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Kawasaki disease (KD) is a febrile disease mainly observed in children aged <5 years, with medium- and small-vessel vasculitis as the main lesion. Although KD has been reported for more than 50 years and great progress has been made in the etiology and pathology of KD in recent years, there is still a lack of specific indicators for the early diagnosis of KD, especially with more difficulties in the diagnosis of incomplete Kawasaki disease (IKD). At present, there are no clear diagnostic criteria for IKD, which leads to the failure of the timely identification and standardized treatment of IKD in clinical practice and even induce the development of coronary artery lesion. This article reviews the concept, epidemiological features, diagnosis, treatment, and follow-up management of IKD, in order to deepen the understanding of IKD among clinical workers and help to improve the clinical diagnosis and treatment of KD in China.
Subject(s)
Child , Humans , Infant , Mucocutaneous Lymph Node Syndrome/therapy , Coronary Vessels , ChinaABSTRACT
Objective To analyze the clinical features of children with Kawasaki disease(KD) in order to find out the trend of its clinical features and to provide guidance for clinical diagnosis and treatment.Methods Children with KD hospitalized in the Pediatric Ward of Peking University Third Hospital from January 2007 to December 2016 were collected and analyzed for their characteristics of onset,clinical features and treatment.The children were divided into 2 groups according to the time:2007 to 2011 group,2012 to 2016 group.The changes in clinical characteristics between the 2 stages were analyzed.Results A total of 337 children with KD were enrolled in this study,including 212 males and 125 females,with a ratio of 1.69 ∶ 1.00.The age of onset was from 2 months to 12 years old.The median age was 24 months.A total of 325(96.4％) children were under 6 years of age.There were 114(33.8％) cases of KD children from 2007 to 2011,among whom incomplete KD (IKD),intravenous gamma globulin non-response and coronary artery injury cases accounted for 23.7％ (27 cases),4.5％ (5 cases),38.6％ (44 cases),respectively;there were 223 (66.2％) cases of KD children from 2012 to 2016,among whom IKD,intravenous gamma globulin non-response and coronary artery injury cases accounted for 38.6％ (86 cases),11.9％ (26 cases),31.4％ (70 cases),respectively.The group of 2012 to 2016 was compared with the the group of 2007 to 2011 and it was found that the proportion of IKD and intravenous gamma globulin non-response cases increased,and the differences were significant (x2 =7.495,4.654,P =0.006,0.031),but the proportion of coronary artery injury cases decreased with no statistical difference (x2 =1.750,P =0.186).Conclusion With the incidence of KD increasing,the proportion of incomplete KD and intravenous gamma globulin non-response KD also showed an upward trend,which should be paid more attention to by clinicians in order to promptly diagnose and implement more targeted treatment.
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Objective To explore the difference of coronary artery lesions (CAL) in complete Kawasaki disease (cKD) and incomplete Kawasaki disease (iKD) in children. Methods The clinical data of 1514 hospitalized children with KD from January 2014 to December 2015 were retrospectively analyzed and compared between the children with cKD and those with iKD. The risk factors of CAL were analyzed. Results There were 1094 cases (72.3％) of cKD and 420 cases (27.7％) of iKD in 1514 children with KD. The incidence of CAL in all KD children was 51.9％. And the incidence of CAL in cKD group and iKD group was 57.2％and 37.9％, respectively, and there was statistical difference (P<0.01). The distribution of different degrees of CAL between cKD group and iKD group was statistically different (P<0.01). The incidence of echo enhancement or small coronary artery aneurysm in cKD group (50.4％) was higher, and the incidence of giant coronary artery aneurysm in iKD group (2.4％) was relatively higher. The incidence of thrombosis in iKD group was 3.3％, which was significantly higher than that in cKD group (0.6％) (P<0.001). The time when CAL was found first by clinical ultrasound echocardiography in cKD group and iKD group were 7.842.97 d and 8.472.89 d, respectively, and there was statistical difference (P<0.05). The most frequent involvement was left main trunk in CAL in children with KD. The proportion of simultaneous involvement of the left and right coronary arteries, only right stem involvement, and whole left coronary artery involvement in were significantly higher cKD group than those in iKD group, while the proportion of left main trunk involvement was significantly higher in iKD group than that in cKD group (all P<0.05). Male and iKD were the high risk factors for CAL, and intravenous infusion of immunoglobulin (IVIG) in 5～10 days of the course of disease was the protective factor for CAL. Conclusions CAL, especially giant coronary artery aneurysms and thrombosis, are more common in children with iKD. In iKD, the left coronary artery is mainly involved in CAL, and in cKD, the simultaneous involvement of left and right coronary arteries is the most common in CAL. Standard use of IVIG can reduce the occurrence of CAL.
