Our previous study suggested that the production of superantigens and heat-shock protein 60 by small intestinal bacteria might play a role in Kawasaki disease (KD). We demonstrated that they were all resistant to comm...Our previous study suggested that the production of superantigens and heat-shock protein 60 by small intestinal bacteria might play a role in Kawasaki disease (KD). We demonstrated that they were all resistant to commonly used antibiotics, except for sulamethoxazole trimethoprim (SMX-TMP). We used SMX-TMP for 7 cases of KD that were unresponsive to intravenous immunoglobulin (IVIG) and studied the antipyretic potency of this treatment. In 6 out of the 7 cases, we demonstrated that antipyretic potency was observed without side effects within 2 days of the initial administration. Antimicrobial therapy using SMX-TMP might represent a novel strategy for cases of KD that are unresponsive to IVIG.展开更多
Kawasaki disease(KD) is a common medium vessel systemic vasculitis that usually occurs in small children. It has a predilection for the coronary arteries, but other medium sized arteries can also be involved. The etio...Kawasaki disease(KD) is a common medium vessel systemic vasculitis that usually occurs in small children. It has a predilection for the coronary arteries, but other medium sized arteries can also be involved. The etiology of this disorder remains a mystery. Though typical presentation of KD is quite characteristic, it may also present as incomplete or atypical disease in which case the diagnosis can be very challenging. As both incomplete and atypical forms of KD can be associated with serious coronary artery complications, the pediatrician can ill afford to miss these diagnoses. The American Heart Association has enunciated consensus guidelines to facilitate the clinical diagnosis and treatment of this condition. However, there are still several issues that remain controversial. Intravenous immunoglobulin remains the cornerstone of management but several other treatment modalities, especially glucocorticoids, are increasingly finding favour. We review here some of the contemporary issues, and the controversies thereon, pertaining to management of KD.展开更多
BACKGROUND Kawasaki disease(KD)is diagnosed based on clinical features.Blood tests and other tests are auxiliary diagnostic tools.Since KD is a disease caused by arterial inflammation,many patients with KD have elevat...BACKGROUND Kawasaki disease(KD)is diagnosed based on clinical features.Blood tests and other tests are auxiliary diagnostic tools.Since KD is a disease caused by arterial inflammation,many patients with KD have elevated levels of inflammatory biomarkers,such as C-reactive protein(CRP),erythrocyte sedimentation rate(ESR),and serum amyloid A protein(SAA)in blood tests.We report our experience of a patient with KD who did not have elevated levels of inflammatory biomarkers.CASE SUMMARY A 1-year-old boy presented with a 3-day history of fever.Five of the six symptoms of KD were observed,except for changes in the lips and oral cavity.Blood tests revealed no elevation in CRP,ESR,or SAA levels.Although the blood test results were atypical,the patient was diagnosed with KD based on clinical symptoms and was admitted to the hospital for treatment.The patient was administered intravenous immunoglobulin(IVIG)and aspirin.Despite commencing treatment,the fever persisted;therefore,additional IVIG was administered,the dosage of aspirin was increased,and ulinastatin was added.Three doses of IVIG were administered and the fever resolved on day 11 of KD symptoms started.Blood tests performed during hospitalization showed normal levels of inflammatory biomarkers.We examined leucine-rich alpha-2-glycoprotein 1-a protein that is elevated during the acute phase of KD.The protein levels did not increase during hospitalization.CONCLUSION This case suggests the need to identify criteria and biomarkers for detecting KD conditions that do not require KD treatment.展开更多
