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.     

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.     

Rising incidence of type 1 diabetes mellitus in children and adolescents in Cyprus in 2000-2004

Introduction:  The incidence of type 1 diabetes mellitus (T1DM) has dramatically increased recently in some countries.
Aim:  To ascertain any changes in the incidence of T1DM in our population during the years 1990–2004.
Methodology:  All newly diagnosed cases of T1DM children under the age of 15 yr were registered and relevant information was obtained. Population demographic data based on the most recent census were used for calculations.
Results:  The overall mean annual incidence of T1DM during this 15-yr period was 11.9/100 000 person-years, with a statistically significant increase in the third 5-yr period (14.9/100 000 person-years).
The incidence during the first (1990–1994) and second (1995–1999) 5-yr periods was 10.5/100 000 person-years (p < 0.001). The overall male:female ratio was 0.94. Seasonal distribution for the first and second 5-yr periods revealed a higher incidence during winter and autumn months. Seasonal variation, however, disappears in the third 5-yr period, where no differences were found between the four seasons.
Conclusion:  The incidence of newly diagnosed T1DM cases has increased during 2000–2004. A seasonal variation during the first and second 5-yr periods was no longer observed in the third 5-yr period.     

Six principal symptoms and coronary artery sequelae in Kawasaki disease

Background:  The relationship between coronary artery sequelae due to Kawasaki disease and the six principal symptoms associated with the latter are still unknown.
Methods:  Among the 16 952 patients reported in the 17th nationwide survey on Kawasaki disease, 14 068 were found to have definite and typical cases with five or six principal symptoms. The proportions of coronary artery aneurysms including dilatation of the artery were compared between the patient group with six symptoms and that with five.
Results:  Percentages for those with all six symptoms (7223) were 4.7% for coronary dilatations (i.e. small aneurysms), 1.7% for coronary aneurysms (i.e. middle-sized aneurysms), and 0.42% for giant coronary aneurysms (i.e. large aneurysms) 1 month after onset, whereas for those with five symptoms (6845), the corresponding percentages were 4.1%, 1.4%, and 0.22%, respectively. For overall observation, the odds ratios were 1.44, 1.28, and 1.70 for the three types of aneurysm, respectively. For male patients, all the odds ratios were >1.0, and some were significant. In contrast, odds ratios <1.0 were observed among those with six symptoms in some subgroups among female patients despite the lack of significance.
Conclusions:  Because odds ratios were elevated – especially for male patients with Kawasaki disease – with all six principal symptoms, patients with the six symptoms are more problematic for coronary sequelae among the definite cases, although there was not a specific symptom for the sequelae.     

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.     

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.     

Shortage of pediatricians in Japan: A longitudinal analysis using physicians' survey data

Background:  Currently, there is a shortage of hospital pediatricians in Japan. In the present study, using data from the Survey of Physicians, Dentists, and Pharmacists in Japan, we analyzed the dynamics and distribution of pediatricians, using a time series approach.
Methods:  The total number of pediatricians, the ratios of hospital-working and female pediatricians, their mean age and geographic distribution in 1974, 1984, 1994, and 2004 were determined. The dynamics of pediatricians were analyzed by identifying the annual number of physicians participating in and withdrawing from pediatrics, and by following up withdrawal rates from pediatrics and movement rates from hospitals. The withdrawal rates of male and female pediatricians registered in 1992, 1994, and 1996 were also analyzed.
Results:  The number of pediatricians per 10 000 children increased from 1.9 to 7.4 between 1974 and 2004. The percentage of women among pediatricians was significantly higher than that of women among all physicians in 2004 ( P < 0.01). The numbers of physicians who withdrew from pediatrics increased from the periods 1985–1994 to 1995–2004. Younger pediatricians tended to leave pediatrics earlier than elder pediatricians. There were no differences in the withdrawal rates of pediatricians between men and women registered in 1992, 1994, and 1996.
Conclusions:  It is anticipated that the number of pediatricians in Japan will decrease in the near future unless practical strategies are implemented to improve the early withdrawal of younger pediatricians and the current working conditions of female pediatricians.     

