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  1. Kawasaki Disease

    MedlinePlus

    ... page from the NHLBI on Twitter. What Is Kawasaki Disease? Kawasaki (KAH-wah-SAH-ke) disease is a ... condition involves inflammation of the blood vessels. In Kawasaki disease, the walls of the blood vessels throughout the ...

  2. Kawasaki disease

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/000989.htm Kawasaki disease To use the sharing features on this page, please enable JavaScript. Kawasaki disease is a rare condition that involves inflammation of ...

  3. Kawasaki disease.

    PubMed

    Kawasaki, Tomisaku

    2006-04-01

    Short history of Kawasaki disease, clinical features (principal symptoms and other significant symptoms or findings), diagnosis, cardiovascular involvement, epidemiology. Pathological features (lesion of vessels and lesion of organs exclusive of vessels), comparison between infantile periarteritis nodosa (IPN)/Kawasaki disease and classic periarteritis nodosa (CPN), etiology, treatment and management of Kawasaki disease are described.

  4. Kawasaki disease

    PubMed Central

    Kawasaki, Tomisaku

    2006-01-01

    Short history of Kawasaki disease, clinical features (principal symptoms and other significant symptoms or findings), diagnosis, cardiovascular involvement, epidemiology. Pathological features (lesion of vessels and lesion of organs exclusive of vessels), comparison between infantile periarteritis nodosa (IPN)/Kawasaki disease and classic periarteritis nodosa (CPN), etiology, treatment and management of Kawasaki disease are described. PMID:25792773

  5. Kawasaki Disease

    MedlinePlus

    ... swollen neck glands, swollen hands and feet, and red eyes, lips, and tongue. Early on, Kawasaki Disease can ... body but more severe in the diaper area. Red, bloodshot eyes without any pus, drainage, or crusting. Tender, swollen ...

  6. Kawasaki Disease

    PubMed Central

    2006-01-01

    Kawasaki disease is an acute febrile, systemic vasculitic syndrome of an unknown etiology that primarily occurs in children younger than five years of age. The principal presentations of Kawasaki disease include fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develops in 15% to 25% of untreated children with the disease, which may later lead to myocardial infarction, sudden death, or ischemic heart disease. Treatment with intravenous gamma globulin (IVIG) is effective, but the mode of action is still unclear. The development of a diagnostic test, a more specific therapy, and ultimately the prevention of this potentially fatal illness in children are all dependent upon the continued advances in determining the etiopathogenesis of this fascinating disorder. PMID:17191303

  7. [Kawasaki's disease].

    PubMed

    Cortes, J; Martínez, B; Montini, C; Barraza, P; Reyes, A

    1989-08-01

    We described a case of Kawasaki's disease in a chilean girl, one year and 5 months old of age, who presented the oral characteristics, cutaneous and systemic manifestation of the condition, that is not very common for the dentist but that it is necessary to know due to the heart complications and the mortality associated with the disease, and it is necessary that the dentist recognize early this condition.

  8. How Is Kawasaki Disease Diagnosed?

    MedlinePlus

    ... page from the NHLBI on Twitter. How Is Kawasaki Disease Diagnosed? Kawasaki disease is diagnosed based on your child's signs and ... are the first to suspect a child has Kawasaki disease. Pediatricians are doctors who specialize in treating children. ...

  9. Genetics Home Reference: Kawasaki disease

    MedlinePlus

    ... LY, Chang WP, Chang WC. ITPKC single nucleotide polymorphism associated with the Kawasaki disease in a Taiwanese ... Kawasaki T, Nakamura Y, Hata A. ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of ...

  10. Kawasaki Syndrome

    PubMed Central

    Rowley, Anne H.; Shulman, Stanford T.

    1998-01-01

    Kawasaki syndrome (KS) is an acute, sometimes fatal vasculitis of young children. KS has replaced acute rheumatic fever as the most common cause of acquired heart disease in children in the United States. The illness is manifested by prolonged fever, conjunctival injection, enanthem, exanthem, erythema and swelling of the hands and feet, and cervical adenopathy. These acute features of illness are self-limiting, but coronary artery abnormalities occur in 20% of untreated patients. The etiology of the illness is unknown, but its clinical and epidemiologic features are most consistent with an infectious cause. Common cardiovascular manifestations of the illness include myocarditis, pericardial effusion, and coronary artery aneurysm formation. Treatment with intravenous gamma globulin (IVGG) and aspirin within the first 10 days of illness reduces the prevalence of coronary artery abnormalities from 20% in those treated with aspirin alone to 4%. Patients who develop coronary artery aneurysms, particularly those who develop giant coronary artery aneurysms, may suffer myocardial infarction secondary to thrombosis or stenosis in the abnormal vessel. Additional research to determine the cause of KS is urgently needed to allow for improved diagnosis, more specific therapy, and prevention of the disorder. PMID:9665974

  11. History of Kawasaki disease.

    PubMed

    Kawasaki, Tomisaku; Naoe, Shiro

    2014-04-01

    We describe a short history of Kawasaki disease. In 1967, we published a paper entitled 'Infantile acute febrile mucocutaneous lymph node syndrome with specific desquamation of the fingers and toes. Clinical observation of 50 cases'; this was the first report on what is now called Kawasaki disease. Since then, many reports on cardiology, treatment, epidemiology, pathology and etiology of Kawasaki disease have been published. Furthermore, a recent Chapel Hill Consensus Statement on Kawasaki disease in the classification of vasculitis is given, along with a figure on the relationship and classification of childhood vasculitis by autopsy material.

  12. Living with Kawasaki Disease

    MedlinePlus

    ... can help lower their risk of future heart disease. (Following a healthy lifestyle is advised for all children, not just those who have Kawasaki disease). Children treated with immune globulin should wait 11 ...

  13. ATYPICAL KAWASAKI DISEASE.

    PubMed

    Ristovski, Ljiljana; Milankov, Olgica; Vislavski, Melanija; Savić, Radojica; Bjelica, Milena

    2016-01-01

    Kawasaki disease is an acute vasculitis which occurs primarily in children under the age of 5. The etiology of the disease is still unknown. Diagnostic criteria for Kawasaki disease are fever and at least four of the five additional clinical signs. Incomplete Kawasaki disease should be taken into consideration in case of all children with unexplained fever for more than 5 days, associated with 2 or 3 of the main clinical findings of Kawasaki disease. The diagnosis of incomplete Kawasaki disease is based on echocardiographic findings indicating the involvement of the coronary arteries. Cardiac complications, mostly coronary artery aneurysm, can occur in 20% to 25% of untreated patients and in 4% of treated patients. CASE REPORT. In this report we present a case of atypical Kawasaki disease in a 3.5-month-old infant. As soon as the diagnosis was made, the patient received high doses of intravenous immunoglobulin, with the initial introduction of ibuprofen, then aspirin with a good clinical response. Due to the presence of aneurysm of coronary arteries, further therapy involved aspirin and clopidogrel over the following 3 months, and then only aspirin for 2 years. There was a gradual regression of the changes in the coronary blood vessels to the normalization of the echocardiographic findings after 2 years. Kawasaki disease is the second most common vasculitis of childhood, so it should be included in the differential diagnosis for any child with a prolonged unexplained fever. Atypical Kawasaki disease should be taken into consideration in cases when not all clinical criteria are present but coronary abnormalities are documented.

  14. Kawasaki Disease: MedlinePlus Health Topic

    MedlinePlus

    ... symptoms. Further problems are rare. NIH: National Heart, Lung, and Blood Institute Start Here Kawasaki Disease (American Academy of ... American Academy of Pediatrics) Kawasaki Disease (National Heart, Lung, and Blood Institute) What Is Kawasaki Disease? (American Heart Association) - ...

  15. Kawasaki Disease (For Parents)

    MedlinePlus

    ... the symptoms and ruling out other conditions. Most kids diagnosed with Kawasaki disease will have a fever lasting 5 or more days and at least four of these symptoms: redness in both eyes changes around the lips, tongue, or mouth changes in ...

  16. What Is Kawasaki Disease?

    MedlinePlus

    ... Do you have questions for the doctor or nurse? Take a few minutes to write your questions for the next time you see your healthcare provider. For example: What can I do to help my child deal with Kawasaki disease? My Questions: We have many other fact sheets ...

  17. Conceptos Basicos Sobre el Propano

    SciTech Connect

    2016-08-01

    El propano provee energia a mas de 143.000 vehiculos en los Estados Unidos y 23 millones en todo el mundo. Flotas de todos los Estados Unidos han implementado con exito el uso de vehiculos que funcionan con gas propano, y en la actualidad varios funcionan gracias a este gas, incluyendo autobuses escolares, lanzaderas y autobuses publicos, asi como tambien furgonetas, taxis, vehiculos utilizados por las fuerzas del orden, barredoras de calles y camiones para uso profesional. El propano tambien se utiliza con frecuencia en aplicaciones fuera de la carretera, tales como montacargas, podadoras y equipos de uso profesional y otros equipos agricolas. Las ventajas del propano incluyen la disponibilidad interna, su rendimiento y el hecho de que genera menos emisiones de gases contaminantes que otras alternativas energeticas.

  18. How Is Kawasaki Disease Treated?

    MedlinePlus

    ... can help lower the risk of future heart disease. (Following a healthy lifestyle is advised for all children, not just those who have Kawasaki disease.) Children who have had immune globulin should wait ...

  19. Kawasaki Disease: Complications, Treatment and Prevention

    MedlinePlus

    ... Aortic Aneurysm More Kawasaki Disease: Complications, Treatment and Prevention Updated:May 8,2017 Complications The possibility of ... problems that did not show up right away. Prevention There is no known prevention for Kawasaki disease. ...

  20. Vaccines and Kawasaki disease.

    PubMed

    Esposito, Susanna; Bianchini, Sonia; Dellepiane, Rosa Maria; Principi, Nicola

    2016-01-01

    The distinctive immune system characteristics of children with Kawasaki disease (KD) could suggest that they respond in a particular way to all antigenic stimulations, including those due to vaccines. Moreover, treatment of KD is mainly based on immunomodulatory therapy. These factors suggest that vaccines and KD may interact in several ways. These interactions could be of clinical relevance because KD is a disease of younger children who receive most of the vaccines recommended for infectious disease prevention. This paper shows that available evidence does not support an association between KD development and vaccine administration. Moreover, it highlights that administration of routine vaccines is mandatory even in children with KD and all efforts must be made to ensure the highest degree of protection against vaccine-preventable diseases for these patients. However, studies are needed to clarify currently unsolved issues, especially issues related to immunologic interference induced by intravenous immunoglobulin and biological drugs.

  1. Kawasaki disease. The mystery continues.

    PubMed Central

    Bradley, D J; Glodé, M P

    1998-01-01

    Kawasaki disease is an acute systemic vasculitis of unknown cause first described in Japan in 1967. It affects children younger than 10 years, predominantly those younger than 3 years. Children of Asian ancestry are more commonly affected than white children. The diagnosis is made when 4 of the following 5 signs or symptoms are present with fever for at least 5 days: polymorphous rash; conjunctival injection; oral mucosal changes; cervical lymphadenitis; and erythema, swelling, or desquamation of the hands and feet. Various symptoms may be seen also, among them pronounced irritability, arthralgia, and abdominal pain. No diagnostic test exists, and clinicians must carefully exclude diseases that mimic Kawasaki disease. In addition to the above clinical findings, Kawasaki disease causes aneurysm formation in medium-sized arteries, particularly the coronary arteries. Found in about a fourth of patients, these lesions may persist, scar with stenosis, or resolve angiographically. The treatment consists of administering high-dose aspiring and intravenous immune globulin, with the goal of relieving acute illness and minimizing sequelae. Although most respond rapidly, almost 10% of children do not improve clinically with treatment. Complications of Kawasaki disease include myocardial infarction, which may occur during acute illness or later, as a result of coronary abnormalities. Images Figure 1. Figure 2. PMID:9448484

  2. Management of Kawasaki disease

    PubMed Central

    Eleftheriou, D; Levin, M; Shingadia, D; Tulloh, R; Klein, NJ; Brogan, PA

    2014-01-01

    Kawasaki disease (KD) is an acute self-limiting inflammatory disorder, associated with vasculitis, affecting predominantly medium-sized arteries, particularly the coronary arteries. In developed countries KD is the commonest cause of acquired heart disease in childhood. The aetiology of KD remains unknown, and it is currently believed that one or more as yet unidentified infectious agents induce an intense inflammatory host response in genetically susceptible individuals. Genetic studies have identified several susceptibility genes for KD and its sequelae in different ethnic populations, including FCGR2A, CD40, ITPKC, FAM167A-BLK and CASP3, as well as genes influencing response to intravenous immunoglobulin (IVIG) and aneurysm formation such as FCGR3B, and transforming growth factor (TGF) β pathway genes. IVIG and aspirin are effective therapeutically, but recent clinical trials and meta-analyses have demonstrated that the addition of corticosteroids to IVIG is beneficial for the prevention of coronary artery aneurysms (CAA) in severe cases with highest risk of IVIG resistance. Outside of Japan, however, clinical scores to predict IVIG resistance perform suboptimally. Furthermore, the evidence base does not provide clear guidance on which corticosteroid regimen is most effective. Other therapies, including anti-TNFα, could also have a role for IVIG-resistant KD. Irrespective of these caveats, it is clear that therapy that reduces inflammation in acute KD, improves outcome. This paper summarises recent advances in the understanding of KD pathogenesis and therapeutics, and provides an approach for managing KD patients in the UK in the light of these advances. PMID:24162006

  3. Kawasaki disease for dermatologists

    PubMed Central

    Gupta, Aman; Singh, Surjit

    2016-01-01

    Kawasaki disease (KD) is a systemic vasculitis that mostly affects children below the age of 5. The vasculitis involves arteries of medium size, especially the coronaries. Various etiologies have been proposed including association with micro-organisms, bacterial superantigens, and genetic factors, however, the exact cause remains unknown. There is no specific laboratory test for KD. Diagnosis is clinical and depends upon the presence of fever for ≥5 days and 4 or more of five principal features, viz. polymorphous exanthem, extremity changes, mucosal changes involving the lips and oral cavity, bilateral bulbar conjunctival injection, and unilateral cervical lymphadenopathy. The term “incomplete KD” refers to the presence of fever and less than four principal clinical features. Recognition of this group of patients is important because it is usually seen in infants and risk of coronary abnormalities is increased probably because of delays in diagnosis. However, what appears to be “incomplete” at a given point of time may not actually be so because some of the features may have already subsided and others may evolve over time. Hence, a detailed dermatological examination is warranted in all cases, especially in incomplete KD, to ensure timely diagnosis. Although KD is a self-limiting disease in most patients, coronary artery abnormalities (CAAs) including coronary dilatations and aneurysms may develop in up to 25% of untreated patients. CAAs are the most common cause of morbidity and mortality in patients with KD. Treatment is aimed at reducing inflammation and consists of intravenous immunoglobulin (IVIG) along with aspirin. Despite treatment, some patients may still develop CAAs, and hence, long-term follow up is of utmost importance. PMID:27990380

  4. [Cardiac and extracardiac complications in Kawasaki disease].

    PubMed

    Kamada, Masahiro

    2014-09-01

    In terms of frequency and prognosis, discussions of the complications of Kawasaki disease have focused on coronary artery aneurysms and stenosis. However, as revealed by autopsy findings, medium and small muscular arteries in various organs could be injured in patients with Kawasaki disease, leading to the development of a variety of complications. Accurate diagnosis of Kawasaki disease can be especially difficult when incomplete Kawasaki disease is accompanied by rare complications involving the central nervous system, digestive system, and other systems in the body, which leads to delay in treatment and subsequent development of coronary artery aneurysms and delayed improvement of the existing complications. Doctors who treat Kawasaki disease should be familiar with the rare but important complications of Kawasaki disease.

  5. Recognition of a Kawasaki Disease Shock Syndrome

    PubMed Central

    Kanegaye, John T.; Wilder, Matthew S.; Molkara, Delaram; Frazer, Jeffrey R.; Pancheri, Joan; Tremoulet, Adriana H.; Watson, Virginia E.; Best, Brookie M.; Burns, Jane C.

    2010-01-01

    objective We sought to define the characteristics that distinguish Kawasaki disease shock syndrome from hemodynamically normal Kawasaki disease. methods We collected data prospectively for all patients with Kawasaki disease who were treated at a single institution during a 4-year period. We defined Kawasaki disease shock syndrome on the basis of systolic hypotension for age, a sustained decrease in systolic blood pressure from baseline of ≥20%, or clinical signs of poor perfusion. We compared clinical and laboratory features, coronary artery measurements, and responses to therapy and analyzed indices of ventricular systolic and diastolic function during acute and convalescent Kawasaki disease. results Of 187 consecutive patients with Kawasaki disease, 13 (7%) met the definition for Kawasaki disease shock syndrome. All received fluid resuscitation, and 7 (54%) required vasoactive infusions. Compared with patients without shock, patients with Kawasaki disease shock syndrome were more often female and had larger proportions of bands, higher C-reactive protein concentrations, and lower hemoglobin concentrations and platelet counts. Evidence of consumptive coagulopathy was common in the Kawasaki disease shock syndrome group. Patients with Kawasaki disease shock syndrome more often had impaired left ventricular systolic function (ejection fraction of <54%: 4 of 13 patients [31%] vs 2 of 86 patients [4%]), mitral regurgitation (5 of 13 patients [39%] vs 2 of 83 patients [2%]), coronary artery abnormalities (8 of 13 patients [62%] vs 20 of 86 patients [23%]), and intravenous immunoglobulin resistance (6 of 13 patients [46%] vs 32 of 174 patients [18%]). Impairment of ventricular relaxation and compliance persisted among patients with Kawasaki disease shock syndrome after the resolution of other hemodynamic disturbances. conclusions Kawasaki disease shock syndrome is associated with more-severe laboratory markers of inflammation and greater risk of coronary artery

  6. Purified protein derivative anergy in Kawasaki disease.

    PubMed

    Kollmann, T R; Klein, E J; Stefanelli, C B; Marcuse, E K

    2001-01-01

    It was previously reported from Italy that all patients with Kawasaki disease had a positive tuberculin intradermal test. In this study from Seattle, WA, nine patients with Kawasaki disease showed no reaction to intradermal tuberculin. The difference in results might be caused by the different tuberculin products.

  7. Estudio de la estructura logica utilizada en la ensenanza y el aprendizaje de los conceptos sobre el comportamiento de gases en el curso introductorio de quimica a nivel universitario

    NASA Astrophysics Data System (ADS)

    Costa Diaz, Agnes

    El estudio que se presenta es de caracter cualitativo, un estudio multicasos donde se estudia la estructura logica utilizada por cuatro (4) profesores universitarios que ensenan el curso introductorio de quimica, en la planificacion, presentacion y evaluacion del tema sobre el comportamiento de los gases. Se utilizaron varias fuentes de informacion como: cuestionarios de profesores y estudiantes, entrevistas, grabaciones videomagnetofonicas, materiales didacticos y una prueba conceptual, entre otros. La informacion recopilada fue analizada de acuerdo al orden logico del contenido presentado, el estilo de ensenanza del profesor, las tecnicas y estrategias utilizadas para el desarrollo de destrezas de pensamiento, el ambiente fisico en el salon de clase y los instrumentos de evaluacion y avaluo. El estudio demuestra que lo que los profesores piensan y planifican para hacer sus presentaciones no necesariamente es lo que ocurre en el salon de clases. El desarrollo de destrezas de pensamiento, que constituye una prioridad de los profesores, no se elaboran efectivamente. El uso de las estrategias de resolucion de problemas numericos predomino. La participacion del estudiante en el salon de clases fue limitada y no se logro demostrar el desarrollo de las destrezas de pensamiento deseadas. Aunque los profesores tienen su propio estilo de ensenanza, el orden logico del contenido presentado en clase fue el mismo o siguio muy de cerca el orden establecido por el libro de texto. Los profesores utilizaron preferentemente la tiza y la pizarra para sus presentaciones y la dinamica en el salon de clases fue esencialmente tradicional. Los profesores hicieron su presentacion y los estudiantes copiaron pasivamente la informacion. Las evaluaciones de los estudiantes fueron esencialmente, pruebas escritas de seleccion multiple de acuerdo con el estilo en que se les enseno. El avaluo fue casi inexistente. La prueba conceptual administrada revela un aprendizaje pobre en los conceptos mas

  8. Kawasaki disease and immunisation: A systematic review.

    PubMed

    Phuong, Linny Kimly; Bonetto, Caterina; Buttery, Jim; Pernus, Yolanda Brauchli; Chandler, Rebecca; Felicetti, Patrizia; Goldenthal, Karen L; Kucuku, Merita; Monaco, Giuseppe; Pahud, Barbara; Shulman, Stanford T; Top, Karina A; Trotta, Francesco; Ulloa-Gutierrez, Rolando; Varricchio, Frederick; de Ferranti, Sarah; Newburger, Jane W; Dahdah, Nagib; Singh, Surjit; Bonhoeffer, Jan; Burgner, David

    2017-03-27

    Kawasaki disease is a complex and potentially serious condition. It has been observed in temporal relation to immunisation. We conducted a systematic literature review using various reference sources to review the available evidence published in the literature. We identified twenty seven publications reporting a temporal association between immunisation and Kawasaki disease. We present a systematic review of data drawn from randomised controlled trials, observational studies, case series and reports, and reviews. Overall there was a lack of standardised case definitions, making data interpretation and comparability challenging. Although a temporal relationship between immunisation and Kawasaki disease is suggested, evidence for an increased risk or a causal association is lacking. Implementation of a standardised Kawasaki disease case definition would increase confidence in the findings and add value to future studies of pre- or post-licensure vaccine safety studies. Copyright © 2016. Published by Elsevier Ltd.

  9. Serum adipokine profiles in Kawasaki disease.

    PubMed

    Kemmotsu, Yasushi; Saji, Tsutomu; Kusunoki, Natsuko; Tanaka, Nahoko; Nishimura, Chiaki; Ishiguro, Akira; Kawai, Shinichi

    2012-02-01

    Adipokines are cytokines derived from adipose tissue. Recently it has been established that adipokines are closely linked to the pathophysiology of not only metabolic diseases, such as diabetes mellitus, obesity, and atherosclerosis, but also to inflammation and immune diseases. In this study we measured serum levels of adipokines in patients with acute Kawasaki disease to investigate the role of adipokines in the pathophysiology of Kawasaki disease. Serum resistin, high-molecular-weight (HMW) adiponectin, leptin, and visfatin levels were measured by enzyme-linked immunosorbent assay in a total of 117 subjects: 56 patients with acute Kawasaki disease, 30 healthy children, and 31 patients with acute infectious diseases. Serum resistin levels in patients with Kawasaki disease were significantly higher than those of healthy children and patients with acute infectious diseases. In contrast, mean serum HMW adiponectin, leptin, and visfatin levels in patients with Kawasaki disease exhibited no statistically significant differences compared with those in healthy children and patients with infectious diseases. Serum resistin levels decreased significantly after administration of intravenous immune globulin. Serum resistin levels on admission were significantly higher in nonresponders compared with responders to intravenous immune globulin therapy. A multivariate model revealed that C-reactive protein was a factor that was significantly related to elevated serum resistin level in patients with Kawasaki disease. In patients with Kawasaki disease, serum resistin levels were elevated, but decreased to nearly normal after intravenous administration of immune globulin. In contrast, serum HMW adiponectin, leptin, and visfatin levels showed no statistically significant changes. These findings suggest that resistin plays an important role, while other adipokines do not play a major role, in the pathogenesis of Kawasaki disease.

  10. Seasonality and Temporal Clustering of Kawasaki Syndrome

    PubMed Central

    Burns, Jane C.; Cayan, Daniel R.; Tong, Garrick; Bainto, Emelia V.; Turner, Christena L.; Shike, Hiroko; Kawasaki, Tomisaku; Nakamura, Yosikazu; Yashiro, Mayumi; Yanagawa, Hiroshi

    2010-01-01

    Background The distribution of a syndrome in space and time may suggest clues to its etiology. The cause of Kawasaki syndrome, a systemic vasculitis of infants and children, is unknown, but an infectious etiology is suspected. Methods Seasonality and clustering of Kawasaki syndrome cases were studied in Japanese children with Kawasaki syndrome reported in nationwide surveys in Japan. Excluding the years that contained the 3 major nationwide epidemics, 84,829 cases during a 14-year period (1987–2000) were analyzed. To assess seasonality, we calculated mean monthly incidence during the study period for eastern and western Japan and for each of the 47 prefectures. To assess clustering, we compared the number of cases per day (daily incidence) with a simulated distribution (Monte Carlo analysis). Results Marked spatial and temporal patterns were noted in both the seasonality and deviations from the average number of Kawasaki syndrome cases in Japan. Seasonality was bimodal with peaks in January and June/July and a nadir in October. This pattern was consistent throughout Japan and during the entire 14-year period. Some years produced very high or low numbers of cases, but the overall variability was consistent throughout the entire country. Temporal clustering of Kawasaki syndrome cases was detected with nationwide outbreaks. Conclusions Kawasaki syndrome has a pronounced seasonality in Japan that is consistent throughout the length of the Japanese archipelago. Temporal clustering of cases combined with marked seasonality suggests an environmental trigger for this clinical syndrome. PMID:15703537

  11. Seasonality and temporal clustering of Kawasaki syndrome.

    PubMed

    Burns, Jane C; Cayan, Daniel R; Tong, Garrick; Bainto, Emelia V; Turner, Christena L; Shike, Hiroko; Kawasaki, Tomisaku; Nakamura, Yosikazu; Yashiro, Mayumi; Yanagawa, Hiroshi

    2005-03-01

    The distribution of a syndrome in space and time may suggest clues to its etiology. The cause of Kawasaki syndrome, a systemic vasculitis of infants and children, is unknown, but an infectious etiology is suspected. Seasonality and clustering of Kawasaki syndrome cases were studied in Japanese children with Kawasaki syndrome reported in nationwide surveys in Japan. Excluding the years that contained the 3 major nationwide epidemics, 84,829 cases during a 14-year period (1987-2000) were analyzed. To assess seasonality, we calculated mean monthly incidence during the study period for eastern and western Japan and for each of the 47 prefectures. To assess clustering, we compared the number of cases per day (daily incidence) with a simulated distribution (Monte Carlo analysis). Marked spatial and temporal patterns were noted in both the seasonality and deviations from the average number of Kawasaki syndrome cases in Japan. Seasonality was bimodal with peaks in January and June/July and a nadir in October. This pattern was consistent throughout Japan and during the entire 14-year period. Some years produced very high or low numbers of cases, but the overall variability was consistent throughout the entire country. Temporal clustering of Kawasaki syndrome cases was detected with nationwide outbreaks. Kawasaki syndrome has a pronounced seasonality in Japan that is consistent throughout the length of the Japanese archipelago. Temporal clustering of cases combined with marked seasonality suggests an environmental trigger for this clinical syndrome.

  12. What Are the Signs and Symptoms of Kawasaki Disease?

    MedlinePlus

    ... Twitter. What Are the Signs and Symptoms of Kawasaki Disease? Major Signs and Symptoms One of the main symptoms during the early part of Kawasaki disease, called the acute phase, is fever. The fever ...

  13. Kawasaki disease: A brief history.

    PubMed

    Burns, J C; Kushner, H I; Bastian, J F; Shike, H; Shimizu, C; Matsubara, T; Turner, C L

    2000-08-01

    Tomisaku Kawasaki published the first English-language report of 50 patients with Kawasaki disease (KD) in 1974. Since that time, KD has become the leading cause of acquired heart disease among children in North America and Japan. Although an infectious agent is suspected, the cause remains unknown. However, significant progress has been made toward understanding the natural history of the disease and therapeutic interventions have been developed that halt the immune-mediated destruction of the arterial wall. We present a brief history of KD, review progress in research on the disease, and suggest avenues for future study. Kawasaki saw his first case of KD in January 1961 and published his first report in Japanese in 1967. Whether cases existed in Japan before that time is currently under study. The most significant controversy in the 1960s in Japan was whether the rash and fever sign/symptom complex described by Kawasaki was connected to subsequent cardiac complications in a number of cases. Pathologist Noboru Tanaka and pediatrician Takajiro Yamamoto disputed the early assertion of Kawasaki that KD was a self-limited illness with no sequelae. This controversy was resolved in 1970 when the first Japanese nationwide survey of KD documented 10 autopsy cases of sudden cardiac death after KD. By the time of the first English-language publication by Kawasaki in 1974, the link between KD and coronary artery vasculitis was well-established. KD was independently recognized as a new and distinct condition in the early 1970s by pediatricians Marian Melish and Raquel Hicks at the University of Hawaii. In 1973, at the same Hawaiian hospital, pathologist Eunice Larson, in consultation with Benjamin Landing at Los Angeles Children's Hospital, retrospectively diagnosed a 1971 autopsy case as KD. The similarity between KD and infantile periarteritis nodosa (IPN) was apparent to these pathologists, as it had been to Tanaka earlier. What remains unknown is the reason for the

  14. Conceptos Basicos Sobre el Gas Natural

    SciTech Connect

    2016-08-01

    El gas natural abastece cerca de 150.000 vehiculos en los Estados Unidos y aproximadamente 22 millones de vehiculos en todo el mundo. Los vehiculos de gas natural (NGV, por sus siglas en ingles) son una buena opcion para las flotas de vehiculos de alto kilometraje, tales como autobuses, taxis, vehiculos de recoleccion de basura, los cuales son alimentados centralmente u operan dentro de un area limitada o a lo largo de una ruta con estaciones de servicio de gas natural. Las ventajas del gas natural como combustible alternativo incluyen su disponibilidad interna, la red de distribucion establecida, un costo relativamente bajo, y los beneficios de las emisiones.

  15. Kawasaki disease - an Indian perspective.

    PubMed

    Singh, Surjit; Kawasaki, Tomisaku

    2009-07-01

    Kawasaki disease (KD) was first reported from Japan in 1967 by a young pediatrician, Tomisaku Kawasaki, while working at the Red Cross Hospital in Tokyo. Soon therafter, Marian Melish independently reported children with a similar clinical profile from Hawaii in the United States. KD has now been reported from all parts of the world, including several centers in India. Based on the epidemiology and clinical features, an infectious etiology has been suspected for long but no definitive causative agent has been implicated so far. Like many other vasculitides, the diagnosis of this condition is based on the recognition of a temporal sequence of clinical features, none of which is pathognomonic in isolation. KD is believed to be the commonest vasculitic disorder of children. Incidence rates as high as 60-150 per 100,000 children below 5 years of age have been reported from several countries. In India (as also perhaps in many other developing countries), however, majority of children with KD continue to remain undiagnosed probably because of the lack of awareness amongst pediatricians. The clinical features of KD can be confused with other common conditions like scarlet fever and the Stevens Johnson syndrome, if the clinician is not careful. Development of coronary artery abnormalities (CAA) is the hallmark of KD and accounts for most of the morbidity and mortality associated with the disease. Prompt recognition of the disease and early initiation of treatment with intravenous immunoglobulin (IVIG) results in significant reduction in the occurrence of CAA. It is, therefore, imperative for the pediatrician to diagnose and treat KD expeditiously. KD should be considered in the differential diagnosis of all febrile illnesses in young children where the fever persists for more than 5-7 days.

  16. Diagnosis and management of kawasaki disease.

    PubMed

    Saguil, Aaron; Fargo, Matthew; Grogan, Scott

    2015-03-15

    Kawasaki disease is an acute, systemic vasculitis that predominantly affects patients younger than five years. It represents the most prominent cause of acquired coronary artery disease in childhood. In the United States, 19 per 100,000 children younger than five years are hospitalized with Kawasaki disease annually. According to U.S. and Japanese guidelines, Kawasaki disease is a clinical diagnosis. Classic (typical) Kawasaki disease is diagnosed based on the presence of a fever lasting five or more days, accompanied by four out of five findings: bilateral conjunctival injection, oral changes such as cracked and erythematous lips and strawberry tongue, cervical lymphadenopathy, extremity changes such as erythema or palm and sole desquamation, and polymorphous rash. Incomplete (atypical) Kawasaki disease occurs in persons with fever lasting five or more days and with two or three of these findings. Transthoracic echocardiography is the diagnostic imaging modality of choice to screen for coronary aneurysms, although other techniques are being evaluated for diagnosis and management. Treatment for acute disease is intravenous immunoglobulin and aspirin. If there is no response to treatment, patients are given a second dose of intravenous immunoglobulin with or without corticosteroids or other adjunctive treatments. The presence and severity of coronary aneurysms and obstruction at diagnosis determine treatment options and the need, periodicity, and intensity of long-term cardiovascular monitoring for potential atherosclerosis.

  17. Sensorineural hearing loss in Kawasaki disease.

    PubMed

    Aggarwal, Varun; Etinger, Veronica; Orjuela, Andres F

    2016-01-01

    Kawasaki disease is a common nonspecific vasculitis seen in childhood. The most significant long-term sequela is coronary artery aneurysm. However, the spectrum of complications involves not only the heart, but also other organs such as the eyes, skin, kidneys, gallbladder, liver, and central nervous system. Sensorineural hearing loss (SNHL) is a relatively unrecognized complication of the disease. Although most of the complications (except coronary artery aneurysm) are self-limiting, SNHL can be persistent. It is, especially important in infants and young children who might not be able to report the hearing deficits and are most likely to have cognitive and speech delays if this hearing loss is not addressed in a timely manner. We report a child with Kawasaki disease who had SNHL during the 2(nd) week of the illness. The aim of this article is to briefly review the pathophysiology behind this hearing loss and strongly emphasize the importance of universal hearing evaluation in all children diagnosed with Kawasaki disease. This screening in children with Kawasaki disease may provide some timely intervention if needed. Since most Kawasaki disease patients will be seen by cardiologists, we hope to create more awareness about this complication to the cardiology community as well.

  18. Kawasaki Disease and Peripheral Gangrene in Infancy.

    PubMed

    Malekzadeh, Iran; Ziaee, Vahid; Sadrosadat, Taravat; Moardinejad, Mohammad-Hassan; Sayadpour-Zanjani, Keyhan

    2015-12-01

    Early diagnosis and treatment of Kawasaki disease as the most common cause of acquired heart disease in childhood, may significantly improve the prognosis. Diagnosing infantile Kawasaki (younger than a year) is difficult because of obscure symptoms; at the same time they are at the higher risk of coronary abnormalities. We report three infants with prolonged (more than 5 days) fever and peripheral gangrene without any other clinical manifestations of Kawasaki disease. Kawasaki was diagnosed due to dilation of coronary artery and other aortic branches, thrombocytosis, and rising of ESR and CRP. All patients were treated with high dose aspirin, IVIG and pulse therapy with methylprednisolone. Additionally, cytotoxic drugs or infliximab were used for two of them because of severe aneurysms in the aortic branches. All 3 patients received aspirin with anti-platelet aggregation dose and 2 patients heparin as an anti-coagulant agent for longtime. After adequate treatment, peripheral gangrene, arterial dilations and aneurysms improved, but during 12 months follow-up coronary aneurysms did not improve completely. Peripheral gangrene must be regarded as an important sign of infantile Kawasaki disease early treatment of which can prevent severe permanent coronary involvements and sequels.

  19. Kawasaki disease: etiopathogenesis and novel treatment strategies.

    PubMed

    Agarwal, Shreya; Agrawal, Devendra K

    2017-03-01

    Kawasaki disease is an acute febrile systemic vasculitis that predominantly occurs in children below five years of age. Its etiopathogenesis is still not clear, but it is thought to be a complex interplay of genetic factors, infections and immunity. Areas covered: This review article discusses in detail Kawasaki disease, with particular emphasis on the recent updates on its pathogenesis and upcoming alternate treatment options. Though self-limiting in many cases, it can lead to severe complications like coronary artery aneurysms and thrombo-embolic occlusions, and hence requires early diagnosis and urgent attention to avoid them. Intravenous immunoglobulin (IVIG) with or without aspirin has remained the sole treatment option for these cases, but 10-15% cases develop resistance to this treatment. Expert commentary: There is a need to develop additional treatment strategies for children with Kawasaki disease. Targeting different steps of pathogenesis could provide us with alternate therapeutic options.

  20. Kawasaki disease shock syndrome: a rare and severe complication of Kawasaki disease.

    PubMed

    Çakan, Mustafa; Gemici, Hakan; Aktay-Ayaz, Nuray; Keskindemirci, Gonca; Bornaun, Helen; İkizoğlu, Tarkan; Çeliker, Alpay

    2016-01-01

    Kawasaki disease is an acute systemic vasculitis that occurs most commonly in young children. It affects medium-sized muscular arteries and the coronary arteries are the predominant site of involvement. Morbidity and mortality is generally due to coronary artery aneurysms that develop during the chronic phase. Although it is well known that Kawasaki disease can cause myocarditis, tachycardia and heart failure during acute stage, Kawasaki disease shock syndrome has been recently described. It is characterized by hypotension, signs and symptoms of poor perfusion and a shock-like state. Herein we describe two cases of Kawasaki disease shock syndrome that were treated in the pediatric intensive care unit and followed a course without morbidity or mortality.

  1. Kawasaki disease in Australia, 1993-95

    PubMed Central

    Royle, J.; Williams, K.; Elliott, E.; Sholler, G.; Nolan, T.; Allen, R.; Isaacs, D.

    1998-01-01

    AIM—To describe the epidemiology, management, and rate of cardiac sequelae of Kawasaki disease in Australia.
DESIGN—Cases were notified to the Australian Paediatric Surveillance Unit, an active national surveillance scheme, from May 1993to June 1995.
RESULTS—139 cases of Kawasaki disease were confirmed. In 1994, the annual incidence was 3.7/100 000 children < 5 years old. Sixteen children were not admitted to hospital. Coronary artery abnormalities were reported in 35 (25%) children. Two patients were diagnosed at postmortem examination. Sixty six per cent of patients were diagnosed within 10 days of onset and 81% of these received intravenous gammaglobulin within 10 days. Forty five of the notified children did not fulfil the study criteria because of streptococcal infection or insufficient clinical criteria. One child with streptococcal infection had coronary artery dilatation.
CONCLUSION—Diagnosis of Kawasaki disease was delayed beyond 10 days in one third of patients, and almost 20% of children who could have received gammaglobulin within 10 days did not. The distinction between Kawasaki disease, streptococcal infection, and other possible diagnoses is problematic in some children.

