Giant coronary aneurysms due to Kawasaki disease: A case−control study

BACKGROUND: Epidemiologic features of giant coronary aneurysm due to Kawasaki disease and its risk factors are still not clear. METHODS: Sixty-six patients with giant coronary aneurysms were reported to a 15th nationwide survey of Kawasaki disease in Japan. With all other patients treated in the same hospital as a control group, odds ratios were calculated for certain potential risk factors. RESULTS: Infant males aged less than 1 year,neutrophil concentration among leukocyte, late administration of intravenous gammaglobulin (IVGG) therapy and additional administration of IVGGwere considered as risk factors of giant coronary aneurysms due to Kawasaki disease. In univariate analysis, use of IVGG therapy and a large amount of IVGG (2500+ mg/kg)elevated the risk, whereas the relationship disappeared after the adjustment. CONCLUSIONS: The observation of 66 cases with giant coronary aneurysms due to Kawasaki disease reported to the nationwide survey provides some risk factors and consideration about the aneurysms.     

Coronary risk factors in Kawasaki disease treated with additional gammaglobulin.

AIMS: To assess the hypothesis that an additional intravenous gammaglobulin (IVGG) infusion, if administered early, may prevent coronary artery lesions (CAL) in patients with Kawasaki disease (KD) who do not respond to initial IVGG therapy. METHODS: Forty four KD patients (17 with CAL and 27 without CAL), treated with additional IVGG because of persistent or recrudescent fever after initial IVGG therapy, were studied. Main outcome measures were the presence of CAL by echocardiography and the number of febrile days before and after start of additional IVGG infusion (pre- and post-additional IVGG). RESULTS: In univariate analyses, risk factors for CAL were the number of febrile days pre-additional IVGG, the number of febrile days post-additional IVGG, the number of days that initial IVGG was divided over, the white blood cell count pre- and post-additional IVGG, and the C reactive protein concentration pre-additional IVGG. In a multivariate analysis, the only independent risk factor was the number of febrile days pre-additional IVGG (> or =10 days; odds ratio 7.86; 95% CI 1.44 to 42.8; p = 0.02). CONCLUSIONS: Among KD patients with persistent or recrudescent fever after initial IVGG therapy, administration of additional IVGG before the first 10 febrile days was associated with a decreased prevalence of CAL, when compared with the prevalence in those who were retreated later. An additional IVGG infusion, if administered early, may prevent CAL in initial IVGG non-responders.     

Coronary risk factors in Kawasaki disease treated with additional gammaglobulin

Aims: To assess the hypothesis that an additional intravenous gammaglobulin (IVGG) infusion, if administered early, may prevent coronary artery lesions (CAL) in patients with Kawasaki disease (KD) who do not respond to initial IVGG therapy. Methods: Forty four KD patients (17 with CAL and 27 without CAL), treated with additional IVGG because of persistent or recrudescent fever after initial IVGG therapy, were studied. Main outcome measures were the presence of CAL by echocardiography and the number of febrile days before and after start of additional IVGG infusion (pre- and post-additional IVGG). Results: In univariate analyses, risk factors for CAL were the number of febrile days pre-additional IVGG, the number of febrile days post-additional IVGG, the number of days that initial IVGG was divided over, the white blood cell count pre- and post-additional IVGG, and the C reactive protein concentration pre-additional IVGG. In a multivariate analysis, the only independent risk factor was the number of febrile days pre-additional IVGG (⩾10 days; odds ratio 7.86; 95% CI 1.44 to 42.8; p = 0.02). Conclusions: Among KD patients with persistent or recrudescent fever after initial IVGG therapy, administration of additional IVGG before the first 10 febrile days was associated with a decreased prevalence of CAL, when compared with the prevalence in those who were retreated later. An additional IVGG infusion, if administered early, may prevent CAL in initial IVGG non-responders.     

Management of Kawasaki disease in the British Isles.