Kawasaki Disease (KD) is a self-limiting systemic vasculitis common among children under five years of age. Diagnosis of the disease is made primarily from the clinical features presented during the illness. Coronary ...Kawasaki Disease (KD) is a self-limiting systemic vasculitis common among children under five years of age. Diagnosis of the disease is made primarily from the clinical features presented during the illness. Coronary artery aneurysm (CAA) is the main complication of this disease which can be prevented largely by the early diagnosis and administration of IVIG (Intravenous Immunoglobulin). Even with first dose of IVIG up to 10% - 20% of patients develop refractory Kawasaki Disease, i.e., remain unresponsive to initial IVIG treatment. Second dose of IVIG is recommended treatment for refractory Kawasaki Disease but other alternative treatments are also being considered to lower the risk of complications. Different drugs are still in trial phase and some others have limited studies on larger population. Most of the study reports that involved newer drugs have limited patients or are from single centers which are very hard to apply to larger population. We will review general treatment approach in refractory Kawasaki disease.展开更多
Background Currently,there are no reliable indicators for predicting intravenous immunoglobulin resistance and coronary artery lesions in the early stage of Kawasaki disease.Methods A total of 300 patients with Kawasa...Background Currently,there are no reliable indicators for predicting intravenous immunoglobulin resistance and coronary artery lesions in the early stage of Kawasaki disease.Methods A total of 300 patients with Kawasaki disease were studied retrospectively.Laboratory data were compared between the intravenous immunoglobulin resistant (29 patients) and responsive groups,and between the groups with coronary artery lesions (48 patients) and without coronary artery lesions.Results The intravenous immunoglobulin resistant group had significantly higher D-dimer,globulin,interleukin-6 and serum ferritin levels in comparison to the intravenous immunoglobulin responder group.D-dimer level had a sensitivity of 87.0% and a specificity of 56.3% for predicting intravenous immunoglobulin resistance at a cutoff point of 1.09 mg/L.Globulin had a sensitivity of 62.1% and a specificity of 82.3% for predicting intravenous immunoglobulin resistance at a cutoff point of 34.7 g/L.Serum ferritin level had a sensitivity of 42.9% and a specificity of 88.8% for predicting intravenous immunoglobulin resistance at a cutoff point of 269.7 ng/mL.The patients with coronary artery lesions had higher D-dimer and tumor necrosis factor-α level.D-dimer level had a sensitivity of 50% and a specificity of 78.6% for predicting coronary artery lesions at a cutoff point of 1.84 mg/L.Based on analysis by multivariate logistic regression,serum ferritin and globulin were independent risks for intravenous immunoglobulin resistance,D-dimer was independent risk for coronary artery lesions.Conclusions Elevated serum ferritin,globulin and D-dimer levels are significantly associated with intravenous immunoglobulin resistance in Kawasaki disease.Moreover,serum D-dimer is significantly increased in Kawasaki disease with coronary artery lesions.展开更多
Background We aimed to explore predictive measures for intravenous immunoglobulin(IVIG)resistance in children with Kawasaki disease(KD).Methods Patients diagnosed with KD were enrolled in this study.Univariate analysi...Background We aimed to explore predictive measures for intravenous immunoglobulin(IVIG)resistance in children with Kawasaki disease(KD).Methods Patients diagnosed with KD were enrolled in this study.Univariate analysis and multiple logistic regression were utilized to analyze the clinical features and laboratory results prior to IVIG-treatment of the two groups.Independent predictors of IVIG resistance were analyzed,and a predictive model for KD children with IVIG resistance was constructed.Results A total of 277 children with KD,180 boys and 97 girls,aged 2-128(median 23)months,were enrolled in the study.Compared with the IVIG-responsive group,the IVIG-resistant group had higher levels of the peripheral neutrophil count,mean platelet volume,mean platelet volume-to-lymphocyte ratio and C-reactive protein,and total serum bilirubin,but lower levels of peripheral lymphocyte count,serum albumin and serum prealbumin.Age(in months),peripheral neutrophil count,lymphocyte count and mean platelet volume and serum albumin were independent indicators for IVIG resistance by multivariate logistic regression analysis.A logistic regression model and a scoring system were set up,where cut-off values of—0.46 and 6.5 points yielded sensitivities of 83.9%and 77.4%,and specificities of 74.8%and 61.0%,respectively.The areas under the curve(AUC)were 0.808 in the logistic regression model,and 0.750 in the scoring system.Conclusion Our model for predicting IVIG-resistant children with KD,involving age(months),peripheral neutrophil count,lymphocyte count and mean platelet volume and serum albumin prior to IVIG-treatment,is helpful for clinical prediction of children with IVIG-resistant KD.展开更多
