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.     

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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。     

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.     

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.     

Intravenous γ-Globulin for Kawasaki Disease

We studied the effect of γ-globulin (IVGG) and aspirin (ASA) on the development of the coronary artery lesions (CAL) of Kawasaki disease (KD) in three different protocols. Within 29 days of the onset of KD the echocardiographic evidence of CAL had developed in 39–42% of the patients in the ASA group, but only in 13.7–20.8% of the patients treated with IVGG (200 or 400 mgγkgX5). In long-term follow-up observation of CAL of these patients the evidence of CAL in both the ASA and the IVGG group regressed gradually; however, the residual rate of CAL was significantly low in the IVGG group at all times up to 24 months after onset. These facts suggest that when using IVGG for KD, we should select a dose of intact γ-globulin, 1,000 mgγkg or more in total, to prevent the occurrence of CAL. We have demonstrated not only a significant reduction in the occurrence of CAL in patients treated with IVGG but a reduction in the residual rate of CAL for two years as compared with those treated by ASA.     

Coronary risk factors in acute Kawasaki disease: Correlation of serum immunoglobulin levels with coronary complications

Abstract Background To determine the usefulness of the IgG z-score (age and sex-standardized serum IgG level) before intravenous gamma globulin therapy (1VGG) in predicting the occurrence or severity of coronary complications in Kawasaki disease (KD).
Methods A case-control study of clinical and laboratory findings with 88 children in the early stage of acute KD who received IVGG (100 or 200 mg/kg for2–5 days) therapy. Of these, 20 cases had persistent coronary arterial lesions (small aneurysm, moderate aneurysm or large aneurysm persisting more than 1 month). The controls comprised 68 children with no coronary aneurysms or transient small aneurysm only observed within 1 month after the onset of KD. The association between serum levels of immunoglobulin G (IgG), IgM, IgA as well as other coronary risk factors previously reported and the occurrence of the coronary arterial lesions was evaluated using logistic regression analysis.
Results: After adjustment for age, gender, total IVGG dose before the 9th illness day and other traditional coronary risk factors, the odds ratio for the persistent coronary aneurysm associated with lower serum IgG r-score (<-0.7485 v.v & -0.7485). was 30.3 (95% confidence interval, 3.8–243.2). Furthermore, the serum IgG z-score was inversely correlated with the severity of the coronary arterial lesion.
Conclusions: The IgG z-score before IVGG therapy in the early stage of KD provides useful information on the risk factors for persistent coronary aneurysm and is a novel, additional indicator for therapy to prevent the coronary complications in acute KD.     

Predictors of coronary artery lesions after intravenous gamma-globulin treatment in Kawasaki disease

OBJECTIVE: We evaluated the efficacy of intravenous gamma-globulin (IVGG) administration for children with Kawasaki disease to establish whether additional, more advanced therapy is needed in intractable cases. STUDY DESIGN: A total of 193 children with Kawasaki disease were studied retrospectively. Patients were admitted 3 to 7 days after the onset of the disease, and IVGG was administered. Laboratory measurements including white blood cell (WBC), neutrophil, and platelet counts and C-reactive protein (CRP) and albumin concentrations were determined before and 2 to 3 days after IVGG treatment. The progression of coronary artery lesions (CALs) was monitored by serial echocardiography until 30 days after treatment. RESULTS: Of 193 children, 24 (12.2 %) had CALs including transient dilatation. In contrast to the other measurements, the WBC count increased in 21 of 24 (87.5%) children with CALs after IVGG therapy. The patients with increased neutrophil count and CRP concentration after IVGG therapy also had CAL formation at a high rate (78.3% and 66.7%, respectively). Among children with normal coronary arteries, elevations of the WBC and neutrophil counts and CRP concentration were observed after IVGG therapy in only 3, 6, and 8 patients, respectively (specificity: 98.2%, 97.0%, and 95.3%, respectively). Furthermore, multiple logistic regression indicated that these variables were useful predictors of CALs in KD. CONCLUSION: Though the introduction of IVGG therapy has improved the prognosis of Kawasaki disease, approximately 10% of patients still develop CALs. The need for more aggressive therapy in IVGG-resistant cases can be recognized early by increases in the WBC and neutrophil counts and serum CRP concentration after IVGG administration.     

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.     

川崎病患儿血清中基质金属蛋白酶-9及其组织抑制物-1的变化

目的评价基质金属蛋白酶-9(MMP-9)及其组织抑制物-1(TIMP-1)在川崎病(KD)发病机制中的作用。方法采用酶联免疫吸附法(ELISA)检测33例KD患儿治疗前后血清MMP-9及TIMP-1的含量,并设置无热、发热对照组;同时检测KD患儿外周血中性粒细胞计数、C反应蛋白(CRP)等指标。结果KD组患儿急性期MMP-9血清水平较对照组升高,合并冠脉损害(CAL)者尤甚,治疗后降至正常;MMP-9的升高与外周血中性粒细胞计数、CRP呈正相关;KD患儿无论是否合并CAL,其急性期TIMP-1血清水平均高于对照组,治疗后虽有所下降,仍较对照组高;MMP-9/TIMP-1比值在KD组急性期与对照组差异无统计学意义,治疗后较无热对照组降低,与发热对照组差异无统计学意义。结论MMP-9作为一种损害因素参与了川崎病的病理生理过程,而TIMP-1可抑制其作用;MMP-9的水平可反映KD的严重程度。     

