Kawasaki disease in Sweden: incidence and clinical features

The incidence and clinical pattern of patients with Kawasaki disease in Sweden and the outcome of treatment with iv immunoglobulin (Sandoglobulin) and aspirin were examined in a national prospective study over a 2-year period. Cases not referred to the study were identified by inquiry. Ninety-nine children were diagnosed as having Kawasaki disease. The annual incidence rate was calculated to be 2.9per 100 000 in children younger than 16 years of age and 6.2 per 100000 in children younger than 5 years of age. The median age of our patients was 2.2 years and the male-to-female ratio was 2.3:1. Cardiac ultrasonography revealed abnormalities in 33% of all patients, and 14% of cases in the prospective study had pronounced abnormalities. In most cases (91%) treatment had a prompt effect on fever and morbidity in general, and side effects were mild. Two infants, identified from the inquiry, died from rupture of an aneurysm in the coronary artery in the acute phase of the disease. The risk of cardiac involvement is obvious and emphasizes the importance of early diagnosis and treatment. Incidence, Kawasaki disease, vasculitis     

Incidence and clinical features of incomplete Kawasaki disease

During the nine-year period from 1983 to 1991, a total of 242 patients (142 males and 100 females) presenting with Kawasaki disease were seen at one hospital. Among them, 25 (10%) patients demonstrated incomplete Kawasaki disease and 17 of these 25 (68%) lacked two of the six principal symptoms of Kawasaki disease, with the most frequently missing symptoms being cervical lymphadenopathy and polymorphous exanthema. The typical laboratory features of Kawasaki disease, such as elevated erythrocyte sedimentation rate, leukocytosis, anemia, positive C-reactive protein and thrombocytosis were also seen in the incomplete cases. None of the 25 patients underwent iv gamma-globulin therapy while in 1 (4%), transient dilatation of the coronary artery was recognized. Incomplete Kawasaki disease may therefore be characterized by a less frequent association of rash, cervical lymphadenopathy and coronary involvement.     

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目的探讨儿童川崎病(KD)的临床、治疗和预后特点。方法对2000—2005年广东省中山市博爱医院儿科收治的100例KD患儿进行随访,对其临床特征、治疗方案以及预后进行回顾分析。结果 (1)临床特点:发病年龄(2.01±1.35)岁,随访年龄(8.2±1.7)岁;男女之比为1.7∶1。典型KD88例,其中再发病例2例;不完全KD12例。心血管系统并发症25例,包括冠状动脉改变22例,心包积液2例,心肌炎1例,其中一过性冠脉扩张18例,冠脉瘤形成4例;非心血管系统并发症包括肝损害34例,胆囊积液3例,麻痹性肠梗阻1例,无菌性脑膜炎3例,面神经麻痹5例,肺炎53例,尿道炎12例,关节炎6例。(2)治疗和疗效:89例病程10d内给予丙种球蛋白(IVIG)2g/kg,12例无效,需追加第2次IVIG,其中2例加用肾上腺皮质激素。11例亚急性期确诊者给予IVIG1～2g/kg,其中3例并发冠脉瘤(P<0.05)。无冠脉损害者病程6～8周停药,18例有冠脉扩张者跟踪至半年至1年后停药,3例中小型冠脉瘤者在病程1～3年停药,1例巨大冠脉瘤者服用阿司匹林至今已7年。(3)随访和预后:56例追踪随访至病程≥5年,最长10年。随访...     

Kawasaki disease: infection, immunity and genetics

Kawasaki disease is an acute multisystem vasculitic syndrome of unknown etiology occurring mostly in infants and children younger than 5 years of age. In developed countries, Kawasaki disease is currently the leading cause of acquired heart diseases in children. However, it is still a mysterious disease. In this article, we reviewed and summarized from the aspects based on infection agents, host immune dysregulation and genetic background intended to establish a feasible infection-immunogenetic pathogenesis for this mysterious disease and also provided the rational strategy to explore optimal treatment of this disease.     

Incomplete and atypical Kawasaki disease in a young infant: severe, recalcitrant disease responsive to infliximab

This report describes a 7-week-old infant with incomplete and atypical Kawasaki disease, an acute vasculitis that predominantly affects infants and children. The patient was refractory to 2 doses of intravenous immunoglobulin and to high-dose intravenous methylprednisolone. He became afebrile only after 2 doses of infliximab. His prolonged, recalcitrant course was complicated by the development of peripheral gangrene and giant coronary artery aneurysms. Infants with incomplete and atypical Kawasaki disease are prone to intravenous immunoglobulin treatment failure and are at risk for the development of coronary artery aneurysms. In such patients, we suggest that consideration be given to early aggressive therapy with corticosteroids or infliximab added to intravenous immunoglobulin.     

