Vasculopathy, Immunodeficiency, and Bone Marrow Failure: The Intriguing Syndrome Caused by Deficiency of Adenosine Deaminase 2
Deficiency of adenosine deaminase 2 (DADA2) is a monogenic form of systemic vasculopathy that often presents during early childhood. Linked to biallelic mutations in ADA2 (previously CECR1), DADA2 was initially described as a syndrome of recurrent fever, livedo racemosa, early-onset strokes, and per...
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Format: | Article |
Language: | English |
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Frontiers Media S.A.
2018-10-01
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Series: | Frontiers in Pediatrics |
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Online Access: | https://www.frontiersin.org/article/10.3389/fped.2018.00282/full |