@article {1680, title = {Defective FOXP3 expression in patients with acute Kawasaki disease and restoration by intravenous immunoglobulin therapy.}, journal = {Clin Exp Rheumatol}, volume = {28}, year = {2010}, month = {2010 Jan-Feb}, pages = {93-7}, abstract = {

OBJECTIVES: The aims of this study were: 1) to investigate forkhead box P3 (FOXP3) expression in patients with Kawasaki disease (KD), exploring possible differences during the acute phase and after defervescence; 2) to evaluate a possible association of the FOXP3 single nucleotide polymorphism (SNP) 543 (SNP ID: rs2232367) with KD.

METHODS: FOXP3 expression was evaluated on 8 children with KD and 15 healthy children by flow cytometry and Real-Time polymerase chain reaction (RT-PCR). FOXP3 SNP 543 was genotyped by denaturing high-performance liquid chromatography (DHPLC) and sequencing on DNA samples from 58 additional children with KD and 114 healthy donors.

RESULTS: The frequencies of CD4+CD25 +FOXP3+ regulatory T cells were significantly (p=0.0002) lower during the acute phase of KD than in sex- and age-matched healthy donors (median \% + SD: 4.8+/-1.3 vs. 7.7+/-1.7) and a similar tendency was revealed for FOXP3 mRNA transcripts (p<0.0001). FOXP3 expression increased significantly, at both protein and mRNA levels, after intravenous immunoglobulin (IVIG) therapy treatment and achieving complete remission of disease (at least 48 hrs; median 9.5 days, range 2-30). Of the 58 patients screened, only one female subject (1.7\%) carried the presence of 543 SNP in heterozygosis (C>T; for a total of 1 allele out of 88), with no difference between KD patients and controls (0.0\%, 0/203 alleles).

CONCLUSIONS: Our data reinforce the notion of an impaired immunoregulation in KD, suggesting also a role of IVIG treatment in modifying the Treg compartment. FOXP3 SNP 543 does not seem to be involved in susceptibility to KD in Italian children.

}, keywords = {Acute Disease, Child, Child, Preschool, Female, Flow Cytometry, Forkhead Transcription Factors, Genotype, Humans, Immunoglobulins, Intravenous, Infant, Italy, Male, Mucocutaneous Lymph Node Syndrome, Polymorphism, Single Nucleotide, Reverse Transcriptase Polymerase Chain Reaction, RNA, Messenger, T-Lymphocytes, Regulatory}, issn = {0392-856X}, author = {Olivito, Biagio and Taddio, Andrea and Simonini, Gabriele and Massai, Cristina and Ciullini, Sara and Gambineri, Eleonora and de Martino, Maurizio and Azzari, Chiara and Cimaz, Rolando} }