2012至2016年单中心川崎病流行病学及临床特征研究

目的了解川崎病(KD)患病情况及临床特征,探讨KD冠状动脉损害(CAL)及IVIG耐药的危险因素。方法回顾性分析华中科技大学同济医学院附属同济医院2012年1月1日至2016年12月31日初诊的KD患儿的临床资料,比较分析KD治疗前后,典型和不完全KD,KD伴或不伴CAL,IVIG敏感或耐药的临床特征,分析CAL发生和IVIG耐药的危险因素。结果725例KD患儿进入本文分析,男∶女为1.61∶1,平均年龄（2.7±2.3）岁;不完全KD 206例（28.4%）,典型KD 519例;CAL 216例（29.8%）,IVIG耐药61例（8.4%）;治疗中仅使用阿司匹林者70例（9.6%）。KD伴CAL的危险因素为IVIG耐药（OR=5.138,95%CI：1.835~14.836）和氨基末端脑钠肽前体（NT-proBNP）≥1 000 pg·mL-1（OR=2.723,95%CI：1.110~6.679）。IVIG耐药的危险因素为出现CAL（OR=2.586,95%CI：1.067~6.271）。结论KD患病人数、CAL和IVIG耐药患儿有增加趋势。IVIG耐药和NT-proBNP≥1 000 pg·mL-1为KD伴CAL的危险因素,而发生CAL为IVIG耐药的危险因素。     

儿童再发川崎病临床特征的Meta分析

目的 探讨儿童再发川崎病（KD）的临床特点,以提高对再发KD的认识。方法 检索PubMed、Web of Science、Embase、中国知网、万方医学网和中国科技期刊数据库中关于儿童再发KD与初发时临床特点的对照研究,根据纳入、排除标准筛选文献,采用RevMan 5.3软件进行Meta分析。根据异质性检验结果选择相应效应模型进行数据合并,计算各观察指标合并比值比（OR）或加权均数差（WMD）及其95%CI。结果 最终纳入9个病例对照研究,KD患儿样本总量为12 059例,其中再发KD患儿206例（男127例,占61.7%;女79例,占38.3%）。Meta分析结果显示,与初发时相比,再发时发热时程缩短（WMD=-1.81,95%CI：-2.99~-0.64）,手足硬肿率降低（OR=0.46,95%CI：0.26~0.80）,差异均具有统计学意义（P < 0.05）;KD患儿再发时与初发时冠状动脉病变发生率的比较差异无统计学意义（OR=1.34,95%CI：0.84~2.14,P=0.22）。结论 目前证据显示再发KD患儿热程更短,手足硬肿率较低;KD再发以男童多见;再发患儿冠状动脉病变发生风险未见明显增高。     

儿童再发川崎病临床特征的Meta分析

目的 探讨儿童再发川崎病（KD）的临床特点,以提高对再发KD的认识。方法 检索PubMed、Web of Science、Embase、中国知网、万方医学网和中国科技期刊数据库中关于儿童再发KD与初发时临床特点的对照研究,根据纳入、排除标准筛选文献,采用RevMan 5.3软件进行Meta分析。根据异质性检验结果选择相应效应模型进行数据合并,计算各观察指标合并比值比（OR）或加权均数差（WMD）及其95%CI。结果 最终纳入9个病例对照研究,KD患儿样本总量为12 059例,其中再发KD患儿206例（男127例,占61.7%;女79例,占38.3%）。Meta分析结果显示,与初发时相比,再发时发热时程缩短（WMD=-1.81,95%CI：-2.99~-0.64）,手足硬肿率降低（OR=0.46,95%CI：0.26~0.80）,差异均具有统计学意义（P < 0.05）;KD患儿再发时与初发时冠状动脉病变发生率的比较差异无统计学意义（OR=1.34,95%CI：0.84~2.14,P=0.22）。结论 目前证据显示再发KD患儿热程更短,手足硬肿率较低;KD再发以男童多见;再发患儿冠状动脉病变发生风险未见明显增高。     