 PMID:9534673

  2. Management of Kawasaki disease in adults.

    PubMed

    Denby, Kara J; Clark, Daniel E; Markham, Larry W

    2017-07-27

    Kawasaki disease is the most common childhood vasculitis in the USA and the most common cause of acquired cardiac disease in children in developed countries. Since the vast majority of Kawasaki disease initially presents at <5 years of age, many adult cardiologists are unfamiliar with the pathophysiology of this disease. This vasculitis has a predilection for coronary arteries with a high complication rate across the lifespan for those with medium to large coronary artery aneurysms. An inflammatory cascade produces endothelial dysfunction and damage to the vascular wall, leading to aneurysmal dilatation. Later, pseudonormalisation of the vascular lumen occurs through vascular remodelling and layering thrombus, but this does not necessarily indicate resolution of disease or reduction of risk for future complications. There is a growing prevalence of Kawasaki disease, making it increasingly relevant for adult cardiologists as this population transitions into adulthood. As the 2017 American Heart Association (AHA) and 2014 Japanese Circulation Society (JCS) guidelines emphasise, Kawasaki disease requires rigorous follow-up with cardiac stress testing and non-invasive imaging to detect progressive stenosis, thrombosis and luminal occlusion that may lead to myocardial ischaemia and infarction. Due to differences in disease mechanisms, coronary disease due to Kawasaki disease should be managed with different pharmacological and non-pharmacological treatment algorithms than atherosclerotic coronary disease. This review addresses gaps in the current knowledge of the disease and its optimal treatment, differences in the AHA and JCS guidelines, targets for future research and obstacles to transition of care from adolescence into adulthood. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  3. Extremely refractory Kawasaki disease with disseminated intravascular coagulation.

    PubMed

    Koh, Young Kwon; Lee, Jae Hee; Park, Yeong Bong

    2017-03-07

    Disseminated intravascular coagulation is a rare complication of Kawasaki disease and appears in <0.1% of Kawasaki disease patients. We report a case of refractory Kawasaki disease complicated with disseminated intravascular coagulation and giant coronary aneurysm. A 5-month-old boy presented with Kawasaki disease with coagulopathy. Although the coagulopathy improved after fresh-frozen plasma and antithrombin-III administration, the fever persisted despite two rounds of intravenous immunoglobulin, along with intravenous methylprednisolone pulse therapy and infliximab administration. Despite all efforts to treatment, the patient had giant coronary aneurysms and died suddenly.

  4. Erythema at BCG Inoculation Site in Kawasaki Disease Patients.

    PubMed

    Rezai, Mohammad Sadegh; Shahmohammadi, Soheila

    2014-08-01

    Kawasaki disease is an acute, self-limiting childhood systemic vasculitis with unknown etiology. Because there is no diagnostic test for Kawasaki disease, the diagnosis is based on clinical criteria. An important clinical sign that is not included in the classical clinical criteria for Kawasaki disease is a reaction at the Bacille Calmette-Guérin inoculation site that are present in about 30-50% of Kawasaki disease patients. of this review was to highlight the usefulness of the inflammation at the Bacille Calmette-Guérin inoculation site for early diagnosis of Kawasaki disease, we conducted a literature review on Medline in PubMed area, Google scholar, Magiran and Scientific Information Database using the search terms "Kawasaki disease, Erythema, BCG, inoculation site, children, cardiac complications, coronary artery lesion, aneurysm, incomplete Kawasaki in 2013. A total of 15 articles had been found. Erythema at the Bacille Calmette-Guérin inoculation site was found in 49.87% of Kawasaki disease patients. Coronary artery abnormalities were found in 10.3% of cases. According to this review, BCGitis is more prevalent than cervical lymphadenopathy and rash and it can be a useful criterion in the diagnosis of incomplete Kawasaki disease in cases not fulfills the classic criteria of at least four of the five findings.

  5. Recognition and management of Kawasaki disease

    PubMed Central

    Han, R K; Sinclair, B; Newman, A; Silverman, E D; Taylor, G W; Walsh, P; McCrindle, B W

    2000-01-01

    Kawasaki disease is the leading cause of acquired heart disease in children in the developed world, with coronary artery aneurysms occurring in up to 25% of untreated cases. The mean annual incidence of Kawasaki disease across Canada is about 13 per 100,000 children less than 5 years of age, based on hospital discharge data from 1990 to 1995. The cause remains unknown, and the diagnosis is based on the same clinical criteria used to describe the disease over 30 years ago. However, nonspecific clinical features, evolving presentations and atypical or incomplete presentations make early diagnosis and timely treatment difficult. Delays in diagnosis and treatment, which occur more frequently in older children, are associated with an increased risk of coronary artery aneurysms. Hence, high diagnostic suspicion and prompt referral are required to reduce the rate of cardiac complications. PMID:10750471

  6. Update on etio and immunopathogenesis of Kawasaki disease.

    PubMed

    Takahashi, Kei; Oharaseki, Toshiaki; Yokouchi, Yuki

    2014-01-01

    This review first discusses the pathogenesis of Kawasaki disease based on the results of recently performed studies aimed at identifying Kawasaki disease-susceptibility genes and the results of analyses of the immune system. Following that, we discuss the findings generated using a murine Kawasaki disease arteritis model and speculate regarding the mechanism of Kawasaki disease onset based on immune function aberrations seen in that model. Recent advances in gene analysis studies of Kawasaki disease are contributing not only to prediction of disease susceptibility but also to improving our understanding of the pathogenesis of Kawasaki disease and development of new improved therapies. In addition, Th17/Treg imbalance is observed in patients with acute-phase Kawasaki disease. Th17/Treg imbalance may be an important factor causing disturbed immunological function. IL-17 induced by Th17 cells have proinflammatory properties and act on inflammatory cells, thereby inducing expression of cytokines and chemokines and resulting in tissue inflammation. Kawasaki disease vasculitis may be triggered by aberrant activation of inflammatory cytokines mediated by IL-17 that is produced by Th17 cells that have been activated by some infectious agent(s).

  7. Risk of coronary artery involvement in Kawasaki disease.

    PubMed

    Soriano-Ramos, María; Martínez-Del Val, Elena; Negreira Cepeda, Sagrario; González-Tomé, María I; Cedena Romero, Pilar; Fernández-Cooke, Elisa; Albert de la Torre, Leticia; Blázquez-Gamero, Daniel

    2016-04-01

    Kawasaki disease refers to systemic vasculitis with risk of coronary artery disease. Our objective is to identify risk factors associated with coronary artery disease in patients with complete and incomplete Kawasaki disease. Descriptive, retrospective study conducted in patients diagnosed with Kawasaki disease in a tertiary-care hospital between 2008 and 2014. The American Heart Association diagnostic criteria were used to define complete and incomplete Kawasaki disease. Thirty-one children were diagnosed with Kawasaki disease; 24 met the criteria for the complete form, and 7, for the incomplete form of this condition. Five had coronary artery disease. One of them had incomplete Kawasaki disease (1/7= 14.3%), and the remaining four had the complete form (4/24= 16.7%). No significant differences were found between both groups (p= 1.0). Patients with coronary artery involvement had a higher C-reactive protein level (median: 16.2 mg/dL versus 8.4 mg/dL, p= 0.047) and lower albuminemia (median: 3.2 mg/dL versus 3.99 mg/dL, p= 0.002). The risk of coronary artery involvement in incomplete Kawasaki disease is similar to that in complete Kawasaki disease; therefore, in patients with the incomplete form, immunoglobulin therapy should not be delayed. In our population, C-reactive protein and albumin levels were related to a higher risk of coronary artery involvement. Sociedad Argentina de Pediatría.

  8. [Aftermaths of lesions of coronary arteries in Kawasaki disease].

    PubMed

    Vostokova, A A; Grunina, E A; Klemenov, A V

    2016-01-01

    Kawasaki disease, also known as cutaneous-mucous-glandular mucocutaneous glandular syndrome, is acute systemic vasculitis of small-to-medium calibre arteries, frequently involving coronary arteries, affect almost exceptionally children, with reports concerning cases of Kawasaki syndrome in 20-to-30-year-old adults being extremely rare. The most serious manifestation of Kawasaki disease is coronaritis and formation of coronary artery aneurysms. The dynamics of the formed coronary aneurysms and, consequently, the fate of patients may be different. Thrombosis of an aneurysm in the early period of the disease and stenosing of the affected coronary artery later on present possible complications of Kawasaki disease and potential causes of myocardial infection in young adults. Increased risk of coronary artery thromboses in Kawasaki disease is conditioned by a decrease in velocity of blood flow and its turbulent pattern in the aneurysms, endothelial dysfunction due to currently existing or endured coronaritis and thrombocytosis typical of this pathology. Predisposing factors of coronary artery stenosing are unfavourable haemodynamic conditions appearing at the sites of the "entry" and "exit" of the aneurysm. Described herein are two case reports of myocardial infarction, one of which being a complication of an acute case of Kawasaki disease in a 29-year-old patient, with the second one being a consequence of coronary artery stenosing in a 25-year-old patient who had endured Kawasaki disease in his childhood.

  9. Kawasaki Disease Presenting as Acute Intestinal Obstruction

    PubMed Central

    Lone, Yasir Ahmad; Menon, Jagadeesh; Menon, Prema; Vaiphei, Kim; Narasimha Rao, Katragadda Lakshmi; Thapa, Baburam; Gupta, Kirti

    2017-01-01

    Kawasaki disease (KD) is an acute febrile illness of childhood associated with vasculitis of medium-sized arteries especially the coronary arteries. Typical clinical features involving the skin, mucous surfaces, etc., occur sequentially over a few days. We report a rare presentation of KD as a surgical abdomen in a 2-year-old boy. Awareness of this presentation is important as it can otherwise lead to a delay in starting potentially life-saving intervention like intravenous immunoglobulins for cardiac complications kept cryptic by the manifest acute abdomen. PMID:28694577

  10. Unusual imaging presentation of infantile atypical Kawasaki disease.

    PubMed

    Kumar, Nishith; Mittal, Mahesh Kumar; Sinha, Mukul; Gupta, Arpita; Thukral, Brij Bhushan

    2016-01-01

    Kawasaki disease is a systemic medium vessel vasculitis of unknown etiology affecting children under 5 years of age. There are no specific diagnostic tests, and thus, the diagnosis of the disease is primarily made on the basis of clinical criteria. Unusual presentations of Kawasaki disease have been variably reported from different parts of the world. However, presentation of the disease in the form of peripheral thromboembolism and florid non-coronary aneurysms has rarely been described This report describes the imaging findings in infantile atypical Kawasaki disease with aneurysms of multiple medium-sized arteries, including coronary arteries, emphasizing the detection of clinically silent aneurysms in the disease.

  11. Cardiac magnetic resonance feature tracking in Kawasaki disease convalescence

    PubMed Central

    Bratis, Konstantinos; Hachmann, Pauline; Child, Nicholas; Krasemann, Thomas; Hussain, Tarique; Mavrogeni, Sophie; Botnar, Rene; Razavi, Reza; Greil, Gerald

    2017-01-01

    Objective: The objective of this study was to determine whether left ventricular (LV) myocardial deformation indices can detect subclinical abnormalities in Kawasaki disease convalescence. We hypothesized that subclinical myocardial abnormalities due to inflammation represent an early manifestation of the disease that persists in convalescence. Background: Myocardial inflammation has been described as a global finding in the acute phase of Kawasaki disease. Despite normal systolic function by routine functional measurements, reduced longitudinal strain and strain rate have been detected by echocardiography in the acute phase. Methods and Results: Peak systolic LV myocardial longitudinal, radial, and circumferential strain and strain rate were examined in 29 Kawasaki disease convalescent patients (15 males; mean [standard deviation] age: 11 [6.6] years; median interval from disease onset: 5.8 [5.4] years) and 10 healthy volunteers (5 males; mean age: 14 [3.8] years) with the use of cardiac magnetic resonance (CMR) feature tracking. Routine indices of LV systolic function were normal in both groups. Comparisons were made between normal controls and (i) the entire Kawasaki disease group, (ii) Kawasaki disease subgroup divided by coronary artery involvement. Average longitudinal and circumferential strain at all levels was lower in patients compared to normal controls. In subgroup analysis, both Kawasaki disease patients with and without a history of coronary involvement had similar longitudinal and circumferential strain at all levels and lower when compared to controls. There were lower circumferential and longitudinal values in Kawasaki disease patients with persisting coronary artery lesions when compared to those with regressed ones. Conclusion: In this CMR study in Kawasaki disease convalescent patients with preserved routine functional indices, we detected lower circumferential and longitudinal strain values compared to normal controls, irrespective of the

  12. Cardiac magnetic resonance feature tracking in Kawasaki disease convalescence.

    PubMed

    Bratis, Konstantinos; Hachmann, Pauline; Child, Nicholas; Krasemann, Thomas; Hussain, Tarique; Mavrogeni, Sophie; Botnar, Rene; Razavi, Reza; Greil, Gerald

    2017-01-01

    The objective of this study was to determine whether left ventricular (LV) myocardial deformation indices can detect subclinical abnormalities in Kawasaki disease convalescence. We hypothesized that subclinical myocardial abnormalities due to inflammation represent an early manifestation of the disease that persists in convalescence. Myocardial inflammation has been described as a global finding in the acute phase of Kawasaki disease. Despite normal systolic function by routine functional measurements, reduced longitudinal strain and strain rate have been detected by echocardiography in the acute phase. Peak systolic LV myocardial longitudinal, radial, and circumferential strain and strain rate were examined in 29 Kawasaki disease convalescent patients (15 males; mean [standard deviation] age: 11 [6.6] years; median interval from disease onset: 5.8 [5.4] years) and 10 healthy volunteers (5 males; mean age: 14 [3.8] years) with the use of cardiac magnetic resonance (CMR) feature tracking. Routine indices of LV systolic function were normal in both groups. Comparisons were made between normal controls and (i) the entire Kawasaki disease group, (ii) Kawasaki disease subgroup divided by coronary artery involvement. Average longitudinal and circumferential strain at all levels was lower in patients compared to normal controls. In subgroup analysis, both Kawasaki disease patients with and without a history of coronary involvement had similar longitudinal and circumferential strain at all levels and lower when compared to controls. There were lower circumferential and longitudinal values in Kawasaki disease patients with persisting coronary artery lesions when compared to those with regressed ones. In this CMR study in Kawasaki disease convalescent patients with preserved routine functional indices, we detected lower circumferential and longitudinal strain values compared to normal controls, irrespective of the coronary artery status.

  13. T-Helper Cytokine Profiles in Patients with Kawasaki Disease.

    PubMed

    Lee, Sang Bum; Kim, Young Hyun; Hyun, Myung Chul; Kim, Yeo Hyang; Kim, Hee Sun; Lee, Young Hwan

    2015-11-01

    Kawasaki disease is an acute systemic vasculitis of which pathogenesis suspected is caused by immune dysregulation. The goal of this study is to evaluate the activation pattern of T helper cell type 1 (Th1) and T helper cell type 2 (Th2) in patients with Kawasaki disease. Prospective study of 60 patients (male 36, female 24) with diagnosis of Kawasaki disease were enrolled. One hundred and eighty blood samples from these patients were collected according to the different clinical stages {before initial intravenous immunoglobulin (IVIG), 5 days after initial IVIG, 2 months after initial IVIG}. The plasma level of Th1 cytokines; interferon-gamma (IFN-γ) & interleukin (IL)-2 and Th2 cytokines; IL-4 & IL-10 were measured by enzyme-liked immunosorbent assay. In all patients, the plasma level of Th1 cytokines (IFN-γ, IL-2) and Th2 cytokines (IL-4 and IL-10) were markedly elevated during the acute stage of Kawasaki disease. Since then, the plasma level of all these cytokines decreased significantly along with the process of clinical stages. Regardless of the existence of coronary artery lesion or no response to initial IVIG treatment, there were no significant differences between them. These data suggest that both Th1 and Th2 cells may be activated simultaneously during the acute stage of Kawasaki disease. Further studies are therefore required to establish the difference of activation pattern of T helper cells between Kawasaki disease and other inflammatory diseases.

  14. Preventing Long-Term Cardiac Damage in Pediatric Patients With Kawasaki Disease.

    PubMed

    Williams, Kelly

    Kawasaki disease is currently the leading cause of long-term cardiac damage in pediatric patients in the United States. Kawasaki disease is diagnosed based on symptomatology and by ruling out other etiology. There is a significant need for an improved, standardized treatment protocol for patients diagnosed with Kawasaki disease and a more rapid initiation of treatment for these patients. Decreasing the cardiac damage caused by Kawasaki disease with timely diagnosis and treatment needs be a principal goal.

  15. Four cases with Kawasaki disease and viral infection: aetiology or association.

    PubMed

    Giray, Tuba; Biçer, Suat; Küçük, Öznur; Çöl, Defne; Yalvaç, Zerrin; Gürol, Yeşim; Yilmaz, Gülden; Saç, Ahmet; Mogol, Yigit

    2016-12-01

    The aetiology of Kawasaki disease has not yet been precisely determined. It has been associated with a variety of bacterial and viral agents. Some viruses including human adenovirus, coronavirus, and parainfluenza virus type 3 have been isolated from patients with Kawasaki disease. Clinical presentation of patients with human coronavirus and adenovirus infections mimics Kawasaki disease. In addition, these viruses may also be detected in Kawasaki disease as a coinfection. In this report, we present four Kawasaki disease patients infected with adenovirus, coronavirus OC43/HKU1 and parainfluenza virus type 3.

  16. Facial palsy in Kawasaki disease. Report of two cases and a review.

    PubMed

    Hattori, T; Tokugawa, K; Fukushige, J; Tasaki, H; Nanri, T; Ueda, K

    1987-11-01

    A case of facial palsy was reported initially in 1974 by Murayama as one of the neurological manifestations in Kawasaki disease. Thereafter, an additional nine case have been documented in Japan. This facial palsy, in the revised "Diagnostic Guideline of Kawasaki Disease" released in 1984, has been added recently as one of the neurological signs and symptoms of Kawasaki disease. This is a report on two cases of Kawasaki disease showing facial palsy with indurative oedema during their clinical course, and also a clinical review of the ten previously reported cases of facial palsy complicating Kawasaki disease.

  17. Hemophagocytosis in the Acute Phase of Fatal Kawasaki Disease in a 4 Month-Old Girl.

    PubMed

    Doğan, Vehbi; Karaaslan, Erhan; Özer, Samet; Gümüşer, Rüveyda; Yılmaz, Resul

    2016-07-01

    Kawasaki disease is a systemic vasculitis predominately affecting coronary arteries. Hemophagocytic lymphohistiocytosis can complicate the course of Kawasaki disease. Rare cases of secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of Kawasaki disease have been reported. We report here a 4 month-old girl with diffuse coronary ectasia and secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of incomplete Kawasaki disease. Due to the large overlap in clinical symptoms, the presence of atypical findings for Kawasaki disease should suggest the possible diagnosis of hemophagocytic lymphohistiocytosis in these patients.

  18. Sensorineural hearing loss in patients with Kawasaki disease.

    PubMed

    Park, Sun Young; Kim, Young Hyun; Kim, Yeo Hyang; Hyun, Myung Chul; Lee, Young Hwan

    2015-11-01

    Kawasaki disease involves acute febrile systemic vasculitis that can cause a variety of symptoms by affecting various organs. Here, we aimed to evaluate the prevalence, causes, and prognosis of sensorineural hearing loss (SNHL) occurring in children with Kawasaki disease. Patients who were diagnosed with Kawasaki disease and received inpatient treatment in the Pediatrics Department at one of three university hospitals in Daegu city from February 2012 to September 2012 were enrolled in the study. The clinical features, hematological results, echocardiography results, audiometry results, and aspirin and salicylic acid serum levels of the patients were evaluated. Of the 59 children enrolled in the study, three showed mild bilateral SNHL on audiometry tests conducted after 48 hours of defervescence; these patients demonstrated normal patterns of recovery on follow-up tests 8 weeks later. Aspirin serum levels were significantly higher in the SNHL group after 48 hours of afebrile condition with high dose aspirin intake (P=0.034). However, no significant differences were found in other laboratory tests or for fever duration (P>0.05). Upon echocardiography, coronary artery abnormality was observed in 9 cases, but none of these patients showed hearing loss. The results indicate that SNHL in children with Kawasaki disease might occur during treatment of the acute phase; this SNHL usually involves mild bilateral hearing loss and recovers naturally. However, this study suggests that determination of the causes and clinical implications of hearing loss in Kawasaki disease requires long-term follow-up studies with more cases.

  19. Can Coronary Artery Involvement in Kawasaki Disease be Predicted?

    PubMed

    Ghelani, Sunil J; Kwatra, Neha S; Spurney, Christopher F

    2013-03-26

    Coronary artery involvement is seen in approximately 15-20% of children with Kawasaki disease. There is conflicting literature regarding the clinical and laboratory findings associated with coronary artery involvement. In this retrospective study, we attempt identification of predictive factors for coronary artery involvement at our institute and review the existing literature. A review of 203 patients (65% males) with Kawasaki disease was performed, of whom 33 (16.3%) had coronary artery involvement. High erythrocyte sedimentation rate, high platelet count, low hematocrit, low albumin levels, and refractory Kawasaki disease showed significant association with coronary artery involvement. High erythrocyte sedimentation rate and refractory Kawasaki disease were found to be independent predictors of coronary artery involvement. Review of literature suggested a wide range of coronary involvement (<5% to >60%), and highly conflicting clinical and laboratory associations. It remains difficult to accurately determine risk of coronary artery involvement, although some laboratory markers may provide information that is helpful for parental counseling and clinical follow up. Future identification of novel biomarkers and host predispositions may further our understanding of coronary artery risks and help personalize therapy for Kawasaki disease.

  20. Angioscopic observation of coronary lesions in Kawasaki disease

    NASA Astrophysics Data System (ADS)

    Ishikawa, Hiromi

    1993-05-01

    Kawasaki disease is an acute systematic angiitis, specifically affecting the coronary arteries in young children. Coronary aneurysms develop in 10 to 15% of patients with Kawasaki disease. The coronary aneurysm frequently changes into coronary stenosis and obstruction. Hitherto, we could observe and access the severity of these coronary lesions with the use of two dimensional echocardiography and selective coronary angiography. Angiography is the most sensitive means available for diagnosing significant coronary lesions and their severity. However, it is insensitive for detecting intraluminal minute and pathologic changes such as intimal thickening, regeneration of endothelium and thrombus. Recent advance in fiberscopie technology, enabled us to observe the inside of coronary artery percutaneously. We tried to use this angioscopic technique for the observation of coronary lesions in eight patients with Kawasaki disease at chronic stage.

  1. Pathogenetic determinants in Kawasaki disease: the haematological point of view.

    PubMed

    Del Principe, Domenico; Pietraforte, Donatella; Gambardella, Lucrezia; Marchesi, Alessandra; Tarissi de Jacobis, Isabella; Villani, Alberto; Malorni, Walter; Straface, Elisabetta

    2017-04-01

    Kawasaki disease is a multisystemic vasculitis that can result in coronary artery lesions. It predominantly affects young children and is characterized by prolonged fever, diffuse mucosal inflammation, indurative oedema of the hands and feet, a polymorphous skin rash and non-suppurative lymphadenopathy. Coronary artery involvement is the most important complication of Kawasaki disease and may cause significant coronary stenosis resulting in ischemic heart disease. The introduction of intravenous immunoglobulin decreases the incidence of coronary artery lesions to less than 5%. The etiopathogenesis of this disease remains unclear. Several lines of evidence suggest that an interplay between a microbial infection and a genetic predisposition could take place in the development of the disease. In this review, we summarize the state of the art of pathogenetic mechanisms of Kawasaki disease underscoring the relevance of haematological features as a novel field of investigation.

  2. [Kawasaki disease: interdisciplinary and intersocieties consensus (clinical guidelines). Brief version].

    PubMed

    2016-08-01

    Kawasaki disease is an acute self-limiting systemic vasculitis. It is the most common cause of acquired heart disease, with the risk of developing coronary artery aneurysms, myocardial infarction and sudden death. Diagnosis is based on the presence of fever in addition to other clinical criteria. The quarter of the Kawasaki disease patients have "incomplete" presentation. Treatment with intravenous immunoglobulin within ten days of fever onset improves clinical outcomes and reduces the incidence of coronary artery dilation to less than 5%. Non-responders to standard therapy have shown a successful response with the use of corticosteroids and/or biological agents. The long-term management must be delineated according to the degree of coronary involvement in a multidisciplinary manner. To facilitate the pediatrician's diagnosis, treatment and monitoring of Kawasaki disease, a group of experts from the Argentine Society of Pediatrics and the Argentine Society of Cardiology carried out a consensus to develop practical clinical guidelines.

  3. Tomographic myocardial perfusion scintigraphy in children with Kawasaki disease

    SciTech Connect

    Spielmann, R.P.; Nienaber, C.A.; Hausdorf, G.; Montz, R.

    1987-12-01

    Myocardial infarction and stenotic coronary lesions are serious late complications in children with Kawasaki disease. For the noninvasive assessment of myocardial perfusion, dipyridamole-redistribution /sup 201/Tl emission computed tomography (ECT) was performed in seven children (age 2 8/12-8 7/12 yr) 3-20 mo after the acute stage of the disease. In all patients, coronary aneurysms had been demonstrated by cross-sectional echocardiography. The scintigrams of six children showed no significant regional reduction of myocardial thallium uptake. These children had remained asymptomatic since the acute stage of Kawasaki disease. Persistent and transient thallium defects were present in one child with documented myocardial infarction. For this patient, obstruction of corresponding coronary vessels was confirmed by contrast angiography. It is suggested, that /sup 201/Tl ECT after dipyridamole-induced vasodilation may be used as a safe alternative to invasive coronary angiography for follow-up investigations in patients with Kawasaki disease.

  4. T-Helper Cytokine Profiles in Patients with Kawasaki Disease

    PubMed Central

    Lee, Sang Bum; Kim, Young Hyun; Hyun, Myung Chul; Kim, Yeo Hyang; Kim, Hee Sun

    2015-01-01

    Background and Objectives Kawasaki disease is an acute systemic vasculitis of which pathogenesis suspected is caused by immune dysregulation. The goal of this study is to evaluate the activation pattern of T helper cell type 1 (Th1) and T helper cell type 2 (Th2) in patients with Kawasaki disease. Subjects and Methods Prospective study of 60 patients (male 36, female 24) with diagnosis of Kawasaki disease were enrolled. One hundred and eighty blood samples from these patients were collected according to the different clinical stages {before initial intravenous immunoglobulin (IVIG), 5 days after initial IVIG, 2 months after initial IVIG}. The plasma level of Th1 cytokines; interferon-gamma (IFN-γ) & interleukin (IL)-2 and Th2 cytokines; IL-4 & IL-10 were measured by enzyme-liked immunosorbent assay. Results In all patients, the plasma level of Th1 cytokines (IFN-γ, IL-2) and Th2 cytokines (IL-4 and IL-10) were markedly elevated during the acute stage of Kawasaki disease. Since then, the plasma level of all these cytokines decreased significantly along with the process of clinical stages. Regardless of the existence of coronary artery lesion or no response to initial IVIG treatment, there were no significant differences between them. Conclusion These data suggest that both Th1 and Th2 cells may be activated simultaneously during the acute stage of Kawasaki disease. Further studies are therefore required to establish the difference of activation pattern of T helper cells between Kawasaki disease and other inflammatory diseases. PMID:26617655

  5. Lie symmetries of the shigesada-Kawasaki-Teramoto system

    NASA Astrophysics Data System (ADS)

    Cherniha, Roman; Davydovych, Vasyl'; Muzyka, Liliia

    2017-04-01

    The Shigesada-Kawasaki-Teramoto system, which consists of two reaction-diffusion equations with variable cross-diffusion and quadratic nonlinearities, is considered. The system is the most important case of the biologically motivated model proposed by Shigesada et al. (J. Theor. Biol.79(1979) 83-99). A complete description of Lie symmetries for this system is derived. It is proved that the Shigesada-Kawasaki-Teramoto system admits a wide range of different Lie symmetries depending on coefficient values. In particular, the Lie symmetry operators with highly unusual structure are unveiled and applied for finding exact solutions of the relevant nonlinear system with cross-diffusion.

  6. Kawasaki disease and juvenile systemic lupus erythematosus.

    PubMed

    Diniz, J C; Almeida, R T; Aikawa, N E; Sallum, A M E; Sakane, P T; Silva, C A

    2012-01-01

    Kawasaki disease (KD) is a common vasculitis in childhood. To the authors' knowledge, only one case of juvenile systemic lupus erythematosus (JSLE)-like onset mimicking KD and another case of KD and JSLE association have previously been described. However, the prevalence of this association of the two diseases was not reported. Therefore, over 27 consecutive years, 5419 patients were followed at the Pediatric Rheumatology Unit and 271 (5%) of them met the ACR classification criteria for JSLE. Two (0.7%) of them were female. These also had KD according to European League against Rheumatism / Paediatric Rheumatology European Society (EULAR/PReS) consensus criteria and are described in this report. One case was a 13-year-old who presented all six KD criteria. Echocardiogram showed pericardial effusion, dilatation and tortuosity of right and left coronary, and her symptoms promptly improved after treatment with intravenous immunoglobulin (IVIG). Lupus diagnosis was established a few days later. Another case was a 4-year-old who had also met all six KD criteria, with improvement after IVIG, and lupus diagnosis was made 1 year later. In conclusion, the frequency of the association between these two autoimmune diseases was rare. The occurrence of a second autoimmune systemic disease in a patient with a history of KD should also be considered. Furthermore, the initial presentation of lupus may mimic KD.

  7. Kawasaki disease with facial nerve paralysis.

    PubMed

    Larralde, Margarita; Santos-Muñoz, Andrea; Rutiman, Ricardo

    2003-01-01

    Kawasaki disease (KD) is a multisystem disorder with varying clinical expression. We describe an instance of facial nerve paralysis in a patient with KD. A 5-month-old boy developed fever, irritability, and diarrhea, treated 8 days later with cefaclor and ibuprofen. Three days later a confluent, erythematous and papular rash appeared, his lips were reddened and swollen, and his white blood count and platelet count were 20,900/mm(3) and 558,000/mm(3), respectively. He was admitted to the hospital with a diagnosis of KD, and an echocardiogram showed a right coronary aneurysm. The patient then developed an acute, right-sided, facial nerve peripheral paralysis that resolved over the next 6 weeks. He was treated with intravenous immune globulin (IVIG) 2 g/kg and aspirin 100 mg/kg/day with improvement of signs and symptoms. This report documents facial nerve paralysis as an uncommon complication of KD and points out that it may be a marker of increased risk of cardiovascular disease in this disorder.

  8. Predicting Factors for Refractory Kawasaki Disease

    PubMed Central

    Do, Young-Sun; Kim, Ki-Won; Chun, Jin-Kyong; Cha, Byung Ho; Namgoong, Mee Kyung

    2010-01-01

    Background and Objectives About 10-15% of Kawasaki disease (KD) is refractory to intravenous immunoglobulin (IVIG) therapy. This study was designed to investigate the predicting factors for refractory KD. Subjects and Methods We reviewed retrospectively the clinical records of 77 patients with typical KD admitted at Wonju Christian Hospital from January, 2005, to December, 2008. The variance of laboratory and demographic parameters between the IVIG-responsive group and IVIG-resistant group were analyzed. Thirteen patients with urinary tract infections were randomly collected as a febrile control group. Results Among 77 patients diagnosed with complete KD, 13 patients (16.9%) were IVIG-resistant. The febrile period and hospital days were significantly longer in the IVIG-resistant group than IVIG-responsive group (p<0.001, p=0.002). Serum levels of albumin and sodium were significantly lower in the IVIG-resistant group (p=0.025). The Kobayashi score could differentiate these two groups (p=0.015). Fewer lymphocytes was observed during the subacute phase in the IVIG-resistant group (p=0.032). Coronary arterial dilatations (CADs) were observed in 10.9% (7/64) of IVIG-responders and 38.5% (5/13) of IVIG-resistant patients (p=0.038). Conclusion The percentage of neutrophils and lymphocytes in patients with KD, in addition to known risk factors for refractory KD, may help predict IVIG-resistance in patients with KD. PMID:20514335

  9. Predicting factors for refractory kawasaki disease.

    PubMed

    Do, Young-Sun; Kim, Ki-Won; Chun, Jin-Kyong; Cha, Byung Ho; Namgoong, Mee Kyung; Lee, Hae Yong

    2010-05-01

    About 10-15% of Kawasaki disease (KD) is refractory to intravenous immunoglobulin (IVIG) therapy. This study was designed to investigate the predicting factors for refractory KD. We reviewed retrospectively the clinical records of 77 patients with typical KD admitted at Wonju Christian Hospital from January, 2005, to December, 2008. The variance of laboratory and demographic parameters between the IVIG-responsive group and IVIG-resistant group were analyzed. Thirteen patients with urinary tract infections were randomly collected as a febrile control group. Among 77 patients diagnosed with complete KD, 13 patients (16.9%) were IVIG-resistant. The febrile period and hospital days were significantly longer in the IVIG-resistant group than IVIG-responsive group (p<0.001, p=0.002). Serum levels of albumin and sodium were significantly lower in the IVIG-resistant group (p=0.025). The Kobayashi score could differentiate these two groups (p=0.015). Fewer lymphocytes was observed during the subacute phase in the IVIG-resistant group (p=0.032). Coronary arterial dilatations (CADs) were observed in 10.9% (7/64) of IVIG-responders and 38.5% (5/13) of IVIG-resistant patients (p=0.038). The percentage of neutrophils and lymphocytes in patients with KD, in addition to known risk factors for refractory KD, may help predict IVIG-resistance in patients with KD.

  10. Recognising Kawasaki disease in UK primary care: a descriptive study using the Clinical Practice Research Datalink.

    PubMed

    Moore, Abigail; Harnden, Anthony; Mayon-White, Richard

    2014-08-01

    Kawasaki disease is a rare childhood illness that can present non-specifically, making it a diagnostic challenge. The clinical presentation of Kawasaki disease has not been previously described in primary care. To describe how children with an eventual diagnosis of Kawasaki disease initially present to primary care in the UK. The Clinical Practice Research Datalink was used to find cases coded as Kawasaki disease. Hospital Episode Statistics, hospital admissions, and hospital outpatient attendances were used to identify the children with a convincing diagnosis of Kawasaki disease. Questionnaires and a request for copies of relevant hospital summaries, discharge letters, and reports were sent to GPs of the 104 children with a diagnosis of Kawasaki disease between 2007 and 2011. Most children presented with few clinical features typical of Kawasaki disease. Of those with just one feature, a fever or a polymorphous rash were the most common. By the time that most children were admitted to hospital they had a more recognisable syndrome, with three or more clinical features diagnostic of Kawasaki disease. Most GPs did not consider Kawasaki disease among their differential diagnoses, but some GPs did suspect that the child's illness was unusual. The study highlighted the difficulty of early diagnosis, with most children having a non-specific presentation to primary care. GPs are encouraged to implement good safety netting, and to keep Kawasaki disease in mind when children present with fever and rashes. © British Journal of General Practice 2014.

  11. Giant coronary artery aneurysms complicating Kawasaki disease in Mexican children.

    PubMed

    Garrido-García, Luis M; Morán-Villaseñor, Edna; Yamazaki-Nakashimada, Marco A; Cravioto, Patricia; Galván, Fernando

    2017-08-29

    One of the most important complications of Kawasaki disease is the development of giant coronary aneurysms. Risk factors for their development are still not clear. A retrospective analysis was conducted at the National Institute of Paediatrics in Mexico City, Mexico. It included all patients with a diagnosis of acute Kawasaki disease between August, 1995 and August, 2015. Clinical and laboratory findings, as well as echocardiographic measurements, were recorded. Patients with giant coronary aneurysms (z-score⩾10) were compared with the rest of the patients. A value of p<0.05 was considered statistically significant. Odds ratios and their 95% confidence intervals were calculated to define risk factors. During the study period, 416 patients were diagnosed with Kawasaki disease. Of them, 34 developed giant coronary aneurysms during the acute stage of the disease. In the multivariate analysis, patients younger than 1 year, those with a higher duration of illness at the time of diagnosis, and those who received additional intravenous immunoglobulin showed a significantly higher frequency of giant coronary aneurysms. One of the main factors associated with the development of giant coronary aneurysms was the delay in the diagnosis of Kawasaki disease. This finding highlights the importance of maintaining a high suspicion of the disease, which would enable an early diagnosis and prompt treatment and decrease the risk for developing giant coronary aneurysms.

  12. Seasonality of Kawasaki disease: a global perspective.

    PubMed

    Burns, Jane C; Herzog, Lauren; Fabri, Olivia; Tremoulet, Adriana H; Rodó, Xavier; Uehara, Ritei; Burgner, David; Bainto, Emelia; Pierce, David; Tyree, Mary; Cayan, Daniel

    2013-01-01

    Understanding global seasonal patterns of Kawasaki disease (KD) may provide insight into the etiology of this vasculitis that is now the most common cause of acquired heart disease in children in developed countries worldwide. Data from 1970-2012 from 25 countries distributed over the globe were analyzed for seasonality. The number of KD cases from each location was normalized to minimize the influence of greater numbers from certain locations. The presence of seasonal variation of KD at the individual locations was evaluated using three different tests: time series modeling, spectral analysis, and a Monte Carlo technique. A defined seasonal structure emerged demonstrating broad coherence in fluctuations in KD cases across the Northern Hemisphere extra-tropical latitudes. In the extra-tropical latitudes of the Northern Hemisphere, KD case numbers were highest in January through March and approximately 40% higher than in the months of lowest case numbers from August through October. Datasets were much sparser in the tropics and the Southern Hemisphere extra-tropics and statistical significance of the seasonality tests was weak, but suggested a maximum in May through June, with approximately 30% higher number of cases than in the least active months of February, March and October. The seasonal pattern in the Northern Hemisphere extra-tropics was consistent across the first and second halves of the sample period. Using the first global KD time series, analysis of sites located in the Northern Hemisphere extra-tropics revealed statistically significant and consistent seasonal fluctuations in KD case numbers with high numbers in winter and low numbers in late summer and fall. Neither the tropics nor the Southern Hemisphere extra-tropics registered a statistically significant aggregate seasonal cycle. These data suggest a seasonal exposure to a KD agent that operates over large geographic regions and is concentrated during winter months in the Northern Hemisphere extra-tropics.

  13. [Geographic distribution of Kawasaki disease throughout Chile].

    PubMed

    Hoyos-Bachiloglu, Rodrigo; García, Álvaro; Morales, Pamela S; Cerda, Jaime; Talesnik, Eduardo; Borzutzky, Arturo

    2016-02-01

    Incidence of Kawasaki disease (KD) in Chile is rising, however the distribution of cases throughout Chile is unknown. To describe the epidemiology of KD in Chile between years 2001 and 2011, and study the geographic distribution of KD cases throughout the country. We reviewed national hospital discharge databases for KD cases (ICD10 code M30.3) in children < 18 years. KD admission rates per 100,000 children < 5 years were calculated for every commune, health district and region, as a proxy of KD incidence. 1,404 KD cases were registered with a national KD incidence rate of 8.7. KD incidence rate increased significantly from 5.9 in 2001-2003 to 10.4 in 2009-2011 (p < 0.001). Regions IX (Araucanía), Metropolitan and VI (O'Higgins) had the highest KD incidence (12.4, 11.1 and 10.5 respectively), and regions III (Atacama), II (Antofagasta) and XII (Magallanes), had the lowest incidence (0.8, 3.9 and 4, respectively). The Eastern Metropolitan Health District, the population with the highest socioeconomic status in Chile, had the highest KD incidence rate (19.8) and concentrated 23.9% of the country's hospital discharges for KD. KD incidence in Chile is heterogeneous, with concentration of caseloads in the central regions and especially in the Eastern Metropolitan Health District. Geographic variations of KD in Chile could be associated with real differences in incidence or with disparities in diagnostic opportunity, and access to specialists and tertiary healthcare centers.

