静脉输注丙种球蛋白防治川崎病冠状动脉病变的疗效

目的评价静脉输注丙种球蛋白(IVIG)治疗和预防川崎病(KD)冠状动脉病变(CAL)的疗效,探讨IVIG疗效的影响因素。方法对314例KD患儿的临床资料进行回顾性对比观察。按治疗将患儿分为阿司匹林(ASA) IVIG组和ASA组,观察两组CAL发生、恢复情况、不同时机不同剂量IVIG治疗KD疗效、临床及实验室指标,急性期出现CAL者分别于病程1,3,6,12个月复查。结果ASA IVIG组CAL发生率34.3%,ASA组56.0%,两组比较P<0.001。应IVIG2.0g/kg或1.0g/kg以及在病程3～10d应用IVIG,CAL发牛率低,P<0.05。22.2?L发生在IVIG治疗后;13.4?L在病程12个月仍不能恢复正常,多数为IVIG治疗开始时间超过10d者。ASA IVIG组住院时间、退热时间、总热程缩短,血小板计数、血沉、C反应蛋白显著降低(P<0.05)。IVIG耐药病例占10.5%。结论IVIG治疗可显著缩短KD病程和降低CAL发生,但对川崎病CAL防治并非人们所预期的那样有效,实际疗效需要再评价。     

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.     

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。     

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.     

Time interval of measles vaccination in patients with Kawasaki disease treated with additional intravenous immune globulin

A standard treatment with a dose of 2 g/kg of intravenous immune globulin (IVIG) is sometimes ineffective in some patients with Kawasaki disease (KD), who are commonly treated with additional IVIG. An interval of 6 to 7 months in Japan and 11 months in the United States is recommended before vaccination against measles after IVIG treatment for KD, but it is not known how long after treatment with additional IVIG. We studied the persistence of measles antibody titers in six episodes of KD in five patients without history of measles infection or vaccination, 4 to 28 months of age, after additional infusion of IVIG. The total dose was 4 g/kg in five episodes and 6 g/kg in one episode. Enzyme immunoassay antibody titers against measles were still positive (400) in all patients tested 3 months after additional infusion of IVIG and positive in one patient and equivocal (200 and <400) in three patients after 6 months, but negative (<200) in all after 9 months following infusion. Neutralization test antibody titers against measles were still positive (1:4) in all patients 3 months after additional infusion of IVIG, but only one after 6 months, and negative (<1:4) in all after 9 months following infusion. Conclusion: we suggest that an appropriate interval between infusion of 4 g/kg of intravenous immune globulin and measles vaccination be 9 months. The 11-month interval recommended in the United States for 2 g/kg may be longer than necessary.Abbreviations EIA enzyme immunoassay - HI hemagglutination inhibition - IVIG intravenous immune globulin - KD Kawasaki disease - NT neutralization test     

Immunoglobulin preparations affect hyponatremia in Kawasaki disease

Hyponatremia frequently occurs in Kawasaki disease (KD). The aim of this study was to investigate the effect of Na content of the intravenous immunoglobulin (IVIG) preparation on serum Na levels in KD. Seventy-eight subjects, of whom 27 had hyponatremia, were split up into two groups: group A receiving IVIG preparations containing high Na (0.9%) and group B receiving IVIG preparations containing trace Na. While the data before IVIG therapy revealed no significant differences in the median serum Na between the groups, an administration of IVIG preparations increased the serum levels of Na in group A (P < 0.01) but not in group B (P > 0.05). Furthermore, the median serum Na level was significantly higher in group A than that in group B (139.0 vs 137.0 mEq/L, respectively, P < 0.01). No significant difference was found in the prevalence of coronary artery lesions between the groups. In conclusion, we should keep it in mind that the IVIG products without Na have an adverse affect on hyponatremia in KD though their efficacy seems to be equivalent to those containing high Na.     

Prediction of non-responsiveness to intravenous high-dose gamma-globulin therapy in patients with Kawasaki disease at onset

Children with Kawasaki disease (n = 82), treated with intravenous immune globulin (IVIG) at a high dose, were classified as IVIG-responsive (defervescence within 5 days of starting IVIG, n = 69) or IVIG-non-responsive (consistent fever over a 6-day period since starting IVIG, n = 13). One patient in the IVIG-responsive group had a coronary artery abnormality during the acute phase (1. 4%) versus 5 in the IVIG-non-responsive group (38.5%). Age, duration of fever before the initiation of IVIG therapy, and laboratory data obtained on admission were tested by the Mann-Whitney U test. Serum levels of C-reactive protein, total bilirubin, lactate dehydrogenase, and gamma-glutamyltranspeptidase were significantly higher (P =.002, P <.001, P <.034, and P <.038, respectively), and the hemoglobin value was significantly lower (P =.025) in patients in the non-responsive group. A multivariate analysis showed that serum levels of C-reactive protein (P =.006), lactate dehydrogenase (P =. 035), and total bilirubin (P =.046) on admission were independent correlates of the success of IVIG therapy. By defining the predictive values, patients with a C-reactive protein level >10 mg/dL, LDH level >590 IU/L, and/or hemoglobin value <10 g/dL are considered non-responsive to IVIG. Additional therapy at an early stage of the disease should be considered for patients who are predicted to be IVIG-non-responsive.     