Kawasaki disease in the British Isles was surveyed by an active reporting scheme, based on all cases reported to the British Paediatric Surveillance Unit that were diagnosed between 1 January and 31 December 1990. The study was prompted by the need to investigate the high case fatality rate of Kawasaki disease of 2% observed in 1988. One hundred and sixty three patients were identified of whom six (3.7%) died. Forty five children (28%) suffered cardiac complications of which 39 (24%) were coronary artery abnormalities; five children were diagnosed at postmortem examination, and coronary artery abnormalities were detected by echocardiography in 34. One hundred and forty nine children (93%) had echocardiography. High thrombocytosis, leucocytosis, duration of fever, and younger age were associated with the presence of coronary artery abnormalities. Erythrocyte sedimentation rate, sex, and the number of diagnostic criteria were not. One hundred and thirty three children (87%) received aspirin. Ninety three children (61%) received intravenous gammaglobulin (IVGG). Children were more likely to receive IVGG if they had thrombocytosis or typical Kawasaki disease. The incidence of coronary artery abnormalities was found to be similar in those treated with IVGG (29%) and those untreated (20%), including those treated within 10 days of onset. This may have reflected selection of the more serious cases to receive IVGG or that Kawasaki disease in the British Isles is a different illness to that experienced elsewhere. It amy be, however, that IVGG is less effective in the treatment of British patients with Kawasaki disease than has been the experience in the United States and Japan. These observations emphasise the need for a therapeutic trial of treatment modalities for Kawasaki disease in the UK and the Republic of Ireland.     

Plasminogen activator inhibitor-1 in patients with Kawasaki disease: diagnostic value for the prediction of coronary artery lesion and implication for a new mode of therapy

Kawasaki disease (KD) in children takes the form of acute systemic vasculitis, which causes coronary artery dilation and aneurysm formation in 10% to 15% of the patients. We have recently shown that matrix metalloproteinases (MMPs) are intimately involved in coronary arterial wall destruction and the resultant formation of coronary artery lesions (CALs) in this disease. Plasminogen activators (PAs) are known to be a major pathway of MMP activation, and this suggests that their inhibitor, plasminogen activator inhibitor-1 (PAI-1), also plays important roles in the development of CALs in KD. The present study was conducted to test the hypothesis that circulating levels of PAI-I are related to CAL formation in KD. Plasma levels of PAI-1 were measured by enzyme-linked immunoassay in 37 KD patients without CALs (group 1) and 7 KD patients with CALs (group 2). Blood samples were obtained before and after i.v. gammaglobulin therapy (IVGG), and in the convalescent stage. Levels of PAI-1 were significantly higher in KD patients before IVGG than in 18 age-matched healthy control subjects (p < 0.01). More importantly, both pre-IVGG and post-IVGG levels of PAI-1 were significantly higher in group 2 than in group 1 (p < 0.01). Furthermore, PAI-1 levels of 9 patients from group 1 who showed pre-IVGG PAI-1 levels higher than the minimum PAI-1 level in group 2 significantly decreased after IVGG, whereas PAI-1 levels of group 2 patients remained persistently elevated, further suggesting a close association between PAI-1 and CAL development in KD. Thus, PAI-1 may be useful as a predictive marker for CAL development in KD. Studies of the effects of PA inhibition on coronary outcome may provide evidence that PA is a viable therapeutic target for the prevention of KD-related CALs.     

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目的探讨川崎病(KD)患儿合并冠状动脉损伤的危险因素。方法按照KD诊断标准,对北京儿童医院2000-01-01—2004-12-31收治的644例KD患儿临床资料、治疗方法以及实验室资料进行分析。结果Pear-son卡方检验显示,患儿性别、发热持续时间、丙种球蛋白使用时间、丙种球蛋白使用剂量、血沉及C反应蛋白与KD合并冠状动脉损伤有关(P<0·05);进一步多因素Logistic回归分析显示,性别、发热持续时间、丙种球蛋白使用剂量与冠状动脉扩张显著独立相关(P<0·05)。结论对男性、发热持续时间长的KD患儿应予足够重视,早期足量使用丙种球蛋白以减少或减轻冠状动脉并发症。     

Effect of Intravenous γ-Globulin on Neutrophil Function in Kawasaki Disease

The effect of intravenous γ-globulin (IVGG) on the neutrophil count and neutrophil chemiluminescence (CL) of patients with Kawasaki Disease (KD) was investigated. Forty patients with KD were enrolled in the study. Ten patients were treated with 100 mg/kg/day of γ-globulin for five days (GG 100 group) and 14 patients were treated with 400 mg/kg/day of γ-globulin (GG 400 group) for five days. These patients also took aspirin. Sixteen patients were treated with aspirin alone (ASA group). The neutrophil counts were significantly lower in the GG 400 and GG 100 groups than in the ASA group, three days, and one and two weeks after the start of treatment. Neutrophil CL of the GG 400 and GG 100 groups was significantly lower than in the ASA group one and two weeks after the start of treatment. In the in vitro study, γ-globulin had a dose-dependent suppressive effect on the neutrophil CL in the early stage. Albumin had similar effects. The suppressive effect of γ-globulin on CL was not specific. These findings suggest that IVGG is effective in reducing the production of active oxygen which is considered responsible for the vascular damage in the early stage of KD.     