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Objective To summarize and discuss the laboratory characteristics of children with incomplete Kawasaki disease(IKD)in recent 10 years and provide some evidence for early diagnosis. Methods A retrospective review was performed on 67 children with IKD and 67 children with respiratory tract infection(bacterial infection)from 2005 to 2015 in the First Hospital of China Medical University. Results The age of chil-dren with IKD varied from 2 months to 11.3 years,with the average age of 31 months. Totally 86.5%of the children were less than 5 years old. The male to female ratio was 2:1. The incidence of the disease in spring and summer was 72%. The incidences of the increasing of white blood cell ,C-reactive protein,alanine aminotransferase,aspartate aminotransferase,glutamyl transpeptidase,and platelet and the decreasing of hemoglobin and serum albumin were 82%,94%,27%,24%,45%,72%,61%,and 94%,respectively. Conclusion The levels of the hemoglobin(393×109/L),C-reactive protein(>74 mg/L),alanine aminotransferase(>51 U/L),and glutamyl transpepti-dase(>43 U/L)may have a certain reference value for the early diagnosis of IKD.
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Kawasaki disease is an acute vasculitis of unknown etiology that predominantly affects children <5 years of age. The incidence and the severity of myocarditis in this disease is variable and depends upon the stage of the disease, acute or chronic. Acute-stage Kawasaki disease shows relatively high incidence of myocarditis, but almost all cases are clinically mild. We describe teenage boy presenting with atypical/incomplete manifestations of Kawasaki disease and developing fulminant myocarditis within a week of illness resulting in death. The case underscores the importance of suspecting Kawasaki disease in a young child presenting with features of myocardial ischemia.
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Background: Recurrent Kawasaki disease is rare. Case characteristics: An eight-month old infant had classic Kawasaki disease with transient coronary artery dilatation. Observations: Recurrence of incomplete Kawasaki disease after two years of initial diagnosis. Outcome: The index episode of Kawasaki disease was resistant to single infusion of immunoglobulin, while repeat episode responded within 24 hours of institution of therapy. Message: Early recognition of recurrent Kawasaki disease requires a high index of suspicion.
ABSTRACT
Kawasaki disease (KD) is an acute systemic vasculitis of unknown aetiology that has largely replaced rheumatic heart disease as a cause of acquired heart disease in children of many developed countries. We report a case of incomplete KD in a threemonth- old girl. The diagnosis of incomplete KD was made after exclusion of conditions with similar presentation. She was treated with intravenous immunoglobulin following which she made an uneventful recovery but demonstrated thrombocytosis in the second week of convalescence. It is important for the treating physicians to become aware of the incomplete KD as prompt diagnosis and early treatment of these patients with intravenous immunoglobulin is vital for the prevention of lethal coronary complications. Physicians need to have a "high index of suspicion" for KD and even, higher for IKD.
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PURPOSE: In 2004, the American Heart Association (AHA) had published an algorithm for the diagnosis of incomplete Kawasaki disease (KD). The aim of the present study was to investigate characteristics of supplemental laboratory criteria in this algorithm. METHODS: We retrospectively examined the medical records of 355 patients with KD who were treated with intravenous immunoglobulin (IVIG) during the acute phase of the disease. Laboratory data were obtained before the initial IVIG administration and up to 10 days after fever onset. In 106 patients, laboratory testing was performed more than twice. RESULTS: The AHA supplemental laboratory criteria were fulfilled in 90 patients (25.4%), and the frequency of laboratory examination (odds ratio [OR], 1.981; 95% confidence interval [CI], 1.391-2.821; P<0.001) was a significant predictor of it. The fulfillment of AHA supplemental laboratory criteria was significantly associated with refractoriness to the initial IVIG administration (OR, 2.388; 95% CI, 1.182-4.826; P=0.013) and dilatation of coronary arteries (OR, 2.776; 95% CI, 1.519-5.074; P=0.001). CONCLUSION: Repeated laboratory testing increased the rate of fulfillment of the AHA supplemental laboratory criteria in children with KD.
