Kawasaki Syndrome and Factors Associated With Coronary Artery Abnormalities in California

BACKGROUND:: Kawasaki syndrome (KS) occurs in children <18 years of age and is the leading cause of acquired heart disease among children in the United States. Understanding the epidemiology of KS and factors associated with coronary artery abnormalities (CAA) may lead to timely diagnosis and treatment of KS and could limit CAA. METHODS:: Epidemiologic characteristics, including risk factors for the development of CAA, among KS and incomplete KS patients <18 years of age with onset during 2000-2009 reported by the California Department of Public Health to the Centers for Disease Control and Prevention's national KS surveillance system were analyzed. RESULTS:: A total of 2056 KS and incomplete KS patients <18 years of age were reported during 2000-2009. The median age of patients was 2 years; 60% of patients were male. Of 1818 patients with race information reported, 56% were white and 28% were Asian/Pacific Islander. Ninety-eight percent of patients received intravenous immunoglobulin. Of 1843 patients with information on cardiac complications, 89 (5%) had coronary artery aneurysms and 341 (19%) had CAA. Characteristics associated with the occurrence of CAA in KS patients were male sex, Asian/Pacific Islander race, age <1 year or 9-17 years, and not receiving intravenous immunoglobulin treatment before the fifth day of illness. CONCLUSIONS:: This study suggests that intravenous immunoglobulin treatment before the fifth day of illness may reduce CAA among KS patients. Timely diagnosis and treatment of KS continue to be important in reducing the occurrence of cardiac complications.     

An evaluation of hospitalizations for Kawasaki syndrome in Georgia

OBJECTIVE: To evaluate and describe the epidemiologic characteristics of Kawasaki syndrome (KS) hospitalizations in Georgia. DESIGN: We reviewed hospital discharge data and corresponding medical records for Georgian patients discharged with a KS diagnosis during 1997 and 1998. RESULTS: During the study period, 233 KS hospital discharges were recorded in Georgia; 177 (76%) were for children younger than 5 years. Twenty-one (9%) of 233 of the hospital discharges represented multiple hospitalizations. Medical records for 211 KS discharges (91%), representing 197 patients (93%), were reviewed. For those 189 patients whose medical records were reviewed and had sufficient information, 139 (74%) either had a documented illness that met the Centers for Disease Control and Prevention (CDC) definition for KS (n = 135) or had coronary artery abnormalities without meeting the CDC definition for KS (atypical KS; n = 4). Eight patients had only a history of KS. Excluding multiple hospitalizations and patients with only a history of KS, 158 hospitalizations were for patients younger than 5 years (14.0 per 100 000 children); 110 of these patients met the KS or atypical KS definition (9.8 per 100 000 children). CONCLUSIONS: Hospital discharge data are useful for KS surveillance. However, analysis of hospital discharge data may slightly overestimate the KS hospitalization rates because some discharges may represent multiple hospitalizations or hospitalizations of patients with only a history of KS. The incidence and epidemiology of KS in Georgia are consistent with findings from other continental US studies. Physicians should exercise their best clinical judgment in identifying and treating patients with KS who may not meet standard case definitions.     

The incidence of Kawasaki syndrome in West Coast health maintenance organizations

BACKGROUND: Kawasaki syndrome (KS) causes an acute vasculitis of unknown etiology. It is a leading cause of acquired heart disease of children in Japan and the United States. METHODS: We examined the incidence of KS in a well-defined population group of children < or =6 years of age, using data collected through the Vaccine Safety Datalink (VSD) project. The VSD database contains information on >1 million children enrolled in four West Coast health maintenance organizations (HMOs). RESULTS: During 1993 through 1996 a total of 234 physician-diagnosed KS patients were reported in the 4 HMOs; 152 (65.0%) were boys and 195 (83.3%) were <5 years of age. The incidence of KS among children <5 years of age in the HMOs ranged from 9.0 to 19.1 per 100,000 person years. KS incidence was higher among boys in 3 of the sites. In the 2 sites with the highest number of KS patients, a seasonal occurrence of KS in winter and early spring was observed. Overall 226 (96.6%) of the KS patients were reported to have been hospitalized; hospitalization rates for children <5 years of age ranged from 9.0 to 16.8 per 100,000 person years. CONCLUSIONS: The incidence of KS in the HMOs was similar to that reported in other population-based studies in the United States and higher than estimates for Australia and several European countries.     