  14. Seasonality of Kawasaki Disease: A Global Perspective

    PubMed Central

    Burns, Jane C.; Herzog, Lauren; Fabri, Olivia; Tremoulet, Adriana H.; Rodó, Xavier; Uehara, Ritei; Burgner, David; Bainto, Emelia; Pierce, David; Tyree, Mary; Cayan, Daniel

    2013-01-01

    Background Understanding global seasonal patterns of Kawasaki disease (KD) may provide insight into the etiology of this vasculitis that is now the most common cause of acquired heart disease in children in developed countries worldwide. Methods Data from 1970-2012 from 25 countries distributed over the globe were analyzed for seasonality. The number of KD cases from each location was normalized to minimize the influence of greater numbers from certain locations. The presence of seasonal variation of KD at the individual locations was evaluated using three different tests: time series modeling, spectral analysis, and a Monte Carlo technique. Results A defined seasonal structure emerged demonstrating broad coherence in fluctuations in KD cases across the Northern Hemisphere extra-tropical latitudes. In the extra-tropical latitudes of the Northern Hemisphere, KD case numbers were highest in January through March and approximately 40% higher than in the months of lowest case numbers from August through October. Datasets were much sparser in the tropics and the Southern Hemisphere extra-tropics and statistical significance of the seasonality tests was weak, but suggested a maximum in May through June, with approximately 30% higher number of cases than in the least active months of February, March and October. The seasonal pattern in the Northern Hemisphere extra-tropics was consistent across the first and second halves of the sample period. Conclusion Using the first global KD time series, analysis of sites located in the Northern Hemisphere extra-tropics revealed statistically significant and consistent seasonal fluctuations in KD case numbers with high numbers in winter and low numbers in late summer and fall. Neither the tropics nor the Southern Hemisphere extra-tropics registered a statistically significant aggregate seasonal cycle. These data suggest a seasonal exposure to a KD agent that operates over large geographic regions and is concentrated during winter

  15. Recent Advances in Kawasaki Disease - Proceedings of the 3rd Kawasaki Disease Summit, Chandigarh, 2014.

    PubMed

    Singh, Surjit; Sharma, Dhrubajyoti; Bhattad, Sagar; Phillip, Saji

    2016-01-01

    Kawasaki disease (KD) is the most common cause of acquired heart disease in children in Japan, North America and Europe. It is now being increasingly recognized from the developing countries as well. If not diagnosed and treated in time, KD can result in coronary artery abnormalities in approximately 15-25% cases. The long-term consequences of these abnormalities may manifest in adults as myocardial ischemia and congestive heart failure. Intravenous immunoglobulin (IVIg) remains the drug of choice for treatment of KD, but several new agents like infliximab, cyclosporine, glucocorticoids and statins are now being increasingly used in these patients. While echocardiography has been the preferred imaging modality hitherto, CT coronary angiography has emerged as an exciting new supplementary option and provides an entirely new dimension to this disease. The incidence of KD has shown a progressive increase in several countries and it is likely that this disease would impact public health programmes in the near future even in the developing countries.

  16. Consortium-Based Genetic Studies of Kawasaki Disease in Korea: Korean Kawasaki Disease Genetics Consortium.

    PubMed

    Lee, Jong-Keuk; Hong, Young Mi; Jang, Gi Young; Yun, Sin Weon; Yu, Jeong Jin; Yoon, Kyung Lim; Lee, Kyung-Yil; Kil, Hong-Rang

    2015-11-01

    In order to perform large-scale genetic studies of Kawasaki disease (KD) in Korea, the Korean Kawasaki Disease Genetics Consortium (KKDGC) was formed in 2008 with 10 hospitals. Since the establishment of KKDGC, there has been a collection of clinical data from a total of 1198 patients, and approximately 5 mL of blood samples per patient (for genomic deoxyribonucleic acid and plasma isolation), using a standard clinical data collection form and a nation-wide networking system for blood sample pick-up. In the clinical risk factor analysis using the collected clinical data of 478 KD patients, it was found that incomplete KD type, intravenous immunoglobulin (IVIG) non-responsiveness, and long febrile days are major risk factors for coronary artery lesions development, whereas low serum albumin concentration is an independent risk factor for IVIG non-responsiveness. In addition, we identified a KD susceptibility locus at 1p31, a coronary artery aneurysm locus (KCNN2 gene), and the causal variant in the C-reactive protein (CRP) promoter region, as determining the increased CRP levels in KD patients, by means of genome-wide association studies. Currently, this consortium is continually collecting more clinical data and genomic samples to identify the clinical and genetic risk factors via a single nucleotide polymorphism chip and exome sequencing, as well as collaborating with several international KD genetics teams. The consortium-based approach for genetic studies of KD in Korea will be a very effective way to understand the unknown etiology and causal mechanism of KD, which may be affected by multiple genes and environmental factors.

  17. Consortium-Based Genetic Studies of Kawasaki Disease in Korea: Korean Kawasaki Disease Genetics Consortium

    PubMed Central

    Hong, Young Mi; Jang, Gi Young; Yun, Sin Weon; Yu, Jeong Jin; Yoon, Kyung Lim; Lee, Kyung-Yil; Kil, Hong-Rang

    2015-01-01

    In order to perform large-scale genetic studies of Kawasaki disease (KD) in Korea, the Korean Kawasaki Disease Genetics Consortium (KKDGC) was formed in 2008 with 10 hospitals. Since the establishment of KKDGC, there has been a collection of clinical data from a total of 1198 patients, and approximately 5 mL of blood samples per patient (for genomic deoxyribonucleic acid and plasma isolation), using a standard clinical data collection form and a nation-wide networking system for blood sample pick-up. In the clinical risk factor analysis using the collected clinical data of 478 KD patients, it was found that incomplete KD type, intravenous immunoglobulin (IVIG) non-responsiveness, and long febrile days are major risk factors for coronary artery lesions development, whereas low serum albumin concentration is an independent risk factor for IVIG non-responsiveness. In addition, we identified a KD susceptibility locus at 1p31, a coronary artery aneurysm locus (KCNN2 gene), and the causal variant in the C-reactive protein (CRP) promoter region, as determining the increased CRP levels in KD patients, by means of genome-wide association studies. Currently, this consortium is continually collecting more clinical data and genomic samples to identify the clinical and genetic risk factors via a single nucleotide polymorphism chip and exome sequencing, as well as collaborating with several international KD genetics teams. The consortium-based approach for genetic studies of KD in Korea will be a very effective way to understand the unknown etiology and causal mechanism of KD, which may be affected by multiple genes and environmental factors. PMID:26617644

  18. Quantitative evaluation of myocardial fibrosis by cardiac integrated backscatter analysis in Kawasaki disease.

    PubMed

    Xie, Lijian; Wang, Renjian; Huang, Min; Zhang, Yongwei; Shen, Jie; Xiao, Tingting

    2016-01-12

    Kawasaki disease is an acute, systemic vasculitis that affects the coronary arteries. However, the relationship between myocardial fibrosis and Kawasaki disease has been completely unknown until now. We aimed to provide quantitative information about myocardial fibrosis using cardiac integrated backscatter in Han race Kawasaki disease patients. Ninety Kawasaki disease patients and 90 healthy control subjects were recruited. Based on Kawasaki disease status, the patients were categorized into 3 groups: acute, subacute, and convalescence phase. Based on coronary artery status, the Kawasaki disease patients were categorized into 3 groups: without coronary artery lesions, with coronary artery dilation, and with coronary artery aneurysms. All subjects underwent two-dimensional and Doppler examinations to measure clinical echocardiographic parameters. Myocardial fibrosis was detected with calibrated integrated backscatter imaging. Left ventricle systolic functions were normal in both the Kawasaki disease and control participants. The myocardial calibrated integrated backscatter values of the left ventricles of the acute (p < 0.001), subacute (p < 0.001) and convalescence phase (p < 0.001) Kawasaki disease patients were significantly greater than those of the healthy controls. The left ventricle myocardial calibrated integrated backscatter values were significantly smaller in the Kawasaki disease patients without coronary artery lesions than in the Kawasaki disease patients with coronary artery dilations and coronary artery aneurysms in different phases. The left ventricle myocardial calibrated integrated backscatter results were positively correlated with coronary artery status in the acute (r = 0.331, p < 0.001), subacute (r = 0.456, p < 0.001) and convalescence phases (r = 0.407, p < 0.001) of Kawasaki disease. Our findings may suggest that myocardial fibrosis occurs during early episodes of Kawasaki disease given uncertainties that

  19. Kawasaki Disease in India, Lessons Learnt Over the Last 20 Years.

    PubMed

    Singh, Surjit; Kawasaki, Tomisaku

    2016-02-01

    Over the last 20 years, Kawasaki disease is being increasingly recognized in India and it may soon replace acute rheumatic fever to become the commonest cause of acquired heart disease amongst children. However, the vast majority of children with Kawasaki disease in India are still not being diagnosed. Diagnosis of Kawasaki disease is based on a constellation of clinical findings which have a typical temporal sequence. All pediatricians must we familiar with the nuances involved in arriving at a diagnosis of Kawasaki disease. With early diagnosis and prompt treatment, the risk of coronary artery abnormalities can be significantly reduced.

  20. Myocardial infarction in a 35-day-old infant with incomplete Kawasaki disease and chicken pox.

    PubMed

    Kossiva, Lydia; Papadopoulos, Marios; Lagona, Evangelia; Papadopoulos, George; Athanassaki, Corina

    2010-10-01

    Kawasaki disease is an acute febrile vasculitis of infancy and early childhood. It is uncommon in early infancy, because a significant proportion of these children do not meet the classical diagnostic criteria at this age. Infants younger than 6 months with persistent fever and some of the criteria of Kawasaki disease should always raise suspicion for Kawasaki disease early to avoid delayed diagnosis with severe cardiac complications. We present a 35-day-old infant with incomplete Kawasaki disease complicated with myocardial infarction during chicken pox.

  1. 76 FR 82039 - Receipt of Petition for Decision That Nonconforming 2000-2003 Kawasaki ZR750 Motorcycles Are...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-12-29

    ...-2003 Kawasaki ZR750 Motorcycles Are Eligible for Importation AGENCY: National Highway Traffic Safety... Kawasaki ZR750 motorcycles that were not originally manufactured to comply with all applicable Federal... whether non-U.S. certified 2000-2003 Kawasaki ZR750 motorcycles are eligible for importation into the...

  2. BCG and Kawasaki disease in Mexico and Japan.

    PubMed

    Gamez-Gonzalez, Luisa Berenise; Hamada, Hiromichi; Llamas-Guillen, Beatriz Adriana; Ruiz-Fernandez, Miguel; Yamazaki-Nakashimada, Marco

    2017-05-04

    Dr. Tomisaku Kawasaki was the first to describe BCG reactivation in Kawasaki Disease (KD), and this sign is present in about 30-50% of KD patients. It is a very specific early sign of the disease and although it has been recognized for decades, its pathophysiology continues to be an enigma. Recently, Yamada et al. reported a severe BCG reaction with tuberculid in 2 Japanese KD patients. We present 2 cases with KD and severe BCG reaction, one from Japan and the other from Mexico and review the policies of administration of BCG in both countries. The BCG vaccine has a worldwide coverage of 88%. Differences in BCG strains and methods of administration may influence BCG reactions in KD. The BCG reaction in the inoculation site may represent the most useful sign in KD.

  3. Chronic granulomatous disease associated with atypical Kawasaki disease.

    PubMed

    Yamazaki-Nakashimada, M A; Ramírez-Vargas, N; De Rubens-Figueroa, J

    2008-01-01

    Chronic granulomatous disease (CGD) is an infrequent inherited disorder characterized by recurrent infections and abnormal granuloma formation. Patients with CGD have an exuberant inflammatory response and an increased risk of developing autoimmunity. We present the case of a 1-year-old boy with CGD who developed several of the characteristic clinical features of Kawasaki Disease. His illness responded to intravenous immunoglobulin, aspirin, and corticosteroids.

  4. Establishment of Kawasaki disease database based on metadata standard

    PubMed Central

    Park, Yu Rang; Kim, Jae-Jung; Yoon, Young Jo; Yoon, Young-Kwang; Koo, Ha Yeong; Hong, Young Mi; Jang, Gi Young; Shin, Soo-Yong; Lee, Jong-Keuk

    2016-01-01

    Kawasaki disease (KD) is a rare disease that occurs predominantly in infants and young children. To identify KD susceptibility genes and to develop a diagnostic test, a specific therapy, or prevention method, collecting KD patients’ clinical and genomic data is one of the major issues. For this purpose, Kawasaki Disease Database (KDD) was developed based on the efforts of Korean Kawasaki Disease Genetics Consortium (KKDGC). KDD is a collection of 1292 clinical data and genomic samples of 1283 patients from 13 KKDGC-participating hospitals. Each sample contains the relevant clinical data, genomic DNA and plasma samples isolated from patients’ blood, omics data and KD-associated genotype data. Clinical data was collected and saved using the common data elements based on the ISO/IEC 11179 metadata standard. Two genome-wide association study data of total 482 samples and whole exome sequencing data of 12 samples were also collected. In addition, KDD includes the rare cases of KD (16 cases with family history, 46 cases with recurrence, 119 cases with intravenous immunoglobulin non-responsiveness, and 52 cases with coronary artery aneurysm). As the first public database for KD, KDD can significantly facilitate KD studies. All data in KDD can be searchable and downloadable. KDD was implemented in PHP, MySQL and Apache, with all major browsers supported. Database URL: http://www.kawasakidisease.kr PMID:27630202

  5. Oral necrotizing microvasculitis in a patient affected by Kawasaki disease.

    PubMed

    Scardina, Giuseppe Alessandro; Fucà, Gerlandina; Carini, Francesco; Valenza, Vincenzo; Spicola, Michele; Procaccianti, Paolo; Messina, Pietro; Maresi, Emiliano

    2007-12-01

    Kawasaki disease (KD) was first described in 1967 by Kawasaki, who defined it as "mucocutaneous lymph node syndrome". KD is an acute systemic vasculitis, which mainly involves medium calibre arteries; its origin is unknown, and it is observed in children under the age of 5, especially in their third year. The principal presentations of KD include fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Within KD, oral mucositis - represented by diffuse mucous membrane erythema, lip and tongue reddening and lingual papillae hypertrophy with subsequent development of strawberry tongue - can occur both in the acute stage of the disease (0-9 days), and in the convalescence stage (>25 days) as a consequence of the pharmacological treatment. KD vascular lesions are defined as systemic vasculitis instead of systemic arteritis. This study analyzed the anatomical-pathological substrata of oral mucositis in a baby affected by Kawasaki disease and suddenly deceased for cardiac tamponade caused by coronary aneurysm rupture (sudden cardiac death of a mechanical type).

  6. Establishment of Kawasaki disease database based on metadata standard.

    PubMed

    Park, Yu Rang; Kim, Jae-Jung; Yoon, Young Jo; Yoon, Young-Kwang; Koo, Ha Yeong; Hong, Young Mi; Jang, Gi Young; Shin, Soo-Yong; Lee, Jong-Keuk

    2016-07-01

    Kawasaki disease (KD) is a rare disease that occurs predominantly in infants and young children. To identify KD susceptibility genes and to develop a diagnostic test, a specific therapy, or prevention method, collecting KD patients' clinical and genomic data is one of the major issues. For this purpose, Kawasaki Disease Database (KDD) was developed based on the efforts of Korean Kawasaki Disease Genetics Consortium (KKDGC). KDD is a collection of 1292 clinical data and genomic samples of 1283 patients from 13 KKDGC-participating hospitals. Each sample contains the relevant clinical data, genomic DNA and plasma samples isolated from patients' blood, omics data and KD-associated genotype data. Clinical data was collected and saved using the common data elements based on the ISO/IEC 11179 metadata standard. Two genome-wide association study data of total 482 samples and whole exome sequencing data of 12 samples were also collected. In addition, KDD includes the rare cases of KD (16 cases with family history, 46 cases with recurrence, 119 cases with intravenous immunoglobulin non-responsiveness, and 52 cases with coronary artery aneurysm). As the first public database for KD, KDD can significantly facilitate KD studies. All data in KDD can be searchable and downloadable. KDD was implemented in PHP, MySQL and Apache, with all major browsers supported.Database URL: http://www.kawasakidisease.kr.

  7. Possible involvement of infection with human coronavirus 229E, but not NL63, in Kawasaki disease.

    PubMed

    Shirato, Kazuya; Imada, Yoshio; Kawase, Miyuki; Nakagaki, Keiko; Matsuyama, Shutoku; Taguchi, Fumihiro

    2014-12-01

    Although human coronavirus (HCoV)-NL63 was once considered a possible causative agent of Kawasaki disease based on RT-PCR analyses, subsequent studies could not confirm the result. In this study, this possibility was explored using serological tests. To evaluate the role of HCoV infection in patients with Kawasaki disease, immunofluorescence assays and virus neutralizing tests were performed. Paired serum samples were obtained from patients with Kawasaki disease who had not been treated with γ-globulin. HCoV-NL63 and two antigenically different isolates of HCoV-229E (ATCC-VR740 and a new isolate, Sendai-H) were examined as controls. Immunofluorescence assays detected no difference in HCoV-NL63 antibody positivity between the patients with Kawasaki disease and controls, whereas the rate of HCoV-229E antibody positivity was higher in the patients with Kawasaki disease than that in controls. The neutralizing tests revealed no difference in seropositivity between the acute and recovery phases of patients with Kawasaki disease for the two HCoV-229Es. However, the Kawasaki disease specimens obtained from patients in recovery phase displayed significantly higher positivity for Sendai-H, but not for ATCC-VR740, as compared to the controls. The serological test supported no involvement of HCoV-NL63 but suggested the possible involvement of HCoV-229E in the development of Kawasaki disease.

  8. Marked eosinophilia in a patient with history of severe atypical Kawasaki disease.

    PubMed

    O'Byrne, Michael L; Cohen, Meryl S

    2013-01-01

    An infant with recent atypical, treatment-refractory Kawasaki disease presented with marked eosinophilia. Workup failed to identify an etiology. The eosinophilia spontaneously resolved. Eosinophilia has been observed in the acute phase of Kawasaki disease, but has not been reported following recovery. © 2012 Wiley Periodicals, Inc.

  9. Incomplete Kawasaki disease associated with complicated Streptococcus pyogenes pneumonia: A case report.

    PubMed

    Leahy, Timothy Ronan; Cohen, Eyal; Allen, Upton D

    2012-01-01

    A three-year-old boy presented with community-acquired pneumonia complicated by empyema. Streptococcus pyogenes (group A streptococcus) was identified on culture of the pleural fluid. The patient improved with antibiotic therapy and drainage of the empyema. During his convalescence, the patient developed persistent fever, lethargy and anorexia. His inflammatory markers were elevated, and repeat cultures were negative. Although the patient had none of the classical mucocutaneous features of Kawasaki disease, an echocardiogram was performed, which revealed coronary artery dilation. The patient was diagnosed with incomplete Kawasaki disease and treated with intravenous immunoglobulin and high-dose acetylsalicylic acid. The fever subsided within 48 h. To the authors' knowledge, the present report is the first report of Kawasaki disease associated with complicated S pyogenes pneumonia. It emphasizes the importance of considering incomplete Kawasaki disease among children with persistent fever, the role of echocardiography in diagnosis, and the potential link between Kawasaki disease and superantigen-producing organisms such as S pyogenes.

  10. Childhood vaccines and Kawasaki disease, Vaccine Safety Datalink, 1996-2006.

    PubMed

    Abrams, Joseph Y; Weintraub, Eric S; Baggs, James M; McCarthy, Natalie L; Schonberger, Lawrence B; Lee, Grace M; Klein, Nicola P; Belongia, Edward A; Jackson, Michael L; Naleway, Allison L; Nordin, James D; Hambidge, Simon J; Belay, Ermias D

    2015-01-03

    Kawasaki disease is a childhood vascular disorder of unknown etiology. Concerns have been raised about vaccinations being a potential risk factor for Kawasaki disease. Data from the Vaccine Safety Datalink were collected on children aged 0-6 years at seven managed care organizations across the United States. Defining exposure as one of several time periods up to 42 days after vaccination, we conducted Poisson regressions controlling for age, sex, season, and managed care organization to determine if rates of physician-diagnosed and verified Kawasaki disease were elevated following vaccination compared to rates during all unexposed periods. We also performed case-crossover analyses to control for unmeasured confounding. A total of 1,721,186 children aged 0-6 years from seven managed care organizations were followed for a combined 4,417,766 person-years. The rate of verified Kawasaki disease was significantly lower during the 1-42 days after vaccination (rate ratio=0.50, 95% CL=0.27-0.92) and 8-42 days after vaccination (rate ratio=0.45, 95% CL=0.22-0.90) compared to rates during unexposed periods. Breaking down the analysis by vaccination category did not identify a subset of vaccines which was solely responsible for this association. The case-crossover analyses revealed that children with Kawasaki disease had lower rates of vaccination in the 42 days prior to symptom onset for both physician-diagnosed Kawasaki disease (rate ratio=0.79, 95% CL=0.64-0.97) and verified Kawasaki disease (rate ratio=0.38, 95% CL=0.20-0.75). Childhood vaccinations' studied did not increase the risk of Kawasaki disease; conversely, vaccination was associated with a transient decrease in Kawasaki disease incidence. Verifying and understanding this potential protective effect could yield clues to the underlying etiology of Kawasaki disease. Copyright © 2014. Published by Elsevier Ltd.

  11. Renormalized dynamics of the Dean-Kawasaki model

    NASA Astrophysics Data System (ADS)

    Bidhoodi, Neeta; Das, Shankar P.

    2015-07-01

    We study the model of a supercooled liquid for which the equation of motion for the coarse-grained density ρ (x ,t ) is the nonlinear diffusion equation originally proposed by Dean and Kawasaki, respectively, for Brownian and Newtonian dynamics of fluid particles. Using a Martin-Siggia-Rose (MSR) field theory we study the renormalization of the dynamics in a self-consistent form in terms of the so-called self-energy matrix Σ . The appropriate model for the renormalized dynamics involves an extended set of field variables {ρ ,θ } , linked through a nonlinear constraint. The latter incorporates, in a nonperturbative manner, the effects of an infinite number of density nonlinearities in the dynamics. We show that the contributing element of Σ which renormalizes the bare diffusion constant D0 to DR is same as that proposed by Kawasaki and Miyazima [Z. Phys. B Condens. Matter 103, 423 (1997), 10.1007/s002570050396]. DR sharply decreases with increasing density. We consider the likelihood of a ergodic-nonergodic (ENE) transition in the model beyond a critical point. The transition is characterized by the long-time limit of the density correlation freezing at a nonzero value. From our analysis we identify an element of Σ which arises from the above-mentioned nonlinear constraint and is key to the viability of the ENE transition. If this self-energy would be zero, then the model supports a sharp ENE transition with DR=0 as predicted by Kawasaki and Miyazima. With the full model having nonzero value for this self-energy, the density autocorrelation function decays to zero in the long-time limit. Hence the ENE transition is not supported in the model.

  12. Renormalized dynamics of the Dean-Kawasaki model.

    PubMed

    Bidhoodi, Neeta; Das, Shankar P

    2015-07-01

    We study the model of a supercooled liquid for which the equation of motion for the coarse-grained density ρ(x,t) is the nonlinear diffusion equation originally proposed by Dean and Kawasaki, respectively, for Brownian and Newtonian dynamics of fluid particles. Using a Martin-Siggia-Rose (MSR) field theory we study the renormalization of the dynamics in a self-consistent form in terms of the so-called self-energy matrix Σ. The appropriate model for the renormalized dynamics involves an extended set of field variables {ρ,θ}, linked through a nonlinear constraint. The latter incorporates, in a nonperturbative manner, the effects of an infinite number of density nonlinearities in the dynamics. We show that the contributing element of Σ which renormalizes the bare diffusion constant D(0) to D(R) is same as that proposed by Kawasaki and Miyazima [Z. Phys. B Condens. Matter 103, 423 (1997)]. D(R) sharply decreases with increasing density. We consider the likelihood of a ergodic-nonergodic (ENE) transition in the model beyond a critical point. The transition is characterized by the long-time limit of the density correlation freezing at a nonzero value. From our analysis we identify an element of Σ which arises from the above-mentioned nonlinear constraint and is key to the viability of the ENE transition. If this self-energy would be zero, then the model supports a sharp ENE transition with D(R)=0 as predicted by Kawasaki and Miyazima. With the full model having nonzero value for this self-energy, the density autocorrelation function decays to zero in the long-time limit. Hence the ENE transition is not supported in the model.

  13. Blood N-terminal Pro-brain Natriuretic Peptide and Interleukin-17 for Distinguishing Incomplete Kawasaki Disease from Infectious Diseases.

    PubMed

    Wu, Ling; Chen, Yuanling; Zhong, Shiling; Li, Yunyan; Dai, Xiahua; Di, Yazhen

    2015-06-01

    To explore the diagnostic value of blood N-terminal pro-brain natriuretic peptide (NT-proBNP) and interleukin-17(IL-17) for incomplete Kawasaki disease. Patients with Kawasaki disease, Incomplete Kawasaki disease and unclear infectious fever were included in this retrospective study. Their clinical features, and laboratory test results of blood NT-proBNP and IL-17 were collected and compared. 766 patients with complete clinical information were recruited, consisting of 291 cases of Kawasaki disease, 74 cases of incomplete Kawasaki disease, and 401 cases of unclear infectious diseases. When the consistency with indicator 2 and 3 in Kawasaki disease diagnosis criteria was assessed with blood IL-17 ?11.55 pg/mL and blood NT-proBNP ? 225.5 pg/dL as the criteria, the sensitivity and specificity for distinguishing incomplete Kawasaki disease and infectious diseases reached 86.5% and 94.8%, respectively. When we chose the consistency with indicator 1 and 2 in Kawasaki disease diagnosis criteria, the appearance of decrustation and/or the BCG erythema, blood IL-17 ?11.55 pg/mL and blood NT-Pro BNP ?225.5 pg/dL as the criteria, the sensitivity and specificity for distinguishing incomplete Kawasaki disease and infectious diseases was 43.2% and 100%, respectively. Blood NT-proBNP and IL-17 are useful laboratory indicators for distinguishing incomplete Kawasaki disease and infectious diseases at the early stage.

  14. Metastable and scaling regimes of one-dimensional Kawasaki dynamics.

    PubMed

    Albarracín, F A Gómez; Rosales, H D; Grynberg, M D

    2016-04-01

    We investigate the large-time scaling regimes arising from a variety of metastable structures in a chain of Ising spins with both first- and second-neighbor couplings while subject to Kawasaki dynamics. Depending on the ratio and sign of these former, different dynamic exponents are suggested by finite-size scaling analyses of relaxation times. At low but nonzero temperatures these are calculated via exact diagonalizations of the evolution operator in finite chains under several activation barriers. In the absence of metastability the dynamics is always diffusive.

  15. Metastable and scaling regimes of one-dimensional Kawasaki dynamics

    NASA Astrophysics Data System (ADS)

    Albarracín, F. A. Gómez; Rosales, H. D.; Grynberg, M. D.

    2016-04-01

    We investigate the large-time scaling regimes arising from a variety of metastable structures in a chain of Ising spins with both first- and second-neighbor couplings while subject to Kawasaki dynamics. Depending on the ratio and sign of these former, different dynamic exponents are suggested by finite-size scaling analyses of relaxation times. At low but nonzero temperatures these are calculated via exact diagonalizations of the evolution operator in finite chains under several activation barriers. In the absence of metastability the dynamics is always diffusive.

  16. The Complexities of the Diagnosis and Management of Kawasaki Disease.

    PubMed

    Rowley, Anne H

    2015-09-01

    Kawasaki disease (KD) must be considered in the differential diagnosis of any child with fever for 4 to 5 days and compatible clinical and laboratory features, and in any infant with prolonged fever and compatible laboratory features, even in the absence of the classic clinical signs. Prompt therapy is required, because delayed or unrecognized KD can lead to lifelong heart disease or death in previously healthy children. Most children with KD respond to a single 2 g/kg dose of intravenous gammaglobulin with oral aspirin, but a small subset require additional therapies to resolve the clinical illness. Copyright © 2015 Elsevier Inc. All rights reserved.

  17. Toxic shock syndrome. Possible confusion with Kawasaki's disease.

    PubMed

    Raimer, S S; Tschen, E H; Walker, M K

    1981-07-01

    Toxic shock syndrome (TSS) is a recently recognized condition associated with toxin-producing strains of Staphylococcus aureus. Patients affected with this syndrome are frequently young and have multisystemic complaints such as fever, headache, edema, myalgia, scarlatiniform rash, conjunctival injection, confusion, diarrhea, oliguria, hypotension and shock, This is followed by desquamation of the skin, especially the palms and soles. The majority of cases reported have been in menstruating women who used vaginal tampons regularly. Because similarities exist between toxic shock syndrome and Kawasaki's disease (mucocutaneous lymph node syndrome), as well as other conditions, proper diagnosis and management are of the utmost importance.

  18. Multimodality Cardiac Imaging in a Patient with Kawasaki Disease and Giant Aneurysms

    PubMed Central

    Srinivasan, Ranjini; Weller, Rachel; Einstein, Andrew J.

    2016-01-01

    Kawasaki disease is a well-known cause of acquired cardiac disease in the pediatric and adult population, most prevalent in Japan but also seen commonly in the United States. In the era of intravenous immunoglobulin (IVIG) treatment, the morbidity associated with this disease has decreased, but it remains a serious illness. Here we present the case of an adolescent, initially diagnosed with Kawasaki disease as an infant, that progressed to giant aneurysm formation and calcification of the coronary arteries. We review his case and the literature, focusing on the integral role of multimodality imaging in managing Kawasaki disease. PMID:27872783

  19. Kidney and Urinary Tract Involvement in Kawasaki Disease

    PubMed Central

    Watanabe, Toru

    2013-01-01

    Kawasaki disease (KD) is a systemic vasculitis and can develop multiple organ injuries including kidney and urinary tract involvement. These disorders include pyuria, prerenal acute kidney injury (AKI), renal AKI caused by tubulointerstitial nephritis (TIN), hemolytic uremic syndrome (HUS), and immune-complex mediated nephropathy, renal AKI associated with either Kawasaki disease shock syndrome or unknown causes, acute nephritic syndrome (ANS), nephrotic syndrome (NS), renal tubular abnormalities, renal abnormalities in imaging studies, and renal artery lesions (aneurysms and stenosis). Pyuria is common in KD and originates from the urethra and/or the kidney. TIN with AKI and renal tubular abnormalities probably result from renal parenchymal inflammation caused by T-cell activation. HUS and renal artery lesions are caused by vascular endothelial injuries resulting from vasculitis. Some patients with ANS have immunological abnormalities associated with immune-complex formation. Nephromegaly and renal parenchymal inflammatory foci are detected frequently in patients with KD by renal ultrasonography and renal scintigraphy, respectively. Although the precise pathogenesis of KD is not completely understood, renal vasculitis, immune-complex mediated kidney injuries, or T-cell immune-regulatory abnormalities have been proposed as possible mechanisms for the development of kidney and urinary tract injuries. PMID:24288547

  20. ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms.

    PubMed

    Onouchi, Yoshihiro; Gunji, Tomohiko; Burns, Jane C; Shimizu, Chisato; Newburger, Jane W; Yashiro, Mayumi; Nakamura, Yoshikazu; Yanagawa, Hiroshi; Wakui, Keiko; Fukushima, Yoshimitsu; Kishi, Fumio; Hamamoto, Kunihiro; Terai, Masaru; Sato, Yoshitake; Ouchi, Kazunobu; Saji, Tsutomu; Nariai, Akiyoshi; Kaburagi, Yoichi; Yoshikawa, Tetsushi; Suzuki, Kyoko; Tanaka, Takeo; Nagai, Toshiro; Cho, Hideo; Fujino, Akihiro; Sekine, Akihiro; Nakamichi, Reiichiro; Tsunoda, Tatsuhiko; Kawasaki, Tomisaku; Nakamura, Yusuke; Hata, Akira

    2008-01-01

    Kawasaki disease is a pediatric systemic vasculitis of unknown etiology for which a genetic influence is suspected. We identified a functional SNP (itpkc_3) in the inositol 1,4,5-trisphosphate 3-kinase C (ITPKC) gene on chromosome 19q13.2 that is significantly associated with Kawasaki disease susceptibility and also with an increased risk of coronary artery lesions in both Japanese and US children. Transfection experiments showed that the C allele of itpkc_3 reduces splicing efficiency of the ITPKC mRNA. ITPKC acts as a negative regulator of T-cell activation through the Ca2+/NFAT signaling pathway, and the C allele may contribute to immune hyper-reactivity in Kawasaki disease. This finding provides new insights into the mechanisms of immune activation in Kawasaki disease and emphasizes the importance of activated T cells in the pathogenesis of this vasculitis.

  1. ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms

    PubMed Central

    Onouchi, Yoshihiro; Gunji, Tomohiko; Burns, Jane C; Shimizu, Chisato; Newburger, Jane W; Yashiro, Mayumi; Nakamura, Yoshikazu; Yanagawa, Hiroshi; Wakui, Keiko; Fukushima, Yoshimitsu; Kishi, Fumio; Hamamoto, Kunihiro; Terai, Masaru; Sato, Yoshitake; Ouchi, Kazunobu; Saji, Tsutomu; Nariai, Akiyoshi; Kaburagi, Yoichi; Yoshikawa, Tetsushi; Suzuki, Kyoko; Tanaka, Takeo; Nagai, Toshiro; Cho, Hideo; Fujino, Akihiro; Sekine, Akihiro; Nakamichi, Reiichiro; Tsunoda, Tatsuhiko; Kawasaki, Tomisaku; Nakamura, Yusuke; Hata, Akira

    2010-01-01

    Kawasaki disease is a pediatric systemic vasculitis of unknown etiology for which a genetic influence is suspected. We identified a functional SNP (itpkc_3) in the inositol 1,4,5-trisphosphate 3-kinase C (ITPKC) gene on chromosome 19q13.2 that is significantly associated with Kawasaki disease susceptibility and also with an increased risk of coronary artery lesions in both Japanese and US children. Transfection experiments showed that the C allele of itpkc_3 reduces splicing efficiency of the ITPKC mRNA. ITPKC acts as a negative regulator of T-cell activation through the Ca2+/NFAT signaling pathway, and the C allele may contribute to immune hyper-reactivity in Kawasaki disease. This finding provides new insights into the mechanisms of immune activation in Kawasaki disease and emphasizes the importance of activated T cells in the pathogenesis of this vasculitis. PMID:18084290

  2. Transient lingual papillitis associated with confirmed herpes simplex virus 1 in a patient with kawasaki disease.

    PubMed

    Krakowski, Andrew C; Kim, Silvia S; Burns, Jane C

    2014-01-01

    We present a case of transient lingual papillitis associated with confirmed herpes simplex virus 1 that developed after a child received intravenous immunoglobulin and infliximab for acute Kawasaki disease.

  3. Kawasaki disease: an evidence based approach to diagnosis, treatment, and proposals for future research

    PubMed Central

    Brogan, P; Bose, A; Burgner, D; Shingadia, D; Tulloh, R; Michie, C; Klein, N; Booy, R; Levin, M; Dillon, M

    2002-01-01

    This article proposes a clinical guideline for the diagnosis and treatment of Kawasaki disease in the UK based on the best available evidence to date, and highlights areas of practice where evidence is anecdotal or based on retrospective data. Future research as proposed by the London Kawasaki Disease Research Group is outlined, and clinicians are invited to prospectively enrol their suspected cases into this collaborative research project. PMID:11919108

  4. Vascular function long term after Kawasaki disease: another piece of the puzzle?

    PubMed

    Pinto, Fátima F; Gomes, Inês; Loureiro, Petra; Laranjo, Sérgio; Timóteo, Ana T; Carmo, Miguel M

    2017-04-01

    Kawasaki disease is an acute systemic vasculitis. Cardiac complications are frequent and include endothelial dysfunction in patients with coronary anomalies. Thus far, endothelial dysfunction in patients with no coronary lesions is poorly understood. Our aim was to access the vascular function in adolescents and young adults long term after Kawasaki disease, but without coronary aneurysms or any other cardiac risk factors. We carried out a single-centre prospective study in a Portuguese population. We evaluated two groups of subjects: (1) Kawasaki disease patients over 11 years of age, diagnosed >5 years ago, with no coronary lesions or any other risk factors for cardiovascular disease; (2) control group of individuals without cardiovascular risk factors. Patients and controls were clinically assessed. Endo-PAT and carotid intima-media thickness assessment were performed to determine vascular function. In total, 43 Kawasaki disease patients were assessed and compared with 43 controls. Kawasaki disease patients presented a decreased reactive hyperaemia index compared with controls (1.59±0.45 versus 1.98±0.41; p<0.001). Augmentation index was similar in both groups (-4.5±7 versus -5±9%; p 0.6). The mean carotid intima-media thickness was not significantly increased in the Kawasaki disease group. There were no statistically significant changes with regard to laboratory data. Children with Kawasaki disease may have long-term sequelae, even when there is no discernible coronary artery involvement in the acute stage of the disease. Further research is needed to assess whether known strategies to improve endothelial function would bring potential benefits to Kawasaki disease patients.

  5. Response of refractory Kawasaki disease to pulse steroid and cyclosporin A therapy.

    PubMed

    Raman, V; Kim, J; Sharkey, A; Chatila, T

    2001-06-01

    We describe a child with aggressive and protracted Kawasaki disease with coronary aneurysms, myocarditis, pericarditis and valvular insufficiency, despite repeated administration of intravenous immunoglobulin. After a transient response to pulse corticosteroids, his disease ultimately subsided with combination therapy with pulse and high dosage corticosteroids and cyclosporin A. Aggressive immunosuppressive therapy with high dosage corticosteroids and cyclosporin A may be beneficial in patients with refractory Kawasaki disease.

  6. [Reactivation of the scar of BCG vaccination in Kawasaki's disease: clinical case and literature review].

    PubMed

    García Pavón, Susana; Staines Boone, Tamara; Hernández Bautista, Víctor; Yamazaki Nakashimada, Marco Antonio

    2006-01-01

    Kawasaki disease is an acute febrile multisystemic vasculitis affecting children that can affect the coronary arteries. Routine BCG vaccination in Mexico leads to a 99% coverage in infants younger than 1 year. We present a case of Kawasaki disease with skin lesions at the site of BCG. Clinicians should be aware of this clinical manifestation that could help diagnose atypical or incomplete cases of the disease.

