Clinical features of patients with Kawasaki disease whose parents had the same disease

OBJECTIVE: To observe the clinical characteristics of patients with Kawasaki disease whose parent also had the same disorder. DESIGN: Cross-sectional study using the data from nationwide surveys of Kawasaki disease in Japan. SETTING: All hospitals with a bed capacity of 100 or more and pediatric departments in Japan. PATIENTS: All patients described on the 16th and 17th surveys covering the 4-year period from January 1, 1999, through December 31, 2002. MAIN OUTCOME MEASURES: We compared clinical details, including sibling case, recurrence, diagnosis, administration of intravenous immunoglobulin, and coronary abnormalities, between patients whose parents had Kawasaki disease and patients with no parental history of Kawasaki disease. We also observed age at onset and sex of affected parent-offspring pairs with Kawasaki disease confirmed by using the data of previous nationwide surveys. RESULTS: The odds for having sibling cases were significantly increased among patients whose parents also had Kawasaki disease (odds ratio, 6.94; 95% confidence interval, 2.77-17.38). Patients with parental Kawasaki disease were more likely to experience recurrent Kawasaki disease, receive additional administration of intravenous immunoglobulin, and experience coronary abnormalities at 1 month after onset. Among confirmed parent-offspring pairs with Kawasaki disease, the mean age at onset of offspring was younger than that of their parents (25.6 vs 41.8 months), despite the lack of statistical significance. CONCLUSIONS: Some cases of Kawasaki disease show familial susceptibility to the disorder. Family history, especially parental history of Kawasaki disease, may be an indicator of disease severity.     

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.     

Re-treatment regimens for acute stage of Kawasaki disease patients who failed to respond to initial intravenous immunoglobulin therapy: Analysis from the 17th nationwide survey

BACKGROUND: Current regimens for re-treatment of patients with Kawasaki disease who failed to respond to the initial intravenous immunoglobulin (IVIG) therapy are still uncertain. The purpose of this study is to reveal what regimens were used as the initial therapy and re-treatment for acute stage of Kawasaki disease in the current Japanese medical setting. METHODS: The 17th nationwide survey on Kawasaki disease covered patients whose onset was in 2001 and 2002. In questionnaires sent to all hospitals with a bed capacity of 100 or more and a pediatric department, several questions related to therapeutic regimens for Kawasaki disease were posed. The authors observed the proportions of hospitals that had regimens for patients who failed to respond to the initial therapy. RESULTS: Among those hospitals that responded to the survey, 1052 (64.1%) reported that at least one patient with Kawasaki disease visited the hospital. Among these 1052 hospitals, 73.3% had a regimen to administer 30-39 mg/kg per day of oral aspirin with initial IVIG. The proportion of hospitals that used 1 g/kg per day of IVIG for 2 days was the largest among the options for the initial treatment. For those patients who fail to respond to the initial therapy, 464 hospitals (44.1%) reported that their pediatricians would use additional IVIG only. The number of hospitals that planned to administer high-dose IVIG and ulinastatin was 185 (17.6%). The number of hospitals having regimens of additional IVIG and steroids was 54 (5.1%). CONCLUSIONS: The current status of the treatment for patients with Kawasaki disease not responding to the initial IVIG therapy in Japan was revealed. A randomized trial of a large sample is needed to ascertain the effectiveness of several options for re-treating Kawasaki disease.     

Kawasaki disease in parents and children

Aim: To estimate the probability that the parents of patients with Kawasaki disease also had a history of the same disease. Methods: Self-reported parents' histories of Kawasaki disease were collected from data of the 16th nationwide survey of the disease conducted in Japan from January 1999 to December 2000. The incidence of Kawasaki disease was calculated by using data reported in all 16 nationwide surveys and live births in the Japanese vital statistics. The expected number of parents with a history of Kawasaki disease in the general population, which was calculated by using the assumed number of parents in the vital statistics and the incidence of this disease, was compared with the observed number. Results: Among 14 163 parent pairs of patients with Kawasaki disease, 33 parents (25 mothers and 8 fathers) had a history of the disease. The number of parents expected to have a history of Kawasaki disease was 16.1 (8.4 mothers and 7.7 fathers). From a Poisson distribution, the probability of the observed number was less than 0.001 among parents or mothers. The prevalence of a recurrence of Kawasaki disease and incidences involving siblings of patients whose parents had a history of the disease were five or six times higher than those of all patients who were reported in the 16th survey.