Prevalence and risk factors of vitamin D deficiency rickets in Hokkaido, Japan

Background:  Resurgence of vitamin D deficiency rickets has been recognized worldwide. While many cases of this disease have been reported in Hokkaido, the northern island of Japan, no prevalence data is available. Here, we investigated the prevalence and risk factors of vitamin D deficiency rickets in Hokkaido.
Methods:  A specially designed questionnaire was sent to 84 major pediatric departments of hospitals in Hokkaido to collect information of the confirmed cases between July 1999 and June 2004.
Results:  Sixty-seven hospitals responded to the questionnaire. Of these, 20 hospitals reported 31 confirmed cases. All the patients were infants and toddlers, less than 4 years of age. The prevalence of cases in a recent year was estimated to be nine in 100 000 children under four years of age. Most of the 31 cases in our study were breast-fed. Eleven cases showed signs of malnutrition due to unbalanced diet or dietary restriction. Furthermore, the prevalence of cases was higher in the northeastern region than in the southwestern region. The number of cases increased gradually from the end of winter to spring.
Conclusions:  This is the first report ascertaining the prevalence of vitamin D deficiency rickets in Hokkaido, Japan. Limited exposure to sunlight and inadequate diet in early childhood are key risk factors of this disease. Thus, it is crucial to introduce active recommendations for vitamin D supplementation based on age, residential area, and to advocate public awareness for preventing this disease.     

Assessment of the burden of paediatric empyema in Australia

Aims:   To investigate the change in incidence of childhood empyema and pneumonia in Australia, and ascertain the management trends in all hospitals caring for children with empyema.
Methods:   The incidences of empyema and pneumonia were calculated for each year between 1993/1994 and 2004/2005 using retrospective primary diagnostic coding from ICD-9 and 10 comprising the Australian National Hospital Morbidity Database for five age groups in patients less than 20 years of age. Hospitals with allocated paediatric beds were surveyed on referral pattern and treatment preferences.
Results:   In this study, 145 562 patients with pneumonia were admitted with a mean (range) incidence of 2306 (1846–2652) per million. The trend towards an overall increase was not statistically significant. Only the 1–4 years old age group demonstrated a significant increase ( P < 0.01, r ²= 0.61). A total of 469 cases of empyema were identified with a mean incidence of 7.35 (4–10.2) per million. There was an overall increase in incidence ( P < 0.05, r ²= 0.51) reflecting an increase in the 1- to 4-year-olds ( P < 0.005, r ²= 0.60) and 15- to 19-year-olds ( P < 0.05, r ²= 0.37). The overall percentage of empyema as a proportion of pneumonia increased from 0.27 to 0.70% (0.48% (0.27–0.70%), P < 0.05, r ²= 0.38). The survey response rate was 75%. Ninety-nine of 121 (82%) hospitals referred children with empyema to a more appropriate centre with wide variations in treatments provided.
Conclusions:   The rise in incidence of empyema reflects that seen in other countries. Furthermore, there are diverse management practices suggesting a clear need for national guidelines.     

Hospital facilities available to patients with Kawasaki disease: Results of a national survey of Kawasaki disease in Japan

