Literature DB >> 22471702

Deficiency of interleukin-1 receptor antagonist responsive to anakinra.

Charlotte Schnellbacher1, Giovanna Ciocca, Roxanna Menendez, Ivona Aksentijevich, Raphaela Goldbach-Mansky, Ana M Duarte, Rafael Rivas-Chacon.   

Abstract

We describe a 3-month-old infant who presented to our institution with interleukin (IL)-1 receptor antagonist deficiency (DIRA), which consists of neutrophilic pustular dermatosis, periostitis, aseptic multifocal osteomyelitis, and persistently high acute-phase reactants. Skin findings promptly improved upon initiation of treatment with anakinra (recombinant human IL-1 receptor antagonist), and the bony lesions and systemic inflammation resolved with continued therapy.
© 2013 Wiley Periodicals, Inc.

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Year:  2012        PMID: 22471702      PMCID: PMC3548019          DOI: 10.1111/j.1525-1470.2012.01725.x

Source DB:  PubMed          Journal:  Pediatr Dermatol        ISSN: 0736-8046            Impact factor:   1.588


  5 in total

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