Conclusion: When compared with parents in the general population, the probability of a history of Kawasaki disease was significantly higher in those parents whose children suffered from the same disease. This suggests that, epidemiologically, a genetic predisposition to Kawasaki disease may be implicated in its occurrence.     

Incidence of Kawasaki disease in northern European countries

Background: The aim of the present study was to compare the epidemiologic features of Kawasaki disease (KD) in three northern European countries and Japan. Methods: Data were obtained from discharge databases for hospitals in Finland, Norway, and Sweden for 1999–2009 and from nationwide epidemiologic surveys in Japan for 1998–2008. Annual incidence for each country was calculated using regional census data. Results: During the 11 year period, 1390 KD patients were recorded in the registries of the three northern European countries. Average annual incidence rates per 100 000 children aged <5 years were: Finland, 11.4; Norway, 5.4; and Sweden, 7.4. Overall, 86.4% of Japanese KD patients were aged <5 years compared to only 67.8% in the four northern European countries (P < 0.001). Conclusions: The incidence of KD in northern Europe was constant over the study period and much lower than in Japan. There was a significant age difference between northern European and Japanese KD patients that remains unexplained.     

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.     

Tendency toward atopy in Kawasaki disease

To evaluate the possible trend towards developing allergic disease in children who had suffered from Kawasaki disease (KD), we evaluated data related to allergy that were collected by parental questionnaire on 1,165 children who had suffered from KD. Comparisons were made with 5,825 sex- and age-matched control children. The incidence of household pets and of cigarette smoking in the family were significantly lower in the children with a history of KD than in those of control children. A family history of allergy was significantly more common in the children with a history of KD (71%) versus the controls (56%) (P < 0.001). The incidence of atopic dermatitis and of allergic rhinitis was significantly higher (by approximately 1.7 times) in the KD children versus the control group (P < 0.01), even in subgroups with no family history of allergy. Conclusion We suggest that a genetic predisposition to atopy may be associated with a susceptibility to KD. Patients with KD tended to develop atopic dermatitis and allergic rhinitis. Received: 27 February / Accepted: 9 July 1996     

川崎病丙种球蛋白治疗策略与心脏损害及冠状动脉病变的相关性研究(1998-2008上海地区调查报告)(英文)

目的 川崎病(Kawasaki disease,KD)是一种病因未明的全身血管炎性综合征,伴冠状动脉病变(coronary artery lesion,CAL);大剂量静脉注射丙种球蛋白(intravenous immunoglobulin,IVIG)治疗KD的临床疗效肯定,但目前IVIG的用法和用量尚存在争议.该研究主要为评价不同IVIG方法治疗.KD的效果,探讨最佳治疗方案.方法 由上海市儿科心血管学组制定统一的KD调查表,发放到上海提供儿科服务的50家医院.回顾性分析1998-2008年上海地区住院KD患者的临床资料.共收集完全符合要求的KD患者资料表格1682例,其中男性1064例(63.3%),女性618例(36.7%);发病年龄(2.57±2.33)岁(0.1～18.8岁).治疗KD的IVIG方案包括1 g/ks×1次、2 g/kg×1次、0.4～0.5 g/kg×5次、1 g/kg×2次、2 g/kg×2次及其他.采用SAS 6.12统计软件包进行统计分析,计数资料采用X2检验计算;计量资料数据以X-±s表示,采用t检验.结果 在KD病程的5～10 d应用IVIG有助于最大化降低KD患者的CAL发生率;所有IVIG的KD患者中,应用方案1g/kg×2次治疗者心脏损害、冠脉病变的发生率均为最低,差异有统计学意义(P<0.05).结论 在KD病程5～10 d以IVIG 1g/kg×2次的剂量,有助于最大化降低KD患者的CAL发生率.     