Septated pericarditis associated with Kawasaki disease: a brief case report

Kawasaki disease (KD) is primarily the systemic vasculitis of childhood that affects mainly the medium-sized arteries, such as the coronary arteries. KD is the leading cause of acquired heart disease, whereas the incidence of rheumatic fever has declined. The most serious complication is coronary artery involvement. Among the children with KD who developed cardiac complications, pericarditis is a rare complication, with an incidence of 0.07%. We report our experience in a 5.5-year-old child with KD complicated with aneurysm of the left anterior descendant coronary artery and septated pericardial effusion, which has not been reported in the literature. The pericardial effusion disappeared very dramatically with intravenous immunoglobulin (IVIG) therapy. We would like to point out that septated pericardial effusion in cases of KD do not need any further therapy other than IVIG and high-dose acetylsalicylic acid.     

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.     

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.     

A multicenter prospective randomized trial of corticosteroids in primary therapy for Kawasaki disease: clinical course and coronary artery outcome

OBJECTIVE: To investigate the role of corticosteroids in the initial treatment of Kawasaki disease (KD). STUDY DESIGN: Between September 2000 and March 2005, we randomly assigned 178 KD patients from 12 hospitals to either an intravenous immunoglobulin (IVIG) group (n = 88; 1 g/kg for 2 consecutive days) or an IVIG plus corticosteroid (IVIG+PSL) group (n = 90). The primary endpoint was coronary artery abnormality (CAA) before a 1-month echocardiographic assessment. Secondary endpoints included duration of fever, time to normalization of serum C-reactive protein (CRP), and initial treatment failure requiring additional therapy. Analyses were based on intention to treat. RESULTS: Baseline characteristics of groups were similar. Fewer IVIG+PSL patients than IVIG patients had a CAA before 1 month (2.2% vs 11.4%; P = .017). The duration of fever was shorter (P < .001) and CRP decreased more rapidly in the IVIG+PSL group than in the IVIG group (P = .001). Moreover, initial treatment failure was less frequent (5.6% vs 18.2%; P = .010) in the IVIG+PSL group. All patients assigned to the IVIG+PSL group completed treatment without major side effects. CONCLUSIONS: A combination of corticosteroids and IVIG improved clinical course and coronary artery outcome without causing untoward effects in children with acute KD.     

肾上腺糖皮质激素联合乌司他丁治疗儿童川崎病的非随机对照临床研究

目的 探讨肾上腺糖皮质激素联合乌司他丁治疗儿童川崎病的临床疗效。方法 根据患儿病情和家长意愿,将2011年1月至2013年12月入院确诊为典型川崎病(KD)的104例患儿分为乌司他丁组(甲基强的松龙+乌司他丁,n=46)和静脉注射丙种球蛋白(IVIG)组(n=58)。观察两组患儿治疗前、治疗后1周、3个月及6个月时的冠状动脉内径的变化,热退时间,再次治疗情况,治疗前、治疗后1周及3周时白细胞(WBC)、血小板(PLT)、血红蛋白(HB)、C反应蛋白(CRP)、血沉的变化以及住院总费用。结果 两组患儿在治疗前、治疗后1周、3个月及6个月时冠状动脉内径比较差异均无统计学意义(P> 0.05)。治疗48 h乌司他丁组患儿体温均正常(100%),正常率高于IVIG组(83%)(PPP结论 甲基强的松龙联合乌司他丁治疗儿童KD没有增加冠状动脉瘤的发生风险;并能大大降低住院费用;与IVIG治疗相比,在KD急性期能更好的控制患儿的实验室指标,缩短发热时间。     

川崎病丙种球蛋白耐药及冠状动脉损伤早期预测指标的研究进展

川崎病是急性全身性血管炎综合征,主要影响冠状动脉.该病的长期预后取决于冠状动脉病变程度.早期应用大剂量静脉丙种球蛋白可以减少冠状动脉病变,丙种球蛋白耐药者冠状动脉病变风险大,早期预测丙种球蛋白耐药及冠脉损伤、及时采取措施对改善预后意义重大.该文对川崎病丙种球蛋白耐药及冠状动脉损伤的预测指标作一介绍.     