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目的探讨10d内已退热的川崎病(KD)患儿应用丙种球蛋白(IVGG)治疗的必要性以及不同剂量IVGG治疗对KD预后的影响。方法研究对象为1999-10—2005-10山东省菏泽市立医院收治的56例KD患儿,所有患儿均为10d内退热后确诊且无冠脉病变。按IVGG治疗剂量分成3组,A组(11例)用1g/kg,B组(26例)用2g/kg,C组(19例)未使用,余治疗相同。对其冠状动脉损害(CAL)情况进行对比。结果病程14~21d时发生CAL例数:A组2例(18·18%),B组4例(15·38%),C组16例(84·21%),A、B组比较差异无显著性意义(P>0·05);A、B组与C组之间差异有非常显著性意义(P<0·01)。随访0·5年CAL例数:A组1例(9·09%),B组1例(3·85%),C组11例(57·89%),A、B组比较差异无显著性意义(P>0·05),而A、B组与C组之间差异有非常显著性意义(P<0·01)。结论10d内一经确诊的KD无论是否已退热均应给予IVGG治疗,对已退热且无冠脉损害的患儿应用总量1g/kg IVGG治疗可以达到满意的效果。     

Predictive risk factors for coronary artery abnormalities in Kawasaki disease

Clinical characteristics to predict the development of coronary artery abnormalities (CAA) in Kawasaki disease (KD) were assessed by reviewing medical records of patients diagnosed with KD at Korea University Medical Center from March 2001 to February 2005. Of the 285 patients diagnosed with KD, 19 developed CAA (6.7%). Compared with the CAA(−) group, the CAA(+) group had a longer duration of fever after intravenous gamma-globulin (IVGG) injection (2.4±2.9 vs. 1.5±1.2 days, p=0.008) and higher C-reactive protein (CRP)(12.3±7.8 vs. 8.7±7.1 mg/dL, p=0.038). In particular, the CAA(+) group tended to have more than 7 days of fever before IVGG and more than 3 days of fever after IVGG (26.3 vs. 5.3%, p<0.001; 26.3 vs. 6.4%, p=0.002). When the IVGG responsiveness was defined by the presence of defervescence within 3 days after IVGG, IVGG-non-responders showed a higher incidence of CAA (22.7 vs. 5.3%, p=0.002). Non-responders had a longer duration of fever after IVGG (5.5±1.9 vs. 1.2±0.6 days, p<0.001) and a significantly increased CRP, AST, ALT and total bilirubin. Multivariate regression analysis for CAA showed that the only factor significantly associated with the development of CAA was total fever that lasted for longer than 8 days (OR=4.052, 95% CI=1.151–14.263, p=0.0293). Conclusively, the most important predictor of CAA in KD is total duration of fever longer than 8 days. Early identification of IVGG non-responders and active therapeutic intervention for fever in KD cases might decrease the incidence of CAA.     

Coronary risks after high-dose γ-globulin in children with Kawasaki disease

OBJECTIVES: The goals of the present study were to develop a predictive coronary risk scoring system after intravenous gamma-globulin (IVGG) therapy of any dose for the different preparations currently used in the treatment of children with Kawasaki disease and to determine the predictive value of the system. The previously reported scoring systems were based on treatment with high-dose IVGG therapy at limited doses and were determined using investigative methods. METHODS: Four hundred and fifty-one patients were randomized into one of three groups and received either i.v. polyethylene glycol-treated human immunoglobulin at a dose of either 200 (n = 147) or 400 mg/kg per day (n = 152) or freeze-dried sulfonated human immunoglobulin at 200 mg/kg per day (n = 152) for 5 consecutive days. We documented 31 cases of coronary abnormalities (CA). Univariate and multivariate logistic regression was performed using 49 clinical variables and the resulting predictive model was validated. RESULTS: The duration of fever (odds (I day)/odds (- 5 days)= 0.158; 95% confidence interval (CI) 0.0385-0.648), hemoglobin (odds (Q1 = 10.3)/odds (Q3 = 11.6) = 3.97; 95% CI 1.92-8.20), IgG (odds (Q1 = 1,900)/odds (Q3=2,658)=2.72, 95% CI 1.18-6.25) and IgA (odds (Q1 =72)/odds (Q3= 160) = 0.415; 95% CI 0.253-0.680) levels after completion of gamma-globulin infusion were independent predictors. The model is quasi-cross validated and has acceptable sensitivity and selectivity. The estimated risk and observed occurrence of CA coincide. CONCLUSIONS: Determinants of the risk of CA after IVGG therapy are a longer duration of fever, a lower IgG level, a higher IgA level and a lower hemoglobin level after IVGG infusion. This model is applicable for IVGG doses from 1 to 2 g/kg and for at least two different gamma-globulin preparations.     