Tendency toward atopy in Kawasaki disease

To evaluate the possible trend towards developing allergic disease in children who had suffered from Kawasaki disease (KD), we evaluated data related to allergy that were collected by parental questionnaire on 1,165 children who had suffered from KD. Comparisons were made with 5,825 sex- and age-matched control children. The incidence of household pets and of cigarette smoking in the family were significantly lower in the children with a history of KD than in those of control children. A family history of allergy was significantly more common in the children with a history of KD (71%) versus the controls (56%) (P?<?0.001). The incidence of atopic dermatitis and of allergic rhinitis was significantly higher (by approximately 1.7 times) in the KD children versus the control group (P < 0.01), even in subgroups with no family history of allergy. Conclusion?We suggest that a genetic predisposition to atopy may be associated with a susceptibility to KD. Patients with KD tended to develop atopic dermatitis and allergic rhinitis.     

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.     

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.     

Self-reported headache in schoolchildren: parents underestimate their children's headaches

Background: Most previous studies of childhood headache have used indirect parental/physician reports to estimate the prevalence of headache in children. Aim: To use direct information from children and to compare the results with data collected from parents. Study design/methods: A cross-sectional questionnaire was sent to parents of 7-12-y-olds in Oslo, Norway. The questionnaire included retrospective reports by the parents of the presence of headache among their children during the past 6 mo. A panel study was subsequently done with daily diaries completed by the children over 6 wk. This was done at school. Results: For 2126 children, both diary reports and parental reports were available. We found a 1-mo prevalence of self-reported headache of 57.6%. Parents, particularly fathers, reported lower headache prevalence than the children. The difference between parental reports and those of the children was larger when the child was a girl. There was a clear difference for the youngest children and parental underreporting for children with the most frequent headaches.

Conclusion: Since indirect parental reports of child headache underestimated headache frequency compared to direct diary-based self-reports, we suggest more widespread use of diary registration of headaches in studies of child headache.     

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.     

1997～2001年四川省川崎病流行病学调查

目的了解四川省川崎病(KD)的发病率和流行病学特点。方法按日本川崎病研究中心提供的川崎病诊断标准、问卷表格和调查方法,对四川省内设有儿科病床的医院进行问卷调查。收集1997年1月1日～2001 年12月31日的川崎病患儿住院资料。结果调查全省212所医院,调查表回收率达到91．5％,全省共报告了KD 患儿1811例,符合川崎病诊断标准1511例。1997～2001年<5岁儿童患病率分别为4．26／10万、5．21／10万、8．57／ 10万、7．70／10万、9．81／10万,5年平均发病率为7．06％。男女之比为1．66:1,发病高峰为1～2岁,17．0％的川崎病患儿有心脏冠脉损伤。典型病例占94．6％,使用静脉丙种球蛋白(IVIG)治疗者占70．9％。结论 1997～2001年四川省5年川崎病的发病率低于日本,与国内其他地区KD流行病学资料相比,发病率高于江苏、陕西和广东,低于北京,有逐年增高趋势。     

Early treatment with intravenous immunoglobulin in patients with Kawasaki disease