283例川崎病的临床分析

目的 总结川崎病住院患儿的临床表现及心血管系统的并发症,为尽早、正确判断川崎病的病情与预后提供临床依据。方法 回顾性研究1992—2002年住院川崎病患儿283例,分析:(1)川崎病各种临床表现的发生率;(2)合并冠状动脉瘤发生的危险因素。结果(1)283例中男186例,女97例;男女比例为1.9:1,年龄分布以婴幼儿为主,3岁以内(包括3岁)占71％;发病季节以春、夏季为主;不典型川崎病55例(19.4％);就诊前平均发热天数6.1 d,除发热外,其他症状出现频率的次序是:口腔黏膜改变(97.5％),淋巴结肿大(95.4％),球结膜充血(91.2％),指、趾端脱皮(89.8％),皮疹(81.5％)。(2)心血管改变情况:治疗前合并冠状动脉(简称冠脉)扩张共103例(36.9％),住院2周后新发生冠脉扩张28例(13.3％);有13例形成动脉瘤(4.7％),在冠状动脉病变人群中占9.9％。分析动脉瘤的形成是与性别、发热持续时间(>8.9 d)有关(P<0.05)。治疗前合并心房、室腔扩大40例(14.3％);心包积液11例(3.9％);合并心电图改变有57例(20.8％)。结论 川崎病心血管并发症出现较早,经治疗后有13.3％新发冠状动脉病变。动脉瘤的形成与性别、发热持续时间有关。     

Kawasaki disease in Thai infants compared with older children

Infants with Kawasaki disease (KD) are at increased risk of having coronary artery abnormalities (CAA). The purpose of this study was to evaluate the clinical features of KD in infants and compare these with findings in older children to determine the risk factors for CAA in infants. All children with KD admitted to a tertiary care hospital between January 1993 and April 2003 were studied retrospectively. Of a total of 51 patients included in the study, 22 (43%) were <1 year of age (mean 8 months, range 2-12 months). All had classical clinical manifestations such as fever, skin rash and mucositis; extremity change occurred in 95%, conjunctivitis in 81% and cervical lymphadenopathy in 27%. Infants had significantly more non-classical symptoms, e.g. diarrhoea (68%), than older children (38%) (p=0.04). The mean number of days before intravenous immunoglobulin (IVIG) treatment was given to infants was 2 days later than in older children. The predictors of CAA in infants were resistance to IVIG treatment (p=0.02) and long duration of fever (p=0.009). Compared with older children, the less typical presentations and delay in diagnosis and treatment in infants might be important factors in CAA in KD.     

Kawasaki syndrome

Kawasaki syndrome is a fascinating worldwide illness of young children. This acute self-limited vasculitis has become the most common cause of acquired heart disease in children in the United States and Japan. KS causes significant coronary artery disease that may lead to myocardial infarction and sudden death. Clinical and epidemiologic features of KS support an infectious cause, but the etiology remains unknown. Clearly, additional research on the cause of KS and its pathogenesis is needed urgently to allow for improved diagnosis; more specific therapy; and, ultimately, prevention of the disorder.     

川崎病并发冠状动脉病变的超声心动图表现的动态观察

目的 探讨川崎病(KD)所引起的冠状动脉扩张(CAD)以及冠状动脉瘤(CAA)的超声心动图特点.方法 用彩色多普勒超声心动图检测KD患儿冠状动脉开口和内径,并动态观察静脉免疫球蛋白治疗前后冠状动脉内径的变化情况.结果 本组46例KD患儿,41例患儿行超声心动图检查,合并CAD 12例,左冠状动脉(LCA)较右冠状动脉(RCA)更易受累及(P<0.05);CAA 4例,均为双侧冠状动脉病变,最大内径10 mm.治疗后6～18 d复查超声心动图,CAD组LCA较治疗前明显回缩(P<0.05),RCA内径无变化(P>0.05);CAA组LCA和RCA内径均无明显变化(P>0.05).对10例冠状动脉病变患儿进行随访,其中8例CAD冠状动脉内径均恢复正常,1例CAA冠状动脉病变加重,1例CAA完全恢复正常.结论 (1)在KD所引起的CAD中,LCA比RCA更易受累及,静脉免疫球蛋白治疗后短期内LCA明显回缩.(2)超声心动图是动态观察KD并发CAD和CAA的无创手段.     