Importance:Patients with Kawasaki disease(KD)experience various extracardiovascular injury complications,which may affect their outcomes.Objective:To investigate the incidence and clinical characteristics of extracard...Importance:Patients with Kawasaki disease(KD)experience various extracardiovascular injury complications,which may affect their outcomes.Objective:To investigate the incidence and clinical characteristics of extracardiovascular complications in children with KD.Methods:The clinical data of patients diagnosed with KD in the First Affiliated Hospital of Guangxi Medical University from January 2003 to January 2021 were reviewed.The clinical characteristics and extracardiovascular complications were compared among patients stratified by age,intravenous immunoglobulin(IVIG)therapy responsiveness,and coronary status.Results:A total of 511 patients with KD were included,357(69.9%)were aged 1–5 years.Children aged<1 year(21.5%)and boys(70.8%)were more likely to have coronary artery lesions(CALs).The incidence of incomplete KD was lowest in 1–5-year-old patients(19.6%).Involvement of the hematological system gradually decreased with age(<1 year,51.8%;1–5 years,36.7%;>5 years,29.5%),whereas the involvement of the joints gradually increased with age(<1 year,2.7%;1–5 years,6.2%;>5 years,20.5%).Nervous system involvement was more common in IVIG non-responders(15.7%[13/83]vs.5.4%[23/428],P=0.001).However,there were no significant differences in extracardiovascular injury complications between patients with or without CALs.Interpretation:KD can involve multiple organ injuries as well as cardiovascular complications,and nervous systerm involvement may be more common in patients unresponsive to IVIG.展开更多
目的探讨静脉注射人免疫球蛋白(intravenous infusion of human immunoglobulin,IVIG)对川崎病(Kawasaki disease,KD)患儿冠状动脉(冠脉)病变发生的影响。方法选择2019年1月至2022年5月在东莞市妇幼保健院住院确诊并治疗的KD患儿,根据...目的探讨静脉注射人免疫球蛋白(intravenous infusion of human immunoglobulin,IVIG)对川崎病(Kawasaki disease,KD)患儿冠状动脉(冠脉)病变发生的影响。方法选择2019年1月至2022年5月在东莞市妇幼保健院住院确诊并治疗的KD患儿,根据有无冠脉病变将患儿分为无冠脉病变组和冠脉病变组。对两组患者的发热时间、IVIG治疗时的病程时间、白细胞、血红蛋白、血小板、C反应蛋白、降钙素原、离子、肝及肾功能等临床资料进行分析。结果KD合并冠脉病变组患者血白细胞、血小板计数高于无冠脉病变组,而白蛋白、羟基酸脱氢酶浓度低于无冠脉病变组,差异均有统计学意义(P<0.05)。KD发热时间<8 d患儿的冠脉病变发生率低于发热时间>10 d的患儿,差异有统计学意义(P<0.05)。病程第5天前应用IVIG治疗患者冠脉病变发生率与病程第7天、第8天及10天后治疗患者比较,差异有统计学意义(P<0.05)。病程第5天及第6天应用IVIG治疗患者的冠脉病变发生率低于病程第10天后应用的患者,差异有统计学意义(P<0.05)。结论应用IVIG时间过晚,白细胞、血小板计数升高,发热时间较长是KD患儿并发冠脉病变的危险因素,而白蛋白、羟基酸脱氢酶浓度的降低也可作为KD并发冠脉病变的预测指标。热程少于8 d,病程7 d内应用IVIG治疗,可减少冠脉病变发生率。展开更多
文摘Our previous study suggested that the production of superantigens and heat-shock protein 60 by small intestinal bacteria might play a role in Kawasaki disease (KD). We demonstrated that they were all resistant to commonly used antibiotics, except for sulamethoxazole trimethoprim (SMX-TMP). We used SMX-TMP for 7 cases of KD that were unresponsive to intravenous immunoglobulin (IVIG) and studied the antipyretic potency of this treatment. In 6 out of the 7 cases, we demonstrated that antipyretic potency was observed without side effects within 2 days of the initial administration. Antimicrobial therapy using SMX-TMP might represent a novel strategy for cases of KD that are unresponsive to IVIG.
文摘Kawasaki disease(KD) is a common medium vessel systemic vasculitis that usually occurs in small children. It has a predilection for the coronary arteries, but other medium sized arteries can also be involved. The etiology of this disorder remains a mystery. Though typical presentation of KD is quite characteristic, it may also present as incomplete or atypical disease in which case the diagnosis can be very challenging. As both incomplete and atypical forms of KD can be associated with serious coronary artery complications, the pediatrician can ill afford to miss these diagnoses. The American Heart Association has enunciated consensus guidelines to facilitate the clinical diagnosis and treatment of this condition. However, there are still several issues that remain controversial. Intravenous immunoglobulin remains the cornerstone of management but several other treatment modalities, especially glucocorticoids, are increasingly finding favour. We review here some of the contemporary issues, and the controversies thereon, pertaining to management of KD.
