High-dose intravenous immunoglobulin downregulates the activated levels of inflammatory indices except erythrocyte sedimentation rate in acute stage of Kawasaki Disease

We evaluated the effects of high-dose intravenous immunoglobulin (IVIG) administration on various protein parameters, including inflammatory profiles, in children with Kawasaki disease (KD). Sixty-three children with KD were treated with IVIG at 2 g/kg over 12 h. Serial examinations of laboratory indices were performed three times: before IVIG treatment, 24 h after IVIG treatment, and 7 days after IVIG treatment. The white blood cell and neutrophil counts showed significant decreases 24 h and 7 days after IVIG administration. The erythrocyte sedimentation rate (ESR) increased significantly 24 h after IVIG, and the elevated level was sustained for 7 days. The levels of hemoglobin, albumin and inflammation-associated proteins, including C-reactive protein, decreased 24 h after IVIG treatment. Inflammation-associated proteins, except transferrin, decreased further to near normal levels after 7 days. On the other hand, IgM and IgA were not affected after 24 h, rather increased significantly after 7 days. High-dose IVIG causes immediate changes in the levels of various proteins, except IgA and IgM, and downregulates the activated levels of inflammatory indices, except ESR, in the acute stage of KD.     

Intravenous immune globulin plus corticosteroids in refractory Kawasaki disease

Background: The aim of the present study was to investigate the efficacy of i.v. immune globulin (IVIG) therapy combined with corticosteroids for additional treatment of acute Kawasaki disease (KD) unresponsive to initial IVIG treatment. Methods: In 50 prospective KD patients, six IVIG non‐responders without clinical improvement within 24–48 h after completion of initial IVIG, received 2 g/kg IVIG concurrently with 2 mg/kg i.v. prednisolone sodium succinate (PSL) until normalization of C‐reactive protein level. Treatment was then changed to oral PSL, which was tapered over time. Clinical and coronary artery lesion (CAL) outcomes were compared with those of 13 IVIG non‐responders who received additional heterogeneous therapies in 125 retrospective KD patients. In addition, the scoring system of Kobayashi et al. for prediction of non‐responsiveness to initial IVIG treatment was retrospectively verified in 175 KD subjects, consisting of 50 prospective and 125 retrospective patients in order to evaluate the efficacy of the re‐treatment regimen. Results: Incidence of CAL in the study patients was lower than in the control patients, although differences were not significant both in the acute stage (within 1 month: 1/6, 16.7% vs 7/13, 53.8%; P= 0.177) and in the convalescent stage (after 1 month: 0/6, 0.0% vs 4/13, 30.8%; P= 0.255). According to the non‐responder prediction system, the scores of six study and 13 control patients before initial IVIG treatment were similar (7.2 ± 1.9 vs 5.3 ± 3.1; P= 0.200). No serious adverse effects related to each treatment were noted in patients of either group. Conclusions: Additional IVIG combined with concurrent PSL appears to be safe and worth evaluation for the treatment of acute KD unresponsive to initial IVIG treatment.     

Association of lower eosinophil-related T helper 2 (Th2) cytokines with coronary artery lesions in Kawasaki disease

Kawasaki disease (KD) is a systemic febrile vasculitis particular coronary artery involvement. Eosinophilia has been found in our and other studies in KD. This study further investigates whether eosinophil-related T helper 2 (Th2) cytokines or the activation marker (eosinophil cationic protein – ECP) is involved in KD with coronary artery lesions (CAL). A total of 95 KD patients were enrolled for this study. Plasma samples were subjected to the measurement of interleukin (IL)-4, IL-5, and eotaxin by Luminex-Bedalyte multiplex beadmates system and to the measurement of ECP by fluoroimmunoassay. Patients with KD had higher eosinophils than controls. Eosinophil-related mediators: IL-4, IL-5, eotaxin, and ECP levels were also higher in KD patients than controls before intravenous immunoglobulin (IVIG) treatment. After IVIG treatment, ECP decreased but IL-4, IL-5, and eotaxin increased significantly. The higher the IL-5 and eosinophil levels after IVIG treatment, the lower rate of CAL was found. Changes of eosinophils after IVIG treatment were positively correlated to changes of IL-5 levels but not ECP levels. An increase of eosinophils and IL-5, but not ECP levels after IVIG treatment, was inversely correlated with CAL formation in KD.     

