Establishment of a new scoring model for IVIG non-response of Kawasaki disease / 国际儿科学杂志

Objective To analyze the possible risk factors of IVIG non-response of Kawasaki disease (KD),Shanghai Children's Hospital and Shanghai Junze Software develop an research platform,which is based on E-Science model.Through the mathematical model by integrating the risk factors to explore the method of effective prediction for IVIG non-response,and to provide the clinical basis for timely and effective treatment and prognosis of the disease.Methods The data of KD children who were hospitalized in Shanghai Children's Hospital from January 2013 to November 2016 were included.The indexes included gender,age,time of IVIG treatment,and laboratory examinations.The multivariate logistic regression was used to analyze the influencing factors of IVIG non-response.The indexes in the model were deduced according to the independent variables of the logistic regression equation.The ROC curve and the area under the curve were calculated for the new prediction model.The sensitivity and specificity of the new prediction model were calculated according to the cutoff value.Finally,the new model was compared with the Kobayashi and Egami scoring model.Results The levels of CRP,NLR,LDH,ALB and FDP in children with KD were influencing factors for IVIG nonresponse (P ＜ 0.05).According to the logistic regression equation,the sensitivity and specificity of the model used to predict IVIG non-response were 69.7％ and 80.4％,respectively,and the area under the ROC curve was 0.825 (95％ CI:0.769-0.882).Kobayashi and Egami scoring models were tested,the sensitivity and specificity of the new scoring system were better than previous ones.Condusion The scoring model established in this study has a good effect in predicting IVIG non-response in KD patients and could be used in clinical practice,and it is worthy to be validated and adjusted by large-scale data.

Serum IL-17A, IL-18, and SCD25 (IL-2R) in early diagnosis of Kawasaki disease / 临床儿科杂志

Objective To investigate the acute phase expression of serum cytokines in children with Kawasaki disease (KD) and its association with coronary artery lesions (CALs). Methods Expression of 13 cytokines in serum of 104 KD patients including 11 cases with CALs and 74 febrile control cases admitted to the hospital from October 2016 to March 2017 were retrospectively analyzed. Results Interleukin (IL)-8, IL-1, IL-2, IL-6, IL-10, IL-17A, IL-18, tumor necrotic factor-α and SCD25 (IL-2R) were declined significantly after intravenous immunoglobulin administration (IVIG) in KD patients (all P<0.05). Compared with the fever control group, Pre-IVIG, an IL-17A level ≥ 0.155 pg/mL had a sensitivity of 50% and 93.2% specificity for predicting KD; IL-18 level ≤ 15.43 pg/mL had a sensitivity of 71.2%, and the specificity was 54.1%; SCD25 (IL-2R) ≥ 29475.29 pg/mL had a 65.4% sensitivity and 81.1% specificity for predicting KD. Before IVIG treatment, the level of IL-10 was significantly lower in KD patients with CALs than in those without CALs. Conclusions Determination of various cytokines profile may be helpful for predicting the disease prognosis and targeting treatment strategies in patients with KD.

Cytokine genetic polymorphisms in Kawasaki disease combined with coronary artery lesions / 国际儿科学杂志

Kawasaki disease(KD) is an acute febrile childhood inflammatory disease,characterized by systemic small artery inflammation as the main pathological changes that can lead to expansion of coronary artery,myocardial infarction and sudden cardiac death.Cytokines secreted by immune cells are small molecules,which can regulate the function of inflammatory cells,play important roles in the process of immune response,stimulate the proliferation and differentiation of cells,and regulate interactions between them.In the acute phase,super antigens activate immune responses.A large number of cytokines and inflammatory mediators are released into the blood,which participate in the occurrence and development of KD and the vascular disease.Many inflammatory cytokines are associated with coronary artery damages in KD patients.In this paper,the roles of various cytokines in the coronary artery lesions of KD are reviewed.

Identification of mutations associated with coronary artery lesion susceptibility in Kawasaki disease by targeted enrichment of genomic region sequencing technique / 中华儿科杂志

Objective@#To screen and identify the mutations in Kawasaki disease by targeted enrichment of genomic region sequencing technique and investigate susceptibility genes associated with coronary artery lesion.@*Method@#This was a case-control study.A total of 114 patients diagnosed as Kawasaki disease treated in Shanghai Children′s Hospital between December 2015 and November 2016 were studied and another 45 healthy children who were physically examined in outpatient department were enrolled as control group. Patients were divided into two groups based on the results of echocardiogram. Peripheral venous blood was obtained from patients and controls. Genomic DNA was extracted. SeqCap EZ Choice libraries were prepared by targeted enrichment of genomic region technology. Then the libraries were sequenced to identify susceptibility genes associated with coronary artery lesion in patients diagnosed as Kawasaki disease.Susceptible genes were identified by Burden test, Pearson chi-square test or Fisher′s exact probability test.@*Result@#There was statistically significant difference in TNFRSF11B(rs2073618)G>C(p.N3K)mutation and GG/GC/CC genotype between Kawasaki disease group and control group(χ2=15.52, P=0.00). There was statistically significant difference in TNFRSF13B(rs34562254)C>T(p.P251L)mutation(χ2=10.40, P=0.01)and LEFTY1(rs360057)T>G(p.D322A)mutation(χ2=8.505, P=0.01)between patients with coronary artery lesions and those without.@*Conclusion@#Targeted enrichment of genomic region sequencing technology can be used to do primary screening for the susceptible genes associated with coronary artery lesions in Chinese Kawasaki patients and may provide theoretical basis for larger sample investigation of risk prediction score standard in Kawasaki disease.