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Absent tibiae—polydactyly—triphalangeal thumbs with fibular dimelia: Variable expression of the Werner (McKusick 188770) syndrome?

✍ Scribed by Vargas, Fernando Regla ;Pontes, Ricardo Lopes ;Llerena, Juan Clinton ;de Almeida, Jose Carlos Cabral


Publisher
John Wiley and Sons
Year
1995
Tongue
English
Weight
459 KB
Volume
55
Category
Article
ISSN
0148-7299

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✦ Synopsis


We report on a family in which the auto-soma1 dominant Werner syndrome (WS) (MIM# 188770) affects ten members in three generations. Besides the absent tibiae the propositus had duplication of the fibulae. Possible pathogenetic mechanism is discussed.