Pulse methylprednisolone therapy in the treatment of immune globulin-resistant Kawasaki disease: case report and review of the literature

A subgroup of patients with Kawasaki disease (KD) did not respond to intravenous immune globulin (IVIG) therapy. Corticosteroid therapy remains a controversial alternative in such cases. We report two young children with KD who failed to respond to three courses of IVIG therapy and subsequently received pulse methylprednisolone as an alternative. One had a satisfactory outcome but the other developed giant coronary aneurysms and had a myocardial infarction 2 months after onset of the illness. A review of relevant literature showed that the timing of initiation of pulse methylprednisolone therapy is important. It is suggested that pulse methylprednisolone therapy should be considered if there is no response to two standard doses of IVIG treatment.     

Intravenous immunoglobulin preparation type: Association with outcomes for patients with acute Kawasaki disease

Manlhiot C, Yeung RSM, Chahal N, McCrindle BW. Intravenous immunoglobulin preparation type: Association with outcomes for patients with acute Kawasaki disease.
Pediatr Allergy Immunol 2010: 21: 515–521.
© 2010 John Wiley & Sons A/S To determine whether two different intravenous immunoglobulin (IVIG) preparations were equally efficacious in the treatment of Kawasaki disease (KD). Single centre retrospective review of all patients treated with IVIG for KD between January 1990 and April 2007. Comparison of IVIG (dose 2 g/kg) from two commercial preparations; Iveegam^® stabilized with sugar (lyophilized, 5 g/ml glucose, pH 6.4–7.2, IgA 10 μg/ml, 5% IgG/ml) and Gamimune^® stabilized through acidification (no sugar, pH 4.0–4.5, IgA 270 μg/ml, 5% 1990–1999, 10% 1999–2007 IgG/ml). Propensity‐adjusted differences in duration of fever after treatment initiation, frequency of retreatment with IVIG, hospital stay and maximum coronary artery z‐score. A total of 954 patients were included, 862 (90%) were treated with Iveegam^® and 92 (10%) were treated with Gamimune^®. Patients’ demographic, clinical and laboratory characteristics were similar between the two groups. In propensity‐adjusted models, Iveegam^® was found to be associated with higher probability of non‐response to IVIG (12% vs. 5%, p = 0.05) and longer median duration of fever after IVIG [1 (1–27) vs. 1 (1–8) days, p = 0.02] than Gamimune^®. Nevertheless, Gamimune^® was found to be associated with longer median duration of hospital stay [5 (2–49) vs. 4 (2–76) days, p < 0.0001] and higher median maximum coronary artery z‐score both at the end of the acute phase (+1.4 vs. +0.8, p < 0.0001) and 6–8 weeks after the acute phase (+0.7 vs. +0.4, p < 0.0001). IVIG preparations with lower IgA content and stabilized with glucose appear to be associated with improved coronary artery outcomes for patients with KD.     

对川崎病患儿静脉注射丙种球蛋白耐药临床预测模型建立的质疑

目的 对川崎病(KD)患儿IVIG耐药预测模型提出质疑。方法 回顾性收集经复旦大学附属儿科医院(我院)首次诊断和治疗的KD病例,全样本人群按7∶3比例随机分为建模组和验证组,通过单因素及多因素Logistic回归分析建立IVIG耐药预测模型并行验证,将KD患儿按性别、年龄、发热天数和KD类型等分层,在不同的分层中单独建模和验证;基于全样本人群验证已发表的11个IVIG耐药预测模型,考察通过临床症状、体征和实验室指标是否能满足临床预测KD患儿IVIG耐药。结果 符合本文纳入和排除标准的1 360例KD患儿进入本文分析。男875例(64.3%);年龄中位数1.8(0.9,3.2)岁;IVIG耐药组和敏感组分别为171和1 189例;建模组和验证组分别为952和408例。建模组和验证组人口学特征、主要临床表现、实验室指标、IVIG耐药率和冠脉病变率差异均无统计学意义(P >0.05);建模组中建立的IVIG耐药模型中,男性、发病年龄≥2岁、N%≥0.75、Hb≥110 g·L^-1各计1分,应用首剂IVIG发热≥5 d、ALB≥34 g·L^-1、Na⁺≥133 mmol·L^-1各计2分,AUC为0.818(95% CI:0.774~0.861),总分≥5时,敏感度和特异度分别为0.767和0.726。验证组中AUC为0.777(95% CI:0.712~0.842),敏感度和特异度分别为0.627和0.776。对11个IVIG耐药预测模型验证,以相应预测界值计算敏感度0.272~0.799,特异度0.412~0.926。结论 基于KD患儿人口学特征、临床症状、体征和实验室指标行KD患儿IVIG耐药预测特异度和敏感度均＜75%,对临床预测KD患儿IVIG耐药作用有限。     

