteriosclerosis Young Age
Presentation/Myocardial Infarction in a Child
Presentation/Juvenile Cataract
Disease Progression
Course/Chronic disorder
Course/Chronic only
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Children/all
Diagnostic Test Results
X-RAY
Xray/Calcified artery/veins finding
Xray/Subcutaneous ossification centers/calcification
Xray/Muscle calcification
Xray/Soft tissue calcification/muscle/subcutaneous
Xray/Distal Clavicle Bone Resorption/Chest
Xray/Periarticular knee calcifications
Associated Diseases & Rule outs
Rule Outs
Scleroderma, progressive systemic
Associated Disease & Complications
Arteriosclerosis
Cancer/malignancy
Cardiomyopathy
Cataract
Cerebral thrombosis
Cerebral vascular accident
Diabetes mellitus
Ischemia, cardiac
Myocardial infarction, acute
Nail dystrophy
Premature aging/senescence
Progeria
Progeria of adult (Werners Syndrome)
Stroke /Young adult
Testicular atrophy
Diabetes mellitus/non-insulin dep/type II
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
Pathophysiology
Pathophysiology/Gene locus 8p11.2
Pathophysiology/Gene locus 8p12-p11.2
Pathophysiology/Gene Locus Identified/OMIM database
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Pathophysiology/Myocardial Ischemia Non-Arteriosclerosis
Pathophysiology/Insulin resistant states
Pathophysiology/Gene locus 8p12
Pathophysiology/Gene locus chromosome 8
Process
PROCESS/Age/senescence disorder (ex).
PROCESS/Autosomal recessive disorder (ex)
PROCESS/Congenital/developmental (category)
PROCESS/Constitutional/essential disorder (ex)
PROCESS/Hereditofamilial (category)
Synonyms
Synonym
Adult prem ageing syndrome, Adult premature ageing syndrome, Adult premature aging syndrome, Adult progeria, Pangeria, Progeria Adult, PROGERIA OF ADULT, Progeria of the adult, Syndrome Werner, Syndrome Werner's, Werner, Werner Syndrome, Werner syndrome (disorder), Werners Syndrome, Werner's Syndrome, WRN, WS, Synonym/Werner's syndrome
Definition

Werner Syndrome; Progeria of Adulthood; WNS; WS; 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 childhood, adolescence, and early adulthood; Children with Werner Syndrome have an abnormally slow growth rate, and there is cessation of growth at puberty; As a result, affected individuals have unusually short stature and low weight even 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 due to aging (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 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[nord 2005]

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External Links Related to Progeria/adult (Werners syndrome)
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Wikipedia
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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