Neonatal-Onset Multisystem Inflammatory Disease Responsive to Interleukin-1β Inhibition
Author(s) -
Raphaela GoldbachMansky,
Natalie Dailey Garnes,
Scott Canna,
A Gelabert,
Janet H. Jones,
Benjamin I. Rubin,
H. Jeffrey Kim,
Carmen C. Brewer,
Christopher Zalewski,
Edythe Wiggs,
Suvimol Hill,
Maria L. Turner,
Barbara I. Karp,
Ivona Aksentijevich,
Frank Pucino,
Scott Penzak,
Margje H. Haverkamp,
Leonard D. Stein,
Barbara S. Adams,
Terry L. Moore,
Robert C. Fuhlbrigge,
Bracha Shaham,
James N. Jarvis,
Kathleen M. O’Neil,
Richard K. Vehe,
Laurie Beitz,
Gregory C. Gardner,
William P. Hannan,
Robert W. Warren,
William Horn,
Joe L. Cole,
Scott M. Paul,
Philip N. Hawkins,
Tuyet Pham,
Christopher S. Snyder,
Robert Wesley,
Steven C. Hoffmann,
Steven M. Holland,
John A. Butman,
Daniel L. Kastner
Publication year - 2006
Publication title -
new england journal of medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 19.889
H-Index - 1030
eISSN - 1533-4406
pISSN - 0028-4793
DOI - 10.1056/nejmoa055137
Subject(s) - anakinra , medicine , erythrocyte sedimentation rate , rash , gastroenterology , c reactive protein , serum amyloid a , immunology , inflammation , disease
Neonatal-onset multisystem inflammatory disease is characterized by fever, urticarial rash, aseptic meningitis, deforming arthropathy, hearing loss, and mental retardation. Many patients have mutations in the cold-induced autoinflammatory syndrome 1 (CIAS1) gene, encoding cryopyrin, a protein that regulates inflammation.
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