Kawasaki syndrome hospitalizations among children in Hawaii and Connecticut

OBJECTIVES: To estimate the incidence and describe recent trends of Kawasaki syndrome (KS) in 2 different areas of the United States. METHODS: Retrospective analysis of Hawaii and Connecticut State KS hospital discharge records for children younger than 5 years. RESULTS: In Hawaii, 175 KS hospitalizations for children younger than 5 years were reported during 1994 through 1997; the annual hospitalization rate per 100,000 children was 47.7. The rate for Hawaiian children younger than 1 year (83.2) was greater than that for 1- to 4-year-old children (39.0), and most hospitalizations occurred prior to age 2 years (median age, 17 months). In Connecticut, 171 KS hospitalizations for children younger than 5 years were reported during 1993 through 1996; the annual hospitalization rate per 100,000 children was 18.8, and the median age at hospitalization was 28 months. For both states, most hospitalizations were for boys. Although no clear seasonality was apparent, monthly peaks occurred in some of the years from December through March. CONCLUSIONS: Kawasaki syndrome seems to remain an endemic disease in the United States. A high KS annual hospitalization rate was seen in Hawaii, especially in children younger than 1 year, whereas in Connecticut, the KS rate was more consistent with those previously reported in the continental United States. Arch Pediatr Adolesc Med. 2000;154:804-808     

Do Kawasaki disease patients without coronary artery abnormalities need a long-term follow-up? A myocardial single-photon emission computed tomography pilot study

Objective:   To determine the frequency and risk factors for long-term myocardial perfusion scintigraphy abnormalities in patients with Kawasaki disease (KD).
Methods:   A cohort of patients with KD at least 3 years after disease onset and with persistent coronary artery aneurysms (CAA) (group 1) or without CAA (group 2) underwent stress–rest myocardial single-photon emission computed tomography (SPECT). Clinical and laboratory parameters at disease onset were considered to assess their predictive value for the development of myocardial perfusion abnormalities.
Results:   Forty patients, 20 in group 1 and 20 in group 2, entered the study. The two groups turned out to be comparable for demographic, clinical and laboratory characteristics. Five patients (12.5%), two in group 1 and three in group 2, had abnormal myocardial perfusion assessed by SPECT. Neither the presence of CAA nor the overall cardiac involvement at the disease onset significantly increased the risk for these abnormalities.
Conclusion:   Cardiac SPECT abnormalities are not unusual in KD and can be found in patients with or without CAA. If confirmed in a larger cohort of patients, these preliminary data indicate that careful long-term cardiac follow-up should be considered, regardless of the presence of CAA.     

Kawasaki syndrome in Hawaii

OBJECTIVE: To describe the incidence and epidemiology of Kawasaki syndrome (KS) in Hawaii. METHODS: Retrospective analysis of the State Inpatient Database for Hawaii residents hospitalized with KS during 1996 through 2001. RESULTS: During 1996 through 2001, 267 persons younger than 18 years of age living in Hawaii were hospitalized with KS; 226 (84.6%) were younger than 5 years of age. The average annual incidence for KS was 45.2 per 100,000 children younger than 5 years of age. The incidence was higher for children younger than 1 year of age than for those 1-4 years of age (74.3 and 37.5 per 100,000). The KS incidence for Asian and Pacific Islander children and for White children was 70.9 and 35.3 per 100,000, respectively. Incidence was highest among Japanese American children living in Hawaii (197.7 per 100,000). Honolulu County had the most KS patients (85.0%) and the highest incidence (53.1 per 100,000) among Hawaii counties. For children younger than 5 years of age hospitalized with KS, the median length of stay was 2 days, and the median hospital charge was $9379. CONCLUSION: During 1996 through 2001, the annual incidence rate for KS among children younger than 5 years of age in Hawaii was the highest in the United States. The incidence among Japanese American children in Hawaii was higher than that among other racial groups in the state and when compared with children living in Japan.     