Since 1970, thirteen nationwide epidemiologic surveys of Kawasaki disease have been conducted, and questions on the current situation of hospitals and their facilities have been included in the survey form to assess the care available to patients with Kawasaki disease. To clarify the characteristics of medical care for Kawasaki disease, this paper summarizes the present condition of hospital facilities available to patients with Kawasaki disease. A questionnaire form including questions about the situation of hospital facilities was sent to all pediatric departments of hospitals with 100 or more beds throughout Japan. The proportion of hospitals in which successive observations with echocardiography (UCG) is available (UCG available hospital) was significantly increased compared with the ninth survey (1985–86), whereas that of the hospitals in which coronary angiography (CAG) is available (CAG available hospital) was significantly decreased. Almost all (96.2%) of the patients were reported from the UCG available hospitals. The proportion of patients reported from hospitals with a set policy for intravenous gamma globulin (IVGG) therapy was 74.5%. The proportion of UCG available and CAG available hospitals and that of hospitals with a set policy for IVGG therapy correlated with the increase in the number of patients. In conclusion, hospital facilities available to patients with Kawasaki disease in Japan have satisfactory capability for diagnosis and management of Kawasaki disease.     

Mortality among patients with a history of Kawasaki disease: The third look

Abstract Background: Long-term prognosis of Kawasaki disease is still unclear.
Methods: In a cohort study, 6576 patients with Kawasaki disease were observed from their first medical encounter because of the disease through the end of 1994, or until death. Standardized mortality ratios (SMR) with 95% confidence intervals (CI) were calculated with vital statistics data of Japan used for the control. Results: Of 6576 patients who met the eligibility criteria, 6550 (99.6%) were followed through either the end of the study or the date of death. Twenty patients (14 male, 6 female subjects) died during the study period; an overall SMR of 1.35 (95% CI 0.82–2.08) was calculated. The SMR was 1.45 (95% CI 0.79–2.44) for male subjects and 1.15 (95% CI 0.42–2.52) for female subjects. During the acute phase of the disease (the first 2 months after the first visit to hospital), the SMR was higher, particularly in male subjects (SMR 10.13, 95% CI 3.72–22.08). After the acute phase, however, both boys and girls had low SMR. Nine of the 20 deaths were caused by Kawasaki disease; there were three deaths as a result of congenital heart diseases and two subjects died of malignant neoplasms of lymphatic or hematopoietic tissues.
Conclusions: Although the mortality rate among those with a history of Kawasaki disease was elevated in Japan, many of the deaths that caused the elevation occurred during the acute phase of the disease. The mortality rate was not increased after the acute phase of the disease.     

Very high and increasing incidence of type 1 diabetes mellitus in Newfoundland and Labrador, Canada

Objective:  To determine the incidence of type 1 diabetes mellitus (T1DM) among children aged 0–14 yr inclusive in the Canadian province of Newfoundland and Labrador (NL).
Methods:  Prospective and retrospective cohort study of the incidence of T1DM in children aged 0–14 yr from 1987 to 2005. Identified cases during this time period were ascertained from several sources and verified using the capture–recapture technique.
Results:  Over the study period, 732 children aged 0–14 yr were diagnosed with T1DM. The incidence of T1DM in this population over the period 1987–2005 inclusive was 35.08 per 100 000 (95% confidence interval: 32.54, 37.62). The incidence over this period increased linearly at the rate of 0.78 per 100 000 per year. There was a significant difference between the incidence of 31.61 per 100 000 for boys in the 0–4-yr age-group and 19.05 per 100 000 for girls in the 0–4-yr age-group (p = 0.001). The incidence was very high throughout the entire province.
Conclusions/Interpretation:  The province of NL has one of the highest incidences of T1DM reported worldwide. The incidence is increasing over the 19-yr study period.     

Kawasaki disease in Korea, 2003-2005

PURPOSE: The aim of this study was to investigate the incidence rate and epidemiologic patterns of Kawasaki disease in Korea for the 3-year-period, 2003-2005. METHODS: The questionnaire for an epidemiologic survey on Kawasaki disease was distributed to all 102 Korean hospitals that conduct pediatric residency programs, and obtained data were analyzed upon receipt. RESULTS: The 9662 patients of Kawasaki disease from 85 hospitals that responded (response rate, 83.3%) consisted of 5877 males and 3785 females (male:female ratio, 1.55:1). The incidence rate per 100,000 children <5-year-old was 104.2 in 2003, 106.4 in 2004, and 104.6 in 2005 (average rate, 105.0). Their mean age of onset was 33.3 months, and the proportions of sibling cases and recurrent cases were 0.29% and 2.0%, respectively. Coronary arterial abnormalities were detected at follow-up by echocardiogram in 18.8% of all such cases including dilatations of 18.0% and aneurysms of 2.5%. CONCLUSION: The average annual rate of incidence, 105.0/100,000 in children <5-year-old is the second highest reported rate in the world.     