  7. Role of the Egami Score in Predicting Intravenous Immunoglobulin Resistance in Kawasaki Disease Among Different Ethnicities.

    PubMed

    Loomba, Rohit S; Raskin, Alexander; Gudausky, Todd M; Kirkpatrick, Edward

    Early treatment with intravenous immunoglobulin (IVIG) is necessary to help reduce the risk of coronary artery abnormalities, such as coronary artery aneurysms and to help alleviate symptoms, in Kawasaki disease. Some patients, however, do not respond to an initial dose of IVIG and require additional doses. Prediction of these IVIG nonresponders may be of assistance in altering initial therapy to make it more effective. The Egami score has been validated in the Japanese population to predict IVIG nonresponders but has shown to be ineffective in US populations. This study evaluates the Egami score in a Midwest US population, subdividing patients by race and the diagnosis of typical or atypical type of Kawasaki disease. Patients were included in the study if they met criteria for Kawasaki disease and received IVIG in the inpatient setting. A total of 182 patients were studied, and in all studied groups, the Egami score had poor sensitivity at predicting IVIG nonresponders. Sensitivity of the score differed between races and differed between typical and atypical Kawasaki disease. The Egami score, as well as other systems, have been validated to predict IVIG nonresponders. These, however, lack sensitivity in the US population. Other scores developed in the United States have also lacked sensitivity, likely due to the absence of race or Kawasaki disease classification as variables. The development of a sensitive scoring system to predict IVIG nonresponders in US populations will require the incorporation of race and Kawasaki disease classification, factors that seem to alter IVIG response.

  8. The serum concentration of soluble interleukin-2 receptor in patients with Kawasaki disease.

    PubMed

    Teraura, Hiroyuki; Kotani, Kazuhiko; Minami, Takaomi; Takeshima, Taro; Shimooki, Osamu; Kajii, Eiji

    2017-03-01

    Kawasaki disease is a febrile disease of childhood that is associated with increased inflammatory cytokines and immunoregulatory abnormalities. While the serum concentrations of soluble IL-2 receptor can change under such pathologies, the relevance of the soluble IL-2 receptor concentration in patients with Kawasaki disease has not been specified. We aimed to summarize the existing studies that reported the soluble IL-2 receptor concentrations in patients with Kawasaki disease. Original articles that were published up to July 2016 were collected using a PubMed/Medline-based search engine. A total of nine articles that reported the serum soluble IL-2 receptor concentrations in acute-phase Kawasaki disease were eligible. All of the articles described a high soluble IL-2 receptor concentration in patients with Kawasaki disease relative to the level of controls or the reference range. Two of five articles on patients with coronary artery aneurysms described a significantly higher soluble IL-2 receptor concentration in patients with coronary artery aneurysms than patients without. Two articles on patients with intravenous immunoglobulin therapy described a significant decrease of the soluble IL-2 receptor concentration after the therapy. Accordingly, the serum soluble IL-2 receptor can be a potent marker of disease activity and therapeutic effects in patients with Kawasaki disease; further studies are thus warranted for its use in the clinical setting.

  9. Revisiting the role of steroids and aspirin in the management of acute Kawasaki disease.

    PubMed

    Dhanrajani, Anita; Yeung, Rae S M

    2017-09-01

    Kawasaki disease is an acute multisystem childhood vasculitis with a predilection for the coronary arteries. The role of corticosteroids and acetyl salicylic acid (ASA) in the treatment of acute Kawasaki disease are matters of ongoing debate and changing attitudes from one extreme to the other. Recent work has provided new evidence to guide our thinking about these two therapeutic agents, which will be the focus of this review. Corticosteroids are effective and well tolerated in Kawasaki disease, both as initial adjunctive treatment in those at high-risk for poor outcome, and as rescue therapy after failed intravenous immunoglobulin (IVIG).Higher doses of ASA (> 30 mg/kg/day) in the acute phase of Kawasaki disease, have no clear benefit over antiplatelet doses in improving coronary outcome. Corticosteroids should be used in patients at high-risk for poor coronary outcome, and in patients who fail IVIG. The absence of widely applicable and validated risk-scoring systems in Kawasaki disease outside of Japan remains a limiting factor to identify high-risk children. Current evidence does not demonstrate any advantage of high-dose over low-dose ASA in the acute phase of Kawasaki disease, in preventing coronary artery aneurysms.

  10. Markers of a recent bocavirus infection in children with Kawasaki disease: "a year prospective study".

    PubMed

    Bajolle, F; Meritet, J-F; Rozenberg, F; Chalumeau, M; Bonnet, D; Gendrel, D; Lebon, P

    2014-12-01

    Retrospective studies and case-reports have suggested the possible role of various viruses in the pathogenesis of the Kawasaki disease. To determine prospectively the incidence of Kawasaki diseases associated with a recent bocavirus infection in the course of a year. Thirty-two children with Kawasaki disease were enrolled in a 13 months prospective study to assess the frequency of human bocavirus type 1 infections. Seasonal shedding of virus, markers of recent infection such as viraemia, viral load, and serum interferon alpha were analyzed. Three of 32 (9%) children had HBoV-DNA in the serum suggesting a recent infection. HBoV-DNA was detected in naso-pharyngeal aspiration of 7/32 (21.8%) children with Kawasaki Disease and six of them (18%) had an increased viral load. No common respiratory viruses were isolated from the 32 patients with the exception of one adenovirus. The seven bocaviruses were identified during the winter-spring season. In addition, 4 of 7 of Kawasaki disease patients shedding bocavirus had detectable interferon alpha in the blood, indicating a possible active or recent viral infection. This study shows that a recent bocavirus infection is concomitant with the onset of some cases of Kawasaki disease. Bocavirus may be a cofactor in the pathogenesis of this disease as previously reported for other infectious agents. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  11. Comorbidity of Kawasaki disease and group a streptococcal pleural effusion in a healthy child: a case report.

    PubMed

    Alhammadi, Ahmed H; Hendaus, Mohamed A

    2013-01-01

    Kawasaki disease is an acute self-limiting vasculitis that affects children. The most dreaded complication of Kawasaki disease reported in the literature over the years is coronary artery disease, which is considered as the main cause of acquired heart disease. However, pulmonary associations with Kawasaki disease have been overlooked. We present a rare, if not unique, case of Kawasaki disease associated with group A streptococcus pleural effusion in the English language literature. A search of the PubMed database was carried out, using a combination of the terms "Kawasaki disease", "pneumonia", and "group A streptococcus". The majority of studies conducted in children with Kawasaki disease have concentrated on the coronary artery implications. Kawasaki disease is considered a self-limiting illness, but can have detrimental consequences if not diagnosed early. When there is a prolonged inflammatory reaction, with no infectious agent identified or remittent fever unresponsive to antibiotics, Kawasaki disease should be taken into consideration. Elevated Vβ2+ T cells compared with healthy controls suggest possible involvement of a superantigen in the etiology of Kawasaki disease, so it is wise that the health care provider concentrates not only on the cardiac consequences, but also on pulmonary associations.

  12. Asymptomatic myocardial infarction in Kawasaki disease: Long-term prognosis

    SciTech Connect

    Shiraishi, I.; Onouchi, Z.; Hayano, T.; Hamaoka, K.; Kiyosawa, N. )

    1991-04-01

    Eight patients with Kawasaki disease who had sustained asymptomatic myocardial infarction 8-15 years ago (mean, 13.1 years) were reexamined by various noninvasive cardiac function tests to assess long-term prognosis. At present, electrocardiograms (ECGs) are normal in six patients. However, all eight patients had a prolonged preejection period (PEP) to left ventricular ejection time (LVET) ratio 30 s after amylnitrate (AN) inhalation. Six patients had perfusion defects by exercise thallium-201 myocardial scintigraphy, and two patients developed ST segment depression in treadmill exercise testing. These patients are symptom-free even though their physical activity has not been restricted. Yet they proved to have serious abnormalities suggesting sequelae of myocardial infarction or existing myocardial ischemia. Judging from the results of noninvasive cardiac function tests and recently performed coronary angiography, five of the eight patients require coronary bypass surgery.

  13. Association of Kawasaki disease with tropospheric wind patterns

    PubMed Central

    Rodó, Xavier; Ballester, Joan; Cayan, Dan; Melish, Marian E.; Nakamura, Yoshikazu; Uehara, Ritei; Burns, Jane C.

    2011-01-01

    The causal agent of Kawasaki disease (KD) remains unknown after more than 40 years of intensive research. The number of cases continues to rise in many parts of the world and KD is the most common cause of acquired heart disease in childhood in developed countries. Analyses of the three major KD epidemics in Japan, major non-epidemic interannual fluctuations of KD cases in Japan and San Diego, and the seasonal variation of KD in Japan, Hawaii, and San Diego, reveals a consistent pattern wherein KD cases are often linked to large-scale wind currents originating in central Asia and traversing the north Pacific. Results suggest that the environmental trigger for KD could be wind-borne. Efforts to isolate the causative agent of KD should focus on the microbiology of aerosols. PMID:22355668

  14. Development of Kawasaki disease in a patient with PFAPA.

    PubMed

    Ninomiya, Takahito; Takada, Hidetoshi; Nagatomo, Yusaku; Nanishi, Etsuro; Nagata, Hazumu; Yamamura, Kenichiro; Doi, Takehiko; Ikeda, Kazuyuki; Hara, Toshiro

    2013-12-01

    Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA) is one of the autoinflammatory diseases of unknown etiology characterized by regularly recurrent fever episodes with attacks lasting 3-6 days every 3-8 weeks associated with at least one of the three cardinal clinical signs: aphthous stomatitis, pharyngitis, and cervical adenitis. Kawasaki disease (KD) is an acute, self-limited systemic vasculitis that occurs predominantly in infants and young children. In most KD patients, i.v. immunoglobulin leads to a rapid amelioration of clinical symptoms and significantly decreases the risk of coronary artery aneurysms. Although the etiology of KD is still unknown, it was reported that innate immunity was activated in the patients. Described herein is a patient with PFAPA who developed KD. This is the first report of KD development in a PFAPA patient. The association between KD and PFAPA may represent a genetic predisposition to dysregulated innate immune response.

  15. [Brain hemorrhage in a patient with Kawasaki disease].

    PubMed

    Yamazaki-Nakashimada, Marco Antonio; Rivas-Larrauri, Francisco; Alcántara-Salinas, Adriana; Hernández-Bautista, Victor; Rodríguez-Lozano, Ana Luisa

    2013-01-01

    Kawasaki disease is an acute, self-limiting vasculitis of unknown origin, characterized by fever, palms and soles edema, cervical lymphadenopathy, strawberry tongue, and non-exudative conjunctivitis. It is a multisystemic vasculitis that affects predominantly infants and young children. The most feared complication is the development of coronary aneurysms that occurs up to 25% of untreated patients; however there are reports of extra coronary involvement. Herein we present the case of a 2 year-old girl who had a severe symptomatology and persistent fever despite intravenous gammaglobulin. Two years later she presented right hemiparesia and headache, with data from CAT and MRI suggestive of brain mass and deviation of the midline, secondary to left frontoparietal haemorrhage that was treated with a craniotomy. She was discharged on prednisone, ASA and rehabilitation.

  16. Common variants in CASP3 confer susceptibility to Kawasaki disease.

    PubMed

    Onouchi, Yoshihiro; Ozaki, Kouichi; Buns, Jane C; Shimizu, Chisato; Hamada, Hiromichi; Honda, Takafumi; Terai, Masaru; Honda, Akihito; Takeuchi, Takashi; Shibuta, Shoichi; Suenaga, Tomohiro; Suzuki, Hiroyuki; Higashi, Kouji; Yasukawa, Kumi; Suzuki, Yoichi; Sasago, Kumiko; Kemmotsu, Yasushi; Takatsuki, Shinichi; Saji, Tsutomu; Yoshikawa, Tetsushi; Nagai, Toshiro; Hamamoto, Kunihiro; Kishi, Fumio; Ouchi, Kazunobu; Sato, Yoshitake; Newburger, Jane W; Baker, Annette L; Shulman, Stanford T; Rowley, Anne H; Yashiro, Mayumi; Nakamura, Yoshikazu; Wakui, Keiko; Fukushima, Yoshimitsu; Fujino, Akihiro; Tsunoda, Tatsuhiko; Kawasaki, Tomisaku; Hata, Akira; Nakamura, Yusuke; Tanaka, Toshihiro

    2010-07-15

    Kawasaki disease (KD; OMIM 611775) is an acute vasculitis syndrome which predominantly affects small- and medium-sized arteries of infants and children. Epidemiological data suggest that host genetics underlie the disease pathogenesis. Here we report that multiple variants in the caspase-3 gene (CASP3) that are in linkage disequilibrium confer susceptibility to KD in both Japanese and US subjects of European ancestry. We found that a G to A substitution of one commonly associated SNP located in the 5' untranslated region of CASP3 (rs72689236; P = 4.2 x 10(-8) in the Japanese and P = 3.7 x 10(-3) in the European Americans) abolished binding of nuclear factor of activated T cells to the DNA sequence surrounding the SNP. Our findings suggest that altered CASP3 expression in immune effecter cells influences susceptibility to KD.

  17. Common variants in CASP3 confer susceptibility to Kawasaki disease

    PubMed Central

    Onouchi, Yoshihiro; Ozaki, Kouichi; Buns, Jane C.; Shimizu, Chisato; Hamada, Hiromichi; Honda, Takafumi; Terai, Masaru; Honda, Akihito; Takeuchi, Takashi; Shibuta, Shoichi; Suenaga, Tomohiro; Suzuki, Hiroyuki; Higashi, Kouji; Yasukawa, Kumi; Suzuki, Yoichi; Sasago, Kumiko; Kemmotsu, Yasushi; Takatsuki, Shinichi; Saji, Tsutomu; Yoshikawa, Tetsushi; Nagai, Toshiro; Hamamoto, Kunihiro; Kishi, Fumio; Ouchi, Kazunobu; Sato, Yoshitake; Newburger, Jane W.; Baker, Annette L.; Shulman, Stanford T.; Rowley, Anne H.; Yashiro, Mayumi; Nakamura, Yoshikazu; Wakui, Keiko; Fukushima, Yoshimitsu; Fujino, Akihiro; Tsunoda, Tatsuhiko; Kawasaki, Tomisaku; Hata, Akira; Nakamura, Yusuke; Tanaka, Toshihiro

    2010-01-01

    Kawasaki disease (KD; OMIM 611775) is an acute vasculitis syndrome which predominantly affects small- and medium-sized arteries of infants and children. Epidemiological data suggest that host genetics underlie the disease pathogenesis. Here we report that multiple variants in the caspase-3 gene (CASP3) that are in linkage disequilibrium confer susceptibility to KD in both Japanese and US subjects of European ancestry. We found that a G to A substitution of one commonly associated SNP located in the 5′ untranslated region of CASP3 (rs72689236; P = 4.2 × 10−8 in the Japanese and P = 3.7 × 10−3 in the European Americans) abolished binding of nuclear factor of activated T cells to the DNA sequence surrounding the SNP. Our findings suggest that altered CASP3 expression in immune effecter cells influences susceptibility to KD. PMID:20423928

  18. Septated pericarditis associated with Kawasaki disease: a brief case report.

    PubMed

    Sonçaği, Arzu; Devrim, Ilker; Karagöz, Tevfik; Dilber, Embiya; Celiker, Alpay; Ozen, Seza; Seçmeer, Gülten

    2007-01-01

    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.

  19. Cellulitis-Like Rash Associated with Kawasaki Disease.

    PubMed

    Itamura, Shinji; Ishiguchi, Yukiko; Kuwabara, Kentaro; Yasui, Kozo; Kamada, Masahiro

    2016-01-01

    We present the case of a 5-year-old-boy who developed a fever and cellulitis-like groin rash 5 days before developing conjunctivitis and 6 to 7 days before other typical signs of Kawasaki disease (KD) appeared. The cellulitis failed to respond to antibiotics and no pathogens were isolated. His fever and clinical signs resolved with intravenous immunoglobulin and high-dose aspirin after discontinuation of antibiotics. Nonbacterial cellulitis is a rare presenting sign of KD, but in the appropriate clinical setting and population, a diagnosis of KD should be considered when cellulitis and fever fail to respond to an appropriate antibiotic regimen and no pathogen can be isolated. © 2015 Wiley Periodicals, Inc.

  20. [Dobutamine stress body surface mapping in Kawasaki disease].

    PubMed

    Seki, T; Zhang, J; Ogawa, S; Hirayama, T

    1994-11-01

    The dobutamine (DOB) stress body surface mapping tests were carried out to detect myocardial ischemia in 23 patients who had Kawasaki disease previously. Eight of 23 patients (group A) had coronary stenosis of 75% or more diameter reduction in major coronary arteries without sufficient collateral flow, as shown by the coronary angiography, but without myocardial infarction. Nine patients (group B) showed no ischemic change exercised 201Tl myocardial scintigram. Six patients (group C) had myocardial infarction due to Kawasaki disease. ST segment potential mapping (0.04 sec after the J point in QRS) and ST-T Isointegral mapping were performed using CVM-3000 system (87 leads), and the following calculations were made: number of leads with horizontal or down-sloping ST depression of 0.10 mV or more, lasting 0.08 sec (nST); row number of the minimum lead in the Isointegral map (Imin); number of positive leads on the seventh row in Isointegral mapping (I-7); number of positive leads on the first row in Isointegral mapping (I-1) and I-7/I-1 ratio. Based on these calculations the criteria for detecting myocardial ischemia (nST < or = 2, Imin < or = 2, I-7/I-1 > or = 1) were created and their usefulness was tested using findings of coronary angiography and exercised 201Tl myocardial scintigram as the golden standard. For the diagnosis of ischemic lesion, the DOB stress body surface mapping test in group A had higher specificity (nST: 100%, Imin: 89%, I-7/I-1: 100% vs. 78%) and higher sensitivity (75%, 50%, 63% vs. 38%), than those by the Treadmill test, while ischemic changes were not detected in group C by this test. From these results it is concluded that it is useful in evaluating ischemic heart disease in children who can not perform Treadmill exercised test adequately.

  1. Cardiovascular imaging in children and adults following Kawasaki disease.

    PubMed

    Dietz, S M; Tacke, C E; Kuipers, I M; Wiegman, A; de Winter, R J; Burns, J C; Gordon, J B; Groenink, M; Kuijpers, T W

    2015-12-01

    Kawasaki disease (KD) is a paediatric vasculitis with coronary artery aneurysms (CAA) as its main complication. Two guidelines exist regarding the follow-up of patients after KD, by the American Heart Association and the Japanese Circulation Society. After the acute phase, CAA-negative patients are checked for cardiovascular risk assessment or with ECG and echocardiography until 5 years after the disease. In CAA-positive patients, monitoring includes myocardial perfusion imaging, conventional angiography and CT-angiography. However, the invasive nature and high radiation exposure do not reflect technical advances in cardiovascular imaging. Newer techniques, such as cardiac MRI, are mentioned but not directly implemented in the follow-up. Cardiac MRI can be performed to identify CAA, but also evaluate functional abnormalities, ischemia and previous myocardial infarction including adenosine stress-testing. Low-dose CT angiography can be implemented at a young age when MRI without anaesthesia is not feasible. CT calcium scoring with a very low radiation dose can be useful in risk stratification years after the disease. By incorporating newer imaging techniques, detection of CAA will be improved while reducing radiation burden and potential complications of invasive imaging modalities. Based on the current knowledge, a possible pathway to follow-up patients after KD is introduced. Key Points • Kawasaki disease is a paediatric vasculitis with coronary aneurysms as major complication. • Current guidelines include invasive, high-radiation modalities not reflecting new technical advances. • Cardiac MRI can provide information on coronary anatomy as well as cardiac function. • (Low-dose) CT-angiography and CT calcium score can also provide important information. • Current guidelines for follow-up of patients with KD need to be revised.

  2. Postnatal Risk of Acquiring Kawasaki Disease: A Nationwide Birth Cohort Database Study.

    PubMed

    Wu, Mei-Hwan; Lin, Min-Tai; Chen, Hui-Chi; Kao, Feng-Yu; Huang, San-Kuei

    2017-01-01

    To investigate the postnatal risk of Kawasaki disease and coronary complications from a nationwide birth cohort in Taiwan, a country with the third-highest incidence of Kawasaki disease worldwide. We enrolled children born between 2000 and 2009 with complete postnatal medical care records for 2000-2014 in the Taiwan national database. Out of a total of 2 150 590 live births, we identified 6690 (62.6% boys) patients with Kawasaki disease. The onset was mostly (93.9%) within the first 5 years of life (median, 16 months; 38% during infancy), but was rare within the first 3 months of life. The overall cumulative incidence of Kawasaki disease by age 5 years was 2.78‰ (3.33‰ for boys and 2.17‰ for girls; P < .001) and exhibited an increasing trend with birth year (from 2.28‰ for 2000 to 3.67‰ for 2009). The incidence ratio was 1.535 in boys and 1.055 in each increasing year. Kawasaki disease recurred more often in younger patients (cumulative incidence, 2.3% in infants vs 1.7% in children aged 1-4 years). Coronary complications occurred in 16.2% of the patients, including 4 cases of acute myocardial infarction (3 occuring during the acute stage and 1 occurring 5 years later). The probability of a major cardiac event (infarction, undergoing percutaneous coronary intervention or coronary artery bypass grafting, or death) by adolescence was 1.9%. The postnatal risk of Kawasaki disease was 3‰-4‰ and increased with every birth year. Patients with Kawasaki disease are at substantial risk for a major cardiac events during childhood. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. Cardiac complications in children with Kawasaki disease in our own experience.

    PubMed

    Gowin, Ewelina; Małecka, Ilona; Stryczyńska-Kazubska, Joanna; Michalak, Michał; Wysocki, Jacek; Górzna-Kamińska, Hanna

    2016-01-01

    Kawasaki disease is the most common cause of acquired heart disease in children in developed countries. The incidence of Kawasaki disease varies from 180 in Japan through 20 in United States to 5-8 in the European countries per 100,000 children younger than 5 years of age. To evaluate cardiac complications in children hospitalised with Kawasaki disease. Retrospective analysis of the medical records of patients hospitalised with Kawasaki disease in the Specialist Mother and Child Healthcare Facility in Poznan (Poland) in 2008-2014. The diagnosis was based on the American Heart Association criteria. Study group included 30 patients (25 boys and 5 girls). The mean age was 49 months; 21 (70%) children were younger than 5 years of age. All patients had oral mucosal lesions, while an elevated leukocyte count was observed in a minority of patients. Cardiac involvement was detected in 18 (60%) patients, aneurysms in 4 (13.3%) patients, coronary artery dilatation in 6 patients, pericarditis in 6 patients, mitral regurgitation in 3 patients, and aortic regurgitation in 2 patients. In 5 children, more than 1 cardiac abnormality was detected. During 12 months of follow-up, coronary artery dilatation resolved in 5 children, and 1 patient developed aneurysm. Our findings suggest that Kawasaki disease should be considered in the differential diagnosis of children with prolonged fever. During the acute stage of the disease, children with Kawasaki disease require regular cardiac evaluation, and long-term care is needed when cardiovascular complications occur. A central case reporting system to monitor all cases of Kawasaki disease in the Polish paediatric population should be introduced.

  4. Pediatric Kawasaki Disease and Adult Human Immunodeficiency Virus Kawasaki-Like Syndrome Are Likely the Same Malady.

    PubMed

    Johnson, Raymond M; Bergmann, Kelly R; Manaloor, John J; Yu, Xiaoqing; Slaven, James E; Kharbanda, Anupam B

    2016-09-01

    Background.  Pediatric Kawasaki disease (KD) and human immunodeficiency virus (HIV)(+) adult Kawasaki-like syndrome (KLS) are dramatic vasculitides with similar physical findings. Both syndromes include unusual arterial histopathology with immunoglobulin (Ig)A(+) plasma cells, and both impressively respond to pooled Ig therapy. Their distinctive presentations, histopathology, and therapeutic response suggest a common etiology. Because blood is in immediate contact with inflamed arteries, we investigated whether KD and KLS share an inflammatory signature in serum. Methods.  A custom multiplex enzyme-linked immunosorbent assay (ELISA) defined the serum cytokine milieu in 2 adults with KLS during acute and convalescent phases, with asymptomatic HIV(+) subjects not taking antiretroviral therapy serving as controls. We then prospectively collected serum and plasma samples from children hospitalized with KD, unrelated febrile illnesses, and noninfectious conditions, analyzing them with a custom multiplex ELISA based on the KLS data. Results.  Patients with KLS and KD subjects shared an inflammatory signature including acute-phase reactants reflecting tumor necrosis factor (TNF)-α biologic activity (soluble TNF receptor I/II) and endothelial/smooth muscle chemokines Ccl1 (Th2), Ccl2 (vascular inflammation), and Cxcl11 (plasma cell recruitment). Ccl1 was specifically elevated in KD versus febrile controls, suggesting a unique relationship between Ccl1 and KD/KLS pathogenesis. Conclusions.  This study defines a KD/KLS inflammatory signature mirroring a dysfunctional response likely to a common etiologic agent. The KD/KLS inflammatory signature based on elevated acute-phase reactants and specific endothelial/smooth muscle chemokines was able to identify KD subjects versus febrile controls, and it may serve as a practicable diagnostic test for KD.

  5. Absence of association of FCGR2A gene polymorphism rs1801274 with Kawasaki disease in Greek patients.

    PubMed

    Chatzikyriakidou, Anthoula; Aidinidou, Louiza; Giannopoulos, Andreas; Papadopoulou-Legbelou, Kyriaki; Kalinderi, Kallirhoe; Fidani, Liana

    2015-04-01

    Kawasaki disease is an acute, febrile syndrome in infancy, characterised by vasculitis of medium-sized arteries, and affects predominantly young children. Family-based studies on Kawasaki disease supports the contribution of genetic factors in disorder manifestation. In a recent genome-wide association study, the polymorphism rs1801274 of FCGR2A [Fc fragment of immunoglobulin G, low-affinity IIa, receptor] gene has been implicated in disease pathogenesis. The aim of the present study was to explore the association of this variant, for the first time, in a group of Kawasaki-diseased patients of Greek origin. A total of 47 Kawasaki-diseased children and 50 control subjects were enrolled in the study. Polymerase chain reaction-restriction fragment length polymorphism assay was performed in rs1801274 genotyping. No association was observed between this polymorphism genotypes' or alleles' distribution between Kawasaki-diseased patients and controls. Furthermore, no association was revealed between this polymorphism and cardiovascular complications in Kawasaki-diseased patients. In the literature, the reported data over this polymorphism association with Kawasaki disease in Caucasian patients are contradictory. In addition, the disease shows low prevalence in the Caucasian populations. Therefore, the independent genetic association studies on rs1801274 with Kawasaki disease in various Caucasian groups increase the amount of genetic data, which could be used in a future meta-analysis, increasing the statistical power of the resultant conclusions.

  6. Primary treatment of incomplete Kawasaki disease with infliximab and methylprednisolone in a patient with a contraindication to intravenous immune globulin.

    PubMed

    Shirley, Debbie-Ann; Stephens, Ina

    2010-10-01

    Incomplete Kawasaki disease was diagnosed in a 3-year-old boy. Because intravenous immune globulin infusion was not tolerated, he was treated with infliximab and methylprednisolone. Coronary aneurysms were not visualized on initial or follow-up echocardiograms. To our knowledge, this is the first report to document the use of infliximab and methylprednisolone as first line therapy for Kawasaki disease.

  7. Comprension de los conceptos de los enlaces ionico y covalente en estudiantes universitarios del primer curso de quimica general

    NASA Astrophysics Data System (ADS)

    Ballesteros Benavides, Maria Elvira

    participantes no lograron diferenciar los dos enlaces quimicos. Esto implica que reflexionemos sobre la necesidad de revisar tanto las competencias cognoscitivas como la forma de ensenar quimica en el salon de clase. Recomendamos indagar y dar importancia al conocimiento previo de los estudiantes, enfatizar la ensenanza de los conceptos y propiciar el aprendizaje con significado.

  8. Kawasaki disease--the importance of prompt recognition and early referral.

    PubMed

    Golshevsky, Daniel; Cheung, Michael; Burgner, David

    2013-07-01

    Kawasaki disease is an acute, febrile vasculitis of childhood that affects medium sized arteries, particularly the coronary arteries. Consequently, it is the leading cause of paediatric-acquired heart disease in developed countries. It is important to have a high index of suspicion for Kawasaki disease in any child with prolonged fever of unknown origin and to refer to a paediatric facility promptly, as timely treatment reduces coronary artery damage. To provide an evidence based review that will help guide the safe and timely recognition, referral and management of typical and incomplete Kawasaki disease. Kawasaki disease is most common in children aged 6 months to 4 years. A high index of suspicion is needed to consider the diagnosis. There are specific diagnostic criteria, though incomplete Kawasaki disease may occur where the child does not meet all diagnostic criteria. There may be co-existing illnesses, which make the diagnosis more difficult. Persistent fever, skin manifestations and extreme irritability may be some cues to consider the diagnosis. If there is strong clinical suspicion the child should be referred, as early treatment significantly decreases the risk of long term cardiac artery damage.

  9. National survey of pediatric hospitalizations due to Kawasaki disease and coronary artery aneurysms in the USA.

    PubMed

    Okubo, Yusuke; Nochioka, Kotaro; Sakakibara, Hiroshi; Testa, Marcia; Sundel, Robert P

    2017-02-01

    Several studies revealed the epidemiology of Kawasaki disease-related hospitalizations among children in the USA and other countries. However, disparities of developing coronary artery aneurysms by race/ethnicity, patient socioeconomic status, and geographic locations remain unknown in the USA. Hospital discharge record data of patients with Kawasaki disease aged 19 years or younger were obtained from the 2003, 2006, 2009, and 2012 Kid's Inpatient Database. The data were weighted to estimate the annual hospitalization rates with respect to age, gender, and race/ethnicity in the USA. Multivariable logistic regression was conducted to ascertain the factors associated with the development of coronary artery aneurysms. Total annual hospitalization rates of Kawasaki disease showed a decreasing trend, ranging from 6.54 per 100,000 children in 2003 to 6.11 per 100,000 children in 2012 (p < 0.001). The proportions of coronary artery aneurysms among patients with Kawasaki disease ranged from 2.25 to 3.20%. Factor associated with the development of coronary artery aneurysms was hospitals in West (OR 2.15, 95% CI 1.42-3.26). Race/ethnicity, health insurance status, and household income were not associated with the development of coronary artery aneurysms. Total hospitalization rates of Kawasaki disease showed a decreasing trend. Children admitted to hospitals in West region were more likely to develop coronary artery aneurysms.

  10. Fluctuations of the Concentration of Cs-137 Aerosol in Chernobyl,Fukushima and Kawasaki

    NASA Astrophysics Data System (ADS)

    Ota, Yohei; Hatano, Yuko; Okada, Yukiko; Hirose, Katsumi

    2017-04-01

    Statistical analysis is applied to a time series of the airborne concentration of Cs-137. In order to extract fractal characteristics of the fluctuations, we employed the Hurst analysis. Interestingly, the Hurst index is around 1/3, which is common to the Chernobyl data, Fukushima data, and Kawasaki data. The Kawasaki data is measured by the Tokyo City University, located at 40km south to Tokyo. We proposed a stochastic differential equation, based on an advection equation with winds fluctuating probabilistically. The averaged solution of the equation is compared with measured data. We found that the index of the power of the time is -4/3, which is common to the three cases, Chernobyl, Fukushima and Kawasaki.

  11. Antiendothelial cell antibodies detected by a cellular based ELISA in Kawasaki disease.

    PubMed Central

    Tizard, E J; Baguley, E; Hughes, G R; Dillon, M J

    1991-01-01

    Kawasaki disease is an acute vasculitic illness of childhood associated with significant morbidity and mortality. A cellular based enzyme linked immunosorbent assay (ELISA) was used to demonstrate the presence of antiendothelial cell antibodies in sera from children with Kawasaki disease. Twenty one of 32 patients with Kawasaki disease had raised IgM antibody titres and four had raised IgG antiendothelial antibody titres. There was a significant difference in the IgM antiendothelial cell antibody titres when comparing the patients with normals and febrile controls. The antibody titre paralleled the disease activity in patients studied serially. There was no relative increase in binding of antiendothelial cell antibodies after cytokine stimulation. These findings may be of importance in further research into the understanding of mechanisms involved in this and other forms of vasculitis in man. PMID:1900406

  12. Recurrent lip swelling as a late presentation of Kawasaki disease: Case report and review of literature.

    PubMed

    Faden, Asmaa

    2013-01-01

    Kawasaki Disease (KD) is an acute systemic vasculitis of unknown etiology. In many developed countries, KD has replaced rheumatic heart disease as the leading cause of acquired heart disease in children. Among the classical criteria for a diagnosis of KD are oral manifestations such as strawberry tongue, erythematous cracked lip, and oropharyngeal mucositis. We report the case of a 24-year-old Saudi female with a history of Kawasaki disease who presented to our oral medicine clinic with recurrent painless swelling of the upper lip. As lip swelling has not previously been reported as an oral manifestation of KD, this case represents a novel presentation of recurrent Kawasaki disease in an adult female.

  13. Prevalence and predictors of coronary artery disease in adults with Kawasaki disease.

    PubMed

    Garg, Jalaj; Krishnamoorthy, Parasuram; Palaniswamy, Chandrasekar; Paudel, Rajiv; Chatterjee, Saurav; Ahmad, Hasan; Snyder, Christopher

    2015-08-01

    Accelerated coronary atherosclerosis in patients with Kawasaki disease, in conjunction with coronary artery aneurysm and stenosis that characterise this disease, are potential risk factors for developing coronary artery disease in young adults. We aimed to determine the prevalence and predictors of coronary artery disease in adult patients with Kawasaki disease. All patients aged 18-55 years of age diagnosed with Kawasaki disease were sampled from Nationwide Inpatient Sample database using International Classification of Diseases 9th revision (ICD 9 code 446.1) from 2009 to 2010. Demographics, prevalence of coronary artery disease, and other traditional risk factors in adult patients with Kawasaki disease were analysed using ICD 9 codes. The prevalence of Kawasaki disease among adults was 0.0005% (n=215) of all in-hospital admissions in United States. The mean age was 27.3 years with women (27.6 years) older than men (27.1 years). Traditional risk factors were hypertension (21%), hyperlipidaemia (15.6%), diabetes (11.5%), tobacco use (8.8%), and obesity (8.8%), with no significant difference between men and women. Coronary artery disease (32.4%), however, was more prevalent in men (44.7%) than in women (12.1%; p=0.03). In multivariate regression analysis, after adjusting for demographics and traditional risk factors, hypertension (OR=13.2, p=0.03) was an independent risk factor of coronary artery disease. There was increased preponderance of coronary artery disease in men with Kawasaki disease. On multivariate analysis, hypertension was found to be the only independent predictor of coronary artery disease in this population after adjusting for other risk factors.

  14. Epidemiologic pictures of Kawasaki disease in Shanghai from 1998 through 2002.

    PubMed

    Huang, Guo-Ying; Ma, Xiao-Jing; Huang, Min; Chen, Shu-Bao; Huang, Mei-Rong; Gui, Yong-Hao; Ning, Shou-Bao; Zhang, Tuo-Hong; Du, Zhong-Dong; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2006-01-01

    Epidemiologic features of Kawasaki disease in China is still not clear. A questionnaire form and diagnostic guidelines for Kawasaki disease were sent to hospitals in Shanghai, which provided with pediatric medical care. All patients with Kawasaki disease diagnosed during January 1998 through December 2002 were recruited in this study. A total of 768 patients with Kawasaki disease were reported. The incidence rates of Kawasaki disease for each year were 16.79 (1998), 25.65 (1999), 28.16 (2000), 28.05 (2001), and 36.76 (2002) per 100,000 children under 5 years of age. The male/female ratio was 1.83:1. The age at onset ranged from 1 month to 18.8 years (median: 1.8 years). The disease occurred more frequently in spring and summer. Fever was the most common clinical symptom, followed by oral changes, extremities desquamate, rash, conjunctive congestion, lymphadenopathy, extremities swelling, and crissum desquamate. Cardiac abnormalities were found in 24.3% of patients. The most common cardiac abnormality was coronary artery lesions including dilatation (68%) and aneurysm (10%). The case-fatality rate at acute stage of the disease was 0.26%. A second onset of the disease occurred in 1.82% of patients. The incidence rate of Kawasaki disease in Shanghai is lower than that reported in Japan, but higher than those in western countries. The increasing trend in incidence, sex distribution and cardiac abnormalities are similar to those in previous reports. The seasonal distribution is similar to the report from Beijing and different from other reports.

  15. A systematic review of validated methods for identifying Kawasaki disease using administrative or claims data.

    PubMed

    Williams, Candice L; Sathe, Nila A; Krishnaswami, Shanthi; McPheeters, Melissa L

    2013-12-30

    To identify and assess algorithms used to identify Kawasaki syndrome/Kawasaki disease in administrative and claims databases. We searched the MEDLINE database from 1991 to September 2012 using controlled vocabulary and key terms related to Kawasaki disease. We also searched the reference lists of included studies. Two investigators independently assessed the full text of studies against pre-determined inclusion criteria. Two reviewers independently extracted data regarding participant and algorithm characteristics. Our searches identified 177 citations of which 22 met our inclusion criteria. All studies used algorithms including International Classification of Diseases, Ninth Revision (ICD-9) code 446.1 either alone, or with evidence of intravenous immunoglobulin (IVIG) administration, or with ICD-10 code M30.3. Six studies confirmed diagnoses by medical chart review. Three of these six studies reported validation statistics, with positive predictive values of 74%, 84%, and 86%, respectively. All studies that reported algorithms used either the ICD-9 code 446.1 either alone, with evidence of IVIG administration or with ICD-10 code M30.3. The ICD-9 code 446.1 alone produced positive predictive values of 74%, 84%, and 86% in separate studies in Georgia and California. The sensitivity of these codes to detect Kawasaki disease is unknown, as no sampling of medical records for missed true cases of Kawasaki disease was done. Further research would be helpful to determine whether the relatively high positive predictive values found in southern California are seen elsewhere and also to evaluate the performance of other codes to identify cases of Kawasaki disease and the sensitivity of the narrow algorithms that have been used to date. Copyright © 2013 Elsevier Ltd. All rights reserved.

