Kawasaki Disease: An Update on Diagnosis and Treatment

Kuo, Ho‐Chang; Yang, Kuender D.; Chang, Wei Chiao; Ger, Luo Ping; Hsieh, Kai‐Sheng

doi:10.1016/j.pedneo.2011.11.003

Cited by 130 publications

(128 citation statements)

References 87 publications

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“…The ratio of males to females was 1.53:1. The standard diagnostic criteria for KD were used (30). None of the subjects had been previously diagnosed with KD and they had not received the standard treatment of intravenous immunoglobulin from the onset of fever.…”

Section: Subjectsmentioning

confidence: 99%

Plasma MASP-1 concentration and its relationship to recovery from coronary artery lesion in children with Kawasaki disease

Song

Zou

et al. 2015

Pediatr Res

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Background: This study investigated prognostic factors for early recovery of coronary artery lesion (CAL) in children with Kawasaki disease (KD). Methods: Patients hospitalized for KD were enrolled less than 2 wk from the onset of illness and divided into two groups: KD with CAL and KD without CAL. The CAL group was further divided into two subgroups according to the degree of CAL: mild (n = 31) and moderate/severe (n = 6) and further divided into two subgroups according to the age: younger than 1 y (n = 9) and older than 1 y (n = 28). Lectin pathway-related factors MASP-1, CD59, and C5b-9 were measured, along with C-reactive protein, white blood cell counts, erythrocyte sedimentation rate, and platelet count. Patients were followed up for 3 mo. Correlation between the measured factors and the length of time of recovery from CAL was analyzed. results: Plasma concentrations of MASP-1 in the CAL group were significantly lower than those without CAL. MASP-1 and gender positively correlated with the recovery time of CAL. There was no difference in MASP-1 between mild and moderate/severe CAL. At 3-mo follow-up, there was a positive correlation between plasma MASP-1 concentration and recovery time of the patients with CAL older than 1 y. conclusion: Plasma MASP-1 concentration at the early stage of KD is predictive of length of time of recovery from CAL. k awasaki disease (KD), also known as mucocutaneous lymph node syndrome, is an acute nonspecific systemic vascular inflammatory syndrome occurring mainly in children under 5 y of age. A considerable volume of research has shown that the pathological process of KD is characterized by immune activation and immune vasculitis of the vascular endothelial cells (1). KD mostly affects the small and mediumsized arteries, especially the coronary artery (2), resulting in coronary artery lesion (CAL), such as dilation, aneurysm formation, or advanced stenosis, and it is the leading cause of death in children with KD (3). It has been reported that 15-25% of untreated KD patients may develop CAL (4). Although treatment with intravenous immunoglobulin within 10 d of onset of fever reduces the incidence of CAL, 5-8% of patients still develop it. The degree of CAL varies considerably between individuals, ranging from mild to moderate ectasia of the coronary artery to giant aneurysm formation. The recovery time of CAL also differs between patients. There is a very little information about the length of time for which different degrees of CAL persists. Little is known on the relationship between the complement system and the recovery time of CAL resulting from KD. Our previous study indicated that the lectin pathway of the complement system is activated in KD. CAL is associated with endothelial cell dysfunction induced by T-cell activation (5). Endothelial cells are a source of complement-related factors and complement regulatory factors (6), and MASP-1 activates endothelial cells directly, which then activates the complement lectin pathway (7). Endothelial cell dysfunction (8) and com...

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Section: Subjectsmentioning

confidence: 99%

Plasma MASP-1 concentration and its relationship to recovery from coronary artery lesion in children with Kawasaki disease

Song

Zou

et al. 2015

Pediatr Res

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“…Down-regulation of inflammation terminates the acute febrile (12). However, acute KD can trigger coronary artery aneurysms, particularly when there is delayed diagnosis and/or treatment (21).…”

Section: Discussionmentioning

confidence: 99%

“…The majority of cases of KD can be successfully treated following IVIG treatment, however the incidence of coronary aneurysm remains at 3-5% (9,10). In addition, cases of KD resistant to IVIG therapy have been reported (11,12). In Japan and the USA, 20-30% of KD cases have been reported to be IVIG-resistant (13).…”

Section: Introductionmentioning

confidence: 99%

Screening of differentially expressed genes associated with Kawasaki disease by microarray analysis

Jiang

Cai

et al. 2017

Experimental and Therapeutic Medicine

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Abstract. Kawasaki disease (KD) is an autoimmune disorder that can induce coronary artery aneurysms, particularly in the case of delayed diagnosis and/or treatment. Early diagnosis is important for treatment and reduces the risk of heart injury. The aim of the present study was to identify differentially expressed genes by comparing the levels of gene expression in human umbilical vein endothelial cells following treatment with plasma from healthy individuals and patients with acute or convalescent KD. Following comparison of the control and acute KD groups, 385 up-regulated and 537 down-regulated genes were identified in the acute KD group. In the convalescent group, 505 and 879 genes were up-regulated and down-regulated, respectively, relative to the control group. Genes involved in the immune system and cell growth factors were up-regulated, while genes functioning in methylation were down-regulated, following treatment with KD plasma. In addition, five potential candidate molecular markers of KD, C-X-C motif chemokine ligand 2 (CXCL2), interleukin (IL) 8, tripartite motif containing 58 (TRIM58), immunoglobulin superfamily member 3 (IGSF3) and runt related transcription factor 1 (RUNX1) were identified by microarray analysis and verified using quantitative polymerase chain reaction. A significant positive correlation was identified between the neutrophil polys and expression levels of four of these candidate genes, including CXCL2, IL8, TRIM58, and IGSF3 (all P<0.01; R 2 ≥0.64). However, only CXCL2 expression was significantly positively correlated with neutrophil polys (P=0.01; R 2 =0.64) and neutrophil bands (P<0.001; R 2 =0.73).These results indicate that CXCL2 serves a crucial role in the injury of endothelial cells by KD plasma.

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“…Some microorganism, especially bacteria and viruses, may be the first "driver" of KD [4], but no specific pathogen and other autoimmune target have been precisely identified [2][3][4][5]. The diagnosis of KD is still based on clinical characteristics, and generally fever plus five specific criteria are required for diagnosis of KD according the suggestion by the American Heart Association [3,6]. So far, no laboratory serum test could auxiliary diagnose KD with specificity [6,7].…”

Section: Introductionmentioning

confidence: 99%

Lactate Dehydrogenase is involved in but not the Target Antigen in Children with Kawasaki Disease

Zhao¹,

Zhao²,

Du³

et al. 2016

J Cardiovasc Med Cardiol

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Kawasaki Disease: An Update on Diagnosis and Treatment

Cited by 130 publications

References 87 publications

Plasma MASP-1 concentration and its relationship to recovery from coronary artery lesion in children with Kawasaki disease

Plasma MASP-1 concentration and its relationship to recovery from coronary artery lesion in children with Kawasaki disease

Screening of differentially expressed genes associated with Kawasaki disease by microarray analysis

Lactate Dehydrogenase is involved in but not the Target Antigen in Children with Kawasaki Disease

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