Medical aid program for chronic pediatric diseases of specified categories in Japan: Current status and future prospects

Background: In 1974 the Medical Aid Program for Chronic Pediatric Diseases of Specified Categories (MAPChD) was established in Japan, and the national registration of MAPChD beneficiaries was initiated in 1998. In 2005, MAPChD became legal. The purpose of the present study was to analyze the newest registration data and discuss the future prospects of MAPChD. The differences in MAPChD content before and after legislation were compared.
Methods: The subjects were MAPChD-registered patients whose guardians had signed a consent document for the children to join a MAPChD research program during the fiscal years 2002–2003. The nationwide electronic registration data were gathered and analyzed, and the official documents about MAPChD were reviewed and summarized.
Results: Given that the overall population was aged 0–19 years, there were four MAPChD beneficiaries for every 1000 children in Japan. Moreover, the prolonged survival of MAPChD patients was accompanied by an increasing prevalence of complications. The percentages of MAPChD beneficiaries who did not sign a consent document for research were 3.1% and 6.3% in 2002 and 2003, respectively. Following the legislation, both inpatients and outpatients younger than 20 years with any of 514 chronic diseases became eligible, and MAPChD began providing welfare services to these patients.
Conclusions: The establishment of a MAPChD database is necessary for further research. It is expected that, in future, more beneficiaries of MAPChD will join the research program. One of the major challenges faced by long-term survivors of childhood MAPChD diseases is making the transition from a specialized pediatric care facility to an adult health care system.     

Epidemiologic picture of Kawasaki disease in Korea, 2000–2002

BACKGROUND: The aim of this study was to investigate the incidence rate and epidemiologic patterns of Kawasaki disease in Korea for a 3 year period during 2000 to 2002. METHODS: An epidemiologic survey on Kawasaki disease was retrospectively performed. The questionnaire was sent to all 112 hospitals having pediatric residency programs, and obtained data were analyzed. RESULTS: The 9150 cases of Kawasaki disease from 92 hospitals which responded (response rate, 82.1%) included 5515 males and 3635 females (male : female ratio, 1.52:1). The incidence rate per 100,000 children <5 years old was 73.7 in 2000, and increased to 90.8 in 2001, and 95.5 in 2002 (average rate, 86.4). The monthly number of patients was slightly higher in May, June and July. Their mean age of onset, the proportion of sibling cases, and a rate of recurrent cases were 30.5 months, 0.17%, and 2.9%, respectively. Coronary arterial abnormalities occurred in 18.6% of cases including dilatations of 17.3% and aneurysms of 3.1%. CONCLUSION: The average annual incidence, 86.4/100 000 in children <5 years old is the second highest rate in the world.     