  16. Kawasaki disease incidence in children and adolescents: an observational study in primary care.

    PubMed

    Hall, Gillian C; Tulloh, Louise E; Tulloh, Robert M R

    2016-04-01

    Kawasaki disease is reported to be increasing in incidence and is the commonest childhood cause of acquired heart disease in the Western world. To determine the current UK incidence of Kawasaki disease across childhood and adolescence; and investigate trends over time and season. An observational, descriptive study in the UK. The Health Improvement Network (THIN) database of primary healthcare records was searched for codes or text indicating Kawasaki disease. Identified records were compared with a study case definition and a date of onset was assigned to cases. The incidence, age/sex distribution, and trend in seasonal and temporal distribution were estimated (2008-2012). A total of 110 episodes of Kawasaki disease in 109 children were identified from 3.9 million person-years observation. The incidence of Kawasaki disease was 2.8 per 100 000 person-years (95% confidence interval [CI] = 2.3 to 3.4) when aged <20 years; 9.1 (95% CI = 7.3 to 11.2) aged <5 years, and 3.0 per 100 000 (95% CI = 2.0 to 4.3) across the age groups when possible cases were included. More cases were identified in males (55%) with one-fifth of cases diagnosed after 5 years of age. There was no statistically significant trend in incidence over the study years (P = 0.10 adjusted for sex and month), or between seasons (P = 0.65 adjusted for year and sex). Although the incidence of Kawasaki disease remains low and has stabilised in the UK, GPs should recognise that the condition occurs throughout childhood and across the seasons. © British Journal of General Practice 2016.

  17. Kawasaki Shock Syndrome in a 12-Year-Old Girl Mimicking Septic Shock.

    PubMed

    Sinhabahu, Vindika Prasad; Suntharesan, Janani; Wijesekara, Dimuthu Saraji

    2016-01-01

    Kawasaki disease is diagnosed when fever lasts for more than 5 days with the presence of four out of five of the following clinical features: bilateral conjunctival congestion, changes in the lips and oral cavity, polymorphous exanthem, changes in peripheral extremities, and acute nonpurulent cervical lymphadenopathy (Nakamura et al., 2012). The average age of onset is 2 years and 90% of patients are below 5 years of age. Boys are more affected than girls (Cox and Sallis, 2009). This case report describes an adolescent female who was initially managed as having septic shock and subsequently found to have Kawasaki shock syndrome.

  18. Kawasaki Shock Syndrome in a 12-Year-Old Girl Mimicking Septic Shock

    PubMed Central

    Suntharesan, Janani; Wijesekara, Dimuthu Saraji

    2016-01-01

    Kawasaki disease is diagnosed when fever lasts for more than 5 days with the presence of four out of five of the following clinical features: bilateral conjunctival congestion, changes in the lips and oral cavity, polymorphous exanthem, changes in peripheral extremities, and acute nonpurulent cervical lymphadenopathy (Nakamura et al., 2012). The average age of onset is 2 years and 90% of patients are below 5 years of age. Boys are more affected than girls (Cox and Sallis, 2009). This case report describes an adolescent female who was initially managed as having septic shock and subsequently found to have Kawasaki shock syndrome. PMID:28101385

  19. Epidemiology of Kawasaki disease in Asia, Europe, and the United States.

    PubMed

    Uehara, Ritei; Belay, Ermias D

    2012-01-01

    Kawasaki disease (KD) is a systemic vasculitis that mainly affects children younger than 5 years. Although Dr. Tomisaku Kawasaki first reported KD over 40 years ago, the cause of the disease remains unknown. Currently, KD has been diagnosed in more than 60 countries, including those in Asia, the Middle East, Latin America, and Africa, as well as in North America and Europe. The purpose of this review is to describe the epidemiologic features of KD--particularly its incidence, seasonality, and the occurrence of coronary artery abnormalities--primarily in Japan and the United States, but also in Europe and other Asian countries.

  20. Epidemiology of Kawasaki Disease in Asia, Europe, and the United States

    PubMed Central

    Uehara, Ritei; Belay, Ermias D.

    2012-01-01

    Kawasaki disease (KD) is a systemic vasculitis that mainly affects children younger than 5 years. Although Dr. Tomisaku Kawasaki first reported KD over 40 years ago, the cause of the disease remains unknown. Currently, KD has been diagnosed in more than 60 countries, including those in Asia, the Middle East, Latin America, and Africa, as well as in North America and Europe. The purpose of this review is to describe the epidemiologic features of KD—particularly its incidence, seasonality, and the occurrence of coronary artery abnormalities—primarily in Japan and the United States, but also in Europe and other Asian countries. PMID:22307434

  1. Evidence of Microvascular Changes in the Retina following Kawasaki Disease

    PubMed Central

    Chen, Katherine Y. H.; Burgner, David P.; Wong, Tien Y.; Saw, Seang Mei; Quek, Swee Chye; Pang, Audrey Y. C.; Leo, Seo Wei; Wong, Inez B.; Zannino, Diana; Curtis, Nigel; Cheung, Michael; Cheung, Carol Y.; Lim, Terence C. W.

    2017-01-01

    It is unclear whether all children with Kawasaki disease (KD) have increased later cardiovascular risk. The retinal microvasculature reflects changes in the microcirculation and is associated with traditional cardiovascular risk factors and events. The aim of this study was to investigate retinal microvascular parameters in two populations of patients with previous KD and control participants. We performed case-control studies of 116 (57 patients and 59 control participants) Australian and 156 (78 patients and 78 control participants) Singaporean individuals, at least two years since their acute illness. Standardised retinal photographs were graded by trained technicians using a semi-automated software, which quantifies the retinal microvasculature (calibre, branching angle, fractal dimensions, and tortuosity). Retinal venules of Singaporean KD patients were 9.67 μm (95% CI 4.87 to 14.51, p < 0.001) larger than control participants following correction for traditional cardiovascular risk factors. An incremental increase in the size of retinal venules in those with coronary artery abnormalities was observed. There was limited evidence that retinal venules were larger in Australian KD patients with coronary artery abnormalities compared to control participants (7.34 μm, 95% CI 1.30 to 15.99, p = 0.10). Differences in retinal microvasculature were particularly evident in Singaporean KD patients. Larger retinal venules may reflect chronic inflammation and endothelial dysfunction, and are associated with coronary artery disease in adults. PMID:28094311

  2. Do predictors of incomplete Kawasaki disease exist for infants?

    PubMed

    No, Sol Ji; Kim, Dong Ouk; Choi, Kyong Min; Eun, Lucy Youngmin

    2013-02-01

    Kawasaki disease (KD), an acute febrile vasculitis, is the most common cause of acquired heart disease in infants and young children. However, the diagnosis of infantile KD can be difficult or delayed due to vague clinical manifestations. This current study aimed to assess the clinical characteristics and cardiac complications of infantile KD. The study retrospectively reviewed the data of 242 patients with KD. The clinical, laboratory, and echocardiographic data between infants and older children were analyzed. The patients were divided into two groups: infants 12 months old or younger and children older than 12 months. The rate of incomplete KD was much more frequent in infants. During all phases, prohormone brain natriuretic peptide (Pro-BNP) levels were higher in infants, as was thrombocytosis. The coronary artery z-score was higher in infants at all phases of KD. On tissue Doppler imaging, the E/E' ratio (ratio of transmitral Doppler early filling velocity to tissue Doppler early diastolic mitral annular velocity) was higher at the septal and lateral annulus in infants. Infant patients with KD are at increased risk for the development of coronary abnormalities and diastolic dysfunction. Higher levels of Pro-BNP and thrombocytosis with diastolic echo parameters of a higher E/E' ratio can help to identify incomplete KD in infants.

  3. Upcoming biomarkers for the diagnosis of Kawasaki disease: A review.

    PubMed

    Parthasarathy, Pavithra; Agarwal, Arnav; Chawla, Karan; Tofighi, Taraneh; Mondal, Tapas K

    2015-11-01

    Kawasaki disease (KD) is a major cause of acquired heart disease among children and increases the risk of myocardial infarction. While the biochemical basis of the disease is unclear, the evidence suggests interplay between a microbial infection and a genetic predisposition in the development of the disease. Diagnosis of KD based on clinical observation is not completely reliable and is problematic due to the time-sensitive nature of the disease. Hence, identification of inflammatory, proteomic, and genetic biomarkers may assist in earlier and more effective diagnosis and treatment. This review of observational studies and clinical trials analyzes biomarkers in recent research that may be used to establish a gold standard test for KD diagnosis. 65 articles in the literature are assessed to investigate these new biomarkers in addition to biomarkers presently in use. ESR≥40mm/h, leukocyte count ≥16∗10(9)/L and increased WBC count are together suggestive of the presence of KD. Among proteomic biomarkers, elevated NT-proBNP and differing levels of several other proteomic biomarkers such as iNOS in monocytes and neutrophils have been observed in KD patients. Genetic polymorphisms of six HLA class I genes have also been linked with the disease, alongside MICA alleles A4 and A5.1. The results suggest that NT-proBNP is currently a very promising biomarker for future investigation; further research is warranted to allow for accurate and early detection of the disease using this biomarker.

  4. Variations in ORAI1 Gene Associated with Kawasaki Disease.

    PubMed

    Onouchi, Yoshihiro; Fukazawa, Ryuji; Yamamura, Kenichiro; Suzuki, Hiroyuki; Kakimoto, Nobuyuki; Suenaga, Tomohiro; Takeuchi, Takashi; Hamada, Hiromichi; Honda, Takafumi; Yasukawa, Kumi; Terai, Masaru; Ebata, Ryota; Higashi, Kouji; Saji, Tsutomu; Kemmotsu, Yasushi; Takatsuki, Shinichi; Ouchi, Kazunobu; Kishi, Fumio; Yoshikawa, Tetsushi; Nagai, Toshiro; Hamamoto, Kunihiro; Sato, Yoshitake; Honda, Akihito; Kobayashi, Hironobu; Sato, Junichi; Shibuta, Shoichi; Miyawaki, Masakazu; Oishi, Ko; Yamaga, Hironobu; Aoyagi, Noriyuki; Yoshiyama, Megumi; Miyashita, Ritsuko; Murata, Yuji; Fujino, Akihiro; Ozaki, Kouichi; Kawasaki, Tomisaku; Abe, Jun; Seki, Mitsuru; Kobayashi, Tohru; Arakawa, Hirokazu; Ogawa, Shunichi; Hara, Toshiro; Hata, Akira; Tanaka, Toshihiro

    2016-01-01

    Kawasaki disease (KD; MIM#61175) is a systemic vasculitis syndrome with unknown etiology which predominantly affects infants and children. Recent findings of susceptibility genes for KD suggest possible involvement of the Ca(2+)/NFAT pathway in the pathogenesis of KD. ORAI1 is a Ca(2+) release activated Ca(2+) (CRAC) channel mediating store-operated Ca(2+) entry (SOCE) on the plasma membrane. The gene for ORAI1 is located in chromosome 12q24 where a positive linkage signal was observed in our previous affected sib-pair study of KD. A common non-synonymous single nucleotide polymorphism located within exon 2 of ORAI1 (rs3741596) was significantly associated with KD (P = 0.028 in the discovery sample set (729 KD cases and 1,315 controls), P = 0.0056 in the replication sample set (1,813 KD cases vs. 1,097 controls) and P = 0.00041 in a meta-analysis by the Mantel-Haenszel method). Interestingly, frequency of the risk allele of rs3741596 is more than 20 times higher in Japanese compared to Europeans. We also found a rare 6 base-pair in-frame insertion variant associated with KD (rs141919534; 2,544 KD cases vs. 2,414 controls, P = 0.012). These data indicate that ORAI1 gene variations are associated with KD and may suggest the potential importance of the Ca(2+)/NFAT pathway in the pathogenesis of this disorder.

  5. Psoriasiform eruptions during Kawasaki disease (KD): A distinct phenotype.

    PubMed

    Haddock, Ellen S; Calame, Antoanella; Shimizu, Chisato; Tremoulet, Adriana H; Burns, Jane C; Tom, Wynnis L

    2016-07-01

    A psoriasis-like eruption develops in a subset of patients with Kawasaki disease (KD). We sought to systematically compare KD-associated psoriasiform eruptions with classic psoriasis and the outcomes of KD in children with and without this rash. This was a retrospective study of 11 KD cases with a psoriasiform eruption matched 1:2 by age, gender, and ethnicity with psoriasis-only and KD-only controls. Genotyping was performed in 10 cases for a deletion of 2 late cornified envelope (LCE) genes, LCE3C_LCE3B-del, associated with increased risk for pediatric-onset psoriasis. Similar to classic psoriasis, KD-associated eruptions were characterized clinically by well-demarcated, scaly pink plaques and histopathologically by intraepidermal neutrophils, suprabasilar keratin 16 expression, and increased Ki-67 expression. They showed less frequent diaper area involvement, more crust and serous exudate, and an enduring remission (91% vs 23% with confirmed resolution; P < .001). Frequency of LCE3C_LCE3B-del and major KD outcomes were similar between cases and controls. The study was limited by the small number of cases, treatment variation, and availability of skin biopsy specimens. Although the overall clinical and histopathologic findings were similar to conventional psoriasis, this appears to be a distinct phenotype with significantly greater propensity for remission. No adverse effect on KD outcomes was noted. Copyright © 2016 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.

  6. Psoriasiform Eruptions During Kawasaki Disease: A Distinct Phenotype

    PubMed Central

    Haddock, Ellen S.; Calame, Antoanella; Shimizu, Chisato; Tremoulet, Adriana H.; Burns, Jane C.; Tom, Wynnis L.

    2016-01-01

    Background A psoriasis-like eruption develops in a subset of patients with Kawasaki disease (KD). Objective To systematically compare KD-associated psoriasiform eruptions with classic psoriasis and the outcomes of KD in children with and without this rash. Methods This was a retrospective study of 11 KD cases with a psoriasiform eruption matched 1:2 by age, gender, and ethnicity with psoriasis-only and KD-only controls. Genotyping was performed in 10 cases for a deletion of two late cornified envelope (LCE) genes, LCE3C_LCE3B-del, associated with increased risk for pediatric-onset psoriasis. Results Similar to classic psoriasis, KD-associated eruptions were characterized clinically by well-demarcated, scaly pink plaques and histopathologically by intraepidermal neutrophils, suprabasilar keratin 16 expression, and increased Ki-67 expression. They showed less frequent diaper area involvement, more crust and serous exudate, and an enduring remission (91% vs. 23% with confirmed resolution; p < 0.001). Frequency of LCE3C_LCE3B-del and major KD outcomes were similar between cases and controls. Limitations The study was limited by the small number of cases, treatment variation, and availability of skin biopsy specimens. Conclusions Although the overall clinical and histopathologic findings were similar to conventional psoriasis, this appears to be a distinct phenotype with significantly greater propensity for remission. No adverse effect on KD outcomes was noted. PMID:26946987

  7. Recurrent Kawasaki Disease in a Child With Retropharyngeal Involvement

    PubMed Central

    Aldemir-Kocabaş, Bilge; Kıcalı, Mücahit Murat; Ramoğlu, Mehmet G.; Tutar, Ercan; Fitöz, Suat; Çiftçi, Ergin; İnce, Erdal

    2014-01-01

    Abstract Kawasaki disease (KD) is a multisystemic vasculitic disease. Recurrent KD is rare and generally presents in a similar clinical picture as the first episode, and early diagnosis with prompt treatment is the key point in preventing associated cardiovascular morbidities. A 9-year-old boy, who was diagnosed with KD when he was 1.5 years’ old, was referred to our hospital for surgical drainage of retropharyngeal abscess. He had a 7-day history of high fever, sore throat, left-sided neck swelling, and restricted neck movements. Subsequently, he was diagnosed with recurrent KD and retropharyngeal involvement. He was successfully treated with a single dose of intravenous immunoglobulin (IVIG) and acetyl salicylic acid. Recurrence is rare and occurs most commonly in children. Atypical presentation, incomplete disease, short duration of fever, and reduced response to IVIG treatment were found to be the risk factors for recurrence. KD can occasionally present with clinical and radiographic findings of deep neck bacterial infection. Unusual presentations in KD may cause delay in diagnosis and increase the risk of life-threatening complications. We describe a case of recurrent KD presenting with a clinical picture resembling retropharyngeal infection who fully recovered after 1 dose of IVIG instead of surgical drainage and antibiotic use. PMID:25546657

  8. INCIDENCE OF KAWASAKI DISEASE IN NORTHERN EUROPEAN COUNTRIES

    PubMed Central

    Salo, Eeva; Griffiths, Elizabeth P.; Farstad, Teresa; Schiller, Bodil; Nakamura, Yosikazu; Yashiro, Mayumi; Uehara, Ritei; Best, Brookie M.; Burns, Jane C.

    2012-01-01

    Background We sought 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 from 1999–2009 and from nationwide epidemiologic surveys in Japan from 1998–2008. Annual incidence for each country was calculated using regional census data. Results During the 11-year period, 1,390 KD patients were recorded in the registries of the three Northern European countries. Average annual incidence rates per 100,000 children less than 5 years were: Finland 11.4, Norway 5.4, and Sweden 7.4. Overall, 86.4% of Japanese KD patients were less than 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. PMID:22726311

  9. Vascular health late after Kawasaki disease: implications for accelerated atherosclerosis

    PubMed Central

    2014-01-01

    Kawasaki disease (KD), an acute vasculitis that primarily affects young children, is the most common acquired paediatric cardiovascular disease in developed countries. While sequelae of arterial inflammation in the acute phase of KD are well documented, its late effects on vascular health are increasingly unveiled. Late vascular dysfunction is characterized by structural alterations and functional impairment in term of arterial stiffening and endothelial dysfunction and shown to involve both coronary and systemic arteries. Further evidence suggests that continuous low grade inflammation and ongoing active remodeling of coronary arterial lesions occur late after acute illness and may play a role in structural and functional alterations of the arteries. Potential importance of genetic modulation on vascular health late after KD is implicated by associations between mannose binding lectin and inflammatory gene polymorphisms with severity of peripheral arterial stiffening and carotid intima-media thickening. The changes in cholesterol and lipoproteins levels late after KD further appear similar to those proposed to be atherogenic. While data on adverse vascular health are less controversial in patients with persistent or regressed coronary arterial aneurysms, data appear conflicting in individuals with no coronary arterial involvements or only transient coronary ectasia. Notwithstanding, concerns have been raised with regard to predisposition of KD in childhood to accelerated atherosclerosis in adulthood. Until further evidence-based data are available, however, it remains important to assess and monitor cardiovascular risk factors and to promote cardiovascular health in children with a history of KD in the long term. PMID:25550701

  10. Predictors of nonresponse to intravenous immunoglobulin therapy in Kawasaki disease

    PubMed Central

    Park, Hyo Min; Hyun, Myung Chul; Lee, Sang Bum

    2013-01-01

    Purpose It has been reported that 10% to 20% of children with Kawasaki disease (KD) will not respond to intravenous immunoglobulin (IVIG) treatment. In this study, we aimed to identify useful predictors of therapeutic failure in children with KD. Methods We examined 309 children diagnosed with KD at the Kyungpook National University Hospital and the Inje University Busan Paik Hospital between January 2005 and June 2011. We retrospectively reviewed their medical records and analyzed multiple parameters in responders and nonresponders to IVIG. Results Among the 309 children, 30 (9.7%) did not respond to IVIG. They had significantly higher proportion of neutrophils, and higher levels of aspartate aminotransferase, alanine aminotransferase (ALT), total bilirubin, and N-terminal fragment of B-type natriuretic peptide than did responders. IVIG-nonresponders had a significantly longer duration of hospitalization, and more frequently experienced coronary artery lesion, and sterile pyuria. No differences in the duration of fever at initial treatment or, clinical features were noted. Conclusion Two independent predictors (ALT≥84 IU/L, total bilirubin≥0.9 mg/dL) for nonresponse were confirmed through multivariate logistic regression analysis. Thus elevated ALT and total bilirubin levels might be useful in predicting nonresponse to IVIG therapy in children with KD. PMID:23482814

  11. IVIG reduced vascular oxidative stress in patients with Kawasaki disease.

    PubMed

    Takatsuki, Shinichi; Ito, Yuka; Takeuchi, Daiji; Hoshida, Hiroshi; Nakayama, Tomotaka; Matsuura, Hiroyuki; Saji, Tsutomu

    2009-07-01

    Oxidative stress (OS) contributes to the acute phase of Kawasaki disease (KD) in a manner that is as yet unknown. In the present study OS in the acute phase of KD was investigated by measuring urinary 8-iso-prostaglandin F2alpha (8-iso-PG) and evaluating its correlation to the efficacy of intravenous immunoglobulin (IVIG) administration. The 62 patients with acute phase of KD were enrolled, as well as 20 healthy children (HC) and 20 with acute febrile illness (FI). Urinary samples were obtained before and after administration of IVIG. The HC and FI groups also had inflammatory markers evaluated at the same time. The 8-iso-PG was significantly elevated in the 62 KD patients (719 +/-335 pg/mg Cr) without IVIG administration compared with those with FI (583 +/-213 pg/mg Cr) as well as HC (443 +/-288 pg/mg Cr) (P<0.01). 40 patients were given 3 different regimens of IVIG: 16 received 2 g/kg for 1 day; 17 received 1 g/kg for 1 day; 7 received 400 mg . kg(-1) . day(-1) for 5 days. All regimens of IVIG reduced the 8-iso-PG level at 7 days after initiation. OS provokes vasculitis in KD, the activation of which was reduced by IVIG. The urinary level of 8-iso-PG is a useful marker of the effectiveness of IVIG in the acute phase of KD.

  12. Variations in ORAI1 Gene Associated with Kawasaki Disease

    PubMed Central

    Suzuki, Hiroyuki; Kakimoto, Nobuyuki; Suenaga, Tomohiro; Takeuchi, Takashi; Hamada, Hiromichi; Honda, Takafumi; Yasukawa, Kumi; Terai, Masaru; Ebata, Ryota; Higashi, Kouji; Saji, Tsutomu; Kemmotsu, Yasushi; Takatsuki, Shinichi; Ouchi, Kazunobu; Kishi, Fumio; Yoshikawa, Tetsushi; Nagai, Toshiro; Hamamoto, Kunihiro; Sato, Yoshitake; Honda, Akihito; Kobayashi, Hironobu; Sato, Junichi; Shibuta, Shoichi; Miyawaki, Masakazu; Oishi, Ko; Yamaga, Hironobu; Aoyagi, Noriyuki; Yoshiyama, Megumi; Miyashita, Ritsuko; Murata, Yuji; Fujino, Akihiro; Ozaki, Kouichi; Kawasaki, Tomisaku; Abe, Jun; Seki, Mitsuru; Kobayashi, Tohru; Arakawa, Hirokazu; Ogawa, Shunichi; Hara, Toshiro; Hata, Akira; Tanaka, Toshihiro

    2016-01-01

    Kawasaki disease (KD; MIM#61175) is a systemic vasculitis syndrome with unknown etiology which predominantly affects infants and children. Recent findings of susceptibility genes for KD suggest possible involvement of the Ca2+/NFAT pathway in the pathogenesis of KD. ORAI1 is a Ca2+ release activated Ca2+ (CRAC) channel mediating store-operated Ca2+ entry (SOCE) on the plasma membrane. The gene for ORAI1 is located in chromosome 12q24 where a positive linkage signal was observed in our previous affected sib-pair study of KD. A common non-synonymous single nucleotide polymorphism located within exon 2 of ORAI1 (rs3741596) was significantly associated with KD (P = 0.028 in the discovery sample set (729 KD cases and 1,315 controls), P = 0.0056 in the replication sample set (1,813 KD cases vs. 1,097 controls) and P = 0.00041 in a meta-analysis by the Mantel-Haenszel method). Interestingly, frequency of the risk allele of rs3741596 is more than 20 times higher in Japanese compared to Europeans. We also found a rare 6 base-pair in-frame insertion variant associated with KD (rs141919534; 2,544 KD cases vs. 2,414 controls, P = 0.012). These data indicate that ORAI1 gene variations are associated with KD and may suggest the potential importance of the Ca2+/NFAT pathway in the pathogenesis of this disorder. PMID:26789410

  13. Gene Transcript Abundance Profiles Distinguish Kawasaki Disease from Adenovirus Infection

    PubMed Central

    Popper, Stephen J.; Watson, Virginia E.; Shimizu, Chisato; Kanegaye, John T.; Burns, Jane C.; Relman, David A.

    2010-01-01

    Background Acute Kawasaki disease (KD) is difficult to distinguish from other illnesses that involve acute rash or fever, in part because the etiologic agent(s) and pathophysiology remain poorly characterized. As a result, diagnosis and critical therapies may be delayed. Methods We used DNA microarrays to identify possible diagnostic features of KD. We compared gene expression patterns in the blood of 23 children with acute KD and 18 age-matched febrile children with 3 illnesses that resemble KD. Results Genes associated with platelet and neutrophil activation were expressed at higher levels in patients with KD than in patients with acute adenovirus infections or systemic adverse drug reactions, but levels in patients with KD were not higher than those in patients with scarlet fever. Genes associated with B cell activation were also expressed at higher levels in patients with KD than in control subjects. A striking absence of interferon-stimulated gene expression in patients with KD was confirmed in an independent cohort of patients with KD. Using a set of 38 gene transcripts, we successfully predicted the diagnosis for 21 of 23 patients with KD and 7 of 8 patients with adenovirus infection. Conclusions These findings provide insight into the molecular features that distinguish KD from other febrile illnesses and support the feasibility of developing novel diagnostic reagents for KD based on the host response. PMID:19583510

  14. Characteristics and Fate of Systemic Artery Aneurysm after Kawasaki Disease.

    PubMed

    Hoshino, Shinsuke; Tsuda, Etsuko; Yamada, Osamu

    2015-07-01

    To determine the long-term outcome of systemic artery aneurysms (SAAs) after Kawasaki disease (KD). We investigated the characteristics and the fate of SAAs in 20 patients using medical records and angiograms. The age of onset of KD ranged from 1 month to 20 months. The interval from the onset of KD to the latest angiogram ranged from 16 months to 24 years. The regression rate of peripheral artery aneurysm and the frequency of stenotic lesions were analyzed by the Kaplan-Meier method in 11 patients who had undergone initial angiography within 4 months. The mean duration of fever was 24 ± 12 days. All 20 patients had at least 1 symmetric pair of aneurysms in bilateral peripheral arteries, and 16 patients had multiple SAAs. The distributions of SAAs was as follows: brachial artery, 30; common iliac artery, 20; internal iliac artery, 21; abdominal aortic aneurysm, 7; and others, 29. The frequencies of regression of SAA and of the occurrence of stenotic lesions at 20 years after the onset of KD were 51% and 25%, respectively (n = 42). The diameter of all SAAs in the acute phase leading to stenotic lesions in the late period was >10 mm. SAAs occurred symmetrically and were multiple in younger infants and those with severe acute vasculitis. The fate of SAAs resembles that of coronary artery aneurysms, and depends on the diameter during the acute phase. Larger SAAs can lead to stenotic lesions in the late period. Copyright © 2015 Elsevier Inc. All rights reserved.

  15. Hemodynamic simulations in coronary aneurysms of children with Kawasaki disease

    NASA Astrophysics Data System (ADS)

    Sengupta, Dibyendu; Burns, Jane; Marsden, Alison

    2009-11-01

    Kawasaki disease (KD) is a serious pediatric illness affecting the cardiovascular system. One of the most serious complications of KD, occurring in about 25% of untreated cases, is the formation of large aneurysms in the coronary arteries, which put patients at risk for myocardial infarction. In this project we performed patient specific computational simulations of blood flow in aneurysmal left and right coronary arteries of a KD patient to gain an understanding about their hemodynamics. Models were constructed from CT data using custom software. Typical pulsatile flow waveforms were applied at the model inlets, while resistance and RCR lumped models were applied and compared at the outlets. Simulated pressure waveforms compared well with typical physiologic data. High wall shear stress values are found in the narrow region at the base of the aneurysm and low shear values occur in regions of recirculation. A Lagrangian approach has been adopted to perform particle tracking and compute particle residence time in the recirculation. Our long-term goal will be to develop links between hemodynamics and the risk for thrombus formation in order to assist in clinical decision-making.

  16. Th1 and Th2 cytokine production is suppressed at the level of transcriptional regulation in Kawasaki disease

    PubMed Central

    Kimura, J; Takada, H; Nomura, A; Ohno, T; Mizuno, Y; Saito, M; Kusuhara, K; Hara, T

    2004-01-01

    To clarify the functional state of T cells in Kawasaki disease, we analysed mRNA expression levels of Th1/Th2 cytokines (IFN-γ and IL-4) along with Th1/Th2-inducing transcription factors, T-bet and GATA-3, which play pivotal roles in the development of Th1 and Th2 cells, respectively. By real-time PCR, IFN-γ mRNA levels in peripheral blood mononuclear cells (PBMNC) were significantly decreased in Kawasaki disease patients compared with those with measles, and tended to be lower than those in healthy controls. T-bet mRNA levels were significantly decreased in patients with Kawasaki disease compared with healthy controls. In addition, IL-4 and GATA-3 mRNA levels were significantly decreased in Kawasaki disease compared with healthy controls. Regulatory cytokine mRNA levels (TGF-β and IL-10) were also decreased in Kawasaki disease. The mRNA levels of IFN-γ showed a significant positive correlation with those of T-bet in Kawasaki disease. These results suggest that the suppressed function of Th1 and Th2, associated with the suppression of both T-bet and GATA-3 gene expression, may be one of the immunological characteristics of Kawasaki disease. PMID:15270865

  17. Evaluation of clinical characteristics of Kawasaki syndrome and risk factors for coronary artery abnormalities among children in Denmark.

    PubMed

    Patel, Amy; Holman, Robert C; Callinan, Laura S; Sreenivasan, Nandini; Schonberger, Lawrence B; Fischer, Thea K; Belay, Ermias D

    2013-04-01

    To examine clinical characteristics, treatment and outcome of Kawasaki syndrome patients in Denmark. A retrospective chart review of hospitalization records for children <15 years of age with a Kawasaki syndrome discharge diagnosis identified through the Danish National Patient Registry during 1994 through June 2008 was conducted. A total of 284 cases <15 years of age were identified as Kawasaki syndrome (n = 279) and atypical Kawasaki syndrome (n = 5); 70.4% were <5 years of age and 64.4% were male. Most patients (91.5%; 258/282) were treated with intravenous immunoglobulin and 74.6% of these patients (191/256) received intravenous immunoglobulin before the 10th day of illness. A total of 37 (13.3%) Kawasaki syndrome patients were diagnosed with coronary artery abnormalities. Not receiving intravenous immunoglobulin treatment before the 10th day of illness, young age and male sex were significantly associated with the development of coronary artery abnormalities. In Denmark, more than one in 10 children with Kawasaki syndrome develop coronary artery abnormalities. Physicians should increase their index of suspicion for early diagnosis and treatment of Kawasaki syndrome among patients susceptible to increased risk of coronary artery abnormalities, particularly in infants who may have a more atypical presentation of the illness. ©2012 The Author(s)/Acta Paediatrica ©2013 Foundation Acta Paediatrica.

  18. [Association of new functional SNP rs72689236 of CASP3 with Kawasaki disease: a meta-analysis].

    PubMed

    Peng, Qian; Chen, Chang-Hui; Wu, Qing; Yang, Yuan

    2013-06-01

    To investigate the association of rs72689236, a new functional single nucleotide polymorphism (SNP) of the gene encoding caspase-3 (CASP3), with the occurrence and development of Kawasaki disease by a meta analysis. A literature search was performed using databases at home and abroad according to inclusion and exclusion criteria, to acquire studies on the relationship between rs72689236 and Kawasaki disease published up to November 2012, including case-control studies and transmission disequilibrium tests. An integrated meta analysis was performed using RevMan 5.1 software after the studies were screened and evaluated. Six studies were extracted for systematic review of the association between rs72689236 and Kawasaki disease. The frequency of allele A of the SNP was significantly higher in patients with Kawasaki disease than in the controls (OR=1.34, 95%CI=1.24-1.46, P<0.001); the risk for Kawasaki disease in children with allele A (AA+AG) increased by approximately 44% compared with children with GG (OR=1.44, 95%CI=1.27-1.65, P<0.001). The frequency of allele A of the SNP was significantly higher in Kawasaki disease patients with coronary artery lesions than in those without coronary artery lesions (OR=1.51, 95%CI=1.10-2.07, P= 0.01); the risk for coronary artery lesions in Kawasaki disease patients with allele A (AA+AG) increased by approximately 59% compared with Kawasaki disease patients with GG (OR=1.59, 95%CI= 1.00-2.53, P=0.05]. No association between this SNP and the therapeutic effect of intravenous immunoglobulin (IVIG) was found in patients with Kawasaki disease. The allele A of functional SNP rs72689236 of CASP3 increases the risk for Kawasaki disease, and it may be used as the genetic marker for susceptibility to coronary artery lesions as a complication of Kawasaki disease. Currently, there is still no sufficient evidence that this SNP has an impact on the therapeutic effect of IVIG in patients with Kawasaki disease, and more studies are needed to

  19. The Clinical Diagnosis and Management of Kawasaki Disease: a Review and Update.

    PubMed

    Zhu, Frank H; Ang, Jocelyn Y

    2016-09-01

    Kawasaki disease is an acute, self-limited vasculitis of childhood and has become the leading cause of acquired pediatric heart disease in the USA. Prompt treatment is essential in reducing cardiac-related morbidity and mortality. The underlying etiology remains unknown. The disease itself may be the characteristic manifestation of a common pathway of immune-mediated vascular inflammation in susceptible hosts. The characteristic clinical features of fever for at least 5 days with bilateral nonpurulent conjunctivitis, rash, changes in lips and oral cavity, changes in peripheral extremities, and cervical lymphadenopathy remain the mainstay of diagnosis. Supplementary laboratory criteria can aid in the diagnosis, particularly in cases of incomplete clinical presentation. Diagnosis of Kawasaki disease can be challenging as the clinical presentation can be mistaken for a variety of other pediatric illnesses. Standard of care consists of intravenous immune globulin and aspirin. Corticosteroids, infliximab, and cyclosporine A have been used as adjunct therapy for Kawasaki disease refractory to initial treatment. There is ongoing research into the use of these agents in the initial therapy of Kawasaki disease.

  20. A case of atypical Kawasaki disease with giant coronary artery aneurysm containing thrombus

    PubMed Central

    Attard, Montalto S; Grech, V

    2016-01-01

    Introduction Kawasaki disease (KD) is an acute febrile, systemic vasculitic syndrome of unknown etiology, occurring primarily in children younger than 5 years of age. Administration of IVIG within the first 10 days after onset of fever in combination with high dose aspirin reduces the risk of coronary artery damage in KD. Though rare, giant aneurysms of the coronary arteries may develop in untreated cases and prove extremely challenging to manage. Case Presentation A 9-month-old Caucasian boy presented to our paediatric emergency department with a 4-week history of intermittent pyrexia and irritability. Typical mucocutaneous signs of Kawasaki Disease were absent upon presentation. A trans-thoracic echocardiogram identified a giant aneurysm of the left anterior descending artery with thrombus formation in-situ and the child was managed with intravenous immunoglobulin, steroids, high dose aspirin therapy and later warfarinisation. Discussion Cardiovascular sequelae of Kawasaki disease include giant coronary artery aneurysms with thrombosis. Enlargement of a coronary aneurysm after the acute phase of Kawasaki disease is uncommon and the outcome of interventional approaches poorly studied. PMID:28405208

  1. Global Spread of Norovirus GII.17 Kawasaki 308, 2014–2016

    PubMed Central

    Chan, Martin C.W.; Hu, Yunwen; Chen, Haili; Podkolzin, Alexander T.; Zaytseva, Ekaterina V.; Komano, Jun; Sakon, Naomi; Poovorawan, Yong; Vongpunsawad, Sompong; Thanusuwannasak, Thanundorn; Hewitt, Joanne; Croucher, Dawn; Collins, Nikail; Vinjé, Jan; Pang, Xiaoli L.; Lee, Bonita E.; de Graaf, Miranda; van Beek, Janko; Vennema, Harry; Koopmans, Marion P.G.; Niendorf, Sandra; Poljsak-Prijatelj, Mateja; Steyer, Andrej; White, Peter A.; Lun, Jennifer H.; Mans, Janet; Hung, Tin-Nok; Kwok, Kirsty; Cheung, Kelton; Lee, Nelson

    2017-01-01

    Analysis of complete capsid sequences of the emerging norovirus GII.17 Kawasaki 308 from 13 countries demonstrated that they originated from a single haplotype since the initial emergence in China in late 2014. Global spread of a sublineage SL2 was identified. A new sublineage SL3 emerged in China in 2016. PMID:28726618

  2. A case of atypical Kawasaki disease with giant coronary artery aneurysm containing thrombus.

    PubMed

    Micallef, Eynaud S; Attard, Montalto S; Grech, V

    2016-01-01

    Kawasaki disease (KD) is an acute febrile, systemic vasculitic syndrome of unknown etiology, occurring primarily in children younger than 5 years of age. Administration of IVIG within the first 10 days after onset of fever in combination with high dose aspirin reduces the risk of coronary artery damage in KD. Though rare, giant aneurysms of the coronary arteries may develop in untreated cases and prove extremely challenging to manage. A 9-month-old Caucasian boy presented to our paediatric emergency department with a 4-week history of intermittent pyrexia and irritability. Typical mucocutaneous signs of Kawasaki Disease were absent upon presentation. A trans-thoracic echocardiogram identified a giant aneurysm of the left anterior descending artery with thrombus formation in-situ and the child was managed with intravenous immunoglobulin, steroids, high dose aspirin therapy and later warfarinisation. Cardiovascular sequelae of Kawasaki disease include giant coronary artery aneurysms with thrombosis. Enlargement of a coronary aneurysm after the acute phase of Kawasaki disease is uncommon and the outcome of interventional approaches poorly studied.

  3. Matrix metalloproteinase haplotypes associated with coronary artery aneurysm formation in patients with Kawasaki disease

    PubMed Central

    Shimizu, Chisato; Matsubara, Tomoyo; Onouchi, Yoshihiro; Jain, Sonia; Sun, Shelly; Nievergelt, Caroline M.; Shike, Hiroko; Brophy, Victoria H.; Takegawa, Tsuyoshi; Furukawa, Susumu; Akagi, Teiji; Newburger, Jane W.; Baker, Annette L.; Burgner, David; Hibberd, Martin L.; Davila, Sonia; Levin, Michael; Mamtani, Manju; He, Weijing; Ahuja, Sunil K.; Burns, Jane C.