血前降钙素在小儿重症感染诊断中的作用

目的　评价血清前降钙素在重症感染诊断中的作用。方法　应用免疫荧光法对 30例重症感染患儿于入院时和入院后 4 8h进行血清前降钙素水平测定 ,并比较血清CRP、白细胞计数对感染疾病的实验室诊断价值。结果　 17例细菌感染患儿血清前降钙素水平明显升高 ,13例病毒感染患儿血前降钙素水平正常或仅有轻度升高。血清CRP在两组间有数据重叠。经有效抗生素治疗后 ,血清前降钙素水平的下降幅度明显大于血清的下降幅度 (P分别为 0 0 2 8和 0 196 )。结论　血前降钙素是鉴定细菌感染和病毒感染的重要指标 ,是判断细菌感染治疗效果的客观依据之一     

Prospective study of 64 cases of Kawasaki's disease

We prospectively studied 64 children with Kawasaki disease. The well recognized signs were seen, but we also found hyperemia of the tympanic membrane in 27%, uveitis in 17%, syndrome of inappropriate secretion of anti-diuretic hormone in 9%, hydrops of the gallbladder in 8%, pulmonary infiltrations in 5% and one death. One patient presented with acute kidney failure, another one presented with stenosis of the renal artery, and 8 presented white blood cells (WBC) in the urinary sediment. Sedimentation was accelerated and there was an important decrease in hemoglobin blood level around the 5th day of illness. The WBC count rose around the 10th day; thereafter, all these results returned to normal while platelets counts rose. Albumin blood level was low, and all the following data were higher than normal: alpha-1-globulin, alpha-2-globulin, IgA, IgM, SGOT, SGPT, bilirubin and CRP. Viral studies were performed in 37 patients; 12 showed positive results.     

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。     

Pentoxifylline and intravenous gamma globulin combination therapy for acute Kawasaki disease

We compared the efficacy of oral administration of pentoxifylline (PTX) and intravenous infusions of gamma globulin (IVGG) combination therapy with that of IVGG in reducing the frequency of coronary-artery lesions (CAL) in children with Kawasaki disease (KD), in a randomized trial. All patients with KD received acetylsalicylic acid (30 mg/kg per day), until the 30th day, after the onset of fever, followed by daily acetylsalicylic acid at a dose of 3-5 mg/kg per day there-after, and intravenous IVGG, 200 mg/kg per day, for 5 consecutive days. In addition, patients randomly assigned to PTX and IVGG combination therapy groups received oral PTX at a dosage of 10 mg/kg per day (low-dose) or 20 mg/kg per day (high-dose), in three divided doses until the 30th day. Patients with KD were all free from CAL prior to treatment. We assessed the presence of CAL by two-dimensional echocardiography which was also done prior to treatment and then twice a week after hospital admission. We detected CAL in 3 of 18 patients (16.7%) in the IVGG therapy group, as compared with 2 of 18 patients (11.1%) in the low-dose PTX and IVGG combination therapy group. There were no significant differences between the two groups. In the next study, we detected CAL in 3 of 21 patients (14.3%) in the IVGG therapy group, as compared with none of 22 patients (0%) in the high-dose PTX and IVGG combination therapy group (² = 6.4, P < 0.02). No adverse side-effects were observed in 79 patients with KD.     