文摘BACKGROUND Kawasaki disease(KD)is diagnosed based on clinical features.Blood tests and other tests are auxiliary diagnostic tools.Since KD is a disease caused by arterial inflammation,many patients with KD have elevated levels of inflammatory biomarkers,such as C-reactive protein(CRP),erythrocyte sedimentation rate(ESR),and serum amyloid A protein(SAA)in blood tests.We report our experience of a patient with KD who did not have elevated levels of inflammatory biomarkers.CASE SUMMARY A 1-year-old boy presented with a 3-day history of fever.Five of the six symptoms of KD were observed,except for changes in the lips and oral cavity.Blood tests revealed no elevation in CRP,ESR,or SAA levels.Although the blood test results were atypical,the patient was diagnosed with KD based on clinical symptoms and was admitted to the hospital for treatment.The patient was administered intravenous immunoglobulin(IVIG)and aspirin.Despite commencing treatment,the fever persisted;therefore,additional IVIG was administered,the dosage of aspirin was increased,and ulinastatin was added.Three doses of IVIG were administered and the fever resolved on day 11 of KD symptoms started.Blood tests performed during hospitalization showed normal levels of inflammatory biomarkers.We examined leucine-rich alpha-2-glycoprotein 1-a protein that is elevated during the acute phase of KD.The protein levels did not increase during hospitalization.CONCLUSION This case suggests the need to identify criteria and biomarkers for detecting KD conditions that do not require KD treatment.
文摘Kawasaki Disease (KD) is a self-limiting systemic vasculitis common among children under five years of age. Diagnosis of the disease is made primarily from the clinical features presented during the illness. Coronary artery aneurysm (CAA) is the main complication of this disease which can be prevented largely by the early diagnosis and administration of IVIG (Intravenous Immunoglobulin). Even with first dose of IVIG up to 10% - 20% of patients develop refractory Kawasaki Disease, i.e., remain unresponsive to initial IVIG treatment. Second dose of IVIG is recommended treatment for refractory Kawasaki Disease but other alternative treatments are also being considered to lower the risk of complications. Different drugs are still in trial phase and some others have limited studies on larger population. Most of the study reports that involved newer drugs have limited patients or are from single centers which are very hard to apply to larger population. We will review general treatment approach in refractory Kawasaki disease.
基金This work was supported,in part,by grants from The National Natural Science Foundation of China(81670251).
文摘Background Currently,there are no reliable indicators for predicting intravenous immunoglobulin resistance and coronary artery lesions in the early stage of Kawasaki disease.Methods A total of 300 patients with Kawasaki disease were studied retrospectively.Laboratory data were compared between the intravenous immunoglobulin resistant (29 patients) and responsive groups,and between the groups with coronary artery lesions (48 patients) and without coronary artery lesions.Results The intravenous immunoglobulin resistant group had significantly higher D-dimer,globulin,interleukin-6 and serum ferritin levels in comparison to the intravenous immunoglobulin responder group.D-dimer level had a sensitivity of 87.0% and a specificity of 56.3% for predicting intravenous immunoglobulin resistance at a cutoff point of 1.09 mg/L.Globulin had a sensitivity of 62.1% and a specificity of 82.3% for predicting intravenous immunoglobulin resistance at a cutoff point of 34.7 g/L.Serum ferritin level had a sensitivity of 42.9% and a specificity of 88.8% for predicting intravenous immunoglobulin resistance at a cutoff point of 269.7 ng/mL.The patients with coronary artery lesions had higher D-dimer and tumor necrosis factor-α level.D-dimer level had a sensitivity of 50% and a specificity of 78.6% for predicting coronary artery lesions at a cutoff point of 1.84 mg/L.Based on analysis by multivariate logistic regression,serum ferritin and globulin were independent risks for intravenous immunoglobulin resistance,D-dimer was independent risk for coronary artery lesions.Conclusions Elevated serum ferritin,globulin and D-dimer levels are significantly associated with intravenous immunoglobulin resistance in Kawasaki disease.Moreover,serum D-dimer is significantly increased in Kawasaki disease with coronary artery lesions.
基金The work was supported by Peking University Clinical Scientist Program(BMU2019LCKXJ001)and Fundamental Research for the Central Universities.