    2010-01-01

    Aneurysms of the vascular wall represent a final common pathway for a number of inflammatory processes including atherosclerosis and idiopathic vasculitis syndromes. Kawasaki disease is an acute, self-limited vasculitis in children and the leading cause of acquired coronary artery aneurysms. We sought to identify shared molecular mechanisms of aneurysm formation by genotyping 8 polymorphisms in MMP-1, 3, 7, 12 and 13 in the gene cluster on Chr.11q22 whose gene products have been implicated in aneurysm formation or are known to have elastase activity. We genotyped 482 US-UK Kawasaki disease patients (aneurysm+: n=111, aneurysm−: n=371) and tested our findings in an independent cohort of 200 Japanese Kawasaki disease patients (aneurysm+: n=58, aneurysm−: n=142). Analysis of the five MMP genes identified modest trends in allele and genotype frequencies for MMP-3 rs3025058 (−/T) and haplotypes containing MMP-3 rs3025058 (−/T) and MMP-12 rs2276109 (A/G) (nominal p= 2-4 × 10−5) that conferred increased risk of aneurysm formation in US-UK subjects. This finding was validated in Japanese subjects and suggests the importance of this locus in aneurysm formation in children with Kawasaki disease. The region encompassing these risk haplotypes is a prime candidate for re-sequencing to look for rare genetic variation that may influence aneurysm formation. PMID:20827277

  4. ECG findings after myocardial infarction in children after Kawasaki disease

    SciTech Connect

    Nakanishi, T.; Takao, A.; Kondoh, C.; Nakazawa, M.; Hiroe, M.; Matsumoto, Y.

    1988-10-01

    Standard 12-lead ECGs were evaluated in 17 children with myocardial infarction and 78 children without myocardial infarction after Kawasaki disease; sensitivity and specificity of the ECG infarction criteria were determined. The presence or absence of myocardial infarction was determined from either clinical examination results (coronary angiography, ventriculography, and thallium-201 myocardial imaging) or autopsy findings. Of seven patients with inferior infarction, abnormally deep Q waves in lead II, III, or aVF were observed in six, but the duration was greater than 0.04 second in only one (14%). The sensitivity and specificity of inferior infarction criteria based on Q wave amplitude were 86% and 97%, respectively. Of eight patients with anterior infarction, seven (88%) had abnormally deep and wide (greater than or equal to 0.04 second) Q waves in anterior chest leads. The sensitivity and specificity of the infarction criteria based on the amplitude and duration of the Q wave were 75% and 99%, respectively. Of seven patients with lateral infarction, Q waves were observed in lead I, aVL, or both in four patients, and in all of these patients Q waves were wider than 0.04 second. In two patients with both inferior and anterior infarction, Q waves were observed only in leads II, III, and aVF; in only one patient were the Q waves wider than 0.04 second. Thus deep Q waves in lead II, III, or aVF that are not wider than 0.04 second may indicate inferior infarction in children. Q waves in lead I, aVL, and chest leads associated with anterolateral infarction are in most instances deep and wide.

  5. Kawasaki disease mimicking a parapharyngeal abscess: a case report.

    PubMed

    Cai, Qianyun; Luo, Rong; Gan, Jing; Zhang, Li; Qu, Yi; Mu, Dezhi

    2015-05-01

    Parapharyngeal abscess (PPA)-like lesion is a very rare manifestation of Kawasaki disease (KD). Here we report a Chinese case of KD initially mimicking PPA, which is the first one reported in Asia.A 3-year-old male patient presented with fever, drooling, and bilateral painful cervical lymphadenopathy for 3 days. Chest X-ray and echocardiogram were normal. With substantial elevation of white blood count and C-reactive protein, purulent cervical lymphadenitis was considered. Symptoms did not improve after treatment with vancomycin, and the patient further developed trismus and restricted neck movement. Neck CT revealed a 2 × 1.5 cm hypodense lesion in the right parapharyngeal space with peripheral enhancement. PPA was suspected and on the 3rd day following admission, the patient received surgical incision and drainage. One milliliter of serous fluid was drained without bacterial growth on cultures. Fever persisted after surgery. As the clinical course proceeded, additional major signs of KD gradually evolved, and on the 6th day following admission the patient completely fulfilled the diagnostic criteria for KD. Rapid clinical improvement was observed following treatment with high-dose immunoglobulin and aspirin. Due to the parapharyngeal operation, the patient was fed milk through a nasogastric tube for 15 days. His neck incision became infected but healed gradually following dressing change and antibiotic treatment. Currently he remains asymptomatic during regular follow-up and repeated echocardiograms are normal.Both pediatricians and otolaryngologists can learn from this case that KD may initially manifest as PPA. Careful observation for major signs of KD during the clinical course can help to achieve a prompt and correct diagnosis. Thus, unnecessary surgery and cardiac complications of KD may be avoided.

  6. [Giant coronary aneurysms in infants with Kawasaki disease].

    PubMed

    Sánchez Andrés, Antonio; Salvador Mercader, Inmaculada; Seller Moya, Julia; Carrasco Moreno, José Ignacio

    2017-08-01

    Kawasaki disease (KD) is an acute vasculitis of unknown origin and predominant in males. The long-term effects of the disease depend on whether there are coronary lesions, particularly aneurysms. The prognosis of patients with giant aneurysms is very poor due to their natural progression to coronary thrombosis or severe obstructive lesions. A series of 8 cases is presented where the epidemiology and diagnostic methods are described. The treatment of the acute and long-term cardiovascular sequelae is also reviewed. A descriptive analysis was conducted on patients admitted to the Paediatric Cardiology Unit of La Fe University Hospital (Valencia) with KD and a coronary lesion. More than one artery was involved in all patients. Although early diagnosis was established in only two cases, none of the patients had severe impairment of ventricular function during the acute phase. Treatment included intravenous gammaglobulin and acetylsalicylic acid at anti-inflammatory doses during the acute phase. A combination of dual antiplatelet therapy and corticosteroids was given in cases of coronary thrombosis. The silent aneurysms continue to persist. KD is the most common cause of acquired heart disease in children. The delay in diagnosis is associated with a greater likelihood of coronary lesions that could increase the risk of cardiovascular events in adulthood. Thus, this subgroup requires close clinical monitoring for a better control of cardiovascular risk factors over time. Copyright © 2016 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  7. Short-term weather variability in Chicago and hospitalizations for Kawasaki disease.

    PubMed

    Checkley, William; Guzman-Cottrill, Judith; Epstein, Leonardo; Innocentini, Nancy; Patz, Jonathan; Shulman, Stanford

    2009-03-01

    : Kawasaki disease exhibits a distinct seasonality, and short-term changes in weather may affect its occurrence. : To investigate the effects of weather variability on the occurrence of this syndrome, we conducted a time-between-events analysis of consecutive admissions for Kawasaki disease to a large pediatric hospital in Chicago. We used gamma regression to model the times between admissions. This is a novel application of gamma regression to model the time between admissions as a function of subject-specific covariates. : We recorded 723 admissions in the 18-year (1986-2003) study period, of which 700 had complete data for analysis. Admissions for Kawasaki disease in Chicago were seasonal: The mean time between admissions was 34% shorter (relative time = 0.66, 95% confidence interval 0.54-0.81) from January-March than from July-September. In 1998, we recorded a larger number of admissions for Kawasaki disease (n = 65) than in other years (mean n = 37). January-March months of 1998 were warmer by a mean of 3 degrees C (1.5 degrees C-4.4 degrees C) and the mean time between admissions was 48% shorter (relative time = 0.52, 0.36-0.75) than in equivalent periods of other study years. : Our findings show that atypical changes in weather affect the occurrence of Kawasaki disease and are compatible with a link to an infectious trigger. The analysis of interevent times using gamma regression is an alternative to Poisson regression in modeling a time series of sparse daily counts.

  8. Differential protein analysis of serum exosomes post-intravenous immunoglobulin therapy in patients with Kawasaki disease.

    PubMed

    Zhang, Li; Song, Qi-Fang; Jin, Jing-Jie; Huang, Ping; Wang, Zhou-Ping; Xie, Xiao-Fei; Gu, Xiao-Qiong; Gao, Xue-Juan; Jia, Hong-Ling

    2017-08-14

    Kawasaki disease, which is characterised by systemic vasculitides accompanied by acute fever, is regularly treated by intravenous immunoglobulin to avoid lesion formation in the coronary artery; however, the mechanism of intravenous immunoglobulin therapy is unclear. Hence, we aimed to analyse the global expression profile of serum exosomal proteins before and after administering intravenous immunoglobulin. Two-dimensional electrophoresis coupled with mass spectrometry analysis was used to identify the differentially expressed proteome of serum exosomes in patients with Kawasaki disease before and after intravenous immunoglobulin therapy. Our analysis revealed 69 differential protein spots in the Kawasaki disease group with changes larger than 1.5-fold and 59 differential ones in patients after intravenous immunoglobulin therapy compared with the control group. Gene ontology analysis revealed that the acute-phase response disappeared, the functions of the complement system and innate immune response were enhanced, and the antibacterial humoral response pathway of corticosteroids and cardioprotection emerged after administration of intravenous immunoglobulin. Further, we showed that complement C3 and apolipoprotein A-IV levels increased before and decreased after intravenous immunoglobulin therapy and that the insulin-like growth factor-binding protein complex acid labile subunit displayed reverse alteration before and after intravenous immunoglobulin therapy. These observations might be potential indicators of intravenous immunoglobulin function. Our results show the differential proteomic profile of serum exosomes of patients with Kawasaki disease before and after intravenous immunoglobulin therapy, such as complement C3, apolipoprotein A-IV, and insulin-like growth factor-binding protein complex acid labile subunit. These results may be useful in the identification of markers for monitoring intravenous immunoglobulin therapy in patients with Kawasaki disease.

  9. Cardiac lesions and initial laboratory data in Kawasaki disease: a nationwide survey in Japan.

    PubMed

    Kuwabara, Masanari; Yashiro, Mayumi; Kotani, Kazuhiko; Tsuboi, Satoshi; Ae, Ryusuke; Nakamura, Yosikazu; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2015-01-01

    Cardiac lesions, such as coronary dilatation, aneurysms, narrowing, myocardial infarction, and valvular lesions, sometimes occur in Kawasaki disease, but most studies have only evaluated cardiac lesions in the later phase of the disease. This study was undertaken to clarify the related factors between cardiac lesions and laboratory data in the initial phase of Kawasaki disease. We conducted a cross-sectional study using data for 26 691 patients from the 22nd nationwide survey of Kawasaki disease in Japan, the observation period of which was from January 2011 through December 2012. We excluded patients with recurrent Kawasaki disease and who were more than seven days from the start of symptoms at admission. We analyzed 23 155 cases (13 353 boys; mean age: 923 ± 734 days) with available laboratory data for white blood cell count, platelet count, serum albumin, and C-reactive protein (CRP). Cardiac lesions were detected in 984 cases (656 boys and 328 girls); lesions were classified as coronary dilatation (764 cases), coronary aneurysm (40), giant coronary aneurysm (6), coronary narrowing (3), and valvular lesions (204). The significant related factors of initial coronary dilatation were male sex (odds ratio [OR] 1.73), older age (OR per 100 days increase 1.03), higher platelet count (OR per 10 000 cells/µL increase 1.006), lower albumin (OR per 1 g/dL increase 0.66), and higher CRP (OR per 1 mg/dL increase 1.02). The factors related to coronary aneurysm were higher platelet count (OR 1.01) and lower albumin (OR 0.34). No factors were significantly related to giant coronary aneurysm. The related factors of valvular lesions were age (OR 0.98), and higher CRP (OR 1.05). Clinicians should consider male sex, older age, higher platelet count, lower albumin levels, and higher CRP levels when assessing risk of cardiac lesions in the initial phase of Kawasaki disease.

  10. Comparation of clinical and paraclinical findings among patient with Kawasaki disease in Bandar abbas Koodakan Hospital in 2011-14

    NASA Astrophysics Data System (ADS)

    Borjali, Davood

    Title: Comparation of clinical and paraclinical findings among patient with Kawasaki disease in Bandar abbas Koodakan Hospital in 2011-14 Kawasaki disease(KD) is a kind of vasculitis diagnosed by clinical manifestation and it caused acquired heart disease in children because of coronary arteries involvement. Method: patient divided to three group of American Japanese and incomplete and also study in two group according to fever days and then clinical features and laboratory data were checked. Result: A total of 150 patients were enrolled during the study period. number of patients with incomplete Kawasaki disease was 128 american group was 28 and Japanese was 4 patients, the most prevalent symptom was scaling of extremities(61 bladder most seen in group with fever more than five days. Keyword: Kawasaki , epidemiology , criteria

  11. An apparent case of undiagnosed donor Kawasaki disease manifesting as coronary artery aneurysm in a pediatric heart transplant recipient.

    PubMed

    Friedland-Little, Joshua; Aiyagari, Ranjit; Gajarski, Robert J; Schumacher, Kurt R

    2013-02-01

    We present a case of coronary ectasia and LAD coronary artery aneurysm with angiographic characteristics of Kawasaki disease in a three-yr-old girl two-yr status post-orthotopic heart transplant. Coronary anomalies were noted during initial screening coronary angiography two yr after transplant. Subsequent review of the donor echocardiogram revealed that the LMCA had been mildly dilated prior to transplant. In the absence of any symptoms consistent with Kawasaki disease in the transplant recipient, this appears to be a case of Kawasaki disease in the organ donor manifesting with coronary anomalies in the transplant recipient. The patient has done well clinically, and repeat coronary angiography has revealed partial regression of coronary anomalies. Given multiple reports in the literature of persistent abnormalities of coronary artery morphology and function after Kawasaki disease, close monitoring is warranted, with consideration of potential coronary protective medical therapies. © 2012 John Wiley & Sons A/S.

  12. Primary percutaneous coronary intervention for acute myocardial infarction in a pediatric patient with giant coronary aneurysm due to Kawasaki disease.

    PubMed

    Mongiovì, Maurizio; Alaimo, Annalisa; Vernuccio, Federica; Pieri, Daniele

    2014-01-01

    We report a case of acute myocardial infarction in an 8-year-old boy with a history of Kawasaki disease and giant coronary aneurysms in the right and left coronary arteries. We performed coronary angiography and percutaneous coronary intervention 4 hours after the onset of symptoms. This case suggests that primary percutaneous coronary intervention might be safe and effective in the long-term treatment of acute myocardial infarction due to coronary sequelae of Kawasaki. © 2013 Wiley Periodicals, Inc.

  13. [Predicting value of thrombospondin-2 for coronary artery dilatation in patients with Kawasaki disease].

    PubMed

    Song, R X; Li, X H; Zhang, T; Cui, X D; Fu, J

    2016-04-01

    To investigate the predictive value of thrombospondin-2 in children with coronary artery dilatation secondary to Kawasaki disease. This study was a retrospective controlled study, the patients were divided into Kawasaki disease group and control group.Patients first time hospitalized in Children's Hospital Affiliated to Capital Institute of Pediatrics during the acute phase of Kawasaki disease were enrolled in Kawasaki disease group from May 2013 to August 2014.Sixty-four Kawasaki disease patients were enrolled into the Kawasaki disease group based on inclusion and exclusion criteria, including 39 males and 25 females, with an average age of 2.3 years.According to the results of echocardiography of children with Kawasaki disease, patients were divided into coronary artery dilatation group and non-coronary artery dilatation group, there were 33 cases in the coronary dilatation (CAD) group, 26 males and 7 females, the average age was 2.0 years, and 31 cases in the non-coronary dilatation (NCAD) group, 13 males and 18 females, the average age was 2.6 years.The control group consisted of children with fever but excluding vascular inflammatory diseases and healthy children, 32 cases in fever group, 19 males and 13 females, mean age was 4.5 years; in which healthy group had 32 cases, 18 males and 14 females and the average age was 3.3 years. Enzyme linked immunosorbent assay (ELISA) method was used to measure the levels of thrombospondin-1 (TSP-1) and TSP-2 in the plasma of each group, and the related laboratory parameters, and general information was analyzed.Multiple sets of normal distribution of data were compared by analysis of unifactorial analysis of variance and the two groups were compared by two independent samples t-test.Analysis of covariance was used to remove the effect of age.The χ(2) test was used to analyze categeorical data and receiver operating characteristic (ROC) curve for evaluating the predictive value of TSP-2. Compared with the febrile group

  14. Evaluation of echogenicity of the heart in Kawasaki disease.

    PubMed

    Nagata, Hazumu; Yamamura, Kenichiro; Uike, Kiyoshi; Nakashima, Yasutaka; Hirata, Yuichiro; Morihana, Eiji; Mizuno, Yumi; Ishikawa, Shiro; Hara, Toshiro

    2014-08-01

    Pathologic studies of the heart in patients with Kawasaki disease (KD) revealed vasculitis, valvulitis, myocarditis, and pericarditis. However, there have been no studies on the quantitative determination of multi-site echogenicity of the heart in KD patients. It is also undetermined whether the degree of echogenicity of each site of the heart in patients with KD might be related to the response to intravenous immunoglobulin (IVIG) treatment. In 81 KD patients and 30 control subjects, we prospectively analyzed echogenicity of the heart. Echogenicity was measured in four sites: coronary artery wall (CAW), mitral valve (MV), papillary muscle (PM), and ascending aortic wall (AAo wall) by the calibrated integrated backscatters (cIBs). The cIB values of all measurement sites at acute phase in KD patients were significantly higher than those in control subjects (KD patients vs control subjects; CAW, 19.8 ± 6.2 dB vs 14.5 ± 2.0 dB, p < 0.05; MV, 23.3 ± 5.3 dB vs 16.0 ± 3.3 dB, p < 0.05; PM, 22.4 ± 5.1 dB vs 12.7 ± 1.9 dB, p < 0.05; AAo wall, 25.3 ± 5.6 dB vs 18.3 ± 3.4 dB, p < 0.05). The cIB values of CAW at the acute phase in IVIG nonresponders were significantly higher than those in responders. Conclusion: Echogenicity of the heart in KD patients at the acute phase increased not only in the coronary artery wall but also in other parts of the heart. Echogenicity of CAW might be helpful in determining the unresponsiveness of IVIG treatment.

  15. Usefulness of real-time 3-dimensional echocardiography to identify and quantify left ventricular dyssynchrony in patients with Kawasaki disease.

    PubMed

    Yu, Yi; Sun, Kun; Xue, Haihong; Chen, Sun; Yang, Jianping

    2013-06-01

    The role of left ventricular (LV) dyssynchrony in Kawasaki disease is unknown. This study sought to establish values for real-time 3-dimensional (3D) echocardiographically derived LV dyssynchrony parameters and identify and quantify LV dyssynchrony in patients with Kawasaki disease. Forty patients hospitalized for Kawasaki disease were analyzed retrospectively, and 40 sex- and age-matched healthy control volunteers were also enrolled. The systolic dyssynchrony index (percentage of the cardiac cycle) from 16 and 12 LV segments on real-time 3D echocardiography was analyzed to calculate LV dyssynchrony (defined as the standard deviation of the time to reach the minimum systolic volume for 16 LV segments) according to a 17-segment model. We analyzed the 3D LV ejection fraction (LVEF), end-diastolic volume, and end-systolic volume in the patients with Kawasaki disease compared to the controls. The 16-segment systolic dyssynchrony index ± SD was significantly higher in the patients with Kawasaki disease: 2.73% ± 0.96% compared to 2.01% ± 0.85% in the controls (P < .05). The 12-segment systolic dyssynchrony index in the patients with Kawasaki disease was 2.65% ± 0.93% compared to 1.98% ± 0.81% in the controls (P< .05). Patients with Kawasaki disease and an LVEF of less than 50% had a significantly higher systolic dyssynchrony index compared to patients with an LVEF of 50% or greater (2.89% ± 0.79% versus 2.26% ± 0.73%; P < .05). The LVEF measured by echocardiography was decreased in the patients with Kawasaki disease, and global systolic function was impaired. The LVEF measured by a biplane method was sufficiently related to the LVEF measured by echocardiography. Real-time 3D echocardiography is a noninvasive and feasible method for identifying and evaluating LV dyssynchrony in children with Kawasaki disease. Left ventricular dyssynchrony is significantly impaired and related to LV systolic function in patients with Kawasaki disease.

  16. Conocimiento de Energía: Principios Esenciales y Conceptos Fundamentales para la Educación de Energía

    SciTech Connect

    2014-06-01

    This is the Spanish version of our Energy Literacy guidebook. Esta guía presenta conceptos de energía que, cuando se entienden y se aplican, ayudan a individuos y a comunidades a tomar decisiones sobre la energía con conocimiento de causa. No es un currículo sino que ofrece un marco sobre el cual puede basarse un plan de estudios. La guía se dirige a cualquier persona que participe en la educación de energía.

  17. Myocardial performance and perfusion during exercise in patients with coronary artery disease caused by Kawasaki disease

    SciTech Connect

    Paridon, S.M.; Ross, R.D.; Kuhns, L.R.; Pinsky, W.W. )

    1990-01-01

    For a study of the natural history of coronary artery lesions after Kawasaki disease and their effect on myocardial blood flow reserve with exercise, five such patients underwent exercise testing on a bicycle. Oxygen consumption, carbon dioxide production, minute ventilation, and electrocardiograms were monitored continuously. Thallium-201 scintigraphy was performed for all patients. One patient stopped exercise before exhaustion of cardiovascular reserve but had no evidence of myocardial perfusion abnormalities. Four patients terminated exercise because of exhaustion of cardiovascular reserve; one had normal cardiovascular reserve and thallium scintiscans, but the remaining patients had diminished cardiovascular reserve. Thallium scintigrams showed myocardial ischemia in two and infarction in one. No patient had exercise-induced electrocardiographic changes. These results indicate that patients with residual coronary artery lesions after Kawasaki disease frequently have reduced cardiovascular reserve during exercise. The addition of thallium scintigraphy and metabolic measurements to exercise testing improved the detection of exercise-induced abnormalities of myocardial perfusion.

  18. Comparative effectiveness of intravenous immunoglobulin from different manufacturing processes on Kawasaki disease.

    PubMed

    Lin, Ming-Chih

    2014-05-01

    The comparative effectiveness of intravenous immunoglobulin (IVIG) for Kawasaki disease was regarded as inconclusive in the international guidelines. However, several new evidences have been published in recent years. A literature search of PubMed was conducted using key words of "Kawasaki disease or mucocutaneous lymph node syndrome" and "immunoglobulin" in combination. Only original articles published after 2004 were selected. A total of 813 papers were found in PubMed. These papers were screened manually by their titles and abstracts. Patients treated with IVIG prepared by betapropiolactonation might have worse outcome (a higher non-responsive rate in one report and a higher rate of coronary aneurysm in two reports). Storage of IVIG in acidic solution might be correlated with a higher rate of coronary aneurysm (two reports). Different processes of preparation and conditions of preservation of IVIG may have profound effects on its clinical effectiveness. Randomized controlled studies are needed to further elucidate this issue.

  19. Equilibrium dynamics of the Dean-Kawasaki equation: Mode-coupling theory and its extension

    NASA Astrophysics Data System (ADS)

    Kim, Bongsoo; Kawasaki, Kyozi; Jacquin, Hugo; van Wijland, Frédéric

    2014-01-01

    We extend a previously proposed field-theoretic self-consistent perturbation approach for the equilibrium dynamics of the Dean-Kawasaki equation presented in [Kim and Kawasaki, J. Stat. Mech. (2008) P02004, 10.1088/1742-5468/2008/02/P02004]. By taking terms missing in the latter analysis into account we arrive at a set of three new equations for correlation functions of the system. These correlations involve the density and its logarithm as local observables. Our new one-loop equations, which must carefully deal with the noninteracting Brownian gas theory, are more general than the historic mode-coupling one in that a further approximation corresponding to Gaussian density fluctuations leads back to the original mode-coupling equation for the density correlations alone. However, without performing any further approximation step, our set of three equations does not feature any ergodic-nonergodic transition, as opposed to the historical mode-coupling approach.

  20. Equilibrium dynamics of the Dean-Kawasaki equation: mode-coupling theory and its extension.

    PubMed

    Kim, Bongsoo; Kawasaki, Kyozi; Jacquin, Hugo; van Wijland, Frédéric

    2014-01-01

    We extend a previously proposed field-theoretic self-consistent perturbation approach for the equilibrium dynamics of the Dean-Kawasaki equation presented in [Kim and Kawasaki, J. Stat. Mech. (2008) P02004]. By taking terms missing in the latter analysis into account we arrive at a set of three new equations for correlation functions of the system. These correlations involve the density and its logarithm as local observables. Our new one-loop equations, which must carefully deal with the noninteracting Brownian gas theory, are more general than the historic mode-coupling one in that a further approximation corresponding to Gaussian density fluctuations leads back to the original mode-coupling equation for the density correlations alone. However, without performing any further approximation step, our set of three equations does not feature any ergodic-nonergodic transition, as opposed to the historical mode-coupling approach.

  1. Validation of the bifurcation diagram in the 2D Ohta–Kawasaki problem

    NASA Astrophysics Data System (ADS)

    Bouwe van den Berg, Jan; Williams, J. F.

    2017-04-01

    We develop a rigorous numerical method to compare local minimizers of the Ohta–Kawasaki functional in two dimensions. In particular, we validate the phase diagram identifying regions of parameter space where rolls are favorable, where hexagonally packed spots have lowest energy and finally where the constant mixed state does. More generally, we present a method to rigorously determine such features in problems where optimal domain sizes are not known a priori.

  2. [Three Cases of Moyamoya Disease with a History of Kawasaki Disease].

    PubMed

    Kawasaki, Toshinari; Arakawa, Yoshiki; Sugino, Toshiya; Mitsuhara, Takafumi; Funaki, Takeshi; Kikuchi, Takayuki; Koyanagi, Masaomi; Yoshida, Kazumichi; Kunieda, Takeharu; Takahashi, Jun C; Takagi, Yasushi; Miyamoto, Susumu

    2015-11-01

    Here, we report three cases of moyamoya disease with a history of Kawasaki disease. A 33-year-old man was found to have stenotic lesions of the internal carotid arteries(ICAs)on both sides at a nearby hospital where he visited complaining of headache and lisping. He had received immunoglobulin therapy for Kawasaki disease at the ages of 1, 2, and 6 years. MRI showed only a chronic ischemic lesion in the white matter. Angiography showed occlusion at the terminal portion of the ICAs on both sides. He was diagnosed with moyamoya disease, but as he had no symptoms and preserved cerebral blood flow (CBF), he was kept under observation. An 8-year-old boy was diagnosed with moyamoya disease and underwent right encephaloduroarteriosynangiosis at a nearby hospital. He had received immunoglobulin therapy for Kawasaki disease at the age of 1 year. His ischemic symptoms worsened. Although MRI detected no apparent ischemic lesion, angiography revealed severe stenosis at the terminal portions of the ICAs on both sides, and 123I-IMP SPECT showed CBF impairment. Bilateral direct bypass was performed. His father was subsequently also diagnosed with moyamoya disease. A 4-year-old girl with epilepsy was diagnosed with moyamoya disease at a nearby hospital. She had been treated with aspirin for Kawasaki disease at the age of 1 year. MRI detected no remarkable ischemic lesions, but angiography revealed mild stenosis at the terminal portions of the ICAs on both sides. Five months later, her ischemic symptoms were worsening with progressing stenotic lesions, and she underwent bilateral direct bypass.

  3. Kawasaki Disease in Latin American Children: Past, Current, and Future Challenges.

    PubMed

    Ulloa-Gutierrez, Rolando; Salgado, Andrea P; Tremoulet, Adriana H

    2014-12-01

    Kawasaki disease (KD) is the leading cause of acquired cardiac disease in children in developed countries and Asia. However, there is a paucity of data available from Latin America. In response to the gap in knowledge about KD in Latin America, a group of pediatric infectious disease researchers from the Kawasaki Disease Research Center at the University of California San Diego and the Sociedad Latinoamericana de Infectología Pediátrica joined efforts during the last decade to address this problem. The Red de Enfermedad de Kawasaki en América Latina (Latin American Kawasaki Disease Network) was launched in 2013 to study the epidemiology of KD among children from the major pediatric tertiary referral hospitals in Latin America. This multinational multicenter network is primarily composed of pediatric infectious diseases, cardiology, rheumatology, and immunology subspecialists and pediatricians from 20 countries, and it is one of the world's largest networks to study the general epidemiology of KD. The first 2 prospective and retrospective multinational multicenter studies looking at the epidemiology of KD in the region were initiated in 2014. Future plans for the network include establishing collaborative research alliances and projects with other centers around the world. To date [ 1], there have been no published studies describing the overall incidence and prevalence of KD in Latin American children. The most important and recent epidemiological study addressing this issue, related to Chile, was published in 2012 [ 2]. Of these, the most recent relevant study addressed the seasonality of KD in different parts of the globe, including some Latin American and Caribbean countries [ 4]. In this document, we briefly summarize relevant available information from Latin America. Although there have been other publications from individual countries that are outside the scope of this communication, the majority of these reports are single case reports, or case series

  4. Identification of the novel Kawasaki 2014 GII.17 human norovirus strain in Italy, 2015.

    PubMed

    Medici, Maria Cristina; Tummolo, Fabio; Calderaro, Adriana; Chironna, Maria; Giammanco, Giovanni Maurizio; De Grazia, Simona; Arcangeletti, Maria Cristina; De Conto, Flora; Chezzi, Carlo; Martella, Vito

    2015-01-01

    Surveillance of noroviruses in Italy identified the novel GII.17 human norovirus strain, Kawasaki 2014, in February 2015. This novel strain emerged as a major cause of gastroenteritis in Asia during 2014/15, replacing the pandemic GII.4 norovirus strain Sydney 2012, but being reported only sporadically elsewhere. This novel strain is undergoing fast diversification and continuous monitoring is important to understand the evolution of noroviruses and to implement the future strategies on norovirus vaccines.

  5. Unusual manifestations of Kawasaki disease with retropharyngeal edema and shock syndrome in a Taiwanese child.

    PubMed

    Fang, Li-Ching; Shyur, Shyh-Dar; Peng, Chun-Chih; Jim, Wai-Tim; Chu, Szu-Hung; Kao, Yu-Hsuan; Chen, Chen-Kuan; Liu, Ling-Chun

    2014-04-01

    We report a 3-year-old girl with Kawasaki disease who presented with retropharyngeal edema and shock syndrome. This is the first reported case in Taiwan. The patient initially presented with fever, cough, and pyuria followed by rapidly progressive enlarged bilateral cervical lymphadenopathy. On the third day of the fever, computed tomography for airway compression sign found widening of the retropharyngeal space mimicking a retropharyngeal abscess. Later, an endotracheal tube was inserted for respiratory distress. A skin rash over her trunk was also noted. On the fifth day of the fever, the clinical course progressed to hypotension and shock syndrome. Because of more swelling of bilateral neck lymph nodes, computed tomography was arranged again and revealed partial resolution of the edematous changes in the retropharyngeal space. Edema of the hands and feet, bilateral bulbar conjunctivitis, and fissured lips were subsequently found. The diagnosis of Kawasaki disease was confirmed on the eighth day of fever. There was no evidence of bacterial infection. She was administered intravenous immunoglobulin (2 mg/kg) and high dose aspirin (100 mg/kg/day). One day later, the fever subsided, and her blood pressure gradually became stable. Heart echocardiography on the Day 13 revealed dilated left coronary artery and mitral regurgitation. Follow-up echocardiography six months later showed normal coronary arteries. To date, the patient has not experienced any complications. This case illustrates that retropharyngeal edema and shock syndrome can be present in the same clinical course of Kawasaki disease. Clinicians and those who work in intensive care units should be aware of unusual presentations of Kawasaki disease to decrease rates of cardiovascular complications. Copyright © 2012. Published by Elsevier B.V.

  6. Epidemiology of Kawasaki disease in Italy: surveillance from national hospitalization records.

    PubMed

    Cimaz, Rolando; Fanti, Eleonora; Mauro, Angela; Voller, Fabio; Rusconi, Franca

    2017-08-01

    Kawasaki disease is a systemic vasculitis with an acute and self-limited course. The incidence of the disease differs widely among ethnic groups and is higher in the Asian populations. In Italy, no recent data are available. We studied the epidemiology of Kawasaki disease in the years 2008-2013 in children 0-14 years old in Italy using hospital ICD-9 discharge codes with a thorough data cleaning for duplicates in order to select the first hospital admission for the disease. The disease peaked in the first 2 years of life, with 85.5% of cases under 5 years. Male/female ratio was 1.4:1. The incidence rate was 5.7 per 100,000 children 0-14 years old and 14.7 for children younger than 5 years. The incidence rose slightly during the study period and had a seasonal distribution, with higher incidence in spring. A coronary artery aneurysm was recorded in 2.2% of the patients younger than 5 years of age. This is the first epidemiologic study on Kawasaki disease incidence in the country of Italy. Figures are in line but slightly higher than those reported for other European countries. What is known: • Kawasaki disease is more common in Asian populations. • European incidence data are scarce. What is new: • Epidemiological data in Italy show similar incidence, albeit slightly higher, than in other European countries. • Incidence data slightly rose in the recent past.

  7. Extensive cervical lymphadenitis mimicking bacterial adenitis as the first presentation of Kawasaki disease

    PubMed Central

    Rossi, Felipe de Souza; da Silva, Marco Felipe Castro; Kozu, Kátia Tomie; Camargo, Luís Fernando Aranha; Rossi, Flávia Feijó Panico; Silva, Clovis Artur; Campos, Lúcia Maria de Arruda

    2015-01-01

    Cervical adenitis >1.5cm in diameter is the less frequently observed criteria in patients with Kawasaki disease and it is usually found in association with other symptoms during the acute phase. Moreover, the finding of fever and lymphadenitis with intense local signs of inflammation and phlegmon is rarely seen as the initial manifestation of Kawasaki disease. We report the case of a 7-year-old boy who had cervical lymphadenitis with adjacent cellulitis and phlegmon mimicking bacterial adenitis as the first presentation of Kawasaki disease. The patient had fever, cervical lymphadenitis with adjacent cellulitis, and severe headache. Cefadroxil was prescribed based on the clinical diagnosis of bacterial adenitis. Because he remained febrile and phlogistic signs worsened, after 1 day of hospitalization, antibiotics were administrated intravenously (ceftriaxone and oxacillin). The computed tomography of the neck showed primary infectious/inflammatory process. On the fourth day, the patient had dry and scaly lips, and treatment with oxacillin was replaced by clindamycin because the patient was still febrile. On the ninth day, he presented non-exudative bilateral conjunctival injection. On the tenth day of febrile disease, a rash appeared on his trunk, hands and feet. Patient’s symptoms resolved after intravenous administration of immunoglobulin (2g/kg/dose), and he was discharged 2 days later. On the 14th day, the patient had lamellar desquamation of fingers. Kawasaki disease should be considered as a differential diagnosis in children with febrile cervical lymphadenitis unresponsive to empiric antibiotics even if they have adjacent cellulitis and phlegmon. PMID:26132362

  8. Genome-wide association study identifies FCGR2A as a susceptibility locus for Kawasaki disease.

    PubMed

    Khor, Chiea Chuen; Davila, Sonia; Breunis, Willemijn B; Lee, Yi-Ching; Shimizu, Chisato; Wright, Victoria J; Yeung, Rae S M; Tan, Dennis E K; Sim, Kar Seng; Wang, Jie Jin; Wong, Tien Yin; Pang, Junxiong; Mitchell, Paul; Cimaz, Rolando; Dahdah, Nagib; Cheung, Yiu-Fai; Huang, Guo-Ying; Yang, Wanling; Park, In-Sook; Lee, Jong-Keuk; Wu, Jer-Yuarn; Levin, Michael; Burns, Jane C; Burgner, David; Kuijpers, Taco W; Hibberd, Martin L

    2011-11-13

    Kawasaki disease is a systemic vasculitis of unknown etiology, with clinical observations suggesting a substantial genetic contribution to disease susceptibility. We conducted a genome-wide association study and replication analysis in 2,173 individuals with Kawasaki disease and 9,383 controls from five independent sample collections. Two loci exceeded the formal threshold for genome-wide significance. The first locus is a functional polymorphism in the IgG receptor gene FCGR2A (encoding an H131R substitution) (rs1801274; P = 7.35 × 10(-11), odds ratio (OR) = 1.32), with the A allele (coding for histadine) conferring elevated disease risk. The second locus is at 19q13, (P = 2.51 × 10(-9), OR = 1.42 for the rs2233152 SNP near MIA and RAB4B; P = 1.68 × 10(-12), OR = 1.52 for rs28493229 in ITPKC), which confirms previous findings(1). The involvement of the FCGR2A locus may have implications for understanding immune activation in Kawasaki disease pathogenesis and the mechanism of response to intravenous immunoglobulin, the only proven therapy for this disease.

  9. Sets of serum exosomal microRNAs as candidate diagnostic biomarkers for Kawasaki disease

    PubMed Central

    Jia, Hong-Ling; Liu, Chao-Wu; Zhang, Li; Xu, Wei-Jun; Gao, Xue-Juan; Bai, Jun; Xu, Yu-Fen; Xu, Ming-Guo; Zhang, Gong

    2017-01-01

    Although Kawasaki disease is the main cause of acquired heart disease in children, no diagnostic biomarkers are available. We aimed to identify candidate biomarkers for diagnosing Kawasaki disease using serum exosomal microRNAs (miRNAs). Using frozen serum samples from a biobank, high-throughput microarray technologies, two-stage real-time quantitative PCR, and a self-referencing strategy for data normalization, we narrowed down the list of biomarker candidates to a set of 4 miRNAs. We further validated the diagnostic capabilities of the identified miRNAs (namely, CT(miR-1246)-CT(miR-4436b-5p) and CT(miR-197-3p)-CT(miR-671-5p)) in 79 samples from two hospitals. We found that this 4-miRNA set could distinguish KD patients from other febrile patients as well as from healthy individuals in a single pass, with a minimal rate of false positives and negatives. We thus propose, for the first time, that serum exosomal miRNAs represent candidate diagnostic biomarkers for Kawasaki disease. Additionally, we describe an effective strategy of screening for biomarkers of complex diseases even when little mechanistic knowledge is available. PMID:28317854

  10. Periodic fever in MVK deficiency: a patient initially diagnosed with incomplete Kawasaki disease.

    PubMed

    Thors, Valtyr S; Vastert, Sebastiaan J; Wulffraat, Nico; van Royen, Annet; Frenkel, Joost; de Sain-van der Velden, Monique; de Koning, Tom J

    2014-02-01

    Mevalonate kinase deficiency (MKD) is a rare autosomal recessive disorder causing 1 of 2 phenotypes, hyperimmunoglobulin D syndrome and mevalonic aciduria, presenting with recurrent fever episodes, often starting in infancy, and sometimes evoked by stress or vaccinations. This autoinflammatory disease is caused by mutations encoding the mevalonate kinase (MVK) gene and is classified in the group of periodic fever syndromes. There is often a considerable delay in the diagnosis among pediatric patients with recurrent episodes of fever. We present a case of an 8-week-old girl with fever of unknown origin and a marked systemic inflammatory response. After excluding infections, a tentative diagnosis of incomplete Kawasaki syndrome was made, based on the finding of dilated coronary arteries on cardiac ultrasound and fever, and she was treated accordingly. However, the episodes of fever recurred, and alternative diagnoses were considered, which eventually led to the finding of increased excretion of mevalonic acid in urine. The diagnosis of MKD was confirmed by mutation analysis of the MVK gene. This case shows that the initial presentation of MKD can be indistinguishable from incomplete Kawasaki syndrome. When fever recurs in Kawasaki syndrome, other (auto-)inflammatory diseases must be ruled out to avoid inappropriate diagnostic procedures, ineffective interventions, and treatment delay.

