title: The anesthetic management of children with neonatal-onset multi-system inflammatory disease.
abstract: BACKGROUND
  Neonatal-onset multi-system inflammatory disease (NOMID), a rare autosomal dominantly inherited disease, belongs to a growing spectrum of autoinflammatory diseases, is characterized by urticarial rash, arthropathy, and chronic aseptic meningitis, and is associated with mutations in the cold-induced autoinflammatory gene, CIAS1, the gene that encodes the protein, cryopyrin. As little is known about the anesthetic considerations of the disease, we sought to identify the main features and respective anesthetic and perioperative implications of NOMID.
  
  
  METHODS
  We examined perianesthetic records of children with NOMID who were anesthetized for invasive diagnostic and therapeutic interventions between 2003 and 2006. In addition, we conducted an extensive literature review of the genetic, clinical, and biochemical abnormalities of the disease.
  
  
  RESULTS
  Seventeen children with NOMID (median age 8 yr, range 9 mo to 11 yr) were anesthetized for diagnostic and therapeutic procedures. All patients had neurological involvement, including increased intracranial pressure, chronic aseptic meningitis, and developmental delay; 7 had bony overgrowth, 15 ocular, and 14 otological manifestations of NOMID. Despite the complexity of the disease, the perioperative course was uncomplicated, and no serious adverse events were observed.
  
  
  CONCLUSIONS
  This study is the first to investigate the anesthetic implications of NOMID, an autoinflammatory disease associated with arthropathy, recurrent fevers, urticarial rash, and chronic aseptic meningitis. While for the pediatric anesthesiologist, the presence of fever and aseptic meningitis might make the conduct of anesthetics for elective procedures less desirable, our findings suggest that without evidence of active infection, even in the presence of fever and chronic aseptic meningitis, general and regional anesthesia may be conducted in patients with NOMID without untoward complications.
authors.affiliation: Department of Anesthesia and Surgical Services, National Institutes of Health Clinical Center, National Institutes of Health, Bethesda, MD 20892-1512, USA.
authors.forename: Christine F
authors.initials: CF
authors.lastname: Lauro
authors.forename: Raphaela
authors.initials: R
authors.lastname: Goldbach-Mansky
authors.forename: Margaret
authors.initials: M
authors.lastname: Schmidt
authors.forename: Zenaide M N
authors.initials: ZM
authors.lastname: Quezado
mesh_terms: D000777:Anesthetics / Q000009:adverse effects / Q000627:therapeutic use*; D020274:Autoimmune Diseases of the Nervous System / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D002352:Carrier Proteins / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D019468:Disease Management; D005260:Female; D006801:Humans; D007223:Infant; D007249:Inflammation / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D008297:Male; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein
chemical_list: D000777:Anesthetics; D002352:Carrier Proteins; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C453881:NLRP3 protein, human