Case of Incomplete Kawasaki Disease with No Symptoms Except Fever Causing the Development of Coronary Aneurysm.

Incomplete Kawasaki disease (iKD), which does not satisfy the standard KD diagnostic criteria because the required number of principal symptoms is not met, sometimes causes coronary aneurysms. Here we report the case of a patient with iKD who presented with only one principal symptom that resulted in the development of coronary aneurysm, as evidenced by angiography.

Case Report: Structural Changes in the Coronary Vessel Wall in a Patient With Incomplete Kawasaki Disease.

Background: Kawasaki disease (KD) is an acute systemic vasculitis of infants and young children that affects medium-sized vessels. Conventional cardiac imaging techniques, such as cardiac catheterization, are useful for characterizing the coronary arterial lesion (CAL) size and luminal diameter of the diseased coronary artery segment in patients with KD, but there are limitations to the visualization of the detailed vascular anatomy. Optical coherence tomography (OCT) is a high-resolution intracoronary arterial imaging modality that can distinguish the three layers of the coronary arterial wall. Several studies have reported coronary artery wall abnormalities in KD patients with coronary arterial aneurysm or regressed aneurysm. However, there have been no reports on changes in the coronary artery wall in cases of incomplete KD without CAL. Case Presentation: We herein report an 11-year-old girl with a history of incomplete KD without coronary arterial aneurysms. She had been diagnosed with perimembranous ventricular septal defect (VSD) after birth and had experienced incomplete KD at 1 year old. During her hospitalization for KD, she did not receive intravenous immunoglobulin (IVIG), because she did not meet the Harada score or criteria for treatment in patients with incomplete KD established by the American Heart Association. No dilatation or coronary artery aneurysm were observed on transthoracic echocardiography in the acute or follow-up period. At 11 years old, she received cardiac catheterization and coronary angiography (CAG) for the evaluation of a VSD and follow-up of KD. CAG demonstrated no aneurysm, dilatation, or significant stenosis of the coronary arteries. We performed an OCT study, which revealed the presence of intimal thickening, disruption of the media, and neovascularization in the left anterior descending artery. Conclusion: OCT demonstrates the structural changes of CA even in the patient with incomplete KD who have not been treated with IVIG.

Child with Kawasaki Disease Complicated by A Single Right Coronary Artery.

A 3-year-old boy was referred to our hospital for management of Kawasaki disease at 5 days of illness. Echocardiographic examination on admission suggested aneurysmal dilation of the right coronary artery and a possible aorta-left main trunk connection. However, detailed echocardiography at 12 days of illness revealed an abnormal bifurcation of the proximal right coronary artery and no real connection of the aorta-left main trunk, all of which indicated the presence of a single right coronary artery. These diagnoses were confirmed by selective coronary angiography, which was performed later. Considering the difficulties in diagnosing congenital coronary anomalies, which may increase the risk of future fatal events, knowing the disease entity of the congenital coronary arterial anomaly is important for the accurate evaluation of coronary arteries in patients with Kawasaki disease. To the best of our knowledge, this is the first case report of a patient with Kawasaki disease complicated by a single right coronary artery; however, following a search of the literature, we found a brief conference abstract written in Japanese relating to the same clinical condition.

Correlation between angiopoietin-like proteins in inflammatory mediators in peripheral blood and severity of coronary arterial lesion in patients with acute myocardial infarction.

The effects of angiopoietin-like protein 2 (Angptl 2) and interleukin-6 (IL-6) in inflammatory mediators on the severity of coronary arterial lesion in patients with acute myocardial infarction were investigated. One hundred and twenty-six patients with acute myocardial infarction admitted to Tianjin Union Medical Center (the myocardial infarction group) and 133 healthy individuals (the control group) were selected for retrospective analysis from January 2013 to December 2015. The levels of Angptl 2 and IL-6 in serum of patients were detected by enzyme linked immunosorbent assay (ELISA), and the correlation analysis between the levels and the degree of coronary stenosis in patients with myocardial infarction was conducted. The expression level of Angptl 2 and IL-6 in the myocardial infarction group was significantly higher than that in the control group P<0.001. In the myocardial infarction group, the expression levels of Angptl 2 and IL-6 were the highest in the patients with severe stenosis, followed by the moderate stenosis, and the lowest in the patients with mild stenosis (P<0.050). Pearson's correlation analysis showed that Angptl 2 and IL-6 were positively correlated with the diameter of coronary stenosis (r=0.696, 0.750, P<0.001). In conclusion, both Angptl 2 and IL-6 are highly expressed in the peripheral blood of patients with acute myocardial infarction and involved in the occurrence and development of the disease. Moreover, Angptl2 and IL-6 are positively correlated with the severity of coronary arterial lesion in patients with acute myocardial infarction, and they are expected to become a target for the diagnosis and treatment of coronary atherosclerosis (CA) in the future.