Selective high dose gamma-globulin treatment in Kawasaki disease: Assessment of clinical aspects and cost effectiveness

BACKGROUND: High-dose intravenous gamma-globulin (IVGG) plus aspirin (ASA) treatment is effective in preventing coronary artery complications in acute Kawasaki disease (KD). However, gamma-globulin is very expensive, especially in Japan. Furthermore the indication for IVGG treatment and the optimal dose of gamma-globulin remain controversial. OBJECTIVES: To examine these two issues, we used Harada's scoring system to investigate whether a single 2 g/kg dose therapy has any advantage over the 5 day 400 mg/kg per day therapy. METHODS: We studied 203 patients with KD who had no coronary artery complications on admission. Of these, 145 patients scored 4 or more on Harada score within the first 9 days of illness and were treated with IVGG treatment. Using a random number table, 72 patients were selected to receive a single 2 g/kg dose (2 g group), while the remaining 73 patients were treated with 400 mg/kg per day for 5 consecutive days (400 mg group). Those who had a Harada score of three or less received no IVGG (non-IVGG group) treatment (58 patients). RESULTS: The incidence rate of coronary artery complications in the 2 g group was significantly lower than in the 400 mg group. The duration of high fever, positive duration of C-reactive protein and the number of hospital days in the 2 g group were each significantly shorter than in the 400 mg group. The total medical expense in the 2 g group was significantly lower than in the 400 mg group. There were no coronary artery complications in the non-IVGG group. CONCLUSIONS: It was found to be clinically more effective and more cost effective to select a patient by Harada's scoring system and, where a score of four or more was obtained, to administer a single 2 g/kg intravenous dose of gamma-globulin for acute KD.     

Patients diagnosed with Kawasaki disease before the fifth day of illness have a higher risk of coronary artery aneurysm

BACKGROUND: A fever lasting for at least 5 days is an essential characteristic of the original diagnostic criteria of Kawasaki disease (KD). However, it is not difficult for an experienced physician to confirm the diagnosis of KD before the fifth day of fever. The aim of this study is to investigate the effect of intravenous gamma globulin therapy (IVGG) in KD initiated before the fifth day of illness. METHODS: A total of 125 patients treated with IVGGwere divided into group A (IVGG was initiated before the fifth day of illness, n= 46) and group B (IVGG was initiated at the fifth day or after, n= 79). Patients' characteristics,laboratory findings, treatments and outcomes were compared between the groups. RESULTS: White blood cell count value, C-reactive protein and Harada's score showed no difference between the groups. A significantly higher average value of alanine aminotransferase(ALT) was observed in group A. Although the treatments were identical in both groups, the average duration of fever from the initial day of IVGG in group A was significantly longer than in group B. The incidence of aneurysm in group A was significantly higher than that in group B. Stepwise regression analysis using aneurysm as a dependent variable revealed that group A and ALT were significant. CONCLUSIONS: Patients diagnosed with KD before the fifth day of illness showed a poor response to IVGG. This observation might be related to high ALT values. Further examination concerning the modification of treatment in such patients is necessary.     

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.     

Early intravenous gamma-globulin treatment for Kawasaki disease: the nationwide surveys in Japan

OBJECTIVE: To determine the optimal period of intravenous gamma-globulin (IVGG) treatment, using the database from nationwide Kawasaki disease surveys in Japan. STUDY DESIGN: We selected patients who first visited a doctor within 3 days of illness and received IVGG treatment within 9 days of illness. We divided these patients into 2 groups: an early group (treated on days 1-4: 4731 cases) and a conventional group (days 5-9: 4020 cases). We compared the rate of additional IVGG and prevalence of cardiac sequelae between these groups. RESULTS: The rate of additional IVGG in the early group was significantly higher than those of the conventional group (OR, 1.12 [95% CI, 1.10-1.16]). There were no significant differences in cardiac sequelae between the two groups. CONCLUSIONS: There is no evidence that IVGG treatment on day 4 or earlier has greater efficacy in preventing cardiac sequelae than treatment on days 5 to 9. In addition, early treatment is likely to result in a greater requirement for additional IVGG. However, there is also no evidence that early treatment increases the prevalence of cardiac sequelae in a clinical practice setting, where additional IVGG can be given to those whose initial treatment fails.     

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.     

