Werner syndrome
Medical condition / From Wikipedia, the free encyclopedia
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Werner syndrome (WS) or Werner's syndrome, also known as "adult progeria",[1] is a rare, autosomal recessive disorder[2] which is characterized by the appearance of premature aging.[3]
Werner syndrome (progeria) | |
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Werner syndrome has an autosomal recessive pattern of inheritance. | |
Specialty | Endocrinology |
Werner syndrome is named after the German scientist Otto Werner.[4] He identified the syndrome in four siblings observed with premature aging, which he explored as the subject of his dissertation of 1904.[5]
It has a global incidence rate of less than 1 in 100,000 live births[6] (although incidence in Japan and Sardinia is higher, affecting 1 in 20,000–40,000 and 1 in 50,000, respectively).[7][8] 1,300 cases had been reported as of 2006.[9] Affected individuals typically grow and develop normally until puberty; the mean age of diagnosis is twenty-four, often realized when the adolescent growth spurt is not observed.[10] The youngest person diagnosed was six years old.[11] The median and mean ages of death are 47–48 and 54 years, respectively.[12] The main causes of death are cardiovascular disease and cancer.[9][10]