Association between IL-35 and coronary arterial lesions in children with Kawasaki disease.

Kawasaki disease (KD) arises due to the acute inflammation and immune system dysfunction. This study investigated the relationship between the serum level of IL-35 and coronary artery lesions (CALs) in patients with KD. We obtained blood samples from 90 children with KD before intravenous immunoglobulin therapy. Levels of IL-35, IL-6, IL-17A, IL-10, MCP-1 and VEGF were measured in 190 cases, including 4 groups: KD with coronary arterial lesions (n = 46), KD without coronary arteries lesions (n = 44), febrile control group (FC, n = 40) and the normal control group (NC, n = 60). White blood cell counts (WBC), red blood cell counts (RBC), hemoglobin, platelet, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) and procalcitonin were tested in all subjects. Levels of IL-35, RBC and hemoglobin significantly decreased, and IL-6, IL-17A, IL-10, MCP-1 and VEGF were significantly elevated in the KD group compared with febrile and control groups. IL-35 serum level even decreased, and ESR, IL-6, MCP-1 and VEGF increased in the KD patients with CALs. Serum levels of IL-35 in KD patients were negatively associated with WBC, CRP, IL-6, IL-17A, IL-10, MCP-1 and VEGF in children with KD. IL-35 may have the effect on inhibiting inflammatory process in KD and further preventing KD patients from coronary artery lesion.

Relationship between IL-27 and coronary arterial lesions in children with Kawasaki disease.

Kawasaki disease (KD) arises due to the disorder of the inflammation response and faulty immune regulation. Interleukin-27 (IL-27) is a novel cytokine with both pro-inflammatory and anti-inflammatory effects. This study investigated the relationship between serum levels of IL-27, Interleukin-17A (IL-17A), Interleukin-10 (IL-10), Interleukin-6 (IL-6), Interleukin-1ß (IL-1ß), tumor necrosis factor-α (TNF-α) and coronary artery lesions (CALs) in patients with KD. We obtained blood samples from 81 children with KD before intravenous immunoglobulin (IVIG) therapy. Levels of IL-27, IL-17A, IL-10, IL-6, IL-1ß and TNF-α were measured in 251 cases, including 4 groups: the normal control group, NC (n = 90), febrile control, FC (n = 80), KD without coronary arteries (n = 41) and KD with coronary arterial lesions (n = 40). White blood cells counts (WBC), red blood cells counts (RBC), hemoglobin, C-reactive protein (CRP), erythrocyte sedimentation rate and procalcitonin (PCT) were tested in all subjects. Levels of IL-27, IL-10, IL-17A, IL-6, IL-1ß and TNF-α were significantly elevated, and RBC and hemoglobin significantly decreased in the group of KD group compared with febrile and control groups. IL-27, IL-6, IL-1ß and TNF-α serum levels are even higher in KD children with CALs. There was positive relationship between serum levels of IL-27 and WBC, CRP, PCT, IL-10, IL-17A, IL-6 and TNF-α in children with KD. The up-regulation of IL-27 may be closely linked to up-regulation of systemic pro-inflammatory markers in acute KD. Morover, IL-27 may be involved in the development of CALs in acute KD.

Structured learning algorithm for detection of nonobstructive and obstructive coronary plaque lesions from computed tomography angiography.