Coronary artery lesions of incomplete Kawasaki disease: a nationwide survey in Japan

Incomplete Kawasaki disease (KD) is associated with delayed diagnosis and treatment, which in turn can lead to the development of coronary artery lesions (CALs). The aim of this study was to determine the epidemiological features of incomplete KD compared with complete KD and to identify risk factors for CALs from incomplete KD patients using data from a nationwide survey of 2007–2008 in Japan. A total of 23,263 patients were classified according to the number of principal clinical signs: 80% (n = 18,620) had complete forms of KD, 14.2% had four principal signs, 4.6% had three signs, and 1.2% had only one or two signs. In comparison with complete KD cases, the prevalence of CAL development tended to be larger and the proportion receiving initial intravenous immunoglobulin (IVIG) treatment were significantly smaller in patients with incomplete forms. In addition, hospital attendance after 7 days of illness or later was significantly associated with CAL development in all incomplete groups (OR: 2.52 in total patients with incomplete KD, 3.26 in those with one or two principal signs, 2.94 in those with three signs, 2.35 in those with four signs). Conclusion The higher prevalence of CALs in incomplete KD reflects difficulties in diagnosis and delays in treatment. More timely diagnosis and treatment of incomplete KD patients could further prevent the development of cardiac lesions.     

川崎病患儿并发冠状动脉病变的危险因素分析

目的：探讨川崎病(KD)并发冠状动脉病变(CAL)的危险因素。方法：收集2006年1月至2012年1月间诊断为KD的527例患儿的临床资料,对15个可能与CAL发生有关的因素进行单因素和多因素logistic回归分析。结果：单因素分析显示,患儿年龄、性别、KD类型、大剂量丙种球蛋白（IVIG）治疗起始时间、对IVIG治疗的反应、使用糖皮质激素、发热持续时间及C反应蛋白等因素在合并和未合并CAL两组患儿中差异有统计学意义（P8岁、男性、非典型KD、IVIG治疗开始于发热后10 d 以上、对IVIG治疗无反应、发热持续时间>10 d为CAL发生的独立危险因素（OR分别为2.076、1.890、1.972、1.426、3.251、2.301、1.694,均P8岁）、男性、非典型KD、IVIG治疗起始时间较晚、对IVIG治疗无反应、发热持续时间较长是CAL发生的独立危险因素。     

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.     

Herpes simplex virus in febrile neutropenic children undergoing chemotherapy for cancer: a prospective cohort study

BACKGROUND: The primary objective of this study was to determine the prevalence of oral herpes simplex virus (HSV) as detected by polymerase chain reaction, in pediatric oncology patients with febrile neutropenia. Our secondary objectives were to describe the association between oral HSV and prolonged fever, neutropenia, mucositis, and response to initial antimicrobial therapy. METHODS: In this prospective cohort study, we obtained a mouth swab and blood specimen from oncology patients with febrile neutropenia, and tested them for HSV by polymerase chain reaction. Prolonged fever was defined as the presence of fever 48 hours after initiation of broad-spectrum antibiotic therapy. RESULTS: Of the 75 oral and blood specimens obtained, only 7 oral swabs (9%) and 2 blood samples (3%) were positive for HSV. Oral HSV was not associated with prolonged fever or neutropenia. However, oral HSV was associated with longer median duration of mucositis (8 days; interquartile range, 0-12 days) compared with negative episodes (0 days; interquartile range, 0-2.5 days); P = 0.005. Oral HSV also was associated with inferior successful response to initial antimicrobial therapy (1 of 7, 14.3%) compared with negative episodes (51 of 67, 76.1%); P = 0.002. CONCLUSIONS: The prevalence of HSV infection in pediatric oncology patients with febrile neutropenia was low and was not associated with prolonged fever. However, oral HSV was associated with prolonged mucositis and poorer response to initial therapy. It is unknown whether early intervention with acyclovir can alter these associations.     

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.     

942例川崎病的临床分析

目的总结川崎病（Kawasaki disease,KD）的临床特征,探讨KD预后与治疗的关系。方法回顾性分析2000年1月—2004年12月期间942例住院KD患儿的临床资料：（1）比较典型KD与不完全性KD（incomplete KD）的临床特征;（2）总结KD对静脉注射免疫球蛋白（intravenous immune globulin,IVIG）治疗无反应的影响因素;（3）随访观察其中的510例KD患儿,比较IVIG 1g／kg和2g／kg治疗的远期疗效。结果（1）942例中,典型KD774例,不完全性KD168例。不完全性KD冠状动脉病变（coronary artery lesion,CAL）发生率较高（P〈0．05）,除肛周脱屑外,其他临床症状发生较少,出现较晚（P〈0．05或0．01）;（2）与IVIG治疗反应敏感组比较,IVIG治疗无反应组的发热时间较长,血红蛋白（Hb）、白蛋白（ALB）、血细胞比容（Hct）及血小板（PLT）较低（P〈0．05或0．01）;（3）WIG 1g／kg和2g／kg治疗组在KD发病后2年内,CAL的恢复率及新发生率,两组差异均无统计学意义（P〉0．05）。结论（1）不完全性KD的CAL发生率较高,肛周脱屑可以作为不完全性KD的早期诊断依据之一;（2）急性期发热时间较长,PLT无升高及Hb、Hct、ALB持续降低是IVIG治疗无反应的影响因素;（3）IVIG 1g／kg和2g／kg治疗KD的疗效在KD发病后2年内相似。