Procalcitonin and C-reactive protein as diagnostic markers of severe bacterial infections in febrile infants and children in the emergency department

OBJECTIVE: To assess the value of procalcitonin (PCT) and C-reactive protein (CRP), compared with that of total white-blood cell count (WBC) and absolute neutrophil count (ANC), in predicting severe bacterial infections (SBIs) in febrile children admitted to Emergency Department. METHODS: A prospective study was conducted in 408 children aged 7-days to 36-months, admitted with fever without source, at a tertiary care Pediatric Emergency Department. PCT, CRP, WBC, and ANC were determined upon admission and compared. Specificity, sensitivity, multilevel likelihood ratios, receiver operating characteristic (ROC) analysis, and multivariate stepwise logistic regression were carried out. RESULTS: SBI was diagnosed in 94 children (23.1%). PCT, CRP, WBC, and ANC were significantly higher in this group than in non-SBI patients. The area under the ROC (AUC) obtained was 0.82 (95% CI: 0.78-0.86) for PCT, 0.85 (95% CI: 0.81-0.88) for CRP (P = 0.358), 0.71 (95% CI: 0.66-0.75) for WBC, and 0.74 (95% CI: 0.70-0.78) for ANC. Only PCT (OR: 1.32; 95% CI: 1.11-1.57; P < 0.001) and CRP (OR: 1.02; 95% CI: 1.01-1.03; P < 0.001) were retained as significant predictors of SBI in a multiple regression model. For infants with fever <8 hours (n = 45), AUC for PCT and CRP were 0.92 (95% CI: 0.80-0.98) and 0.75 (95% CI: 0.60-0.87), respectively (P = 0.056). CONCLUSION: Both PCT and CRP are valuable markers in predicting SBI in children with fever without source and they perform better than WBC and ANC. PCT appears more accurate at the beginning of infections, but overall CRP may be the most convenient marker for its better sensitivity and feasibility.     

Assessment of Risk Factors for Korean Children with Kawasaki Disease

Kawasaki disease (KD) is the most common cause of acquired heart disease in children. Intravenous immunoglobulin (IVIG) is the standard therapy for KD, but more than 10% of KD patients do not respond to IVIG and are at high risk for the development of coronary artery lesions (CALs). To identify clinical and genetic risk factors associated with CAL development and IVIG nonresponsiveness, this study analyzed the clinical data for 478 Korean KD patients. Multivariate logistic regression analysis showed that incomplete KD, IVIG nonresponse, fever duration of 7?days or longer, and the CC/AC genotypes of the rs7604693 single nucleotide polymorphism (SNP) in the PELI1 gene were significantly associated with the development of CALs, with odds ratios (ORs) ranging from 2.06 to 3.04. The risk of CAL formation was synergistically increased by the addition of individual risk factors, particularly the genetic variant in the PELI1 gene. Multivariate analysis also showed that a serum albumin level of 3.6?g/dl or lower was significantly associated with nonresponsiveness to IVIG [OR, 2.76; 95% confidence interval (CI), 1.34-5.68; P?=?0.006]. Conclusively, incomplete KD, IVIG nonresponsiveness, long febrile days, and the rs7604693 genetic variant in the PELI1 gene are major risk factors for the development of CALs, whereas low serum albumin concentration is an independent risk factor for IVIG nonresponsiveness.     

Management and outcome of persistent or recurrent fever after initial intravenous gamma globulin therapy in acute Kawasaki disease

OBJECTIVE: To determine differences in clinical characteristics, laboratory findings, and cardiac complications between patients with acute Kawasaki disease who received additional treatment for persistent or recurrent fever vs those who did not. DESIGN: Nonconcurrent case series; medical record review. SETTING: Tertiary care pediatric hospital. PATIENTS: One hundred eighty-five consecutive patients diagnosed as having acute Kawasaki disease at The Hospital for Sick Children, Toronto, Ontario, from 1995 to 1997. MAIN OUTCOME MEASURE: Prevalence of cardiac complications. RESULTS: Twenty-one patients (11%) received additional treatment with intravenous gamma globulin (IVGG) with or without intravenous methylprednisolone for persistent fever lasting for more than 48 hours or recurrent fever after initial treatment with IVGG. Patients who received additional treatment did not differ significantly from other patients regarding age, sex, race, or diagnostic criteria. Compared with the patients who did not receive additional therapy, the patients who received additional treatment had shorter median interval from fever onset to initial dose of IVGG (5 vs 6 days; P=.006) and longer total days of fever (9 vs 6 days; P<.001). Initial laboratory investigations did not differ significantly. On initial echocardiography, patients who received additional therapy were significantly more likely to have pericardial effusion (33% vs 15%; P=.04), ventricular dysfunction (14% vs 2%; P= .002), and coronary artery ectasia (76% vs 43%; P=.004) but not aneurysms (10% vs 5%; P= .47). At 12 months after diagnosis, there were no significant differences between the 2 groups regarding the prevalence of coronary artery ectasia or aneurysms. CONCLUSION: Patients receiving additional treatment for persistent or recurrent fever have similar demographic and clinical characteristics, greater initial cardiac involvement, and similar overall outcomes.     