  11. A male infant had subdural effusion and paroxysmal supraventricular tachycardia during the febrile episode of Kawasaki disease: a case report and literature review.

    PubMed

    Chou, Chia-Pei; Lin, I-Chun; Kuo, Kuang-Che

    2016-05-28

    Kawasaki disease is an acute, febrile, self-limiting, inflammatory systemic vasculitis seen in early childhood, most commonly in those below 5 years of age. In Kawasaki disease, the coronary arteries are most commonly affected, which may lead to asymptomatic coronary artery ectasia or formation of an aneurysm. Paroxysmal supraventricular tachycardia(PSVT) is a severe and rare cardiovascular complication of Kawasaki disease. A case of Kawasaki disease presenting with unusual findings, including subdural effusion and PSVT is reported. This is a 4-month-10-day-old boy presents with anterior fontanelle bulging and moderate bilateral subdural effusion at the acute stage of Kawasaki disease and PSVT at the subacute stage of Kawasaki disease. The subdural effusion was resolution after intravenous immunoglobulin(IVIG) administration. And the PSVT was subsided after administered 3 doses of adenosine, 1 dose of amiodarone loading and Propranolol twice per day use. At 1-year follow-up has made a complete recovery with no arrhythmia episodes, developmental effects or abnormal neurologic findings. Subdural effusion in the acute stage of Kawasaki disease may be an inflammatory response. It may resolves spontaneously after anti-inflammatory treatment such as IVIG infusion. PSVT is a severe cardiovascular complication of Kawasaki disease. In those who taking aspirin, we need to carefully observe the heart rhythm and PSVT side effects, especially in the first month.

  12. Usefulness of Neutrophil to Lymphocyte Ratio in Prediction of Coronary Artery Lesions in Patients with Kawasaki Disease.

    PubMed

    Demir, Fikri; Karadeniz, Cem; Özdemir, Rahmi; Yozgat, Yılmaz; Çelegen, Kübra; Karaaslan, Utku; Demirol, Mustafa; Meşe, Timur; Ünal, Nurettin

    2015-10-01

    Kawasaki disease is an inflammatory condition. Neutrophil to lymphocyte ratio is a marker reflecting inflammation. The aim of the study is to evaluate usefulness of neutrophil to lymphocyte ratio in diagnosis of Kawasaki disease and in prediction of coronary artery lesions. Retrospective cross-sectional study. Seventy-five children with Kawasaki disease and 66 controls were retrospectively enrolled. Their leukocyte, neutrophil, and lymphocyte counts were recorded. Abnormally distributed data were shown as median (interquartile range). Cases having coronary artery diameter two standard deviation above mean were diagnosed to have coronary artery lesions. Median age of Kawasaki disease patients was 34 months. Twentyfive of those (33.33%) had incomplete Kawasaki disease and twenty-six (34.66%) had coronary artery lesions. Leukocyte [12.61 (6.09)×10(3)/µL vs. 8.48 (5.58)×10(3)/µL], neutrophil [6.73 (4.10) ×10(3)/µL vs. 4.62 (5.47)×10(3)/µL], and lymphocyte [4.04 (2.91)×10(3)/µL vs. 3.02 (2.57) ×10(3)/µL] counts were significantly higher in Kawasaki disease patients compared to controls (all p values <0.01). However, there was not significant difference between patients and controls regarding neutrophil to lymphocyte ratio [1.72 (1.22) vs. 1.71 (1.88)]. Findings of Kawasaki disease and incomplete Kawasaki disease cases did not differ, while comparison of patients with and without coronary artery lesions revealed significantly higher neutrophil to lymphocyte ratio values in former group [2.02 (1.63) vs. 1.50 (1.28), p=0.01]. The cut-off neutrophil to lymphocyte ratio value for prediction of coronary artery lesions was determined as 1.32. Neutrophil to lymphocyte ratio values in Kawasaki patients with coronary lesions were significantly higher than the ones without and values greater than 1.32 were useful in prediction of coronary lesions.

  13. Relationship between carotid intima‐media thickness and arterial stiffness in children after Kawasaki disease

    PubMed Central

    Cheung, Y‐f; Wong, S J; Ho, M H K

    2007-01-01

    Background Evidence of premature atherosclerosis and systemic arterial stiffening in patients after Kawasaki disease is accumulating. Aim To test the hypothesis that carotid intima‐media thickness (IMT), a surrogate marker of atherosclerosis, is associated with systemic arterial stiffness in children after Kawasaki disease. Methods A cohort of 72 patients was studied, comprising 26 patients with Kawasaki disease and coronary aneurysms (group I), 24 patients with Kawasaki disease and normal coronary arteries (group II) and 22 healthy age‐matched children (group III). The carotid IMT, carotid artery stiffness index, brachioradial pulse wave velocity (PWV), fasting total cholesterol, high‐density lipoprotein (HDL) cholesterol and low‐density lipoprotein (LDL) cholesterol were determined and compared among the three groups. Results The carotid IMT was related to indices of arterial stiffness, and significant determinants of carotid IMT were identified by multivariate analysis. The mean (standard deviation (SD)) carotid IMT of both group I (0.41 (0.04) mm) and group II (0.39 (0.04) mm) was significantly greater than that of group III (0.36 (0.04) mm; p<0.001 and p = 0.008, respectively). For the entire cohort, carotid IMT correlated positively with LDL cholesterol (r = 0.31, p = 0.009), carotid artery stiffness index (r = 0.40, p = 0.001) and brachioradial PWV (r = 0.28, p = 0.016), but not with age, body mass index, systemic blood pressure, and HDL and total cholesterol. Multiple linear regression analysis identified carotid artery stiffness index (β = 0.25, p = 0.028) and subject grouping (β = −0.39, p = 0.001; model R2 = 0.29) as significant correlates of carotid IMT. Conclusion The increased carotid IMT in children after Kawasaki disease is associated with systemic arterial stiffening. PMID:16820386

  14. Oxidative stress and Kawasaki disease: how is oxidative stress involved from the acute stage to the chronic stage?

    PubMed

    Yahata, Tomoyo; Hamaoka, Kenji

    2017-01-01

    Inflammation and oxidative stress are closely related. Further, oxidative stress plays an important role in the pathology of inflammation-based Kawasaki disease. An excessive in vivo production of reactive oxygen species increases oxidative stress in the body, which triggers an endless vicious spiral of inflammation reactions and reactive oxygen metabolites. This presumably forms diffuse vasculitis in the acute phase. Acute inflammation and oxidative stress can be rapidly controlled by treatments; however, they may remain for a long time. This has recently been identified as a problem in the chronic phase of Kawasaki disease. Generally, the presence of vascular inflammation and oxidative stress impairs blood vessels, leading to the onset of atherosclerosis, which is a widely recognized risk. The current discussion focuses on whether the same is valid for blood vessels in the chronic phase of Kawasaki disease.

  15. Ruptured pediatric posterior cerebral artery aneurysm 9 years after the onset of Kawasaki disease: a case report.

    PubMed

    Tanaka, Satoshi; Sagiuchi, Takao; Kobayashi, Ikuo

    2007-06-01

    A 12-year-old boy who had a history of Kawasaki disease 9 years ago experienced a subarachnoid hemorrhage by ruptured right posterior cerebral artery aneurysm. On day 1 operation, as the aneurysm was very fragile and bled easily, two intraoperative ruptures, including a very premature rupture, were encountered. As a result, a left hemiparesis especially severe in the left hand was caused by the right anterior thalamic infarction due to the occlusion of a thalamo-perforating artery arising near the neck of the aneurysm. The histopathological examination of the intraoperative excised aneurysmal dome disclosed the thickening of the endothelial inner due to the endothelial hypertrophy and the invasion of inflammatory cells. This finding of the aneurysm was partially mimicking the finding of the coronary artery of the patients with Kawasaki disease. The combination of cerebral aneurysm and Kawasaki disease has never been reported until now, and the etiology of the aneurysm of this patient is unclear.

  16. Meta-analysis of the relationship between single nucleotide polymorphism rs72689236 of caspase-3 and Kawasaki disease.

    PubMed

    Xing, Yanlin; Wang, Hong; Liu, Xiaomei; Yu, Xianyi; Chen, Rui; Wang, Ce; Yu, Xuexin; Sun, Le

    2014-10-01

    Kawasaki disease is a pediatric systemic vasculitis of unknown etiology, for which a genetic influence is suspected. But whether single nucleotide polymorphism (SNP) of caspase-3 rs72689236 is associated with Kawasaki disease is controversial. The aim of our study is to assess the association between the SNP of caspase-3 and risk for Kawasaki disease. We searched PubMed, MEDLINE, EMBASE, Springer, Elsevier Science Direct, Cochrane Library Google scholar, CNKI (China National Knowledge Infrastructure, in Chinese) and Wanfang database (in Chinese) to identify studies investigating the association between rs72689236 polymorphism and Kawasaki disease occurrence. There were five eligible studies, which included 4,241 (case group 1,560; control group 2,681) participants in this meta-analysis. Pooled odds ratios (ORs) and 95 % confidence intervals (95 % CIs) were calculated in a fixed-effects model (the Mantel-Haenszel method) or a random-effects model (the DerSimonian and Laird method) when appropriate. Significant associations were found under the overall ORs for A-allele comparison (A vs. G, pooled OR 1.33, 95 % CI 1.21-1.46), AA versus GG comparison (pooled OR 1.64, 95 % CI 1.35-2.00), GA versus GG comparison (pooled OR 1.42, 95 % CI 1.24-1.63), recessive model (AA vs. GG + GA, pooled OR 1.37, 95 % CI 1.15-1.64) and dominant model (AA + GA vs. GG, pooled OR 1.47, 95 % CI 1.29-1.67). This meta-analysis suggested that SNP rs72689236 of caspase-3 might be associated with susceptibility of Kawasaki disease and the allele A might increase the risk of Kawasaki disease in Asian samples such as Japanese and Chinese. In addition, individual studies with large sample size are needed to further evaluate the associations in various ethnic populations.

  17. Comparison of early and late intravenous gamma globulin treatment of Kawasaki disease on fever and cardiovascular complications

    PubMed Central

    Mohammadzadeh, Iraj; Noei, Somayyeh; Babazadeh, Kazem; Zamani, Hassan; Barari-Savadkoohi, Rahim; Alizadeh-Navaei, Reza

    2016-01-01

    Background: Cardiac involvement was the major leading cause of death in patients with Kawasaki and IVIG administration reduces cardiac complications. The objective of this study was to determine the frequency of cardiovascular complications and duration of fever with regard to the time of intravenous immunoglobulin (IVIG) administration of patients with Kawasaki disease. Methods: This follow-up study was done on all patients with Kawasaki disease who were hospitalized at Amirkola Children’s Hospital between 2006 and 2011. Diagnosis of Kawasaki was clinical and included fever more than 5 days with 4 of 5 signs containing mucosal changes, scaling and skin rash, bilateral nonexudative conjunctivitis, cervical lymph adenopathy and edema in lower extremities. After diagnosis of Kawasaki, all patients received standard treatment (intravenous immunoglobulins and aspirin) and undergoing cardiac echocardiography in 2 weeks, 2 months and 6 months. Information including age, sex, sign of diseases, laboratory findings, and cardiac complications in echocardiography were recorded. Results: This study was performed on 100 patients (61 boys and 39 girls) with Kawasaki disease. The mean age of children was 2.8±2.6 years. Cardiac complication rate was 47% at the onset of the disease and had reached to 7% at the end of the sixth month (P=0.000). Distribution of cardiovascular complications in the second week, the second month and the sixth month after treatment was not significantly different according to the start of time of treatment (p>0.05). Duration of fever in patients who received treatment before 10th day (1.5±1.3) did not have significant difference (P=0.78) with patients who received after 10th day (1.6±0.9). Conclusion: Result shows that most of patients (99%) responded to the treatment with IVIG and ASA and cardiovascular complication ratio decreased. There was not significant relationship between duration of fever and time of IVIG treatment initiation. PMID:27757208

  18. [Identification of mutations associated with coronary artery lesion susceptibility in Kawasaki disease by targeted enrichment of genomic region sequencing technique].

    PubMed

    Zhu, D Y; Song, S R; Xie, L J; Qiu, F; Yang, J; Xiao, T T; Huang, M

    2017-07-02

    Objective: To screen and identify the mutations in Kawasaki disease by targeted enrichment of genomic region sequencing technique and investigate susceptibility genes associated with coronary artery lesion. Method: This was a case-control study.A total of 114 patients diagnosed as Kawasaki disease treated in Shanghai Children's Hospital between December 2015 and November 2016 were studied and another 45 healthy children who were physically examined in outpatient department were enrolled as control group. Patients were divided into two groups based on the results of echocardiogram. Peripheral venous blood was obtained from patients and controls. Genomic DNA was extracted. SeqCap EZ Choice libraries were prepared by targeted enrichment of genomic region technology. Then the libraries were sequenced to identify susceptibility genes associated with coronary artery lesion in patients diagnosed as Kawasaki disease.Susceptible genes were identified by Burden test, Pearson chi-square test or Fisher's exact probability test. Result: There was statistically significant difference in TNFRSF11B(rs2073618)G>C(p.N3K)mutation and GG/GC/CC genotype between Kawasaki disease group and control group(χ(2)=15.52, P=0.00). There was statistically significant difference in TNFRSF13B(rs34562254)C>T(p.P251L)mutation(χ(2)=10.40, P=0.01)and LEFTY1(rs360057)T>G(p.D322A)mutation(χ(2)=8.505, P=0.01)between patients with coronary artery lesions and those without. Conclusion: Targeted enrichment of genomic region sequencing technology can be used to do primary screening for the susceptible genes associated with coronary artery lesions in Chinese Kawasaki patients and may provide theoretical basis for larger sample investigation of risk prediction score standard in Kawasaki disease.

  19. Comparison of early and late intravenous gamma globulin treatment of Kawasaki disease on fever and cardiovascular complications.

    PubMed

    Mohammadzadeh, Iraj; Noei, Somayyeh; Babazadeh, Kazem; Zamani, Hassan; Barari-Savadkoohi, Rahim; Alizadeh-Navaei, Reza

    2016-01-01

    Cardiac involvement was the major leading cause of death in patients with Kawasaki and IVIG administration reduces cardiac complications. The objective of this study was to determine the frequency of cardiovascular complications and duration of fever with regard to the time of intravenous immunoglobulin (IVIG) administration of patients with Kawasaki disease. This follow-up study was done on all patients with Kawasaki disease who were hospitalized at Amirkola Children's Hospital between 2006 and 2011. Diagnosis of Kawasaki was clinical and included fever more than 5 days with 4 of 5 signs containing mucosal changes, scaling and skin rash, bilateral nonexudative conjunctivitis, cervical lymph adenopathy and edema in lower extremities. After diagnosis of Kawasaki, all patients received standard treatment (intravenous immunoglobulins and aspirin) and undergoing cardiac echocardiography in 2 weeks, 2 months and 6 months. Information including age, sex, sign of diseases, laboratory findings, and cardiac complications in echocardiography were recorded. This study was performed on 100 patients (61 boys and 39 girls) with Kawasaki disease. The mean age of children was 2.8±2.6 years. Cardiac complication rate was 47% at the onset of the disease and had reached to 7% at the end of the sixth month (P=0.000). Distribution of cardiovascular complications in the second week, the second month and the sixth month after treatment was not significantly different according to the start of time of treatment (p>0.05). Duration of fever in patients who received treatment before 10(th) day (1.5±1.3) did not have significant difference (P=0.78) with patients who received after 10(th) day (1.6±0.9). Result shows that most of patients (99%) responded to the treatment with IVIG and ASA and cardiovascular complication ratio decreased. There was not significant relationship between duration of fever and time of IVIG treatment initiation.

  20. Th17- and Treg-related cytokine and mRNA expression are associated with acute and resolving Kawasaki disease.

    PubMed

    Guo, M M-H; Tseng, W-N; Ko, C-H; Pan, H-M; Hsieh, K-S; Kuo, H-C

    2015-03-01

    Kawasaki disease is a vasculitis most commonly afflicting children <5 years of age. Many autoimmune diseases are associated with up-regulation of T helper (Th) 17 cells, and down-regulation Treg cells. Few studies have examined the Th17/Treg expression in Kawasaki disease. Blood samples were obtained from 186 children with Kawasaki disease at 24 h before IVIG therapy, followed by 3 days and 21 days after IVIG therapy. Thirty children with an acute febrile infectious disease and 30 healthy children were obtained as control. Plasma levels of Th17- and Treg-related cytokines including IL-6, IL-17A, IL-10, TGF-β, and mRNA expression levels of RORγt and Foxp3 were tested. Patients with Kawasaki disease had higher levels of plasma IL-17A (25.35 ± 3.21 vs 7.78 ± 1.78 pg/ml, P < 0.001) and IL-6 (152.29 ± 21.94 vs 38.63 ± 12.40 pg/ml, P < 0.001) when compared to the febrile control group. IVIG resulted in a reduction in IL-6 and IL-17A at both 3 and 21 days after IVIG therapy. FoxP3 levels increased significantly 3 days after IVIG therapy (2.28 ± 0.34 vs 0.88 ± 0.14, P < 0.001). IVIG resistance was associated with higher levels of IL-10 and IL-17A. Kawasaki disease was associated with higher IL-17A and IL-6, a cytokine profile similar to other autoimmune diseases. IVIG therapy resulted in increased expression of Treg-related FoxP3. IVIG resistance was associated with higher levels of IL-10 and IL-17A. Our findings provide further evidence that Kawasaki disease is an autoimmune-like disease. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  1. N-terminal pro-brain natriuretic peptide can be an adjunctive diagnostic marker of hyper-acute phase of Kawasaki disease.

    PubMed

    Kwon, Hyuksool; Lee, Jin Hee; Jung, Jae Yun; Kwak, Young Ho; Kim, Do Kyun; Jung, Jin Hee; Chang, Ikwan; Kim, Kyuseok

    2016-12-01

    The purpose of this study was to determine whether the serum N-terminal pro-brain natriuretic peptide (NT-proBNP) level could be a useful marker for Kawasaki disease in the pediatric emergency department (PED) and in the presence of fever duration of 4 days or less (hyper-acute phase of Kawasaki disease). Medical records of patients who were 1 month to 15 years old of age and presented at the PED with suspected Kawasaki disease from January 1, 2010, to December 31, 2014, were collected retrospectively. Two hundred thirty-nine patients with a history of fever for 4 days or less were diagnosed with Kawasaki disease, as well as 111 patients with other febrile diseases, and were enrolled. The NT-proBNP level was significantly higher in patients with Kawasaki disease (Kawasaki disease vs. other febrile disease group, 444.8 (189.7-951.5) vs. 153.4 (68.9-287.6) pg/mL; p < 0.001), and a cutoff value of 244.7 pg/mL yielded a sensitivity and specificity of 68.6 and 70.3 %, respectively. The area under the curve of the NT-proBNP for predicting Kawasaki disease was 0.763 (95 % CI 0.712-0.814).

  2. Two Cases of Super-Giant Coronary Aneurysms after Kawasaki Disease

    PubMed Central

    Lee, Joowon; Kwon, Bo Sang; Bae, Eun Jung; Noh, Chung Il

    2014-01-01

    Acute giant coronary aneurysm after Kawasaki disease (KD) is a catastrophic complication that can be fatal and very difficult to manage. However, no fixed consensus has been reached for the management of super-giant coronary aneurysms in the acute setting. Here, we report the successful management of young children with super-giant coronary aneurysms after KD. Based on our experience, hemodynamic stabilization to prevent further coronary dilation or rupture and strict anticoagulation to avoid thrombus formation are mandatory in the management of this condition. PMID:24497892

  3. Biphasic thallium 201 SPECT-imaging for the noninvasive diagnosis of myocardial perfusion abnormalities in a child with Kawasaki disease--a case report

    SciTech Connect

    Hausdorf, G.; Nienaber, C.A.; Spielman, R.P.

    1988-02-01

    The mucocutaneous lymph node syndrome (Kawasaki disease) is of increasing importance for the pediatric cardiologist, for coronary aneurysms with the potential of thrombosis and subsequent stenosis can develop in the course of the disease. The authors report a 2 1/2-year-old female child in whom, fourteen months after the acute phase of Kawasaki disease, myocardial infarction occurred. Biphasic thallium 201 SPECT-imaging using dipyridamole depicted anterior wall ischemia and inferolateral infarction. This case demonstrates that noninvasive vasodilation-redistribution thallium 201 SPECT-imaging has the potential to predict reversible myocardial perfusion defects and myocardial necrosis, even in small infants with Kawasaki disease.

  4. The first case of Kawasaki disease in a 20-month old baby following immunization with rotavirus vaccine and hepatitis A vaccine in China: A case report.

    PubMed

    Yin, Shi; Liubao, Peng; Chongqing, Tan; Xiaomin, Wan

    2015-01-01

    Kawasaki disease (KD) after rotavirus and hepatitis A vaccination has not previously been reported in a baby in China. Herein, we describe a 20-month-old child who developed Kawasaki disease after receiving her second dose of Lanzhou lamb rotavirus vaccine (LLR) and her first dose of freeze-dried live attenuated hepatitis A vaccine. The case report was conducted by collecting and analyzing the hospital in-patient medical records and reviewing both the domestic and foreign pertinent literature. These findings will be important to note this possible side effect and to further investigate the association between the above 2 vaccines and Kawasaki disease.

  5. Information Systems for Government and Business: Trends, Issues, Challenges. Proceedings of the Kawasaki International Seminar on the Information Systems Challenge for Government and Business (2nd, Kawasaki City, Japan, July 21-24, 1987).

    ERIC Educational Resources Information Center

    United Nations Centre for Regional Development, Nagoya (Japan).

    The 25 papers in this proceedings were presented by national and international information systems experts, development planners, and scholars at a seminar that was jointly sponsored by the United Nations Centre for Regional Development and the city of Kawasaki, Japan. Designed to reach a wider group of interested planning practitioners and…

  6. Percutaneous coronary intervention for acute myocardial infarction in a pediatric patient with coronary aneurysm and stenosis due to Kawasaki disease.

    PubMed

    Drossner, David M; Chappell, Clay; Rab, Tanveer; Kim, Dennis

    2012-06-01

    We report the case of an acutely ill 3-year-old female, with a previous medical history of Kawasaki disease, who presented to care with an acute myocardial infarction. We describe the coordinated therapies employed by pediatric and adult cardiologists aimed to establish coronary revascularization.

  7. Intravenous immunoglobulin treatment responsiveness depends on the degree of CD8+ T cell activation in Kawasaki disease.

    PubMed

    Ye, Qing; Gong, Fang-Qi; Shang, Shi-Qiang; Hu, Jian

    2016-10-01

    Kawasaki disease (KD) has become the most common cause of acquired heart disease in children and is also a risk factor for ischemic heart disease in adults. However, Kawasaki disease lacks specific laboratory diagnostic indices. Thus, this study analyzed the T cell activation profiles of Kawasaki disease and assessed their value in the diagnosis of Kawasaki disease and the prediction of intravenous immunoglobulin (IVIG) sensitivity. We analyzed human leukocyte antigen-DR (HLA-DR), CD69 and CD25 expression on peripheral blood CD4+ and CD8+ T cells during the acute phase of KD. We compared the percentages of HLA-DR+/CD69+/CD25+ T cells in the CD4+ and CD8+ T cell populations of IVIG-effective and IVIG-resistant groups. Receiver operating characteristic curves were used to assess the diagnostic value of the above parameters. The median percentage of CD8+HLA-DR+ T cells and the median ratio of CD8+HLA-DR+ T cells/CD8+CD25+ T cells were significantly elevated in the patient group compared with those in the control group during the acute phase of KD. Regarding the diagnosis of Kawasaki disease, the area under the ROC curve was 0.939 for the percentage of CD8+HLA-DR+ T cells. There was a significant difference in the ratio of CD8+HLA-DR+ T cells/CD8+CD69+ T cells between IVIG-resistant patients and IVIG-sensitive patients. Regarding IVIG sensitivity, the area under the ROC curve was 0.795 for it. Excessive CD8+ T cell activation, as well as an imbalance between CD8+ T cell activation and inhibition, underlies the pathogenesis of Kawasaki disease. The percentage of CD8+ HLA-DR+ T cells may be used as an index to diagnose Kawasaki disease. IVIG inhibits CD8+ T cell activation, but excessive CD8+ T cell activation may cause IVIG resistance. The ratio of CD8+HLA-DR+ T cells/CD8+CD69+ T cells may be used as a predictor of IVIG sensitivity.

  8. Hospitalisation with Infection, Asthma and Allergy in Kawasaki Disease Patients and Their Families: Genealogical Analysis Using Linked Population Data

    PubMed Central

    Webster, Rebecca J.; Carter, Kim W.; Warrington, Nicole M.; Loh, Angeline M.; Zaloumis, Sophie; Kuijpers, Taco W.; Palmer, Lyle J.; Burgner, David P.

    2011-01-01

    Background Kawasaki disease results from an abnormal immunological response to one or more infectious triggers. We hypothesised that heritable differences in immune responses in Kawasaki disease-affected children and their families would result in different epidemiological patterns of other immune-related conditions. We investigated whether hospitalisation for infection and asthma/allergy were different in Kawasaki disease-affected children and their relatives. Methods/Major Findings We used Western Australian population-linked health data from live births (1970–2006) to compare patterns of hospital admissions in Kawasaki disease cases, age- and sex-matched controls, and their relatives. There were 295 Kawasaki disease cases and 598 age- and sex-matched controls, with 1,636 and 3,780 relatives, respectively. Compared to controls, cases were more likely to have been admitted at least once with an infection (cases, 150 admissions (50.8%) vs controls, 210 admissions (35.1%); odds ratio (OR) = 1.9, 95% confidence interval (CI) 1.4–2.6, P = 7.2×10−6), and with asthma/allergy (cases, 49 admissions (16.6%) vs controls, 42 admissions (7.0%); OR = 2.6, 95% CI 1.7–4.2, P = 1.3×10−5). Cases also had more admissions per person with infection (cases, median 2 admissions, 95% CI 1–5, vs controls, median 1 admission, 95% CI 1–4, P = 1.09×10−5). The risk of admission with infection was higher in the first degree relatives of Kawasaki disease cases compared to those of controls, but the differences were not significant. Conclusion Differences in the immune phenotype of children who develop Kawasaki disease may influence the severity of other immune-related conditions, with some similar patterns observed in relatives. These data suggest the influence of shared heritable factors in these families. PMID:22140498

  9. Acute Myocardial Ischemia in Adults Secondary to Missed Kawasaki Disease in Childhood

    PubMed Central

    Rizk, Sherif RY; El Said, Galal; Daniels, Lori B; Burns, Jane C; El Said, Howaida; Sorour, Khaled A; Gharib, Soliman; Gordon, John B

    2015-01-01

    Coronary artery aneurysms that occur in 25% of untreated Kawasaki disease (KD) patients may remain clinically silent for decades and then thrombose resulting in myocardial infarction. Although KD is now the most common cause of acquired heart disease in children in Asia, the United States, and Western Europe, the incidence of KD in Egypt is unknown. We tested the hypothesis that young adults in Egypt presenting with acute myocardial ischemia may have coronary artery lesions due Kawasaki disease (KD) in childhood. We reviewed a total of 580 angiograms of patients ≤ 40 years of age presenting with symptoms of myocardial ischemia. Coronary artery aneurysms were noted in 46 patients (7.9 %) of whom nine presented with myocardial infarction. The likelihood of antecedent KD as the cause of the aneurysms was classified as definite (n=10), probable (n=29), or equivocal (n=7). Compared to the definite and probable groups, the equivocal group had more traditional cardiovascular risk factors, smaller sized aneurysms, and fewer coronary arteries affected. In conclusion, in a major metropolitan center in Egypt, 6.7% of adults age 40 years or younger undergoing angiography for evaluation of possible myocardial ischemia had lesions consistent with antecedent KD. Because of the unique therapeutic challenges associated with these lesions, adult cardiologists should be aware that coronary artery aneurysms in young adults may be due to missed KD in childhood. PMID:25555655

  10. Dipyridamole-thallium-201 tomography documenting improved myocardial perfusion with therapy in Kawasaki disease

    SciTech Connect

    Nienaber, C.A.; Spielmann, R.P.; Hausdorf, G.

    1988-12-01

    Thallium-201 tomographic perfusion studies after pharmacologic vasodilation were performed in seven children (aged 2 years 8 months to 8 years 7 months), 3 to 20 months after the acute stage of the disease. In all patients coronary aneurysms were seen on cross-sectional echocardiograms. The scintigrams of six children showed no significant regional reduction of myocardial thallium-201 uptake. These children had remained asymptomatic in the follow-up period after the acute inflammatory stage of Kawasaki disease. Persistent and transient thallium defects were present in one child with acute posterolateral myocardial infarction; obstruction of two coronary vessels supplying the defect zones was confirmed by contrast angiography. After 8 months of treatment a follow-up nuclear scan showed marked reduction in the size of the defect and almost complete abolishment of the ischemic reaction. Thus tomographic thallium-201 perfusion scintigraphy in conjunction with vasodilation stress is useful to assess myocardial perfusion in children with Kawasaki disease and demonstrates marked improvement in regional perfusion after adequate medical therapy.

  11. Population-Based Study of the Association between Urbanization and Kawasaki Disease in Taiwan

    PubMed Central

    Chang, Wei-Pin; Chang, Wei-Chiao

    2013-01-01

    Background. It is unclear if the prevalence of Kawasaki disease (KD) correlates with the degree of urbanization. We hypothesized that the prevalence of KD is more pronounced in urban versus rural environments. Methods. The National Health Insurance (NHI) program was implemented in Taiwan in 1995 and covers most of the population (>99%). We used the NHI database to investigate the epidemiological features of KD. A total of 115 diagnosed patients with KD from 1997 to 2010 were included, together with 1,150 matched controls without KD. Chi-square analyses were performed to investigate the difference between modern city and rural environments. Results. Of the 1265 sampled subjects (claims data from 1,000,000 random subjects), the mean age of the KD study group and control group was 2.08 ± 1.66 and 2.08 ± 1.64 years, respectively. After matching for age, sex, and same index date, no statistically significant differences in urbanization level and geographical location of the patients' residence were observed. Conclusion. Urbanization did not appear to be an important effect modifier of Kawasaki disease in Taiwan. PMID:23864819

  12. Novel predictors of intravenous immunoglobulin resistance in Chinese children with Kawasaki disease.

    PubMed

    Fu, Pei-pei; Du, Zhong-dong; Pan, Yue-song

    2013-08-01

    The purpose of this study was to develop a predictive scoring system to identify intravenous immunoglobulin resistance in children with Kawasaki disease, to implement additional therapies early in the course of their illness and prevent coronary artery lesions. We performed a retrospective review of children with Kawasaki disease treated within 10 days of fever onset. To identify independent predictors of intravenous immunoglobulin resistance, multivariable logistic regression models were constructed using variables selected by univariable analysis. The independent predictors were combined into a new scoring system and compared with 2 existing systems. The discriminatory capacity of the scoring system was assessed using the area under the receiver operating characteristic curves. By logistic regression analysis, polymorphous exanthema, changes around the anus, days of illness at initial treatment, percentage of neutrophils, C-reactive protein levels, albumin levels, and total bilirubin proved to be independent predictors of intravenous immunoglobulin resistance. The new scoring system gave an area under the receiver operating characteristic curve of 0.672. In this scoring system, 2 risk strata were identified: low risk, with scores of 0-3, and high risk, with scores of ≥4. The sensitivity was 54.1% and the specificity was 71.2%. The new scoring system had a higher specificity and sensitivity for Chinese children, compared with the Kobayashi scoring system and the Egami scoring system, but, unfortunately, the new scoring system was not good enough to be widely used because of its low sensitivity.

  13. Damage spreading on the 3-12 lattice with competing Glauber and Kawasaki dynamics

    NASA Astrophysics Data System (ADS)

    Guo, Z. Z.; Wu, Xiao-Wei; Wang, Chun-An

    2006-06-01

    The damage spreading of the Ising model on the 3-12 lattice with competing Glauber and Kawasaki dynamics is studied. The difference between the two kinds of nearest-neighboring spin interactions (interaction between two 12-gons, or interaction between a 12-gon and a triangle) are considered in the Hamiltonian. It is shown that the ratio of the interaction strength F between the two kinds of interactions plays an important role in determining the critical temperature T_{d} of phase transition from frozen to chaotic. Two methods are used to introduce the bond dilution on the Ising model on the 3-12 lattice: regular and random. The maximum of the average damage spreading < Drangle_{max} can approach values lower than 0.5 in both cases and the reason can be attributed to the `survivors' among the spins. We have also, for the first time, presented the phase diagram of the mixed G-K dynamics in the 3-12 lattice which shows what happens when going from pure Glauber to pure Kawasaki.

  14. Pattern formation with repulsive soft-core interactions: Discrete particle dynamics and Dean-Kawasaki equation

    NASA Astrophysics Data System (ADS)

    Delfau, Jean-Baptiste; Ollivier, Hélène; López, Cristóbal; Blasius, Bernd; Hernández-García, Emilio

    2016-10-01

    Brownian particles interacting via repulsive soft-core potentials can spontaneously aggregate, despite repelling each other, and form periodic crystals of particle clusters. We study this phenomenon in low-dimensional situations (one and two dimensions) at two levels of description: by performing numerical simulations of the discrete particle dynamics and by linear and nonlinear analysis of the corresponding Dean-Kawasaki equation for the macroscopic particle density. Restricting to low dimensions and neglecting fluctuation effects, we gain analytical insight into the mechanisms of the instability leading to clustering which turn out to be the interplay among diffusion, the intracluster forces, and the forces between neighboring clusters. We show that the deterministic part of the Dean-Kawasaki equation provides a good description of the particle dynamics, including width and shape of the clusters and over a wide range of parameters, and analyze with weakly nonlinear techniques the nature of the pattern-forming bifurcation in one and two dimensions. Finally, we briefly discuss the case of attractive forces.

  15. The Γ-Limit of the Two-Dimensional Ohta-Kawasaki Energy. I. Droplet Density

    NASA Astrophysics Data System (ADS)

    Goldman, Dorian; Muratov, Cyrill B.; Serfaty, Sylvia

    2013-11-01

    This is the first in a series of two papers in which we derive a Γ-expansion for a two-dimensional non-local Ginzburg-Landau energy with Coulomb repulsion, also known as the Ohta-Kawasaki model, in connection with diblock copolymer systems. In that model, two phases appear, which interact via a nonlocal Coulomb type energy. We focus on the regime where one of the phases has very small volume fraction, thus creating small "droplets" of the minority phase in a "sea" of the majority phase. In this paper we show that an appropriate setting for Γ-convergence in the considered parameter regime is via weak convergence of the suitably normalized charge density in the sense of measures. We prove that, after a suitable rescaling, the Ohta-Kawasaki energy functional Γ-converges to a quadratic energy functional of the limit charge density generated by the screened Coulomb kernel. A consequence of our results is that minimizers (or almost minimizers) of the energy have droplets which are almost all asymptotically round, have the same radius and are uniformly distributed in the domain. The proof relies mainly on the analysis of the sharp interface version of the energy, with the connection to the original diffuse interface model obtained via matching upper and lower bounds for the energy. We thus also obtain an asymptotic characterization of the energy minimizers in the diffuse interface model.

  16. Generalized competing Glauber-type dynamics and Kawasaki-type dynamics

    NASA Astrophysics Data System (ADS)

    Zhu, Han; Zhu, Jian-Yang; Zhou, Yang

    2002-09-01

    In this paper, we have given a systematic formulation of a generalized competing mechanism: The Glauber-type single-spin transition mechanism, with probability p, simulates the contact of the system with the heat bath, and the Kawasaki-type spin-pair redistribution mechanism, with probability 1-p, simulates an external energy flux. These two mechanisms are natural generalizations of Glauber's single-spin flipping mechanism and Kawasaki's spin-pair exchange mechanism respectively. On the one hand, the proposed mechanism is, in principle, applicable to arbitrary systems, while on the other hand, our formulation is able to contain a mechanism that just directly combines single-spin flipping and spin-pair exchange in their original form. Compared with the conventional mechanism, the proposed mechanism does not assume the simplified version and leads to a greater influence of temperature. The fact, order for lower temperature and disorder for higher temperature, will be universally true. In order to exemplify this difference, we applied the mechanism to the one-dimensional Ising model and obtained analytical results. We also applied this mechanism to the kinetic Gaussian model and found that above the critical point there will be only paramagnetic phase, while below the critical point, the self-organization as a result of the energy flux will lead the system to an interesting heterophase, instead of the initially guessed antiferromagnetic phase. We studied this process in details.

  17. Evaluation of myocardial deformation in patients with Kawasaki disease using speckle-tracking echocardiography during mid-term follow-up.

    PubMed

    Dedeoglu, Reyhan; Barut, Kenan; Oztunc, Funda; Atik, Sezen; Adrovic, Amra; Sahin, Sezgin; Cengiz, Dicle; Kasapcopur, Ozgur

    2017-04-05

    Speckle-tracking echocardiography is a recently developed technique for the evaluation of myocardial deformation or strain. Our objective was to examine strain through a mid-term follow-up of patients with Kawasaki disease.

  18. Persistent high fever for more than 10 days during acute phase is a risk factor for endothelial dysfunction in children with a history of Kawasaki disease.

    PubMed

    Mori, Yasuhiko; Katayama, Hiroshi; Kishi, Kanta; Ozaki, Noriyasu; Shimizu, Tatsuo; Tamai, Hiroshi

    2016-07-01

    Endothelial dysfunction has previously been reported in children with a history of Kawasaki disease, but the determinants of endothelial function in Kawasaki disease patients are still unknown. In this study, we investigated endothelial function in Kawasaki disease patients and attempted to identify risk factors for persistent endothelial dysfunction. Using high-resolution ultrasound, we measured the percent flow-mediated dilatation, an arterial response to reactive hyperemia, to evaluate endothelial function in 67 patients with a history of Kawasaki disease and 28 age- and sex-matched control subjects. We divided the Kawasaki disease patients into a group with impaired endothelial function (the percent flow-mediated dilatation below -2 standard deviations of the control group) and a group with normal endothelial function (the percent flow-mediated dilatation more than -2 standard deviations of control). Logistic multiple regression analysis was performed to identify independent predictors of impaired endothelial function. In Kawasaki disease patients, the percent flow-mediated dilatation was significantly lower than in the control subjects (9.8±3.6%, compared with 13.1±3.4%, p<0.01). In 13 Kawasaki disease patients (3 patients with coronary artery lesions and 10 patients without coronary artery lesions), the percent flow-mediated dilatation was below -2 standard deviations of control. Logistic multiple regression analysis showed that a febrile period of longer than 10 days during the acute phase was the significant risk factor for endothelial dysfunction (odds ratio: 8.562; 95% confidence interval: 1.366-53.68). Presence of coronary artery lesions was not a determinant of endothelial dysfunction. Systemic endothelial dysfunction exists in children with a history of Kawasaki disease, and a febrile period of longer than 10 days during the acute phase is an independent predictor of endothelial dysfunction irrespective of coronary artery involvement. Copyright

  19. The incidence of Kawasaki disease after vaccination within the UK pre-school National Immunisation Programme: an observational THIN database study.