The functions of endothelial progenitor cells were significantly improved after treatment with intravenous immunoglobulin and aspirin in children with Kawasaki disease

We sought to determine the effects of treatment with intravenous immunoglobulin (IVIG) and aspirin on the functions of endothelial progenitor cells (EPCs) in patients with Kawasaki disease (KD) as well as its relationship with concentrations of tumor necrosis factor-?? (TNF-??) and high-sensitivity C-reactive protein (hs-CRP). Ten KD patients in the acute phase of their disease were recruited. We investigated EPC functions in children with KD before and after treatment with IVIG and aspirin. In vitro assays were used to measure the functions, including proliferation, adhesion, and migration activities, of EPCs. Plasma levels of TNF-?? and hs-CRP were also assessed. All of the data were assessed before and at 7 days after treatment initiation. EPC functions after 7 days of treatment with IVIG and aspirin were significantly improved than they were before treatment with IVIG and aspirin. Treatment with IVIG and aspirin significantly decreased TNF-?? and hs-CRP concentrations. There was a significant linear regression relationship between decreased plasma TNF-?? levels, hs-CRP levels, and increased functions of circulating EPCs. The results of our study indicate that the functions of circulating EPCs improved after treatment with IVIG and aspirin, which may be related to decreased concentrations of TNF-?? and hs-CRP.     

Serum albumin level predicts initial intravenous immunoglobulin treatment failure in Kawasaki disease

Objectives: Kawasaki disease (KD) is a systemic vasculitis primarily affecting children who are <5 years old. Intravenous immunoglobulin (IVIG) is the standard therapy for KD. However, many patients with KD still show poor response to initial IVIG treatment. This study was conducted to investigate the risk factors for initial IVIG treatment failure in KD. Methods: Children who met KD diagnosis criteria and were admitted for IVIG treatment were retrospectively enrolled for analysis. Patients were divided into IVIG‐responsive and IVIG‐resistant groups. Initial laboratory data before IVIG treatment were collected for analysis. Results: A total of 131 patients were enrolled during the study period. At 48 h after completion of initial IVIG treatment, 20 patients (15.3%) had an elevated body temperature. Univariate analysis showed that patients who had initial findings of high neutrophil count, abnormal liver function, low serum albumin level (≤2.9 g/dL) and pericardial effusion were at risk for IVIG treatment failure. Multivariate analysis with a logistic regression procedure showed that serum albumin level was considered the independent predicting factor of IVIG resistance in patients with KD (p = 0.006, OR = 40, 95% CI: 52.8–562). There was no significant correlation between age, gender, fever duration before IVIG treatment, haemoglobin level, total leucocyte and platelet counts, C‐reactive protein level, or sterile pyuria and initial IVIG treatment failure. The specificity and sensitivity for prediction of IVIG treatment failure in this study were 96% and 34%, respectively. Conclusion: Pre‐IVIG treatment serum albumin levels are a useful predictor of IVIG resistance in patients with KD.     