川崎病并发巨大冠状动脉瘤急性期临床特征与危险因素分析

目的以川崎病(KD)并发巨大冠状动脉瘤(GCAA)的病例对照研究,分析KD急性期的临床特征,并探讨并发GCAA的危险因素。方法选取2001年5月至2009年5月在广州市妇女儿童医疗中心儿童医院住院的KD并发GCAA患儿为GCAA组;选取同期KD并发中小冠状动脉瘤(CAA)患儿为对照组。对两组患儿的临床特征进行比较,对可能影响GCAA发生的因素进行单因素分析,并进行多因素Logistic逐步回归分析。结果GCAA组纳入22例,其中男19例,女3例,发病年龄3个月至10岁,平均(2.9±2.8)岁。中小CAA组纳入65例,其中男51例,女14例。Pearson χ2检验结果提示,年龄≤6个月或≥5岁、发热时间>14 d、延误诊断、确诊前单独使用糖皮质激素、Hb降低、ESR升高和ALB降低与GCAA发生相关(P均<0.05)。多因素Logistic逐步回归分析提示,延误诊断（OR＝2.998,95%CI：1.004～8.950,P=0.047),确诊前单独使用糖皮质激素（OR＝6.556,95%CI：1.561～28.542,P=0.010),ESR≥100 mm·h-1(OR＝3.591,95%CI：1.164～11.079,P=0.026)为发生GCAA的独立危险因素。结论延误诊断、确诊前单独使用糖皮质激素和ESR≥100 mm·h-1为KD并发GCAA的独立危险因素。     

An update on Kawasaki disease II: Clinical features,diagnosis, treatment and outcomes

This is the second of two updates on Kawasaki disease. The first review focused on epidemiology and aetio‐pathogenesis. Here, we review the clinical features and diagnosis of Kawasaki disease, as well as recent evidence on treatment, follow‐up and cardiovascular outcomes.     

Prolonged fever and pyuria: a urinary tract infection presentation of incomplete Kawasaki disease

We report two patients with incomplete Kawasaki disease that presented as apparent urinary tract infection. Persistent fever and pyuria were the initial presentation without concomitant signs suggestive of Kawasaki disease; thus the patients were treated as urinary tract infection. Fever persisted despite antibiotic treatment. Diagnostic criteria of Kawasaki disease were not fulfilled for these two patients, yet aneurysmal dilatation of the coronary artery was noted 10 and 18 d, respectively, after the onset of fever. The diagnosis of incomplete Kawasaki disease was assigned when the coronary artery abnormality was detected. Fever subsided within 24 h of administration of intravenous immunoglobulin. Conclusion: This report highlights the potentially misleading presentation of fever and pyuria as the sole initial manifestation of incomplete Kawasaki disease. Echocardiography is indicated to detect coronary artery abnormality when fever persists in such patients after adequate antibiotic treatment and thorough urological evaluation.     

川崎病与病原微生物关系的meta分析

目的系统评价与川崎病发病可能相关的病原。方法全面检索CNKI、PubMed、EMbase、Medline数据库中1979年1月至2010年10月的川崎病病因中病原检测的相关文献,语种限中文和英文。纳入原始研究、质量评价并提取资料,剔除不符合研究标准的文献,对所纳入的研究数据采用RevMan5软件进行统计学分析。结果纳入12篇文献,共研究5种病原菌,包括593例川崎病患儿和1152例对照组儿童。Meta分析结果显示,金黄色葡萄球菌和A组链球菌,其合并OR值为4.26(95%CI:2.54~7.16,P<0.05)。人类微小病毒B19,其合并OR值为6.58(95%CI:2.37~18.25,P<0.05)。支原体和衣原体,其合并OR值为3.41(95%CI:1.99~5.84,P<0.05)。人类冠状病毒NL-63,其合并OR值为0.75(95%CI:0.20~2.78,P>0.05)。结论病原体感染参与川崎病发病,相关病原有金黄色葡萄球菌、A组链球菌,支原体和衣原体,人类微小病毒B19,而人类冠状病毒NL-63与川崎病发生无相关性。上述结果尚需进一步证实。     

Correlation of serum antigen and antibody concentration with clinical features in HIV infection.

Serum antigen and antibody values were studied in 164 infants and children infected perinatally with HIV. HIV antigens p17, p24, gp41, and gp120 were determined in sera by immunoblot and antigen capture assays. Lymphocyte blast transformation, serum immunoglobulins, and circulating immune complexes were also evaluated. Altogether 50 patients had HIV antigens measured: 31 (62%) patients had p17 antigen in the serum and 29 (58%) had p24 antigen present. In 19 (38%) and nine (18%) patients, respectively, gp120 and gp41 were detected. All four HIV antigens were detected in seven (14%) patients. There was a positive correlation between the concentration of each HIV sequential specimens were outcome. When sequential specimens were analysed, 120 (73%) patients had p24 antigen present. Patients with stage P2B and P2D (Centers for Disease Control classification) had the highest concentrations of p24 antigen with a mean of approximately 200 pg/ml. Altogether 70% of patients with a p24 antigen concentration of greater than 30 pg/ml eventually died or had severe clinical disease within six to 24 months. Infants under 15 months of age with a p24 antigen concentration as low as 5 pg/ml also did poorly. Increased immunoglobulins and decreases in mitogenic responses and absolute CD4+ lymphocyte counts were more prevalent in patients with raised p24 antigen. Raised concentrations of circulating immune complexes were seen in the symptomatic phase of the disease whereas in the terminal stage of the disease raised serum antigen and a decrease in circulating immune complexes and absolute CD4+ lymphocyte count were evident. Loss of p24 and/or p17 antibody as well as a decreasing ELISA optical density for HIV antibody also signalled progression of the disease.     