OBJECTIVES: To determine if a shorter interval between Kawasaki disease (KD) treatment with intravenous immunoglobulin (IVIG) and fever onset results in increased treatment failures, need for adjunctive therapy, or development of coronary artery lesions. STUDY DESIGN: Patients with KD (n = 178; 89 matched pairs) diagnosed between 1987 and 1999 were included in this case-control study. All patients had fever plus at least 4 of the 5 clinical criteria for KD. Eighty-nine patients who received IVIG at day 5 or earlier were matched to patients diagnosed within 4 weeks and given IVIG at days 6 to 9 of fever. Compiled data from a detailed chart review included demographics, clinical features, fever duration, investigations, disease course, and response to therapy. Differences between matched case and control pairs were analyzed by means oft tests and McNemar tests. RESULTS: No demographic differences were noted between the two groups. Patients treated on day 5 or less of fever had a shorter total fever duration (5.2 +/- 1.9 days vs 8.0 +/- 1.8 days, P <.0001), longer fever after IVIG treatment (1.5 +/- 1.9 days vs 0.8 +/- 1.3 days, P =.008), and less coronary artery ectasia at 1 year after KD onset (4% vs 16%, P =.02). There was no significant difference between cases and control patients in the number of patients with KD recrudescence, need for repeat courses of IVIG, need for corticosteroids, length of hospitalization, or development of coronary artery aneurysms within the first 3 months. Patients who were treated on day 5 or less of fever had higher levels of serum albumin (36 +/- 5 g/L vs 33 +/- 5 g/L, P <.01) and serum ALT (115 +/- 155 U/L vs 46 +/- 49 U/L, P <.001) as well as a lower platelet count (354 +/- 131 vs 403 +/- 166, P =.02) than did control patients during the acute phase. CONCLUSIONS: Early treatment of KD resulted in less coronary ectasia at 1 year after KD onset but was not associated with a quicker resolution of fever, an increased number of treatment failures, an increased need for adjunctive therapy, length of hospitalization, nor development of coronary artery lesions. In children with fever and classic clinical and laboratory findings of KD, treatment with IVIG on or before 5 days of fever resulted in better coronary outcomes and decreased the total length of time of clinical symptoms.     

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目的探讨首次丙种球蛋白静脉注射(IVIG)无反应性川崎病(KD)的发生率及危险因素,及其再治疗方法的选择。方法总结2000—2004年北京45家医院KD患儿的临床资料,IVIG无反应性定义为首次IVIG治疗36h后体温仍超过38·5℃。结果1107例患儿纳入研究对象,1092例有急性期治疗资料,1052例(96·3%)接受IVIG治疗,135例对首次IVIG治疗无反应,发生率12·8%(135/1052)。Logistic回归分析发现血沉、GPT、WBC、发病至用IVIG的时间、血浆白蛋白及IVIG治疗剂量,是IVIG无反应性的独立危险因素(P<0·05)。对IVIG无反应者8例给第2剂2g/kg IVIG,5例热退;114例给1g/kg剂量IVIG治疗,35例(30·7%)热退;11例给400~600mg/kg IVIG,1例(9·1%)热退;2例给糖皮质激素,2例均热退。4种再治疗方法间比较,差异有统计学意义(P=0·015)。第2剂2g/kg IVIG治疗较其它再治疗所需进一步IVIG或激素治疗次数少,体温恢复快。结论约12·8%KD患儿对初次IVIG治疗无反应。血沉、WBC和GPT、血浆白蛋白、IVIG使用方法及起病至用IVIG的时间,是IVIG无反应的独立危险因素。对初次IVIG无反应患儿推荐使用第2剂2g/kgIVIG,对2次2g/kgIVIG治疗仍无效者可以选用糖皮质激素治疗。     

静脉丙种球蛋白无反应性川崎病的治疗及危险因素分析

目的探讨首次丙种球蛋白静脉注射(IVIG)无反应性川崎病(KD)的发生率及危险因素,及其再治疗方法的选择。 方法总结2000—2004年北京45家医院KD患儿的临床资料,IVIG无反应性定义为首次IVIG治疗36h后体温仍超过38.5℃。 结果1107例患儿纳入研究对象,1092例有急性期治疗资料,1052例(96.3%)接受IVIG治疗,135例对首次IVIG治疗无反应,发生率12.8%(135/1052)。Logistic回归分析发现血沉、GPT、WBC、发病至用IVIG的时间、血浆白蛋白及IVIG治疗剂量,是IVIG无反应性的独立危险因素(P<0.05)。对IVIG无反应者8例给第2剂2g/kg IVIG,5例热退;114例给1g/kg剂量IVIG治疗,35例(30.7%)热退;11例给400~600mg/kg IVIG,1例(9.1%)热退;2例给糖皮质激素,2例均热退。4种再治疗方法间比较,差异有统计学意义(P=0.015)。第2剂2g/kg IVIG治疗较其它再治疗所需进一步IVIG或激素治疗次数少,体温恢复快。 结论约12.8%KD患儿对初次IVIG治疗无反应。血沉、WBC和GPT、血浆白蛋白、IVIG使用方法及起病至用IVIG的时间,是IVIG无反应的独立危险因素。对初次IVIG无反应患儿推荐使用第2剂2g/kgIVIG,对2次2g/kg IVIG治疗仍无效者可以选用糖皮质激素治疗。     