Kawasaki syndrome hospitalizations in Ireland, 1996 through 2000

OBJECTIVE: To describe the epidemiologic characteristics and estimate the incidence of Kawasaki syndrome (KS) among children in Ireland. METHODS: Hospital discharge records with a KS diagnosis among patients <18 years of age were examined using Ireland's Hospital In-Patient Enquiry database for 1996 through 2000. RESULTS: During the study period 265 hospitalizations associated with KS among children <18 years of age were recorded in Ireland. Of those, 194 (73%) occurred among children <5 years of age. The median age of patients at admission was 2 years. The average annual KS hospitalization rate for children <5 years of age was 15.2 per 100 000 children, and among that group the hospitalization rate was higher for infants <1 year of age than for children 1 to 4 years of age (19.7 and 16.0 per 100 000 children, respectively). Most KS hospitalizations occurred among children <5 years of age and among boys. The highest monthly number of hospitalizations occurred during the months of November through January. No deaths associated with KS were reported among hospitalized children. CONCLUSION: Hospital discharge data provide useful information on the epidemiology of KS in Ireland. The hospitalization rate for KS in Ireland is similar to rates in the United States and may be higher than those in other European countries, although the European studies differ in methodologies and time periods.     

Prevalence of a metabolic syndrome phenotype in adolescents: findings from the third National Health and Nutrition Examination Survey, 1988-1994

BACKGROUND: In adults the metabolic syndrome imposes a substantial risk for type 2 diabetes mellitus and premature coronary heart disease. Even so, no national estimate is currently available of the prevalence of this syndrome in adolescents. OBJECTIVE: To estimate the prevalence and distribution of a metabolic syndrome among adolescents in the United States. DESIGN AND SETTING: Analyses of cross-sectional data obtained from the Third National Health and Nutrition Examination Survey (1988-1994), which was administered to a representative sample of the noninstitutionalized civilian population of the United States. PARTICIPANTS: Male and female respondents aged 12 to 19 years (n = 2430). MAIN OUTCOME MEASURES: The prevalence and distribution of a metabolic syndrome among US adolescents, using the National Cholesterol Education Program (Adult Treatment Panel III) definition modified for age. RESULTS: The overall prevalence of the metabolic syndrome among adolescents aged 12 to 19 years was 4.2%; 6.1% of males and 2.1% of females were affected (P=.01). The syndrome was present in 28.7% of overweight adolescents (body mass index [BMI], >/=95th percentile) compared with 6.8% of at-risk adolescents (BMI, 85th to <95th percentile) and 0.1% of those with a BMI below the 85th percentile (P<.001). Based on population-weighted estimates, approximately 910 000 US adolescents have the metabolic syndrome. CONCLUSIONS: Perhaps 4% of adolescents and nearly 30% of overweight adolescents in the United States meet these criteria for a metabolic syndrome, a constellation of metabolic derangements associated with obesity. These findings may have significant implications for both public health and clinical interventions directed at this high-risk group of mostly overweight young people.     

Lack of effect of gamma-globulin infusion on circulating immune complexes in patients with Kawasaki syndrome

Previous studies have demonstrated circulating immune complexes (CICs) in sera of 70% of Kawasaki syndrome (KS) patients but the pathogenic role of these complexes in the illness is unknown. Infusions of gamma-globulin (GG) have been shown recently to reduce the prevalence of coronary artery aneurysms in KS patients. One of the proposed mechanisms of action of GG therapy is through an effect on CICs. We analyzed paired sera for CICs from 29 patients with KS, 19 of whom were treated with aspirin and GG and 10 of whom received aspirin only. Assay systems used for CIC detection were Raji-mu enzyme immunoassay, C1q solid phase assay-enzyme immunoassay and F(ab')2-anti-C3-mu enzyme immunoassay. Overall 20 of 29 patients had CICs detected by at least two of three assays used. Of the 19 GG-treated patients 14 had CICs detected: 5 in both paired sera; 2 in pretherapy sera; and 7 posttherapy specimens. There was no correlation between duration of fever, presence of myocarditis or the development of coronary artery aneurysms and the presence of CICs in the serum of KS patients. Although CICs can be found frequently in the sera of KS patients there seems to be no association between the presence of CICs and the duration of fever or the development of coronary artery aneurysms.     

