Mutant Adenosine Deaminase 2 in a Polyarteritis Nodosa Vasculopathy


Elkan P. N. , Pierce S. B. , Segel R., Walsh T., Barash J., Padeh S., et al.

NEW ENGLAND JOURNAL OF MEDICINE, cilt.370, ss.921-931, 2014 (SCI İndekslerine Giren Dergi)

  • Cilt numarası: 370 Konu: 10
  • Basım Tarihi: 2014
  • Doi Numarası: 10.1056/nejmoa1307362
  • Dergi Adı: NEW ENGLAND JOURNAL OF MEDICINE
  • Sayfa Sayısı: ss.921-931

Özet

BackgroundPolyarteritis nodosa is a systemic necrotizing vasculitis with a pathogenesis that is poorly understood. We identified six families with multiple cases of systemic and cutaneous polyarteritis nodosa, consistent with autosomal recessive inheritance. In most cases, onset of the disease occurred during childhood.