Subject(s)
Child , Humans , American Heart Association , Coronary Vessels , Diagnosis , Dilatation , Fever , Immunoglobulins , Immunoglobulins, Intravenous , Medical Records , Mucocutaneous Lymph Node Syndrome , Retrospective StudiesABSTRACT
Objectives To explore the clinical features of incomplete Kawasaki disease (IKD) which presents with sterile pyuria in infant, in order to achieve early diagnosis and treatment. Methods A retrospective study of 15 infants with IKD was conducted in comparison with 29 patients who were diagnosed with KD according to the diagnostic criteria of American Heart Association 2004. Results Both KD and IKD groups had recurrent fever and poor response to antibiotic therapy. Hemoglobin level was signiifcantly lower in IKD group than in KD group at the onset or 7-d after onset (P<0.05). C-reaction protein (CRP) level was signiifcantly higher in IKD group than that in KD group at 7-d after onset (P<0.05). There were 4 patients with coronary artery abnormality, 2 patients with coronary artery dilation and 2 patients with coro-nary artery aneurysm. Conclusions For infant <1 year old who has pyuria ifrstly, continuous fever for over 5 days when treated by antibiotics and increasing serum inlfammatory markers, incomplete Kawasaki disease should be suspected and the cardiac ultrasound is recommended.
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Objective To analyze the changes in clinical characteristics of Kawasaki disease (KD) between the first and the second decade and improve the diagnosis and treatment of incomplete KD.Methods Retrospective analysis of clinical data of children with KD was performed in the Second Affiliated Hospital of Guangzhou Medical Coliege during the recent 20 years (between Dec.1991 and Dec.2011).The changes in clinical manifestations of KD were compared between the first l0 years and the second l0 years.A total of 270 hospitalized patients diagnosed as KD were included in this study.The patients admitted after Dec.2001 were assigned as the observation group (192 cases) and those admitted before Dec.2001 were assigned as the control group (78 cases).The epidemiologic characteristics,clinical manifestations,impairment of organs,and laboratory findings were compared between the 2 groups and statistic analysis was performed on the available date.Results 1.The rate of KD patients ＜ 1-year-old and the incidence of incomplete KD in the observation group were significantly higher than those in the control group (all P ＜0.01) ; 2.The incidences of rash,changes in oral mucosa,congestion of conjunctiva,hardening edema of hands and feet,lymph node enlargement in the observation group were significantly lower than those in the control group (all P ＜ 0.01) ; 3.Incidence of coronary artery impairment in the observation group was significantly higher than that in the control group (P ＜ 0.05) ; 4.There were no significant differences for inflammation index between the 2 groups (all P ＞ 0.05).Conclusions The incidence of KD and the number of cases with incomplete KD increased in the last decade,especially in younger infants,and the incidence of coronary arteries impairment is increased.To incomplete KD,combining clinical manifestations with laboratory findings of inflammation index are needed for early diagnosis.
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Se presenta el caso de un joven de doce años, con diagnóstico de enfermedad de Kawasaki (EK) incompleta, quien presentó, en el trascurso de la misma, un cuadro clínico compatible con hepatitis aguda. El diagnóstico definitivo se retrasó por la necesidad de descartar otros diagnósticos diferenciales relacionados con la hepatitis aguda. Aunque en la EK puede ocurrir una disfunción hepática, caracterizada por aumento de las transaminasas y bilirrubinas, el cuadro clínico de una hepatitis aguda es muy raro en el curso de esta enfermedad.
We report the case of a 12 year old boy with incomplete Kawasaki disease (KD). During the course of his illness he presented with symptoms of acute hepatitis, which delayed the final diagnosis. Although patients with KD may present with elevation of hepatic enzymes and bilirubin, associated acute hepatitis is of rare occurrence in this entity.
Subject(s)
Adolescent , Adolescent , Hepatitis/pathology , Mucocutaneous Lymph Node Syndrome , Bilirubin , TransaminasesABSTRACT
Objectives To study demography, presentation, treatment, complications and prognosis of children with Kawasaki disease (KD). Design A descriptive ongoing case study. Method All children clinically suspected to have KD, following admission to professorial unit at Lady Ridgeway Hospital for Children or when seen in the private sector, from November 2001 to September 2002, were included in the study. The demographic details, presenting features, treatment and complications were recorded. Children with coronary arteritis were reviewed periodically to analyse long-term effects. Results 19 children were suspected to have KD. Mean age was 3.9 years. 53% were males. Fever, conjunctivitis and mucocutaneous lesions were the commonest presenting features. 14 (74%) had coronary artery changes. 5 (26%) patients fulfilled the criteria for diagnosis. Intravenous immunoglobulin was used as treatment in 8 (42%) cases.