Clinical survey of congenital cytomegalovirus infection in Japan

Abstract Background The clinical features of congenital cytomegalovirus (CMV) infection in countries with a higher percentage of maternal seropositivity for CMV has rarely been reported. We conducted a national survey for the first time in Japan to investigate the prevalence of congenital CMV infection.
Methods: Questionnaires were sent in 1994 to pediatricians and obstetricians of 3398 hospitals with either more than 100 beds or a neonatal intensive care unit (NICU). The questionnaire asked for the number of new cases in 1992 and 1993, maternal status of CMV infection, diagnostic methods, clinical manifestations at birth, sequelae and prognosis.
Results. A total of 46 cases of CMV infection were reported for the years 1992 and 1993 by 1448 hospitals; of these 39 were symptomatic. The annual incidence of symptomatic disease was 1.6 cases/100 000 live births. Major clinical manifestations such as low birthweight, hepatosplenomegaly, petechiae and intracranial calcification were noted at birth in38–50% of symptomatic neonates. Sequelae, such as hearing loss, mental retardation and motor disability developed in 71% of survivors. Thirty-five percent of the 49 infected infants had either died or had severe disability. Several clinical manifestations at birth, including petechiae/thrombocytopenia, were significantly associated with severe sequelae or a poor prognosis.
Conclusion: The lower frequency of clinical findings at birth may be attributed to the higher seroprevalence of pregnant women in Japan than in Europe and the United States.     

Case–control study of giant coronary aneurysms due to Kawasaki disease: The 19th nationwide survey

Background: The risk factors for recently reported cases of giant coronary aneurysms due to Kawasaki disease have not been elaborated. Methods: Fifty‐three patients with giant coronary aneurysms, diagnosed as Kawasaki disease in 2005 and 2006, were selected from the 19th nationwide survey of the disease in Japan. With all the other patients recorded at the same hospitals as a control group, OR and their 95%CI were calculated to delineate the risk factors. Results: In multivariate analyses, patients aged younger than 1 year (OR compared with 1–2‐year‐olds = 6.57) and those older than 5 years (OR compared with 1–2‐year‐olds = 4.24), those who received additional intravenous immunoglobulin (IVIG) without the use of steroid (OR = 8.38) and those who received steroid administration with or without the additional use of IVIG (OR = 220.51 and 83.83, respectively), showed significantly higher OR for giant coronary aneurysms. As for IVIG therapy, the additional use of IVIG (OR = 14.84), total dosage of IVIG exceeding 2500 mg/kg (OR compared with 1500–2499 mg/kg = 12.26) and the duration of IVIG administration for more than 3 days (OR = 30.12), were found to significantly increase the risk of developing giant aneurysms in univariate analyses that were adjusted for sex and age. Conclusions: The observation of 53 patients with giant coronary aneurysms due to Kawasaki disease among those included in the nationwide survey presented some risk factors, together with considerations about the associated aneurysms.     

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.     

上海地区1998－2002年川崎病流行病学特征

目的 对上海地区川崎病发病情况进行调查，了解中国发达地区川崎病的流行病学特征。方法 参照日本流行病学调查方案，制作统一调查表和诊断指南，调查对象为1998年1月1日－2002年12月31日上海地区50家有儿科临床服务的二级及二级以上医院收治的所有川崎病病例。结果 调查表回收率100％，将18例不符合表格填写要求者剔除后，对768例进行分析。上海地区5岁以下儿童川崎病发病率呈逐年增高趋势， 1998年为16.79/10万，1999年为25.65/10万，2000年为28.16/10万，2001年为28.05/10万，2002年为36.76/10万。男女性别比为1.83:1；发病年龄为1个月～18.8岁（中位数1.8岁），发病年龄高峰为9.6个月；全年均可发病，但以春夏之交较多见。主要症状表现为发热持续5 d以上者最为常见（99.3％），其后依次为口唇、口腔黏膜损害（83.5％）、指（趾）端脱皮（82.9％）、皮疹（81.0％）、结膜充血（78.4％）、颈部淋巴结肿大（69.3％）、肢端充血硬肿（48.1％），45.2％的病例有肛周脱皮。心血管损害发生率24.3％，以冠状动脉扩张最为常见，占68％，其次为冠状动脉瘤，占10%。急性期病死率为0.26%，死因为冠状动脉瘤破裂和急性心力衰竭。再发率为1.82%。 结论 上海地区川崎病发病率明显低于日本，但高于西方国家。发病率呈增高趋势，性别分布和心血管损害与其他报道相似。发病季节分布与北京地区的报道相似，但与其他报道不同。