{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,30]],"date-time":"2025-11-30T09:10:08Z","timestamp":1764493808784,"version":"3.40.5"},"reference-count":29,"publisher":"American Academy of Pediatrics (AAP)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,11,1]]},"abstract":"<jats:p>Neonatal onset multisystem inflammatory disease (NOMID)\/chronic infantile neurologic cutaneous and articular (CINCA) syndrome is a rare, early-onset autoinflammatory disorder and the most severe form of cryopyrin-associated periodic syndrome, which is associated with overproduction of interleukin (IL)-1\u03b2. This is a case report of a 70-day-old boy, who was diagnosed with NOMID\/CINCA syndrome and who has been treated with anti\u2013IL-1\u03b2 monoclonal antibody (canakinumab) since then, despite his early infancy. The patient presented with fever, aseptic meningitis, and rash. The clinical manifestations combined with the elevated acute-phase reactants strengthened the suspicion of the diagnosis of NOMID\/CINCA syndrome. Specific immunologic workup revealed high levels of serum amyloid A and IL-6. The clinical diagnosis was confirmed by the detection of a de novo mutation of the CIAS1\/NLR3 gene (p.Thr348Met), and canakinumab was started at a dose of 4 mg\/kg, higher than the recommended dose for older age. White blood cell, serum amyloid A, C-reactive protein, and IL-6 levels quickly decreased and became normal within a month, and the clinical condition of the patient improved significantly. The infant remains without recurrence of disease or further complications and with satisfactory mental development with anti\u2013IL-1\u03b2 monoclonal antibody treatment for &amp;gt;2 years. This report indicates the importance of early diagnosis of NOMID\/CINCA syndrome and medication with IL-1 blockers as soon as possible for the improvement of the prognosis of cryopyrin-associated periodic syndrome and of a better patient outcome.<\/jats:p>","DOI":"10.1542\/peds.2013-3185","type":"journal-article","created":{"date-parts":[[2014,10,28]],"date-time":"2014-10-28T03:47:28Z","timestamp":1414468048000},"page":"e1468-e1473","source":"Crossref","is-referenced-by-count":11,"title":["Successful Management of Cryopyrin-Associated Periodic Syndrome With Canakinumab in Infancy"],"prefix":"10.1542","volume":"134","author":[{"given":"Maria","family":"Kanariou","sequence":"first","affiliation":[{"name":"Department of Immunology-Histocompatibility, Specialized Center and Referral Center for Primary Immunodeficiencies\u2013Pediatric Immunology,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sofia","family":"Tantou","sequence":"additional","affiliation":[{"name":"Department of Immunology-Histocompatibility, Specialized Center and Referral Center for Primary Immunodeficiencies\u2013Pediatric Immunology,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ioanna","family":"Varela","sequence":"additional","affiliation":[{"name":"Department of Immunology-Histocompatibility, Specialized Center and Referral Center for Primary Immunodeficiencies\u2013Pediatric Immunology,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria","family":"Raptaki","sequence":"additional","affiliation":[{"name":"Department of Immunology-Histocompatibility, Specialized Center and Referral Center for Primary Immunodeficiencies\u2013Pediatric Immunology,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chrissa","family":"Petropoulou","sequence":"additional","affiliation":[{"name":"B 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