Pulse methylprednisolone with gammaglobulin as an initial treatment for acute Kawasaki disease

Approximately 15–20% of patients with Kawasaki disease (KD) are not responsive to high-dose intravenous gammaglobulin (IVIG). We have previously reported a predictive method for identifying IVIG-non-responsive patients (high-risk KD patients). We determined the safety and effectiveness of pulse methylprednisolone with high-dose IVIG (mPSL+IVIG) as a primary treatment for high-risk KD patients. Sixty-two high-risk KD patients were treated with pulse methylprednisolone 30 mg/kg over 2 h, followed by IVIG 2 g/kg over 24 h (mPSL+IVIG group) and were compared with a historical control group of 32 high-risk patients treated with IVIG 2 g/kg alone at the participating hospitals before this study was opened (IVIG group). High-risk patients were identified with at least two of three predictors (C-reactive protein ≥7 mg/dL, total bilirubin ≥0.9 mg/dL or aspartate aminotransferase ≥200 IU/L). Sixty-six percent (95% confidence interval [CI] 54–78%) of patients had a prompt defervescence in the mPSL+IVIG group compared with 44% (95% CI 26–62%) for the IVIG group (p = 0.048). Coronary artery lesions were observed in 24.2% (95% CI 13.2–35.2%) and 46.9% (95% CI 28.6–65.2%) of patients in the mPSL+IVIG and IVIG groups, respectively (p = 0.025). This is the first report showing that mPSL+IVIG is effective and safe as a primary treatment for high-risk KD patients.     

大剂量丙种球蛋白对川崎病肾上腺髓质素及相关基因表达的影响

目的观察川崎病(Kawasaki disease,KD)患儿静脉用丙种球蛋白(IVIG)治疗前后肾上腺髓质素(adrenomedullin,ADM)及其他相关基因的表达变化,以探讨IVIG治疗KD的作用机制。方法采集40例KD患儿IVIG治疗前3 d及治疗后3 d外周血标本,男25例,女15例;发病年龄10～22个月,平均(16.7±5.4)个月;IVIG治疗方案为1 g/kg×2次。选取其中4例患儿的外周血进行芯片检测基因表达谱,聚类分析法筛选相关基因;同时选择ADM基因,对其余36例KD行ELISA法确认该基因表达变化。结果 4例KD患儿芯片分析发现,IVIG治疗后42个相关炎性基因表达下调(P<0.05)。ELISA法检测发现32例KD患儿血清ADM基因表达在IVIG治疗前明显高于治疗后(P<0.05),与芯片分析相一致;其余4例KD血清ADM浓度在治疗后明显高于治疗前(P<0.05),且均在IVIG治疗后出现冠脉扩张。结论 IVIG可能下调了急性期KD相关炎性基因表达,从而抑制冠脉损害的发生;治疗后ADM基因表达增高可能是预测KD冠脉扩张的线索之一。     

静脉注射不同剂量丙种球蛋白治疗川崎病的临床研究

目的 评价静脉注射丙种球蛋白（intravenous immune globulin,IVIG）1g／kg单次静脉注射治疗川崎病（Kawasaki disease,KD）的临床效果。方法 242例KD患儿随机分为IVIG1s／kg组与IVIG 2g／kg组,对两种治疗方法的疗效进行前瞻性对比研究。分别采用IVIG 1g／kg和2s／kg单次静脉注射,观察患儿总热程、退热时间、黏膜充血、手足肿胀和颈淋巴结肿大消退时间,监测外周血白细胞计数（white blood cells count,WBC）、血小板计数（platelet count,PLT）、血清丙种球蛋白（immunoglobulin,Ig）、C反应蛋白（Creacting protein,CRP）、血沉（erythrocyte sedimentation rate,ESR）、心电图（electrocardiogram,ECG）和冠状动脉病变（coronary artery lesion,CAL）恢复情况,并对治疗前后组内结果、治疗后组间结果进行比较。结果 IVIG 1g／kg组平均热程为10．6d,WBC、PLT、CRP、ESR及ECG异常率与治疗前比较显著降低（P〈0．001）,IVIG1g／kg组与IVIG2g／kg组比较差异无统计学意义（P〉0．05）。WIG1g／kg组CAL发生率为29．5％（36／122）,随访1年有87．5％的CAL恢复正常,12．5％未能恢复正常,其中9．4％为IVIG耐药病例;IVIG2g／kg组CAL发生率为24．2％（29／120例）,随访1年有89．3％的CAL恢复正常,10．7％未能恢复正常,均为IVIG耐药病例,两组比较,差异亦无统计学意义（P〉0．05）。结论 IVIG1g／kg单次静脉注射治疗KD,可有效缓解临床症状,减低CAL发生率,减轻心血管系统损害,与IVIG2g／kg比较具有同样的近期和远期治疗效果。     

