@article {8483, title = {Describing Kawasaki shock syndrome: results from a retrospective study and literature review.}, journal = {Clin Rheumatol}, volume = {36}, year = {2017}, month = {2017 Jan}, pages = {223-228}, abstract = {

Kawasaki shock syndrome (KSS) is a rare manifestation of Kawasaki disease (KD) characterized by systolic hypotension or clinical signs of poor perfusion. The objectives of the study are to describe the main clinical presentation, echocardiographic, and laboratory findings, as well as the treatment options and clinical outcomes of KSS patients when compared with KD patients. This is a retrospective study. All children referred to two pediatric rheumatology units from January 1, 2012, to December 31, 2014, were enrolled. Patients were divided into patients with or without KSS. We compared the two groups according to the following variables: sex, age, type of KD (classic, with less frequent manifestations, or incomplete), clinical manifestations, cardiac involvement, laboratory findings, therapy administered, response to treatment, and outcome. Eighty-four patients with KD were enrolled. Of these, five (6~\%) met the criteria for KSS. Patients with KSS had higher values of C-reactive protein (p = 0.005), lower hemoglobin levels (p = 0.003); more frequent hyponatremia (p = 0.004), hypoalbuminemia (p = 0.004), and coagulopathy (p = 0.003); and increase in cardiac troponins (p = 0.000). Among the KSS patients, three had a coronary artery involvement, but none developed a permanent aneurysm. Intravenous immunoglobulin resistance was more frequent in the KSS group, although not significantly so (3/5, 60~\% vs. 23/79, 30~\%, P = NS). None of the five cases was fatal, and all recovered without sequelae. KSS patients are more likely to have higher rates of cardiac involvement. However, most cardiovascular abnormalities resolved promptly with therapy.

}, keywords = {C-Reactive Protein, Child, Child, Preschool, Echocardiography, Female, Heart Failure, Hemoglobins, Humans, Immunoglobulins, Intravenous, Male, Mucocutaneous Lymph Node Syndrome, Retrospective Studies, Shock, Syndrome}, issn = {1434-9949}, doi = {10.1007/s10067-016-3316-8}, author = {Taddio, Andrea and Rossi, Eleonora Dei and Monasta, Lorenzo and Pastore, Serena and Tommasini, Alberto and Lepore, Loredana and Bronzetti, Gabriele and Marrani, Edoardo and Mottolese, Biancamaria D{\textquoteright}Agata and Simonini, Gabriele and Cimaz, Rolando and Ventura, Alessandro} } @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} }