title: Discrimination between incomplete and atypical Kawasaki syndrome versus other febrile diseases in childhood: results from an international registry-based study.
abstract: OBJECTIVE
  Kawasaki syndrome (KS) is an acute systemic vasculitis of unknown origin predominantly affecting young children. Early diagnosis is crucial to prevent cardiac complications. However, the differential diagnosis of patients with the incomplete or atypical form of the disease poses a heavy challenge for the paediatrician. Our aim was to evaluate the prevalence of incomplete and atypical cases among children with KS and to identify clinical and laboratory variables that may help differentiate incomplete and atypical KS from other febrile diseases at this age.
  
  
  METHODS
  We established an international registry to recruit patients with KS, including those with incomplete and atypical forms. The control group included age-matched febrile children admitted to the hospital with a variety of diseases mimicking KS. The aim was to define clinical or laboratory clues to help in the discrimination of incomplete and atypical KS patients from others.
  
  
  RESULTS
  Two hundred and twenty-eight patients with incomplete KS (78%) and atypical KS (22%) were compared to 71 children with other febrile diseases. Patients with incomplete and atypical KS presented a statistically significant higher frequency of mucosal changes, conjunctivitis, extremity abnormalities and perineal desquamation compared to the group of other febrile diseases. In addition, C-reactive protein and platelet counts were significantly higher in incomplete and atypical KS compared to the other group.
  
  
  CONCLUSIONS
  This is the largest series of incomplete and atypical KS patients of non East-Asian ancestry: we suggest that in patients with the aforementioned clinical features and laboratory evidence of systemic inflammation in terms of increased C-reactive protein and platelet counts an echocardiogram should be performed and diagnosis of KS considered.
authors.affiliation: Department of Internal Medicine, University of Florence, Italy. falcini@unifi.it
authors.forename: Fernanda
authors.initials: F
authors.lastname: Falcini
authors.forename: Seza
authors.initials: S
authors.lastname: Ozen
authors.forename: Silvia
authors.initials: S
authors.lastname: Magni-Manzoni
authors.forename: Marco
authors.initials: M
authors.lastname: Candelli
authors.forename: Laura
authors.initials: L
authors.lastname: Ricci
authors.forename: Giorgia
authors.initials: G
authors.lastname: Martini
authors.forename: Ruben J
authors.initials: RJ
authors.lastname: Cuttica
authors.forename: Sheila
authors.initials: S
authors.lastname: Oliveira
authors.forename: Giovanni Battista
authors.initials: GB
authors.lastname: Calabri
authors.forename: Francesco
authors.initials: F
authors.lastname: Zulian
authors.forename: Angela
authors.initials: A
authors.lastname: Pistorio
authors.forename: Francesco
authors.initials: F
authors.lastname: La Torre
authors.forename: Donato
authors.initials: D
authors.lastname: Rigante
mesh_terms: D000704:Analysis of Variance; D015415:Biomarkers / Q000097:blood; D002097:C-Reactive Protein / Q000032:analysis; D016022:Case-Control Studies; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D042241:Early Diagnosis; D005060:Europe / Q000453:epidemiology; D005260:Female; D005334:Fever / Q000097:blood / Q000175:diagnosis* / Q000453:epidemiology / Q000276:immunology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D018836:Inflammation Mediators / Q000097:blood; D007557:Israel / Q000453:epidemiology; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000097:blood / Q000175:diagnosis* / Q000453:epidemiology / Q000276:immunology; D010976:Platelet Count; D011237:Predictive Value of Tests; D015995:Prevalence; D011379:Prognosis; D012042:Registries; D013020:South America / Q000453:epidemiology; D014421:Turkey / Q000453:epidemiology
chemical_list: D015415:Biomarkers; D018836:Inflammation Mediators; D002097:C-Reactive Protein