免疫球蛋白无应答川崎病再次免疫球蛋白、英夫利昔单抗和激素治疗队列研究

背景 IVIG无应答KD患儿的治疗方案包括再次IVIG、英夫利昔单抗(IFX)和激素(IMP),目前不同治疗方案的疗效评价不一.目的 IVIG无应答KD患儿再次IVIG、IFX和IMP治疗效果比较.研究设计队列研究.方法 以IVIG无应答KD患儿为队列人群,再次治疗和补救治疗(再次治疗无反应)在江西省儿童医院(我院)中...     

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. Serum samples were obtained before and after initial IVIG. Using a commercial chemiluminescence enzyme immunoassay, we examined the serum levels of two cytokines related to T‐cell activation and the severity of inflammation: soluble interleukin‐2 receptor and interleukin‐6. Results: There were no significant differences in the serum levels of the two cytokines before initial IVIG among the three groups, but significant intergroup differences were evident after initial IVIG in the serum levels of soluble interleukin‐2 receptor (P < 0.01, Group A > C) and interleukin‐6 (P < 0.01, Group A > B > C). Conclusions: Our results show that marker of T‐cell activation is elevated most markedly in KD patients resistant to both initial and additional IVIG, and suggest that T cells may be activated in refractory KD.     

人血丙种球蛋白和阿司匹林治疗对川崎病患儿循环内皮祖细胞功能的影响

目的：研究人血丙种球蛋白(IVIG)和阿司匹林(ASP)治疗对川崎病患儿循环内皮祖细胞功能的影响及其可能的机制。方法：抽取10例川崎病患儿IVIG和ASP治疗前及治疗7 d后的外周血,将单个核细胞诱导培养内皮祖细胞。利用MTT法、改良Boyden小室法、细胞培养板贴壁法测定内皮祖细胞的增殖、迁移和贴壁功能。同时测定血浆中肿瘤坏死因子-α（TNF-α）和超敏C反应蛋白（hs-CRP）的浓度。结果：IVIG和ASP治疗7 d后循环内皮祖细胞的功能较治疗前显著提高。IVIG和ASP治疗7 d后血浆中TNF-α和hs-CRP 的浓度显著降低。治疗后TNF-α和hs-CRP 浓度的降低与内皮祖细胞功能的提高呈显著正相关关系。结论：IVIG和ASP治疗可显著改善川崎病患儿循环内皮祖细胞的功能,其机制可能与TNF-α和hs-CRP的浓度降低有关。     

血清心脏肌钙蛋白I在儿童川崎病中的诊断价值

目的：探讨静脉注射免疫球蛋白(IVIG)在川崎病(KD) 治疗前后血清心肌肌钙蛋白I(cTnI)浓度变化的临床意义。方法：检测 KD组 (n=58) 及对照组 (n=23) 患儿血清 cTnI、肌酸激酶(CK)、乳酸脱氢酶( LDH)与谷草转氨酶(GOT)的浓度。结果：KD组血清cTnI浓度为(0.62±1.08) μg/L,明显高于对照组(0.06±0.06) μg/L,两者差异有显著性意义(P0.05)。IVIG治疗后cTnI浓度为(0.08±0.15) μg/L,明显低于治疗前(0.62±1.08) μg/L,差异有显著性意义(P0.05)。结论：cTnI是一项早期诊断 KD患儿急性心肌损伤的有价值的方法,可为早期静脉注射免疫球蛋白减少心血管病变提供依据,并可作为 IVIG疗效观察指标。     

Analysis of potential risk factors associated with nonresponse to initial intravenous immunoglobulin treatment among Kawasaki disease patients in Japan

