Neonatal-Onset Multisystem Inflammatory Disease Responsive to Interleukin-1β Inhibition
نویسندگان
چکیده
منابع مشابه
Neonatal-onset multisystem inflammatory disease responsive to interleukin-1beta inhibition.
BACKGROUND 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. METHODS We selected 18 patients with neonatal-onset multisyst...
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The clinical features, the underlying CIAS1 mutation, and the results of cytokine analyses are described for a 10-year-old German boy with neonatal-onset multisystem inflammatory disease, whose condition improved with age. Disease onset occurred at 26 months of age with predominantly cutaneous (urticarial rash) and neurologic (headache, chronic meningitis) symptoms including early bilateral opt...
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Introduction NOMID/CINCA is the most severe phenotype of cryopyrin-associated periodic syndrome (CAPS), characterized by persistence of inflammation-mediated symptoms and overproduction of interleukin (IL)-1b, associated with significant morbidity, if untreated. In CAPS-patients early initiation of anti-IL1b-treatment appears to prevent severe disease sequelae. However, canakinumab as a 1-line ...
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ژورنال
عنوان ژورنال: Pediatrics
سال: 2007
ISSN: 0031-4005,1098-4275
DOI: 10.1542/peds.2007-0846kkkk