对川崎病患儿静脉注射丙种球蛋白耐药临床预测模型建立的质疑

目的 对川崎病(KD)患儿IVIG耐药预测模型提出质疑。方法 回顾性收集经复旦大学附属儿科医院(我院)首次诊断和治疗的KD病例,全样本人群按7∶3比例随机分为建模组和验证组,通过单因素及多因素Logistic回归分析建立IVIG耐药预测模型并行验证,将KD患儿按性别、年龄、发热天数和KD类型等分层,在不同的分层中单独建模和验证;基于全样本人群验证已发表的11个IVIG耐药预测模型,考察通过临床症状、体征和实验室指标是否能满足临床预测KD患儿IVIG耐药。结果 符合本文纳入和排除标准的1 360例KD患儿进入本文分析。男875例(64.3%);年龄中位数1.8(0.9,3.2)岁;IVIG耐药组和敏感组分别为171和1 189例;建模组和验证组分别为952和408例。建模组和验证组人口学特征、主要临床表现、实验室指标、IVIG耐药率和冠脉病变率差异均无统计学意义(P >0.05);建模组中建立的IVIG耐药模型中,男性、发病年龄≥2岁、N%≥0.75、Hb≥110 g·L^-1各计1分,应用首剂IVIG发热≥5 d、ALB≥34 g·L^-1、Na⁺≥133 mmol·L^-1各计2分,AUC为0.818(95% CI:0.774~0.861),总分≥5时,敏感度和特异度分别为0.767和0.726。验证组中AUC为0.777(95% CI:0.712~0.842),敏感度和特异度分别为0.627和0.776。对11个IVIG耐药预测模型验证,以相应预测界值计算敏感度0.272~0.799,特异度0.412~0.926。结论 基于KD患儿人口学特征、临床症状、体征和实验室指标行KD患儿IVIG耐药预测特异度和敏感度均＜75%,对临床预测KD患儿IVIG耐药作用有限。     

Variations in the Number of CCL3L1 Gene Copies and Kawasaki Disease in Korean Children

High-dose intravenous immunoglobulin (IVIG) therapy is the highly effective and standard treatment for Kawasaki disease (KD). However, ~20?% of KD patients have persistent fever or recurrence of fever after the initial IVIG treatment, which increases the risk for coronary artery lesions (CALs). Furthermore, the mechanism of IVIG resistance in KD patients still is unknown. The number of CC chemokine ligand 3-like 1 (CCL3L1) gene copies is reported to be associated with KD and IVIG resistance in Japanese patients. In addition, the authors observed significant upregulation of the CCL3L1 gene expression after in vitro immunoglobulin treatment in B cell lines derived from KD patients. Therefore, this study of 459?KD patients and 496 healthy control subjects tested whether the number of CCL3L1 gene copies is associated with a risk of KD, CALs, and/or IVIG resistance in Korean KD patients. However, the number of CCL3L1 gene copies was not associated with KD (P?=?0.18), CAL formation (P?=?0.062), or the IVIG resistance (P?=?0.90). Therefore, the results indicate that the number of CCL3L1 gene copies does not have a role in susceptibility to KD or CALs nor with IVIG resistance in Korean KD patients.     

Treatment of Kawasaki syndrome: a comparison of two dosage regimens of intravenously administered immune globulin

Because intravenously administered immune globulin (IVIG) is effective in reducing the incidence of coronary artery aneurysms in Kawasaki syndrome when given at a dose of 400 mg/kg daily for 4 days, we undertook a multicenter clinical trial comparing two dosage regimens of IVIG. Patients were randomly assigned to receive IVIG at either 400 mg/kg daily for 4 days (22 patients) or 1 gm/kg as a single dose (22 patients). All patients received aspirin therapy, and all were enrolled within 7 days of onset of fever. The presence of coronary artery aneurysms was evaluated by means of two-dimensional echocardiography before infusion; at days 4 to 6, 14 to 21, and 42 to 49 after infusion; and at 1 year. Coronary artery aneurysms were detected in 3 of the 44 patients, including one patient receiving 400 mg/kg and two patients receiving 1 gm/kg (p value not significant). No giant aneurysms were detected. No major side effects occurred with either dosage regimen. Patients receiving the 1 gm/kg dose had a faster resolution of fever and were discharged from the hospital approximately 1 day sooner than the 400 mg/kg group (p = 0.01). Although the relatively small sample size in this trial does not allow for a more definitive statement regarding the occurrence of coronary artery aneurysms, it appears that the 1 gm/kg dose is associated with a more rapid clinical improvement and a shorter hospital stay.