Neonatal Onset Multisystem Inflammatory Disease
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...
متن کاملSafe and effective canakinumab-treatment of neonatal onset multisystem inflammatory disease (NOMID)/ chronic infantile neurologic cutaneous and articular (CINCA)
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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Very-early-onset inflammatory bowel disease (VEO-IBD) has a distinct phenotype and should be considered a specific entity. VEO-IBD presents with very severe clinical pictures and is frequently known by an indeterminate colitis whose clinical remission is unmanageable. This study examines the case of a neonate with VEO-IBD, not responding to medical and surgical treatment. A 7-day-old Iranian fe...
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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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ژورنال
عنوان ژورنال: Arthritis & Rheumatism
سال: 1983
ISSN: 0004-3591,1529-0131
DOI: 10.1002/art.1780260515