Vasa vasorum enhancement on optical coherence tomography in Kawasaki disease.

Background: Patients with Kawasaki disease (KD) prone to develop coronary artery aneurysm (CAA) with unknown etiology. We aimed to disclose the relationship between vasa vasorum (VV) and intimal thickening using optical coherence tomography (OCT) in KD.

Methods: Forty-three coronary artery branches of 21 patients with KD were examined by OCT.

Analysis of Age, Sex, Lack of Response to Intravenous Immunoglobulin, and Development of Coronary Artery Abnormalities in Children With Kawasaki Disease in Japan.

Importance: Initial intravenous immunoglobulin (IVIG)-refractory status and prolonged fever are established risk factors for the development of coronary artery abnormalities (CAAs) among patients with acute-phase Kawasaki disease (KD). However, whether different risk factors exist for initial unresponsiveness to IVIG and CAA development remains unclear.

Objective: To evaluate whether different risk factors exist for initial unresponsiveness to IVIG and CAA development among patients with KD (stratified by age at disease onset).

Intimal thickening and disruption of the media occur in the arterial walls of coronary arteries not associated with coronary arterial aneurysms in patients with Kawasaki disease.

Background: Coronary artery aneurysm (CAA) is an important complication of Kawasaki disease (KD) that is associated with arterial structure damage. However, few studies have examined structural changes in coronary arteries that are not associated with CAA.

Methods: We examined coronary arteries in KD patients with CAAs who underwent follow-up coronary angiography (CAG) and optical coherence tomography (OCT).

Z-score is a possible predictor of the risk of coronary artery lesion development in patients with Kawasaki disease in Japan.

Risk factors for coronary artery lesion (CAL) development in patients with Kawasaki disease (KD) include male sex, age < 12 months, intravenous immunoglobulin (IVIG) resistance, and delayed diagnosis.. We aimed to explore the relationship between CAL development and Z-score.

Seasonal Variation in Epidemiology of Kawasaki Disease-Related Coronary Artery Abnormalities in Japan, 1999-2017.

Background: Epidemiological studies show a U-shaped tendency in Kawasaki disease (KD)-related coronary artery abnormalities (CAAs) across age categories. Since studies suggest seasonal variations in KD onset, this study aimed to clarify the epidemiologic features of CAAs, considering the seasons of KD-occurrence.

Methods: We analyzed 2,106 (males = 1,215, females = 891) consecutive KD cases from October 1999 through September 2017 using our electronic database of annual surveys, targeting all hospitals with pediatric departments across Wakayama, Japan.

[Cyclosporin A treatment for refractory Kawasaki disease].

The association between functional polymorphism of inositol 1,4,5-trisphosphate 3- kinase-C(ITPKC) and susceptibility to Kawasaki disease(KD) and formation of coronary arterial lesions was reported in 2008. Since ITPKC acts as a negative regulator of T-cell activation, activated T cells may play a pivotal role in the pathogenesis of KD. Cyclosporin A(CsA), which potently suppresses the activity of T cells through negative regulation of the nuclear factor of activated T cells(NFAT) pathway, may be a promising candidate for the treatment of refractory KD.

Evaluation of coronary arterial lesions due to Kawasaki disease using optical coherence tomography.

Optical coherence tomography (OCT) is a high-resolution intracoronary arterial imaging modality. We describe 2 patients who were admitted to undergo coronary angiography and OCT for follow-up of Kawasaki disease with coronary artery aneurysms. OCT clearly demonstrated thrombus, stenosis, fibrotic intimal thickening with lamellar calcification, and partial disappearance of the tunica media at the aneurysm site.

Epidemiologic features and prognostic factors of coronary artery lesions associated with Kawasaki disease based on a 13-year cohort of consecutive cases identified by complete enumeration surveys in Wakayama, Japan.

Background: To clarify the contribution of patient age to the development of coronary artery lesions (CALs) associated with Kawasaki disease (KD), epidemiologic features and prognostic factors were investigated using hospital-based complete enumeration surveys in a specific area.