BACKGROUND: Some Kawasaki disease (KD) patients do not respond to initial treatment with intravenous immunoglobulin (IVIG). The purpose of this study was to determine potential risk factors associated with IVIG nonresponse among KD patients in Japan. METHODS: Data were obtained from questionnaires used for the 18th nationwide KD survey of patients who visited hospitals in Japan from 2003 through 2004. Data for patients who met the case definition for KD and received 2 g/kg single infusion IVIG as the initial treatment within 10 days of illness were analyzed. IVIG nonresponders were defined as patients who needed secondary treatment after initial IVIG administration. RESULTS: Among 15,940 KD patients in Japan during 2003-2004, 6330 patients received 2 g/kg single infusion IVIG within 10 days of illness onset. IVIG nonresponders accounted for 20.3% of them (n = 1286). Male sex [odds ratio (OR), 1.21, 95% confidence interval (CI), 1.06-1.37], receipt of the initial IVIG before the fifth day of illness (OR: 1.89, 95% CI: 1.66-2.15), and having recurrent KD (OR: 1.38, 95% CI: 1.00-1.90) were significantly associated with IVIG nonresponse. In addition, IVIG nonresponders had significantly higher risks for coronary artery aneurysms (OR: 10.38, 95% CI: 6.98-15.45) or giant coronary artery aneurysms (OR: 54.06, 95% CI: 12.84-227.65). CONCLUSIONS: Physicians should consider potential IVIG nonresponse among recurrent KD patients or KD patients diagnosed and treated before the fifth day of illness, particularly if they are boys and have laboratory values associated with nonresponse such as low platelet count, and elevated alanine aminotransferase and C-reactive protein. Some of these patients may benefit from administration of the alternative secondary treatment early during the illness along with the initial IVIG treatment.     

激素与静脉丙种球蛋白及其联合应用治疗静脉丙种球蛋白无反应川崎病的回顾性对照研究

目的 探讨激素对静脉丙种球蛋白（IVIG）无反应川崎病（KD）患儿的治疗价值。方法 回顾性收集重庆医科大学附属儿童医院IVIG无反应KD住院患儿,根据再治疗情况分为IVIG组、激素组和IVIG+激素组;将应用激素者根据激素应用途径分为静脉滴注序贯口服激素组和口服激素组。统计各组患儿的总热程、平均热退时间、治疗前后实验室检查结果并计算治疗前后的差值与治疗前值的比值（即差值比△）、急性期及出院后6、12、24个月冠状动脉病变和随访中的血栓形成情况。结果 IVIG无反应KD患儿143例进入本文分析。IVIG组107例,激素组12例, IVIG+激素组24例,3组性别、年龄、体重差异无统计学意义。IVIG+激素组治疗后WBC高于IVIG组,△WBC、△PLT均低于IVIG组,△CRP高于IVIG组,总热程长于IVIG组;急性期冠状动脉瘤发生率及随访至6个月时冠状动脉扩张发生率高于IVIG组,差异均有统计学意义;激素组△WBC低于IVIG组,总热程长于IVIG组,差异均有统计学意义。静脉滴注序贯口服激素组和口服激素组各18例,2组性别、年龄、体重差异无统计学意义,口服激素组再次治疗前和再次治疗后CRP均低于静脉滴注序贯口服激素组, 口服激素组△PLT高于静脉滴注序贯口服激素组,2组随访时点冠状动脉扩张和冠状动脉瘤发生率差异均无统计学意义。随访病例中,静脉滴注序贯口服激素组有2例出现血栓,经积极抗凝治疗后血栓消失。结论 IVIG无反应KD患儿再治疗时应用激素或再次IVIG无反应后应用激素,与单纯IVIG相比急性期治疗效果相近,且均不增加远期冠状动脉损伤的发生率;选择普通剂量口服或者静脉滴注序贯口服疗法临床效果相近,但静脉滴注序贯口服激素较口服激素有更高的血栓形成风险。     