文摘Background We aimed to explore predictive measures for intravenous immunoglobulin(IVIG)resistance in children with Kawasaki disease(KD).Methods Patients diagnosed with KD were enrolled in this study.Univariate analysis and multiple logistic regression were utilized to analyze the clinical features and laboratory results prior to IVIG-treatment of the two groups.Independent predictors of IVIG resistance were analyzed,and a predictive model for KD children with IVIG resistance was constructed.Results A total of 277 children with KD,180 boys and 97 girls,aged 2-128(median 23)months,were enrolled in the study.Compared with the IVIG-responsive group,the IVIG-resistant group had higher levels of the peripheral neutrophil count,mean platelet volume,mean platelet volume-to-lymphocyte ratio and C-reactive protein,and total serum bilirubin,but lower levels of peripheral lymphocyte count,serum albumin and serum prealbumin.Age(in months),peripheral neutrophil count,lymphocyte count and mean platelet volume and serum albumin were independent indicators for IVIG resistance by multivariate logistic regression analysis.A logistic regression model and a scoring system were set up,where cut-off values of—0.46 and 6.5 points yielded sensitivities of 83.9%and 77.4%,and specificities of 74.8%and 61.0%,respectively.The areas under the curve(AUC)were 0.808 in the logistic regression model,and 0.750 in the scoring system.Conclusion Our model for predicting IVIG-resistant children with KD,involving age(months),peripheral neutrophil count,lymphocyte count and mean platelet volume and serum albumin prior to IVIG-treatment,is helpful for clinical prediction of children with IVIG-resistant KD.
基金Guangxi Medical and health key discipline construction project,Grant/Award Number:2019(19)Guangxi Clinical Research Center for Pediatric disease,Grant/Award Number:AD22035219。
文摘Importance:Patients with Kawasaki disease(KD)experience various extracardiovascular injury complications,which may affect their outcomes.Objective:To investigate the incidence and clinical characteristics of extracardiovascular complications in children with KD.Methods:The clinical data of patients diagnosed with KD in the First Affiliated Hospital of Guangxi Medical University from January 2003 to January 2021 were reviewed.The clinical characteristics and extracardiovascular complications were compared among patients stratified by age,intravenous immunoglobulin(IVIG)therapy responsiveness,and coronary status.Results:A total of 511 patients with KD were included,357(69.9%)were aged 1–5 years.Children aged<1 year(21.5%)and boys(70.8%)were more likely to have coronary artery lesions(CALs).The incidence of incomplete KD was lowest in 1–5-year-old patients(19.6%).Involvement of the hematological system gradually decreased with age(<1 year,51.8%;1–5 years,36.7%;>5 years,29.5%),whereas the involvement of the joints gradually increased with age(<1 year,2.7%;1–5 years,6.2%;>5 years,20.5%).Nervous system involvement was more common in IVIG non-responders(15.7%[13/83]vs.5.4%[23/428],P=0.001).However,there were no significant differences in extracardiovascular injury complications between patients with or without CALs.Interpretation:KD can involve multiple organ injuries as well as cardiovascular complications,and nervous systerm involvement may be more common in patients unresponsive to IVIG.
文摘目的探讨静脉注射人免疫球蛋白(intravenous infusion of human immunoglobulin,IVIG)对川崎病(Kawasaki disease,KD)患儿冠状动脉(冠脉)病变发生的影响。方法选择2019年1月至2022年5月在东莞市妇幼保健院住院确诊并治疗的KD患儿,根据有无冠脉病变将患儿分为无冠脉病变组和冠脉病变组。对两组患者的发热时间、IVIG治疗时的病程时间、白细胞、血红蛋白、血小板、C反应蛋白、降钙素原、离子、肝及肾功能等临床资料进行分析。结果KD合并冠脉病变组患者血白细胞、血小板计数高于无冠脉病变组,而白蛋白、羟基酸脱氢酶浓度低于无冠脉病变组,差异均有统计学意义(P<0.05)。KD发热时间<8 d患儿的冠脉病变发生率低于发热时间>10 d的患儿,差异有统计学意义(P<0.05)。病程第5天前应用IVIG治疗患者冠脉病变发生率与病程第7天、第8天及10天后治疗患者比较,差异有统计学意义(P<0.05)。病程第5天及第6天应用IVIG治疗患者的冠脉病变发生率低于病程第10天后应用的患者,差异有统计学意义(P<0.05)。结论应用IVIG时间过晚,白细胞、血小板计数升高,发热时间较长是KD患儿并发冠脉病变的危险因素,而白蛋白、羟基酸脱氢酶浓度的降低也可作为KD并发冠脉病变的预测指标。热程少于8 d,病程7 d内应用IVIG治疗,可减少冠脉病变发生率。