Visual identification of coronary arterial lesion from three-dimensional coronary computed tomography angiography (CTA) remains challenging. We aimed to develop a robust automated algorithm for computer detection of coronary artery lesions by machine learning techniques. A structured learning technique is proposed to detect all coronary arterial lesions with stenosis [Formula: see text]. Our algorithm consists of two stages: (1) two independent base decisions indicating the existence of lesions in each arterial segment and (b) the final decision made by combining the base decisions. One of the base decisions is the support vector machine (SVM) based learning algorithm, which divides each artery into small volume patches and integrates several quantitative geometric and shape features for arterial lesions in each small volume patch by SVM algorithm. The other base decision is the formula-based analytic method. The final decision in the first stage applies SVM-based decision fusion to combine the two base decisions in the second stage. The proposed algorithm was applied to 42 CTA patient datasets, acquired with dual-source CT, where 21 datasets had 45 lesions with stenosis [Formula: see text]. Visual identification of lesions with stenosis [Formula: see text] by three expert readers, using consensus reading, was considered as a reference standard. Our method performed with high sensitivity (93%), specificity (95%), and accuracy (94%), with receiver operator characteristic area under the curve of 0.94. The proposed algorithm shows promising results in the automated detection of obstructive and nonobstructive lesions from CTA.

Changes of myeloid related protein-8/myeloid related protein-14 expressions in children with Kawasaki disease / 中华实用儿科临床杂志

Objective To investigate the expression changes of myeloid-related protein-8 (MRP-8) and myeloid-related protein-14 (MRP-14) in children with Kawasaki disease (KD) and to obtain laboratory diagnostic serum markers and new targets for its drug therapy.Methods A total of 46 patients with KD(KD group) were enrolled from Jul.2009 to Dec.2010 and divided into the coronary artery dilatation(CAD) group(n =15) and the normal coronary artery group(n =31) ;Meanwhile,25 febrile patients with acute respiratory tract infection but without disease in the circulatory,blood,immune systems formed the non-KD febrile group.Twenty healthy children from the out-patient department formed the healthy control group.Peripheral venous blood was collected in the acute and subacute stage of KD.Levels of MRP-8/MRP-14 were detected with enzyme-linked immunosorbnent assay (ELISA).Gene expressions of MRP-8,MRP-14 in leukocytes were analyzed by semi-quantitative reverse transcription-polymerase chain reaction(RTPCR).Results The serum levels of MRP-8/MRP-14 along with mRNA expressions of MRP-8 and MRP-14 in the leukocytes in the out-patient acute and subacute stage of KD were significantly higher than those in the non-KD febrile group and the healthy control group(all P ＜ 0.05) ;There was no significant difference between non-KD febrile group and healthy control group (P ＞ 0.05).The serum levels of MRP-8/MRP-14 along with mRNA expressions of MRP-8 and MRP-14 in leukocyte in actue stage of KD were significantly higher than those in subacute stage(all P ＜ 0.001).The serum levels of MRP-8/MRP-14 as well as mRNA expressions of MRP-8 and MRP-14 in the acute and the subacute stage of CAD group were significantly higher than those in the normal coronary artery group(P ＜ 0.05).Conclusions MRP-8/MRP-14 may probably play a role in the pathogenesis of KD and can be used as a diagnostic indicator for KD;MRP-8/MRP-14 may be involved in the formation of coronary artery lesion and can be used as an effective predictor for the coronary artery lesion.

Manifestation of Coronary Artery Lesions after Immunoglobulin Re-treatment in Initial Immunoglobulin-resistant Kawasaki Disease / 소아과

PURPOSE: To evaluate the predictable factors for why initial intravenous immune globulin(IVIG) therapy failed and the outcome of coronary lesions after additional IVIG retreated in initial IVIG-resistant Kawasaki disease(KD). METHODS: Retrospective studies were performed on 284 cases of KD treated with one episode of high-dose IVIG and 63 cases with additional IVIG retreatment at this hospital from January 2000 to June 2003. 2D echocardiogram was done at admission, two months later and every two or three months when coronary lesion had improved more than two months later. RESULTS: In 69(24.3%) of 284 cases with post-first course of IVIG therapy, 19(43.2%) of 63 cases with additional IVIG retreatment, coronary abnormality had been shown by initial 2D-echocardiogram examined at acute stage. In five(1.8%) of 284 patients with post-first course of IVIG therapy, and in three(4.8%) of 63 cases with additional IVIG retreatment, coronary lesions still remained at follow-up echocardiogram. Even though there was a tendency of increased coronary lesions in the group with additional IVIG retreatment, there was no significant differences in the incidence of coronary lesions between the two groups. There were no significant differences in age, sex, and other clinical findings between the two groups. CONCLUSION: Combination therapy with high doses of IVIG and aspirin is generally effective as a standard treatment for KD but not always. Coronary lesion did not increased despite additional IVIG therapy for initial IVIG-resistant KD. There was no predictable factor for initial IVIG-resistant KD.