Methods: Consecutive KD cases identified between October 1999 and September 2012 in Wakayama Prefecture, Japan, were analyzed. The primary outcome measure was the presence/absence of CALs (giant aneurysm, mid- or small-sized aneurysm, and dilatation) on echocardiography 1 month after disease onset.

Inflammatory cytokine profiles during Cyclosporin treatment for immunoglobulin-resistant Kawasaki disease.

Background: Kawasaki disease (KD) is an acute systemic vasculitis occurring in medium-sized arteries, especially coronary arteries. Patients with KD who fail to respond to standard therapy with intravenous immunoglobulin (IVIG) face a higher risk of developing coronary artery lesions. Cyclosporin A (CsA) is one treatment option for IVIG-resistant KD.

A genome-wide association study identifies three new risk loci for Kawasaki disease.

We performed a genome-wide association study (GWAS) of Kawasaki disease in Japanese subjects using data from 428 individuals with Kawasaki disease (cases) and 3,379 controls genotyped at 473,803 SNPs. We validated the association results in two independent replication panels totaling 754 cases and 947 controls. We observed significant associations in the FAM167A-BLK region at 8p22-23 (rs2254546, P = 8.

Cyclosporin A treatment for Kawasaki disease refractory to initial and additional intravenous immunoglobulin.

Background: There are still no definite treatments for refractory Kawasaki disease (KD). In this pilot study, we evaluated the use of cyclosporin A (CyA) treatment in patients with refractory KD.

Methods: We prospectively collected clinical data of CyA treatment (4-8 mg/kg/d, oral administration) for refractory KD patients using the same protocol among several hospitals.

Marker of T-cell activation is elevated in refractory Kawasaki disease.

Background: The aim of this study was to investigate whether T-cell activation is involved in the pathogenesis of Kawasaki disease (KD) resistant to intravenous immunoglobulin (IVIG) treatment.

Methods: Serum samples were obtained from 27 patients who fulfilled the diagnostic criteria for KD. These 27 patients were divided into three groups according to their responses to IVIG: Group A, nine patients who showed no response to either initial IVIG or additional IVIG; Group B, six patients who did not respond to initial IVIG but did respond to additional IVIG; Group C, 12 patients who responded to initial IVIG.

Common variants in CASP3 confer susceptibility to Kawasaki disease.

Kawasaki disease (KD; OMIM 611775) is an acute vasculitis syndrome which predominantly affects small- and medium-sized arteries of infants and children. Epidemiological data suggest that host genetics underlie the disease pathogenesis. Here we report that multiple variants in the caspase-3 gene (CASP3) that are in linkage disequilibrium confer susceptibility to KD in both Japanese and US subjects of European ancestry.

Detection of multiple superantigen genes in stools of patients with Kawasaki disease.

Objectives: To investigate whether superantigens (SAgs) are involved in the development of Kawasaki disease (KD) by examining SAg genes in the stool of patients with KD.

Study Design: Stool specimens were obtained from 60 patients with KD and 62 age-matched children (36 children with acute illness and 26 healthy children). Total DNA was extracted from these stool samples.

Water retention in the acute phase of Kawasaki disease: relationship between oedema and the development of coronary arterial lesions.

Unlabelled: Despite intravenous immunoglobulin therapy, a certain percentage of patients with Kawasaki disease (KD) still develop coronary arterial lesions (CAL). In an effort to find new combined therapies to reduce the incidence of CAL, we focused on the oedema which can be an important sign of the increased vascular permeability in KD. A total of 127 patients with KD were included in the retrospective study.

Detection of auto-antibodies against a 70 kDa protein derived from vascular smooth muscle cells in patients with Kawasaki disease.

Unlabelled: The aetiology and pathogenesis of Kawasaki disease (KD) remain unknown. To investigate the pathogenesis of vasculitis in KD, we tested for the presence of auto-antibodies against a component of vascular smooth muscle cells in the sera of patients with KD. Sera from 48 patients with KD, as well as sera from 14 sick children and from 22 healthy children, were examined for reactivity to both coronary arterial wall tissues and cultured smooth muscle cells (CSMC) derived from human coronary artery, using immunofluorescence and Western immunoblot assay techniques.