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目的分析川崎病(KD)并发冠状动脉瘤(CAA)的高危因素及预后。方法回顾性分析重庆医科大学附属儿童医院1993年1月至2009年12月3902例住院KD患儿的临床资料,对与冠状动脉损害发生有关的因素进行计数资料χ2检验、计量资料t检验,将单因素分析筛选出的变量行多因素Logistic回归分析;对随访的46例CAA患儿病后1、3、6个月和1、2、3、4、5年及5年以上CAA的恢复情况及不同剂量静脉注射丙种免疫球蛋白(IVIG)的远期疗效进行χ2检验。结果 (1)单因素分析结果:性别、年龄、热程、IVIG使用时机、血红蛋白(Hb)、红细胞沉降率(ESR)、血清白蛋白与KD并发CAA有关(P<0.05),将单因素分析筛选出的上述因素作为自变量进一步行多因素分析,结果显示,Hb、ESR、IVIG使用时机与KD并发CAA呈独立相关性(P<0.05),而性别、年龄、热程、血清白蛋白与KD并发CAA非独立相关(P>0.05);(2)小型CAA、中型CAA、巨大CAA的回缩时间逐渐延长(P<0.05),年龄及IVIG的剂量与CAA的回缩时间无统计学相关性(P>0.05)。结论 Hb<100 g/L、ESR>50 mm/h、I...     

Prevalence of coronary artery abnormality in incomplete Kawasaki disease

BACKGROUND: The aim of the present study was to determine the prevalence of coronary artery abnormality (CAA) and other clinical features in patients with incomplete Kawasaki disease (iKD) using the data from the 17th Japanese nationwide survey of KD. METHODS: iKD was defined as the presence of four or fewer of the principal symptoms of the Japanese diagnostic guidelines, regardless of whether the patient had CAA. A total of 15,857 cases were analyzed. RESULTS: Among 15,857 cases, 83.9% of patients had five to six principal symptoms (complete KD: cKD), and 16.1% had iKD. The prevalence of CAA in cKD was 14.2%, and 18.4% in iKD. The prevalence of CAA in patients with four principal symptoms was 18.1%, which was higher than in cKD cases (14.2%). Although the reliability of the data has some limitations, the prevalence of CAA among patients with one to three symptoms was 19.3%. Among all CAA patients, 14% had four symptoms, and 6% had only one to three symptoms. CONCLUSION: Incomplete KD should not be equated with mild KD. Patients with four principal symptoms were comparable to cKD with respect to CAA occurrence. In patients with one to three symptoms also, especially in those under 1 year and older than 4 years of age, other significant symptoms and laboratory findings of the guidelines are very important in making a correct and early diagnosis of iKD so as to prevent CAA.     

Lack of association between Kawasaki syndrome and Chlamydia pneumoniae infection: an investigation of a Kawasaki syndrome cluster in San Diego County

BACKGROUND: The etiology of Kawasaki syndrome (KS), the leading cause of acquired coronary artery disease in children, is unknown. Recent studies have suggested that Chlamydia pneumoniae, a common respiratory pathogen associated with an increased risk of heart disease, might lead to KS. OBJECTIVE: To assess whether KS was associated with an elevated risk of having a current or antecedent infection with C. pneumoniae. METHODS: Blood, urine and pharyngeal specimens from KS patients in San Diego County, CA, during a period of high KS incidence were analyzed for evidence of recent C. pneumoniae infection by culture, PCR and serology. Specimens collected from two control groups, family members of KS patients and age-matched children attending outpatient clinics for well child visits, were similarly analyzed. RESULTS: Thirteen cases were identified. Forty-five outpatient controls and an average of three family members per patient were enrolled in the study. All specimens tested negative for the presence of C. pneumoniae by PCR and culture except for one blood specimen from the mother of a case-patient. Serologic analysis of patients and a subset of outpatient and family controls revealed no evidence of current C. pneumoniae infection; 4 of 13 adult family controls had IgG titers consistent with past exposure to C. pneumoniae. Case patients were no more likely than outpatient controls to have had a respiratory illness in the preceding 2 months (11 of 13 patients vs. 35 of 45 controls; odds ratio, 1.57; 95% confidence interval, 0.3 to 11.9). CONCLUSIONS: We found no evidence that C. pneumoniae infection was associated with KS.     