Iv gamma globulin treatment of Kawasaki disease in Japan: results of a nationwide survey

The administration of iv gamma globulin (IVGG) for Kawasaki disease was investigated throughout Japan in 1993 by obtaining information from the pediatric departments of 2652 hospitals with more than 100 beds. A total of 1826 hospitals (68.9%) responded, reporting on 11 221 patients who were diagnosed during the survey period from January 1991 to December 1992. There were 8958 patients (79.8%) who received IVGG treatment. The most common treatment modality was 200mg/kg (29.6%), followed by 400mg/kg (18.7%) and 300mg/kg (12.9%), all for 5 days. The distributions of total dose were: 1000 mg/kg or less, 45.7%; 1001-1500 mg/kg, 27.3%; and over 1500 mg/kg, 23.8%. For all patients to whom IVGG was administered, treatment was started in 53.8% by day 5 of illness and in 86.1 % by day 7. The proportion of those with cardiac sequelae was higher in patients who were treated with IVGG, possibly due to the fact that those who were more severely affected were more likely to be treated with IVGG. Epidemiology, gamma globulin treatment, Japan, Kawasaki disease     

Intravenous γ-Globulin Treatment in Kawasaki Disease

A multicenter randomized controlled study was carried out to assess the effectiveness of different, doses and kinds of γ-globulin in Kawasaki disease. Gamma globulin lowered the incidence of coronary artery abnormalities. The effect of γ-globulin was dose dependent. The intact type was more effective than the pepsin treated type. To establish the indications for γ-globulin, a study was made of patients who received neither γ-globulin nor indomethacin and who, within nine days of onset of illness, satisfied at least four of the following criteria: (1) WBC: more than 12,000/mm; (2) platelet count: less than 35×10⁴γmm; (3) CRP: more than 3 +; (4) Hct: less than 35%; (5) albumin: less than 3.5 g/dl (6) age: 12 months or less; (7) male sex. This prospective study is continuing. Of 143 children, 73.4% received γ-globulin, and only two demonstrated small dilatations of the coronary arteries in children who did not receive γ-globulin. These guidelines seem satisfactory to establish the indications for γ-globulin in Kawasaki disease.     

Epidemiology and Risk Factors for Coronary Artery Abnormalities in Children With Complete and Incomplete Kawasaki Disease During a 10-Year Period

Kawasaki disease (KD) is an acute systemic vasculitis of childhood. The diagnosis is based on clinical criteria. However, the presentation of KD is incomplete/atypical for approximately 20 % of patients. Kawasaki disease is complicated with coronary artery lesions (CALs) and considered the most common cause of acquired heart disease in children. The medical records of children discharged with KD from a tertiary pediatric hospital in Athens, Greece, during a decade (2001–2010) were retrospectively analyzed. During the study period, KD was diagnosed for 86 children younger than 14 years of age. Complete diagnostic criteria were fulfilled by 64 of the children (74.4 %), whereas 25.6 % were considered incomplete cases. Cardiovascular complications were detected in 48 children (55.8 %) and CALs in 28 children (32.6 %). The prevalence of CALs did not differ significantly between complete and incomplete/atypical KD (42.2 vs 4.5 %; P = 0.001). Logistic regression analysis showed that erythema in the lips and oral cavity was associated with the development of CALs [odds ratio (OR), 3.03; 95 % confidence interval (CI), 1.051–8.783; P = 0.040]. Conversely, children with incomplete/atypical KD (OR, 0.092; 95 % CI, 0.010–0.816; P = 0.032) and previous antibiotic treatment (OR, 0.17; 95 % CI, 0.036–0.875; P = 0.034) were less likely to experience CALs. Children with an incomplete/atypical presentation of KD or before antibiotic treatment may be at lower risk for the development of CALs. Future multicenter studies may help to establish this association better.