中性粒细胞与淋巴细胞比值及血小板与淋巴细胞比值对于川崎病IVIG敏感性的预测价值

目的分析中性粒细胞与淋巴细胞比值(NLR)及血小板与淋巴细胞比值(PLR)对川崎病(KD)免疫球蛋白(IVIG)敏感性的预测作用。方法采用回顾性队列研究,以404例初诊川崎病患者为研究对象,收集IVIG治疗前后的血常规、NLR和PLR值,通过ROC曲线确定NLR和PLR在预测川崎病IVIG不敏感的最佳cut-off值;用logistic回归确定IVIG不敏感的独立预测因素。结果 404例川崎病患者中31例IVIG不敏感。与IVIG敏感组相比,IVIG不敏感组的冠状动脉扩张发生率明显增高(P0.01);启用IVIG时的病程较短(P0.05);IVIG治疗前后的NLR、PLR水平以及CRP均较高(P0.05)。IVIG治疗前、后的NLR和PLR对川崎病IVIG不敏感最佳预测值分别为4.36、162和1.45、196。多元回归分析显示IVIG治疗前的病程、CRP,以及IVIG治疗前后的NLR和PLR是IVIG不敏感的独立预测因素。结论 NLR和PLR可能作为川崎病患者丙球不敏感的预测指标。     

Steroid pulse therapy for Kawasaki disease unresponsive to additional immunoglobulin therapy

BACKGROUND:

The optimal management of Kawasaki disease (KD) unresponsive to intravenous immunoglobulin (IVIG) therapy remains unclear.

OBJECTIVE:

To prospectively evaluate the efficacy and safety of intravenous methylprednisolone pulse (IVMP) therapy in KD cases unresponsive to additional IVIG.

METHODS:

KD patients who initially received IVIG (2 g/kg/24 h) and acetylsalicylic acid within nine days after disease onset were studied. Patients who did not respond received additional IVIG (2 g/kg/24 h), and those who still did not respond were given IVMP (30 mg/kg/day) for three days, followed by oral prednisolone. The response to treatment, echocardiographic findings and adverse effects were evaluated.

RESULTS:

Among 412 KD cases, 74 (18.0%) were treated with additional IVIG; 21 (28.4%) of the latter cases subsequently received IVMP followed by prednisolone. All cases became afebrile soon after IVMP infusion and did not have a high-grade fever during treatment with prednisolone for two to six weeks. Four weeks after disease onset, coronary artery lesions (CAL) were diagnosed according to the Japanese Ministry of Health and Welfare or the American Heart Association criteria in two of the 21 cases treated with IVMP plus prednisolone; among all 412 cases, three (0.7%) and eight (1.9%) had CAL according to each criteria, respectively. All CAL regressed completely one year after disease onset. Adverse effects of IVMP, such as hypothermia and sinus bradycardia, resolved spontaneously.

CONCLUSIONS:

In KD patients unresponsive to additional IVIG, IVMP promptly induced defervescence, and subsequent oral prednisolone suppressed recurrence of fever. IVMP followed by prednisolone therapy may prevent CAL, without severe adverse effects.     