Incidence of Kawasaki disease in Japan: the nationwide surveys of 1999–2002

BACKGROUND: The purpose of the present study was to describe the results of nationwide epidemiologic surveys of Kawasaki disease for the 4 year period 1999-2002. METHODS: The design is a retrospective incidence survey. The patients reported in these two surveys are all new patients who were reported during the two study periods (1999-2000 and 2001-2002), although the data were collected retrospectively. A questionnaire was sent to all pediatric departments of hospitals with 100 beds or more throughout Japan, requesting data on patients with Kawasaki disease, such as sex, age, date of first hospital visit, recurrence, and cardiac lesions. RESULTS: The total number of patients reported in the 4 year period 1999-2002 was 32 266 (18 604 male, 13 662 female), with an average annual incidence of 137.7 per 100 000 children younger than 5 years old. The male/female ratio was 1.30. The incidence peaked at 9-11 months of age, and the proportion of patients under 1 year of age was 26%. The monthly distribution had a high peak in January and a gradual increase in summer. Geographically, the high-incidence areas were limited to certain prefectures and moved from year to year. The cardiac lesions at acute stage and cardiac sequelae occurred more in children under 1 year and older than 4 years. Among the principal symptoms, fever persisting >or=5 days occurred most commonly, followed by conjunctival congestion, changes in lips and oral cavity, polymorphous exanthema, and changes of extremities. Cervical lymphadenopathy occurred less. CONCLUSION: More than 32 000 patients with Kawasaki disease during the 4 year period 1999-2002 were reported to the nationwide incidence surveys. The number of patients is steadily increasing despite the decrease of children. The seasonal variation, geographical distribution, and age-specific distribution support the infection theory for the etiology of Kawasaki disease.     

Alteration of biochemical profiles after high-dose intravenous immunoglobulin administration in Kawasaki disease

Intravenous immunoglobulin (IVIG) has been used as an immunomodulatory treatment for several immune-mediated diseases. Early effects of high-dose IVIG treatment on biochemical profiles including lipids and proteins were evaluated in patients with Kawasaki disease (KD). Twelve children with KD (9 boys) were treated with IVIG at 2 g/kg over a period of 12 h. Serial sera samples were collected from the patients four times: before IVIG treatment and 2 h, 24 h and 7 d after IVIG treatment. The samples were frozen at -20°C before biochemical analysis. A significant decrease in albumin concentration was found 2 h and 24 h after IVIG treatment, but this recovered to the pretreatment level after 7 d. Total cholesterol and triglyceride increased slightly after 7 d. A significant decrease in HDL-cholesterol and C-reactive protein was seen 2 h and 24 h after IVIG treatment.

Conclusion : High-dose IVIG affects immediate changes in protein profiles and HDL-cholesterol in KD. Changes in HDL-cholesterol induced by IVIG may be the result of changes in systemic protein metabolism.     

Coronary Involvement in Infants with Kawasaki Disease Treated with Intravenous γ-Globulin

Aim To analyze the clinical spectrum and the incidence of coronary involvement in infants with typical Kawasaki's disease (KD). Patients and Methods A retrospective study was performed on children one year of age or younger diagnosed from February 1992 to January 2006 with typical KD. Children with incomplete forms of the disease were not included. Results Twenty-five infants were diagnosed with KD during the study period. The median age of the patients was 10 months (range, 4–12 months). All children but one received intravenous gammaglobulin (IVIG), 84% before the 10th day of disease. Seven patients (28%) required the administration of more than one dose of IVIG, because persistence of fever. Coronary artery disease (CAD) was recorded in 6 cases (24%), five of them being boys. All patients with CAD were treated with ASA plus IVIG and 84% of them received this therapy within the first 10 days of the KD onset. Conclusions In spite of the exclusion of our study of incomplete presentations and of an early administration of IVIG in our patients, we have observed a high rate of infants who developed CAD, which is similar to the one reported in children who do not receive IVIG.