Generalized arterial calcification of infancy: two siblings with prolonged survival
β Scribed by Giovanni Ciana; Antonella Trappan; Bruno Bembi; Alessandra Benettoni; Giampaolo Maso; Floriana Zennaro; Nico Ruf; Dirk Schnabel; Frank Rutsch
- Book ID
- 106120738
- Publisher
- Springer
- Year
- 2005
- Tongue
- English
- Weight
- 196 KB
- Volume
- 165
- Category
- Article
- ISSN
- 0340-6997
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## Abstract Generalized arterial calcification of infancy (GACI) (OMIM no. 208000) is characterized by calcification of the major arteries and soft tissues and associated with mutations in the __ENPP1__ gene. Most affected patients die within the first 6 months of life although prolonged survival i
## Generalized arterial calcification of infancy (GACI ,) is characterized by calcification of the internal elastic lamina of large and medium-sized arteries and stenosis due to myointimal proliferation. Although survival to adulthood has been reported, most patients die within the first six months