基质金属蛋白酶9及组织抑制物1对预测和早期诊断川崎病冠状动脉病变的意义

目的探讨川崎病（Kawasaki disease,KD）血清基质金属蛋白酶9（matrix metallopmteinase-9,MMP-9）及其特异性组织抑制物1（tissue inhibitor of metalloproteinase-1,TIMP-1）水平的动态变化在冠状动脉病变（coronary artery lesion,CAL）的预测和早期诊断中的临床价值。方法实验组KD合并CAL患儿15例,未合并CAL患儿44例,急性期静脉注射免疫球蛋白（intravenous immunoglobulin,IVIG）前后、亚急性期及恢复期各抽取1次外周静脉血,对照组为20例正常体检儿童。ELISA双抗体法测定血清MMP-9与TIMP-1含量。结果KD患儿急性期血清MMP-9含量、TIMP-1含量及MMP-9／TIMP-1均较正常对照组增高（P〈0、01）;IVIG干预后KD患儿血清MMP-9含量与MMP-9／TIMP-1比值降低（P〈0．01）;KD合并CAL患儿IVIG干预前血清MMP-9含量及血清MMP-9／TIMP-1比值高于无CAL患儿（P〈0．01）。结论川崎病冠状动脉病变患儿血清MMP-9含量与MMP-9／TIMP-1比值在急性期高于无冠状动脉病变患儿,提示MMP-9含量的急剧升高及与TIMP-1相互拮抗作用的失代偿可能是川崎病冠状动脉病变的高风险因素,动态监测血清MMP-9含量和（或）MMP-9／TIMP-1比值对预测和早期诊断川崎病合并冠脉病变具有重要的临床意义。     

Steroid Pulse Therapy for Children With Intravenous Immunoglobulin Therapy–Resistant Kawasaki Disease: A Prospective Study

Patients with Kawasaki disease (KD) who did not respond to the initial IVIG are known to have higher risk for developing coronary arterial lesions (CALs). Our aim is to clarify whether patients with initial IVIG resistant KD may benefit from methylprednisolone pulse therapy (MPT) in comparison with re- treatment of IVIG (2nd IVIG). A total of 237 patients (median age: 2 years 2 months; range 1 months–10 years) with KD were initially treated with IVIG (2 g/kg). Among them, 41 patients (22 %) were assessed as IVIG resistance: these patients were allocated to either group A receiving MPT (n = 14) or group B receiving the 2nd IVIG (n = 27). Patients with resistant to the additional therapy (MPT or 2nd IVIG) were received second IVIG (group A) or MPT (group B). Changes in leukocyte count, C-reactive protein and albumin before and after an additional therapy were significantly greater in group A than those in group B. However, the prevalence of CALs did not differ between the groups (36 % in group A and 26 % in group B, p > 0.05). There was no significant difference in the medical cost between the groups (median cost: 92,032 JPY in group A and 97,331 JPY in group B). MPT does not reduce the risk of development to CAL and does not seem to be beneficial as single agent therapy for IVIG resistant KD.     

Comparison of the Laboratory Data Between Kawasaki Disease and Enterovirus After Intravenous Immunoglobulin Treatment

Kawasaki disease (KD) is a systemic vasculitis of unknown etiology. The laboratory findings before and after intravenous immunoglobulin (IVIG) in KD have been discussed, but the characteristics of IVIG therapy still are unclear. This study aimed to compare laboratory data from patients with KD and enterovirus (EV) infection to evaluate the differences after IVIG therapy. The study enrolled 171 KD patients and 38 EV patients treated with a single dose of IVIG from 2003 to 2010. Laboratory data including total white blood cell counts (WBC) and hemoglobin (Hb), platelet, segment, lymphocyte, eosinophil, and monocyte levels were analyzed. Compared with the KD patients, the EV patients had higher Hb, lymphocyte, and monocyte levels and lower eosinophil levels before IVIG treatment (p?<?0.05). After IVIG treatment, the KD patients had lower Hb and segment levels but higher platelet, lymphocyte, and eosinophil levels than the EV patients (p?<?0.05). In the KD patients, the platelet, eosinophil, and monocyte levels increased after IVIG treatment, whereas Hb, WBC, and segment levels decreased significantly (p?<?0.001). In the EV patients, eosinophil levels increased after IVIG treatment, whereas WBC and Hb levels decreased significantly (p?<?0.05). The study results provide evidence that eosinophilia may be related to IVIG therapy in KD and EV patients. The KD patients had higher eosinophil levels both before and after IVIG therapy than the EV patients, which may have been due to the inflammatory mechanism of KD. The KD patients had higher platelet levels than the EV patients, suggesting that platelets are involved in the inflammatory response to KD.