Time to focus on outcome assessment tools for childhood vasculitis

More than 40 years have passed since Kawasaki syndrome (KS) was first described. Yet KS still remains an enigmatic illness which damages the coronary arteries in a quarter of untreated patients and is the most common cause of childhood-acquired heart disease in developed countries. Many gaps exist in our knowledge of the etiology and pathogenesis of KS, making improvements in therapy difficult. In addition, many KS features and issues still demand further efforts to achieve a much better understanding of the disease. Some of these problem areas include coronary artery injuries in children not fulfilling the classic diagnostic criteria, genetic predisposition to KS, unpredictable ineffectiveness of current therapy in some cases, vascular dysfunction in patients not showing echocardiographic evidence of coronary artery abnormalities in the acute phase of KS, and risk of potential premature atherosclerosis. Also, the lack of specific laboratory tests for early identification of the atypical and incomplete cases, especially in infants, is one of the main obstacles to beginning treatment early and thereby decreasing the incidence of cardiovascular involvement. Transthoracic echocardiography remains the gold-standard for evaluation of coronary arteries in the acute phase and follow-up. In KS patients with severe vascular complications, more costly and potentially invasive investigations such as coronary CT angiography and MRI may be necessary. As children with KS with or without heart involvement become adolescents and adults, the recognition and treatment of the potential long term sequelae become crucial, requiring that rheumatologists, infectious disease specialists, and cardiologists cooperate to develop specific guidelines for a proper evaluation and management of these patients. More education is needed for physicians and other professionals about how to recognize the long-term impact of systemic problems related to KS.     

Visualization of coronary arteries in patients after childhood Kawasaki syndrome: value of multidetector CT and MR imaging in comparison to conventional coronary catheterization

Background After childhood Kawasaki syndrome (KS) the coronary arteries undergo a lifelong dynamic pathological change, and follow-up coronary artery imaging is essential. At present, conventional coronary catheterization (CCC) and angiography is still regarded as the gold standard. Less-invasive methods such as multidetector CT angiography (MDCT-A) and MRI have been used sporadically. Objective To compare the diagnostic quality of MDCT-A and MRI with that of CCC for coronary imaging in a group of patients with coronary artery pathology after childhood KS. Materials and methods A total of 16 patients (aged 5–27 years) underwent CCC and 16-row MDCT-A and 14 patients MRI (1.5 T). Results There was 100% agreement between MDCT-A and CCC in the detection of coronary aneurysms and stenoses. MDCT-A was superior for the visualization of calcified lesions. MRI and CCC showed 93% agreement for the detection of aneurysms. Visualization of coronary artery stenoses was difficult using MRI—one stenosis was missed. Conclusion MDCT-A has excellent correlation with CCC regarding all changes affecting the coronary arteries in the follow-up of childhood KS. In comparison to MDCT-A and CCC, MRI is less precise in the detection of stenotic lesions. Due to its high image quality and ease of performance MDCT-A should be the primary diagnostic modality in patients following childhood KS. Raoul Arnold and Sebastian Ley contributed equally to the work reported here.     

