title: Fibroblast growth factor 23 (FGF23) gene polymorphism in children with Kawasaki syndrome (KS) and susceptibility to cardiac abnormalities.
abstract: BACKGROUND
  Fibroblast Growth Factor (FGF) 23 influences endothelial integrity and few reports have studied the association between FGF23 and Kawasaki syndrome (KS), a childhood vasculitis displaying a high risk of subsequent cardiac abnormalities (CaA).
  
  
  OBJECTIVE
  To investigate the genetic variation in the FGF23 gene in a cohort of KS children and its association with serum FGF23 levels and eventual development of CaA, including both coronary artery dilatations and aneurysms.
  
  
  METHODS
  84 Italian KS children were recruited; 24/84 (28.6%) developed CaA. Each patient underwent evaluation of serum FGF23 levels and FGF23 genotype: the frequency of the c.212-37insC (rs3832879) polymorphism in intron 1 was examined and compared with sex, age at disease onset, fever duration, laboratory data, and occurrence of CaA. Univariate statistical analysis of categorical parameters was performed by the Pearson's Chi-square test or Fisher's exact test as appropriate. Parametric variables were assessed by Student's t-test for unpaired data. Independent predictors of disease were studied by a logistic regression model.
  
  
  RESULTS
  28/84 patients carried the FGF23 polymorphism (33.3%) and had higher serum FGF23 levels (p < 0.01). FGF23 polymorphism was significantly associated with CaA compared to wild type FGF23 children (respectively, p = 0.03 and p = 0.05). The comparison with demographical, clinical or laboratory data was not significant.
  
  
  CONCLUSIONS
  The prevalent segregation of the c.212-37insC polymorphism in children with CaA advocates a possible functional FGF23 role in the predisposition to higher serum levels of FGF23 and potential occurrence of any coronary artery abnormalities in KS.
authors.affiliation: Department of Internal Medicine, Section of Rheumatology, Transition Clinic, University of Florence, Florence, Italy. falcini@unifi.it.
authors.forename: Fernanda
authors.initials: F
authors.lastname: Falcini
authors.forename: Donato
authors.initials: D
authors.lastname: Rigante
authors.forename: Laura
authors.initials: L
authors.lastname: Masi
authors.forename: Marcello
authors.initials: M
authors.lastname: Covino
authors.forename: Francesco
authors.initials: F
authors.lastname: Franceschelli
authors.forename: Gigliola
authors.initials: G
authors.lastname: Leoncini
authors.forename: Giusyda
authors.initials: G
authors.lastname: Tarantino
authors.forename: Marco
authors.initials: M
authors.lastname: Matucci Cerinic
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Brandi
mesh_terms: D017677:Age Distribution; D000704:Analysis of Variance; D002675:Child, Preschool; D015331:Cohort Studies; D016001:Confidence Intervals; D005260:Female; D000089703:Fibroblast Growth Factor-23; D005346:Fibroblast Growth Factors / Q000235:genetics*; D020022:Genetic Predisposition to Disease / Q000453:epidemiology*; D014644:Genetic Variation; D006330:Heart Defects, Congenital / Q000453:epidemiology* / Q000235:genetics* / Q000503:physiopathology; D006801:Humans; D015994:Incidence; D007223:Infant; D007558:Italy; D016015:Logistic Models; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D015999:Multivariate Analysis; D016133:Polymerase Chain Reaction / Q000379:methods; D011110:Polymorphism, Genetic; D011379:Prognosis; D018570:Risk Assessment; D017678:Sex Distribution; D018709:Statistics, Nonparametric
chemical_list: C000717427:FGF23 protein, human; D005346:Fibroblast Growth Factors; D000089703:Fibroblast Growth Factor-23