Central core disease: clinical, pathological, and genetic features

Central core disease (CCD) is a dominantly inherited congenital myopathy allelic to malignant hyperthermia (MH) caused by mutations in the RYR1 gene on chromosome 19q13.1. Eleven individuals with RYR1 mutations are described. Four index cases showed features consistent with a congenital myopathy (hypotonia, delayed motor milestones, and skeletal abnormalities including congenital hip dislocation and scoliosis). All four cases and subsequently seven other family members were found to possess novel mutations in the RYR1 gene. The degree of disability varied from one clinically normal individual, to another who had never achieved independent ambulation (the only patient with a de novo mutation). Four cases showed a mild reduction in vital capacity, repeated nocturnal polysomnography showed hypoxaemia in one case. A variety of muscle biopsy features were found; central cores were absent in the youngest case, and the biopsy specimens from two others were more suggestive of mini-core myopathy. In all cases missense mutations in exons 101, 102, and 103 of the RYR1 gene on were found. Future laboratory diagnosis of suspected cases and family members will be less invasive and more accurate with DNA analysis. Clinicians, especially paediatricians and orthopaedic surgeons, should be aware of this disorder because of the potential risk of MH.     

Stool antigen test for diagnosis of Helicobacter pylori infection in children with symptomatic disease: a prospective study

OBJECTIVE: Noninvasive tests for the diagnosis of Helicobacter pylori (Hp) infection in children are limited by low accuracy rates and lack of validation. Existing studies indicate that the stool antigen test (HpSA) has an acceptable level of accuracy for the diagnosis of Hp infection in adults but not children. The aim of this study was to evaluate the accuracy of the HpSA test for the detection of Hp infection in U.S. children. METHODS: Children requiring upper endoscopic procedures were prospectively recruited from two pediatric gastroenterology clinics. Stool samples were collected from each participant before endoscopy. The presence of Hp infection was determined by positive histologic findings and positive rapid urease test (RUT). The presence of Hp organisms in stool was determined by an enzyme-linked immunosorbent assay using a commercially available polyclonal antibody kit (Meridian Diagnostics, Cincinnati, OH, U.S.A.). Results of the stool antigen test were compared with histology findings and RUT results. RESULTS: One hundred twenty-one children (mean age, 10.1 +/- 3.7 years) participated, of whom 9 (7.4%) had Hp infection. Histologic findings and RUT results were concordant in 95% of the children. Per study protocol, HpSA had a sensitivity, specificity, positive and negative predictive value, and accuracy rate of 67%, 99%, 86%, 97%, and 96.5%, respectively. CONCLUSION: HpSA, a polyclonal antibody test, had a low sensitivity for infection in children in the United States and at present cannot replace histologic findings as the gold standard for the diagnosis of Hp infection in the pediatric population.     

Central core disease: clinical, pathological, and genetic features.

Central core disease (CCD) is a dominantly inherited congenital myopathy allelic to malignant hyperthermia (MH) caused by mutations in the RYR1 gene on chromosome 19q13.1. Eleven individuals with RYR1 mutations are described. Four index cases showed features consistent with a congenital myopathy (hypotonia, delayed motor milestones, and skeletal abnormalities including congenital hip dislocation and scoliosis). All four cases and subsequently seven other family members were found to possess novel mutations in the RYR1 gene. The degree of disability varied from one clinically normal individual, to another who had never achieved independent ambulation (the only patient with a de novo mutation). Four cases showed a mild reduction in vital capacity, repeated nocturnal polysomnography showed hypoxaemia in one case. A variety of muscle biopsy features were found; central cores were absent in the youngest case, and the biopsy specimens from two others were more suggestive of mini-core myopathy. In all cases missense mutations in exons 101, 102, and 103 of the RYR1 gene on were found. Future laboratory diagnosis of suspected cases and family members will be less invasive and more accurate with DNA analysis. Clinicians, especially paediatricians and orthopaedic surgeons, should be aware of this disorder because of the potential risk of MH.