Children with sickle cell disease and human immunodeficiency virus-1 infection: use of inpatient care services in the United States

BACKGROUND: The purpose of this study was to describe hospital use patterns of children with sickle cell disease (SCD) and human immunodeficiency virus type-1 (HIV) infection in the United States. METHODS: Hospital discharges of children with 1 or both of the 2 conditions (SCD and HIV infection) were analyzed using nationally weighted data from the 1994 to 2003 Nationwide Inpatient Databases of the Healthcare Cost and Utilization Project. Demographic and hospital characteristics, length of stay, charges and the most frequent diagnoses and procedures performed during the hospitalization were compared. Multivariate logistic regression was used to analyze the effects of age, sex and HIV infection on number of hospitalizations for selected conditions. RESULTS: There were an estimated 686 hospitalizations of children with SCD and HIV infection in the United States in the 10-year period 1994-2003; these hospitalizations aggregated in the South (78.2%) and their expected payer was mostly Medicaid/Medicare (82.0%). Their average length of stay was longer than that of children with SCD alone (8.0 days vs. 4.3 days, respectively), and the mean charges associated with the hospitalization were also higher ($18,291 vs. $9584). Compared with patients with SCD without HIV, HIV infection conferred a higher risk for hospitalizations for bacterial infections and sepsis (odds ratio 2.75; 95% CI, 1.66-4.6), but less of a risk for vaso-occlusive crises (odds ratio 0.32; 95% CI, 0.22-0.48). Inpatient case-fatality rate of children with SCD and HIV was no different from that of children with SCD alone, but lower than that of the rest of children with HIV infection. CONCLUSIONS: Hospitalized children with SCD and HIV infection have higher odds of infection than those with SCD alone. Their inpatient case-fatality rate is lower than that of children with HIV infection alone. These findings should be considered in designing appropriate interventions for this population.     

川崎病合并冠状动脉瘤患儿应用华法林治疗的安全性

目的探讨川崎病合并冠状动脉瘤患儿应用华法林治疗的安全性和转归。方法选择2011至2020年湖南省人民医院收治的川崎病合并巨大冠状动脉瘤、1支冠状动脉血管内发生多个冠状动脉瘤或冠状动脉内发生血栓的患儿共21例进行前瞻性研究,应用华法林治疗,将国际标准化比值(INR)目标范围控制在2.0~3.0。在治疗开始时,治疗后1、2、3、4周及2、3、6、12个月,观察治疗前后冠状动脉瘤直径、数量、部位和冠状动脉内血栓的变化,并监测INR、心电图、肌酸激酶同工酶(CK-MB)、肌钙蛋白I。实施规范的华法林出血风险培训与管理,并根据家长实际落实情况,分为落实组和未落实组,比较2组患儿出血的发生情况。组间比较采用秩和检验或Fisher确切概率法。结果21例患儿男15例、女6例,发病年龄为2月龄至6岁。入组时评估冠状动脉病变为Ⅱ级有4例、Ⅲ级有7例、Ⅳ级有7例、Ⅴ级有3例。10例发生血栓的患儿临床发现血栓的时间为病程第4天至4个月。应用华法林治疗后,其剂量分布在0.06~0.10 mg/(kg·d),INR为1.80~2.59,10例有血栓的患儿有8例消失,2例未消失的Ⅴ级患儿的血栓有不同程度机化。治疗后,4例Ⅱ级病变患儿的冠状动脉均恢复正常;7例Ⅲ级病变患儿中有3例冠状动脉瘤消失,4例无改变;7例Ⅳ级病变患儿中5例冠状动脉瘤缩小为Ⅲ级,2例无改变;3例Ⅴ级病变患儿瘤体无改变。治疗后均没有新发血栓及新冠状动脉瘤出现。21例患儿治疗前后心电图无特殊改变,治疗前后肌钙蛋白I和CK-MB差异均无统计学意义[0.07(0~3.01)比0.04(0~0.29)μg/L,20.6(11.2~58.2)比29.0(16.7~47.0)U/L,Z=0.932、1.906,均P>0.05]。华法林出血风险管理落实组患儿出血发生率明显低于未落实组,差异有统计学意义(2/15比4/6,Fisher值=5.689,P=0.031)。结论应用INR 2.0~3.0为目标范围,结合川崎病并冠状动脉瘤的严重程度来调整华法林剂量,并予以规范而严格的管理与培训,可增加川崎病患儿华法林治疗的安全性,改善患儿的冠状动脉病变,治疗血栓及预防新血栓,降低出血风险。