    PubMed

    Hall, Gillian C; Tulloh, Robert Mr; Tulloh, Louise E

    2016-11-01

    To provide expected incidence rates of Kawasaki disease after vaccination in routine clinical practice and as recommended within a pre-school National Immunisation Programme (NIP). A post-immunisation risk period when Kawasaki disease onset might be associated with vaccination was defined as 28 days. Immunisation records for children under 6 years were identified from The Health Improvement Network (THIN) database of electronic UK primary health care records (2008-2012) and linked to previously validated cases of Kawasaki disease with an assigned date of onset. Kawasaki disease incidence in the risk period after a complete NIP recommended set of vaccinations was estimated for five vaccination stages individually and in total. A total of 642 170 complete pre-school immunisation stages from 275 986 children were included. Six cases of Kawasaki disease had onset in the risk period after any NIP stage providing an incidence of 12.8 per 100 000 person years (95%CI 5.7, 28.4). The incidence after any single immunisation stage ranged from 0 to 27.4 (95%CI 8.8, 84.8) per 100 000 person years. There were few cases of Kawasaki disease in the risk period after any NIP vaccination combination. The incidence rates will aid in the interpretation of clinical trials and post-marketing surveillance of new vaccines. Copyright © 2016 John Wiley & Sons, Ltd. Copyright © 2016 John Wiley & Sons, Ltd.

  20. Coronary Artery Aneurysm Measurement and Z Score Variability in Kawasaki Disease.

    PubMed

    Ronai, Christina; Hamaoka-Okamoto, Akiko; Baker, Annette L; de Ferranti, Sarah D; Colan, Steven D; Newburger, Jane W; Friedman, Kevin G

    2016-02-01

    Coronary artery (CA) Z scores are commonly used for clinical decisions in Kawasaki disease, including treatment, anticoagulation, and duration and frequency of follow-up. The aim of this study was to evaluate CA measurement reproducibility, Z score calculation variability, and the impact of variability on management. Twenty-one patients with Kawasaki disease with right CA (RCA) or left anterior descending CA (LAD) Z scores of 1.5 to 3 (group 1) were randomly selected, and all patients with Kawasaki disease with Z scores of 7 to 14 for either the RCA or LAD (n = 20; group 2) were included from March 2008 to May 2014. Two echocardiographers measured left main CA, LAD, and RCA dimensions. The inter- and intraobserver reliability of absolute measurements was calculated, and the CA Z scores derived from three commonly used formulas were compared. Median age at echocardiography was 1.2 years (range, 0.2-11.5 years), and 68% of subjects (n = 28) were male. Interobserver reliability was high for the LAD (intraclass correlation coefficient [ICC], 96.79%) and RCA (ICC, 93.31%) and lower for the left main CA (ICC, 73.54%). Intraobserver reliability was also high for the LAD and RCA (ICC, 99.08% and 97.74%) and lower for the left main CA (ICC, 80.88%). Calculated Z scores were similar among the three formulas for group 1 but varied markedly in group 2. Calculated Z scores using the same CA measurement in each of the three formulas resulted in different clinical management in up to seven of 21 group 1 patients (22%) and in up to 10 of 20 group 2 patients (50%). Although CA measurements have high inter- and intraobserver agreement, CA Z scores vary dramatically on the basis of the Z score formula at larger CA dimensions. Discrepancies in CA Z score calculators may affect clinical decision making. Copyright © 2016 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

  1. Crystalline keratopathy due to intravenous immunoglobulin in a 12-year-old girl with Kawasaki disease.

    PubMed

    Viswanathan, Vijay; Agashe, Prachi; Jain, Vandana; Nair, Akshay Gopinathan

    2016-10-01

    Kawasaki disease (KD) is an acute, self-limiting, medium-vessel vasculitis that occurs predominantly in young children. The treatment of KD consists of intravenous immunoglobulin (IVIG) along with aspirin and, in IVIG-resistant cases, corticosteroids. Crystalline keratopathy with corneal deposition of the immunoglobulin is a rare complication of IVIG therapy. We report the case of a 12-year-old girl who received IVIG for KD and developed visual complaints, which were attributable to crystalline keratopathy and corneal edema. Cessation of IVIG and treatment with topical and systemic corticosteroids reversed the finding. At final follow-up, vision in both eyes was normal. Copyright © 2016 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

  2. [Use of the urinary trypsin inhibitor ulinastatin for acute Kawasaki disease].

    PubMed

    Kawamura, Yoichi; Kanai, Takashi; Takeshita, Seiichiro; Nonoyama, Shigeaki

    2014-09-01

    Intravenous immunoglobulin (IVIG) therapy is widely recognized as standard treatment for Kawasaki disease(KD). However, about 20 % of KD patients are resistant to IVIG and are considered to be a high risk group for coronary artery lesions (CAL). Ulinastatin(UTI) is one of the neutrophil elastase inhibitors used for patients with pancreatitis or circulatory shock, and several studies have shown its efficacy for KD. Recently, we demonstrated that initial UTI treatment combined with IVIG decreased the number of patients requiring addi- tional rescue treatment and the occurrence of CAL. In this study, no severe adverse events occurred. Further research and a prospective trial are needed to prove the clinical efficacy and demonstrate the limits of UTI in patients with KD.

  3. Kawasaki disease and the emerging coronary artery disease epidemic in India: is there a correlation?

    PubMed

    Singh, Surjit; Aulakh, Roosy; Kawasaki, Tomisaku

    2014-04-01

    Although Kawasaki disease (KD) is now being increasingly reported from India, the vast majority of children with KD are still not being diagnosed and treated. A recent study from Chandigarh has shown that the incidence of KD is at least 4.54/100,000 children below 15 y of age. Extrapolations of this figure suggest that a minimum of 17,417 new cases of KD would be occurring every year in our country. A significant proportion of these children may develop coronary artery abnormalities. These children would then be at risk of developing myocardial ischemia as young adults. It is authors' contention that (undiagnosed) KD in childhood may be contributing to the growing pool of coronary artery disease (CAD) in India. Similarly, a missed diagnosis of KD in childhood should be considered as a possibility while evaluating adults with CAD, especially when there are no overt risk factors and no family history of the disease.

  4. Transient unilateral oculomotor palsy and severe headache in childhood Kawasaki disease.

    PubMed

    Thapa, Rajoo; Mallick, Debkrishna; Biswas, Biswajit; Chakrabartty, Subroto

    2011-01-01

    Transient affliction of the cranial nerves may at times be either the presenting feature or complication of otherwise uncomplicated Kawasaki disease (KD) in infants and children. The present report describes a 6 year 9 month old boy with classical KD who developed right-sided oculomotor nerve palsy (manifested by ipsilateral ptosis and medial rectus palsy) resulting in symptoms like severe nausea, intense frontal headache and double vision. The palsy resolved within 5 days of intravenous immunoglobulin therapy, with no residual ophthalmological abnormality at 6 weeks. Besides increased intracranial pressure, which commonly occurs during the course of KD in children, secondary to aseptic meningitis, intense headache in such children may have cranial nerve paresis as accentuating factors.

  5. [Clinical utility of ulinastatin, urinary protease inhibitor in acute Kawasaki disease].

    PubMed

    Saji, Tsutomu

    2008-02-01

    Ulinastatin, a trypsin inhibitor, is useful as a first-line or a second-line treatment regimen including alternative therapy for IVIG-resistant or IVIG nonresponder Kawasaki disease (KD) patients. Mechanisms involving protections against tissue organs and endthelial cell and anti-inflammatory effects by ulinastatin, are dependent on the inhibition of PMN-derived elastase, tumor necrosis factor alpha (TNFalpha), and other proinflammatory cytokines/interleukins(IL-1, IL-6, IL-8). Ulinastatin also suppresses the activation of PMN cells, macrophages, and platelets. Although almost no statistical data related to the definitive effect in acute stage of KD, ulinastatin have shown possible effects, but not always, in a part of KD patients. The indications of clinical use include shock and pancreatitis. Off-label uses of ulinastatin have been reported in hematological, hepatic, renal, OB/Gy diseases and cardiovascular diseases including vasculitis syndromes. The efficacy of ulinastatin in aKD remained to be investigated.

  6. Downregulation of Th17 cells and the related cytokines with treatment in Kawasaki disease.

    PubMed

    Rasouli, Manoochehr; Heidari, Behzad; Kalani, Mehdi

    2014-11-01

    Given the inflammatory nature of Kawasaki disease (KD) and the pro-inflammatory properties of Th17, this study aimed to determine the frequency of Th17 cells and the levels of corresponding cytokines in acute phase of KD and to evaluate their alterations one and eight weeks after treatment. Th17 and the related cytokine levels were measured in 21 KD patients and 42 positive and negative controls, using flow cytometry and ELISA, respectively. Th17, IL-17, IL-22 and IL-23 were significantly higher (P<0.05) in patients compared with negative controls, but no significant differences (P>0.05) with the positive controls. Furthermore, Th17, IL-17, IL-22 and IL-23 were significantly higher in patients before treatment than those one and eight weeks after. Considering the downregulation of Th17 and its related cytokines with aspirin and intravenous immunoglobulin therapy implies the probable role of Th17 in KD pathogenesis.

  7. Thermodynamics of the one-dimensional parallel Kawasaki model: Exact solution and mean-field approximations

    NASA Astrophysics Data System (ADS)

    Pazzona, Federico G.; Demontis, Pierfranco; Suffritti, Giuseppe B.

    2014-08-01

    The adsorption isotherm for the recently proposed parallel Kawasaki (PK) lattice-gas model [Phys. Rev. E 88, 062144 (2013), 10.1103/PhysRevE.88.062144] is calculated exactly in one dimension. To do so, a third-order difference equation for the grand-canonical partition function is derived and solved analytically. In the present version of the PK model, the attraction and repulsion effects between two neighboring particles and between a particle and a neighboring empty site are ruled, respectively, by the dimensionless parameters ϕ and θ. We discuss the inflections induced in the isotherms by situations of high repulsion, the role played by finite lattice sizes in the emergence of substeps, and the adequacy of the two most widely used mean-field approximations in lattice gases, namely, the Bragg-Williams and the Bethe-Peierls approximations.

  8. Strict management of a pregnant patient with giant coronary artery aneurysm due to Kawasaki disease.

    PubMed

    Taniguchi, Kosuke; Ono, Hiroshi; Sato, Anna; Kinomoto, Satoko; Tagawa, Naomi; Umehara, Nagayoshi; Kato, Hitoshi; Sago, Haruhiko

    2015-10-01

    Coronary artery aneurysms (CAA) may occur in Kawasaki disease (KD). Patients with giant CAA (diameter >8 mm), in particular, have higher risk of myocardial infarction. Previous reports have demonstrated the necessity of anticoagulation therapy in such cases. The management of patients with KD complicated by giant CAA later in life, however, remains controversial. Here, we describe the strict management in the case of a 28-year-old pregnant Japanese woman with KD with giant CAA (diameter, 11 mm). Instead of warfarin, the patient was given low-dose aspirin and i.v. unfractionated heparin during pregnancy to prevent thrombosis in the giant CAA. At 38 weeks of gestation, she had spontaneous delivery of a healthy baby. No thrombotic or bleeding complications were observed. The strict anticoagulation therapy resulted in successful pregnancy and delivery without any adverse events.

  9. Hemodynamic simulations in coronary aneurysms of a patient with Kawasaki Disease

    NASA Astrophysics Data System (ADS)

    Sengupta, Dibyendu; Marsden, Alison; Burns, Jane

    2010-11-01

    Kawasaki Disease is the leading cause of acquired pediatric heart disease, and can cause large coronary artery aneurysms in untreated cases. A simulation case study has been performed for a 10-year-old male patient with coronary aneurysms. Specialized coronary boundary conditions along with a lumped parameter heart model mimic the interactions between the ventricles and the coronary arteries, achieving physiologic pressure and flow waveforms. Results show persistent low shear stress in the aneurismal regions, and abnormally high shear at the aneurysm neck. Correlation functions have been derived to compare wall shear stress and wall shear stress gradients with recirculation time with the idea of localizing zones of calcification and thrombosis. Results are compared with those of an artificially created normal coronary geometry for the same patient. The long-term goal of this work is to develop links between hemodynamics and thrombotic risk to assist in clinical decision-making.

  10. Blinded case-control study of the relationship between human coronavirus NL63 and Kawasaki syndrome.

    PubMed

    Dominguez, Samuel R; Anderson, Marsha S; Glodé, Mary P; Robinson, Christine C; Holmes, Kathryn V

    2006-12-15

    We conducted a blinded, case-control, retrospective study in pediatric patients hospitalized at The Children's Hospital, Denver, Colorado, to determine whether human coronavirus (HCoV)-NL63 infection is associated with Kawasaki syndrome (KS). Over the course of a 7-month period, nasopharyngeal-wash samples from 2 (7.7%) of 26 consecutive children with KS and 4 (7.7%) of 52 matched control subjects tested positive for HCoV-NL63 by reverse transcription-polymerase chain reaction. These data suggest that, although HCoV-NL63 was circulating in children in our community during the time of the study, the prevalence of infection with HCoV-NL63 was not greater in patients with KS than in control subjects.

  11. In Vitro Validation of Patient-Specific Hemodynamic Simulations in Coronary Aneurysms Caused by Kawasaki Disease

    PubMed Central

    Kung, Ethan; Kahn, Andrew M.; Burns, Jane C.; Marsden, Alison

    2014-01-01

    To perform experimental validation of computational fluid dynamics (CFD) applied to patient specific coronary aneurysm anatomy of Kawasaki disease. We quantified hemodynamics in a patient-specific coronary artery aneurysm physical phantom under physiologic rest and exercise flow conditions. Using phase contrast MRI (PCMRI), we acquired 3-component flow velocity at two slice locations in the aneurysms. We then performed numerical simulations with the same geometry and inflow conditions, and performed qualitative and quantitative comparisons of velocities between experimental measurements and simulation results. We observed excellent qualitative agreement in flow pattern features. The quantitative spatially and temporally varying differences in velocity between PCMRI and CFD were proportional to the flow velocity. As a result, the percent discrepancy between simulation and experiment was relatively constant regardless of flow velocity variations. Through 1D and 2D quantitative comparisons, we found a 5–17% difference between measured and simulated velocities. Additional analysis assessed wall shear stress differences between deformable and rigid wall simulations. This study demonstrated that CFD produced good qualitative and quantitative predictions of velocities in a realistic coronary aneurysm anatomy under physiological flow conditions. The results provide insights on factors that may influence the level of agreement, and a set of in vitro experimental data that can be used by others to compare against CFD simulation results. The findings of this study increase confidence in the use of CFD for investigating hemodynamics in the specialized anatomy of coronary aneurysms. This provides a basis for future hemodynamics studies in patient-specific models of Kawasaki disease. PMID:25050140

  12. Clinical manifestations of Kawasaki disease shock syndrome: a case-control study.

    PubMed

    Chen, Pei-Shin; Chi, Hsin; Huang, Fu-Yuan; Peng, Chun-Chih; Chen, Ming-Ren; Chiu, Nan-Chang

    2015-02-01

    Kawasaki disease shock syndrome (KDSS) is a severe condition related to Kawasaki disease (KD), and sometimes it is difficult to diagnose. This is a case-control study to ascertain the clinical presentations, risk factors, and clinical outcomes of children who had KDSS. Children who were hospitalized during 2001-2011 with the diagnosis of KD combined with hypotension, sepsis, or shock were retrospectively reviewed and were defined as case patients. For each case patient, three season-matched patients diagnosed as having KD with normal blood pressure were identified to serve as control patients. Demographic characteristics, clinical presentations, laboratory features, therapies, and outcomes were analyzed. Nine KDSS patients and 27 control patients were identified. The average age of patients with KDSS was 3.2 ± 3.2 years. Compared with controls, KDSS patients were less likely to have a diagnosis of KD at admission (22.2% vs. 66.7%) and had a higher risk of coronary artery dilatation (77.8% vs. 11.1%). Risk factors for KDSS included higher neutrophil counts and proportions of bands, higher C-reactive protein (CRP), and lower platelet counts. All case patients received aspirin therapy; eight patients received intravenous immunoglobulin therapy, with two receiving more than one course. Seven KDSS patients required fluid resuscitation, and eight patients required vasoactive infusions. Patients with KDSS may have uneven clinical course and may be misdiagnosed in the beginning. They may have more prominent inflammatory markers in the early phase and higher risk of coronary artery dilatation. Copyright © 2013. Published by Elsevier B.V.

  13. Urine proteomics for discovery of improved diagnostic markers of Kawasaki disease

    PubMed Central

    Kentsis, Alex; Shulman, Andrew; Ahmed, Saima; Brennan, Eileen; Monuteaux, Michael C; Lee, Young-Ho; Lipsett, Susan; Paulo, Joao A; Dedeoglu, Fatma; Fuhlbrigge, Robert; Bachur, Richard; Bradwin, Gary; Arditi, Moshe; Sundel, Robert P; Newburger, Jane W; Steen, Hanno; Kim, Susan

    2013-01-01

    Kawasaki disease (KD) is a systemic vasculitis of unknown etiology. Absence of definitive diagnostic markers limits the accuracy of clinical evaluations of suspected KD with significant increases in morbidity. In turn, incomplete understanding of its molecular pathogenesis hinders the identification of rational targets needed to improve therapy. We used high-accuracy mass spectrometry proteomics to analyse over 2000 unique proteins in clinical urine specimens of patients with KD. We discovered that urine proteomes of patients with KD, but not those with mimicking conditions, were enriched for markers of cellular injury such as filamin and talin, immune regulators such as complement regulator CSMD3, immune pattern recognition receptor muclin, and immune cytokine protease meprin A. Significant elevations of filamin C and meprin A were detected in both the serum and urine in two independent cohorts of patients with KD, comprised of a total of 236 patients. Meprin A and filamin C exhibited superior diagnostic performance as compared to currently used markers of disease in a blinded case-control study of 107 patients with suspected KD, with receiver operating characteristic areas under the curve of 0.98 (95% confidence intervals [CI] of 0.97–1 and 0.95–1, respectively). Notably, meprin A was enriched in the coronary artery lesions of a mouse model of KD. In all, urine proteome profiles revealed novel candidate molecular markers of KD, including filamin C and meprin A that exhibit excellent diagnostic performance. These disease markers may improve the diagnostic accuracy of clinical evaluations of children with suspected KD, lead to the identification of novel therapeutic targets, and allow the development of a biological classification of Kawasaki disease. PMID:23281308

  14. Platelet Endothelial Cell Adhesion Molecule-1 Gene Polymorphisms are Associated with Coronary Artery Lesions in the Chronic Stage of Kawasaki Disease.

    PubMed

    Lu, Wen-Hsien; Huang, Sin-Jhih; Yuh, Yeong-Seng; Hsieh, Kai-Sheng; Tang, Chia-Wan; Liou, Huei-Han; Ger, Luo-Ping

    2017-05-01

    Kawasaki disease is the most common cause of pediatric acquired heart disease. The role of platelet endothelial cell adhesion molecule-1 in the inflammatory process has been documented. To date, no report has investigated the relationship between coronary artery lesions of Kawasaki disease and platelet endothelial cell adhesion molecule-1 polymorphisms. A total of 114 Kawasaki disease children with coronary artery lesions and 185 Kawasaki disease children without coronary artery lesions were recruited in this study. The TaqMan assay was conducted to identify the genotype in this case-control study. In three single nucleotide polymorphisms (Leu125Val, Ser563Asn, and Arg670Gly) of platelet endothelial cell adhesion molecule-1, we found that the Leu-Ser-Arg haplotype was associated with a significantly increased risk for coronary artery lesions in the chronic stage (odds ratio 3.05, 95% confidence interval 1.06-8.80, p = 0.039), but not for coronary artery lesions in the acute stage. Analysis based on the diplotypes of platelet endothelial cell adhesion molecule-1 also showed that Kawasaki disease with one or two alleles of Leu-Ser-Arg had a significantly increased risk of chronic coronary artery lesions (odds ratio 3.38, 95% confidence interval 1.11-10.28, p = 0.032) and had increased platelet counts after Kawasaki disease was diagnosed, as compared to those with other diplotypes. The haplotype of platelet endothelial cell adhesion molecule-1 Leu-Ser-Arg might be associated with the increased platelet counts and the following risk of chronic coronary artery lesions in a dominant manner in Kawasaki disease.

  15. Predictive value of red blood cell distribution width for coronary artery lesions in patients with Kawasaki disease.

    PubMed

    Xu, Haiyan; Fu, Songling; Wang, Wei; Zhang, Qing; Hu, Jian; Gao, Lichao; Zhu, Weihua; Gong, Fangqi

    2016-08-01

    Recent studies have shown that elevated red blood cell distribution width is associated with poor outcome in cardiovascular diseases. In order to assess the predictive value of red blood cell distribution width, before treatment with intravenous immunoglobulins, for coronary artery lesions in patient with Kawasaki disease, we compared 83 patients with coronary artery lesions and 339 patients without coronary artery lesions before treatment with intravenous immunoglobulin. Clinical, echocardiographic, and biochemical values were evaluated along with red blood cell distribution width. A total of 422 consecutive patients with Kawasaki disease were enrolled into our study. According to receiver operating characteristic curve analysis, the optimal red blood cell distribution width cut-off value for predicting coronary artery lesions was 14.55% (area under the curve was 0.721; p=0.000); eighty-three patients (19.7%) had coronary artery lesions, and 70% of the patients with coronary artery lesions had red blood cell distribution width level >14.55%. Logistic regression analysis revealed that fever duration >14 days (odds ratio was 3.42, 95% confidence interval was 1.27-9.22; p=0.015), intravenous immunoglobulin resistance (odds ratio was 2.33, 95% confidence interval was 1.02-5.29; p=0.04), and red blood cell distribution width >14.55% (odds ratio was 3.49, 95% confidence interval was 2.01-6.05; p=0.000) were independent predictors of coronary artery lesions in patients with Kawasaki disease. In Conclusion, red blood cell distribution width may be helpful for predicting coronary artery lesions in patients with Kawasaki disease.

  16. An adult case of Kawasaki disease with multiplex coronary aneurysms and myocardial infarction: the role of transesophageal echocardiography.

    PubMed

    Habon, T; Toth, K; Keltai, M; Lengyel, M; Palik, I

    1998-07-01

    Kawasaki disease (mucocutaneous lymph node syndrome) is an acute inflammatory disease that primarily affects infants and young children. In spite of proper therapy, coronary aneurysms develop in 10 to 25% of cases. Adult diagnosis of coronary aneurysm, presumably caused by Kawasaki disease, is rare. A 37-year-old male patient with previous inferior wall myocardial infarction (MI) was admitted with an acute anterior wall MI. Coronary angiography, performed 2 weeks after successful thrombolytic therapy, showed right coronary artery occlusion and multiplex (left main, left anterior descending, left circumflex, right coronary artery) giant coronary aneurysms. Transthoracic echocardiography was unable to detect the aneurysms. Transesophageal echocardiography (TEE) visualized a large left main coronary aneurysm with an occlusive thrombus and measured low flow velocity (0.2 m/s) in the proximal left anterior descending artery. At 4 weeks control, TEE showed marked regression of the thrombus, and it was not detectable after 6 months of oral anticoagulation with acenocumarol (International Normalized Ratio: 3-3.5) and standard postinfarction therapy. After 2 years of follow-up, the patient has no symptoms, and myocardial ischemia could not be provoked by stress tests [treadmill, dipyridamole single-photon emission computed tomography (SPECT)]. We conclude that, for diagnosis and follow-up of adult Kawasaki disease, transesophageal echocardiography is indicated. The importance and efficacy of long-term anticoagulant treatment should be emphasized in this disease.

  17. Kawasaki Disease

    MedlinePlus

    ... doctor may give your child high doses of aspirin to lower the fever. Aspirin also helps with the rash and the joint ... might give your child a lower dose of aspirin for several weeks to reduce the chance of ...

  18. Kawasaki Disease With Giant Coronary Aneurysms Requiring a Ventricular Assist Device to Separate From Extracorporeal Membrane Oxygenation: Coronary Issues Can Be a Pediatric Problem Too!

    PubMed

    Adler, Adam C; Kodavatiganti, Ramesh

    2016-08-15

    Kawasaki disease, although common in children, may rarely affect the coronary arteries, leading to aneurysm formation and potential for coronary thrombus formation. Extremely rarely, coronary aneurysms from Kawasaki disease can thrombose, resulting in ischemic myocardium. We present a case of a 31-month-old patient requiring a left ventricular assist device after thrombosis of giant coronary aneurysms led to ischemic cardiomyopathy. At the termination of the surgical procedure, we encountered 2 periods of ventricular assist device dropout requiring intervention. With the increase in the number of pediatric patients with assist devices, we review the basic care for a patient requiring emergent surgery.

  19. Transluminal Attenuation Gradient for Thrombotic Risk Assessment in Kawasaki Disease Patients with Coronary Artery Aneurysms

    NASA Astrophysics Data System (ADS)

    Grande Gutierrez, Noelia; Kahn, Andrew; Burns, Jane; Marsden, Alison

    2014-11-01

    Kawasaki Disease (KD) can result in coronary aneurysms in up to 25% of patients if not treated early putting patients at risk of thrombus formation, myocardial infarction and sudden death. Clinical guidelines for administering anti-coagulation therapy currently rely on anatomy alone. Previous studies including patient specific modeling and computer simulations in KD patients have suggested that hemodynamic data can predict regions susceptible to thrombus formation. In particular, high Particle Residence Time gradient (PRTg) regions have shown to correlate with regions of thrombus formation. Transluminal Attenuation Gradient (TAG) is determined from the change in radiological attenuation per vessel length. TAG has been used for characterizing coronary artery stenoses, however this approach has not yet been used in aneurysmal vessels. The aim of this study is to analyze the correlation between TAG and PRTg in KD patients with aneurysms and evaluate the use of TAG as an index to quantify thrombotic risk. Patient specific anatomic models for fluids simulations were constructed from CT angiographic image data from 3 KD aneurysm patients and one normal control. TAG values for the aneurysm patients were markedly lower than for the non-aneurysmal patient (mean -18.38 vs. -2). In addition, TAG values were compared to PRTg obtained for each patient. Thrombotic risk stratification for KD aneurysms may be improved by incorporating TAG and should be evaluated in future prospective studies.

  20. Assessment Of Coronary Artery Aneurysms Using Transluminal Attenuation Gradient And Computational Modeling In Kawasaki Disease Patients

    NASA Astrophysics Data System (ADS)

    Grande Gutierrez, Noelia; Kahn, Andrew; Shirinsky, Olga; Gagarina, Nina; Lyskina, Galina; Fukazawa, Ryuji; Owaga, Shunichi; Burns, Jane; Marsden, Alison

    2015-11-01

    Kawasaki Disease (KD) can result in coronary artery aneurysms (CAA) in up to 25% of patients, putting them at risk of thrombus formation, myocardial infarction and sudden death. Clinical guidelines recommend CAA diameter >8 mm as the arbitrary criterion for initiating systemic anticoagulation. KD patient specific modeling and flow simulations suggest that hemodynamic data can predict regions at increased risk of thrombosis. Transluminal Attenuation Gradient (TAG) is determined from the change in radiological attenuation per vessel length and has been proposed as a non-invasive method for characterizing coronary stenosis from CT Angiography. We hypothesized that CAA abnormal flow could be quantified using TAG. We computed hemodynamics for patient specific coronary models using a stabilized finite element method, coupled numerically to a lumped parameter network to model the heart and vascular boundary conditions. TAG was quantified in the major coronary arteries. We compared TAG for aneurysmal and normal arteries and we analyzed TAG correlation with hemodynamic and geometrical parameters. Our results suggest that TAG may provide hemodynamic data not available from anatomy alone. TAG represents a possible extension to standard CTA that could help to better evaluate the risk of thrombus formation in KD.

  1. Classification of coronary artery tissues using optical coherence tomography imaging in Kawasaki disease

    NASA Astrophysics Data System (ADS)

    Abdolmanafi, Atefeh; Prasad, Arpan Suravi; Duong, Luc; Dahdah, Nagib

    2016-03-01

    Intravascular imaging modalities, such as Optical Coherence Tomography (OCT) allow nowadays improving diagnosis, treatment, follow-up, and even prevention of coronary artery disease in the adult. OCT has been recently used in children following Kawasaki disease (KD), the most prevalent acquired coronary artery disease during childhood with devastating complications. The assessment of coronary artery layers with OCT and early detection of coronary sequelae secondary to KD is a promising tool for preventing myocardial infarction in this population. More importantly, OCT is promising for tissue quantification of the inner vessel wall, including neo intima luminal myofibroblast proliferation, calcification, and fibrous scar deposits. The goal of this study is to classify the coronary artery layers of OCT imaging obtained from a series of KD patients. Our approach is focused on developing a robust Random Forest classifier built on the idea of randomly selecting a subset of features at each node and based on second- and higher-order statistical texture analysis which estimates the gray-level spatial distribution of images by specifying the local features of each pixel and extracting the statistics from their distribution. The average classification accuracy for intima and media are 76.36% and 73.72% respectively. Random forest classifier with texture analysis promises for classification of coronary artery tissue.

  2. Ethnic Kawasaki Disease Risk Associated with Blood Mercury and Cadmium in U.S. Children

    PubMed Central

    Yeter, Deniz; Portman, Michael A.; Aschner, Michael; Farina, Marcelo; Chan, Wen-Ching; Hsieh, Kai-Sheng; Kuo, Ho-Chang

    2016-01-01

    Kawasaki disease (KD) primarily affects children <5 years of age (75%–80%) and is currently the leading cause of acquired heart disease in developed nations. Even when residing in the West, East Asian children are 10 to 20 times more likely to develop KD. We hypothesized cultural variations influencing pediatric mercury (Hg) exposure from seafood consumption may mediate ethnic KD risk among children in the United States. Hospitalization rates of KD in US children aged 0–4 years (n = 10,880) and blood Hg levels in US children aged 1–5 years (n = 713) were determined using separate US federal datasets. Our cohort primarily presented with blood Hg levels <0.1 micrograms (µg) per kg bodyweight (96.5%) that are considered normal and subtoxic. Increased ethnic KD risk was significantly associated with both increasing levels and detection rates of blood Hg or cadmium (Cd) in a linear dose-responsive manner between ethnic African, Asian, Caucasian, and Hispanic children in the US (p ≤ 0.05). Increasing low-dose exposure to Hg or Cd may induce KD or contribute to its later development in susceptible children. However, our preliminary results require further replication in other ethnic populations, in addition to more in-depth examination of metal exposure and toxicokinetics. PMID:26742052

  3. Incidence, epidemiology and clinical features of Kawasaki disease in Catalonia, Spain.

    PubMed

    Sánchez-Manubens, Judith; Antón, Jordi; Bou, Rosa; Iglesias, Estíbaliz; Calzada-Hernandez, Joan

    2016-01-01

    To assess the incidence, epidemiology and clinical features of Kawasaki disease (KD) in Catalonia (northeast region of Spain). This was an observational population-based study including all Paediatric Units in Catalonia, under both public and private management. Retrospective data retrieval was performed for 10 years (2004-2013). A 12-month (March 2013 to March 2014) prospective collection of new cases of KD was carried out to determine the incidence of KD. Data from 399 patients over the 10-year study period was analysed, revealing that 233 (58.4%) had complete KD, 159 (39.8) incomplete KD and 7 (1.7%) were considered atypical KD. Mean annual incidence was 3.5/105 children <14 years old (yo) and 8/105 children <5 yo (mean age 37±33 months, range 1.3-191.3). KD was more frequent in boys (59.6%, p<0.001) and in rural areas (p<0.001). Patients with IVIG non-responsiveness, need of a 2(nd) IVIG dose, delay of treatment >10(th) day of illness, ages <1 yo and >8 yo and the presence of sterile piuria, aseptic meningitis, abdominal pain and uveitis at diagnosis were found to have higher risk of coronary aneurisms (CAA) (p<0.05). This is the first population-based study on the epidemiology of KD in the western Mediterranean area. Incidence, clinical features and treatment plans in our cohort are similar to those described in other European studies.

  4. Pregnancy in women with a history of Kawasaki disease: management and outcomes.

    PubMed

    Gordon, C T; Jimenez-Fernandez, S; Daniels, L B; Kahn, A M; Tarsa, M; Matsubara, T; Shimizu, C; Burns, J C; Gordon, J B

    2014-10-01

    To characterise the obstetrical management and outcomes in a series of women with a history of Kawasaki disease (KD) in childhood. Retrospective case series. Tertiary healthcare setting in the USA. Women with a history of KD in childhood. Women completed a detailed health questionnaire and participated in research imaging studies as part of the San Diego Adult KD Collaborative Study. Obstetrical management, complications during pregnancy and delivery, and infant outcomes. Ten women with a history of KD in childhood carried a total of 21 pregnancies to term. There were no cardiovascular complications during labour and delivery despite important cardiovascular abnormalities in four of the ten subjects. Pregnancy was complicated by pre-eclampsia and the post-partum course was complicated by haemorrhage in one subject each. Two of the 21 progeny subsequently developed KD. Women with important cardiovascular sequelae from KD in childhood should be managed by a team that includes both a maternal-fetal medicine specialist and a cardiologist. Pre-pregnancy counselling should include delineation of the woman's current functional and structural cardiovascular status and appropriate adjustment of medications, but excellent outcomes are possible with appropriate care. Review of the English and Japanese literature on KD and pregnancy revealed the occurrence of myocardial infarction during pregnancy in women with missed KD and aneurysms that were not diagnosed until their acute event. Our study highlights the need for counselling with regard to the increased genetic risk of KD in offspring born to these mothers. © 2014 Royal College of Obstetricians and Gynaecologists.

  5. Severe Kawasaki disease in a 3-month-old patient: a case report

    PubMed Central

    2013-01-01

    Background Kawasaki disease is a multi-system vasculitis which usually occurs in children under 5 years of age. In infants under three months of age, it is very rare and usually associated with a high incidence of incomplete or atypical forms, often unresponsive to treatment. This condition increases the risk of cardiovascular complications such as coronary artery aneurysms. Case presentation We describe a 3-month-old infant who developed early and severe aneurysms in three coronary arteries despite a timely administration of intravenous immunoglobulins, followed by three days of intravenous methylprednisolone. Conclusion This case report underlines that the development of coronary artery aneurysm correlates with a delayed diagnosis and treatment, incomplete or atypical forms of the disease, and additionally the severity of clinical presentation, especially in cases of very young infants below 3 months of age. Our case is notable because of the very young age of the patient, the severity of clinical presentation with an early development of coronary artery aneurysms and the unresponsiveness to the therapy. PMID:24294914

  6. CFD research and systems in Kawasaki Heavy Industries and its future prospects

    NASA Astrophysics Data System (ADS)

    Hiraoka, Koichi

    1990-09-01

    KHI Computational Fluid Dynamics (CFD) system is composed of VP100 computer and 2-D and 3-D Euler and/or Navier-Stokes (NS) analysis softwares. For KHI, this system has become a very powerful aerodynamic tool together with the Kawasaki 1 m Transonic Wind Tunnel. The 2-D Euler/NS software, developed in-house, is fully automated, requires no special skill, and was successfully applied to the design of YXX high lift devices and SST supersonic inlet, etc. The 3-D Euler/NS software, developed under joint research with NAL, has an interactively operated Multi-Block type grid generator and can effectively generate grids around complex airplane shapes. Due to the main memory size limitation, 3-D analysis of relatively simple shape, such as SST wing-body, was computed in-house on VP100, otherwise, such as detailed 3-D analyses of ASUKA and HOPE, were computed on NAL VP400, which is 10 times more powerful than VP100, under KHI-NAL joint research. These analysis results have very good correlation with experimental results. However, the present CFD system is less productive than wind tunnel and has applicability limitations.

  7. Cardiovascular risk factors of early atherosclerosis in school-aged children after Kawasaki disease

    PubMed Central

    Cho, Hyun Jeong; Yang, Soo In; Kim, Kyung Hee; Kim, Jee Na

    2014-01-01

    Purpose The aim of this study was to determine whether school-aged children with Kawasaki disease (KD) have an increased risk for early atherosclerosis. Methods The study included 98 children. The children were divided into the following groups: group A (n=19), KD with coronary arterial lesions that persisted or regressed; group B (n=49), KD without coronary arterial lesions; and group C (n=30), healthy children. Anthropometric variables and the levels of biochemical markers, including total cholesterol, high-density lipoprotein cholesterol (HDL-C), low-density lipoprotein cholesterol (LDL-C), apolipoprotein A, apolipoprotein B, homocysteine, high-sensitivity C-reactive protein (hs-CRP), and brachial artery stiffness using pulse wave velocity were compared among the three groups. Results There were no significant differences in blood pressure and body index among the three groups. Additionally, there was no sex-specific difference. Moreover, the levels of triglyceride, HDL-C, apolipoprotein A, and hs-CRP did not differ among the three groups. However, the levels of total cholesterol (P=0.018), LDL-C (P=0.0003), and apolipoprotein B (P=0.029) were significantly higher in group A than in group C. Further, the level of homocysteine and the aortic pulse wave velocity were significantly higher in groups A and B than in group C (P=0.0001). Conclusion School-aged children after KD have high lipid profiles and arterial stiffness indicating an increased risk for early atherosclerosis. PMID:25045363

  8. Cardiovascular risk factors of early atherosclerosis in school-aged children after Kawasaki disease.

    PubMed

    Cho, Hyun Jeong; Yang, Soo In; Kim, Kyung Hee; Kim, Jee Na; Kil, Hong Ryang

    2014-05-01

    The aim of this study was to determine whether school-aged children with Kawasaki disease (KD) have an increased risk for early atherosclerosis. The study included 98 children. The children were divided into the following groups: group A (n=19), KD with coronary arterial lesions that persisted or regressed; group B (n=49), KD without coronary arterial lesions; and group C (n=30), healthy children. Anthropometric variables and the levels of biochemical markers, including total cholesterol, high-density lipoprotein cholesterol (HDL-C), low-density lipoprotein cholesterol (LDL-C), apolipoprotein A, apolipoprotein B, homocysteine, high-sensitivity C-reactive protein (hs-CRP), and brachial artery stiffness using pulse wave velocity were compared among the three groups. There were no significant differences in blood pressure and body index among the three groups. Additionally, there was no sex-specific difference. Moreover, the levels of triglyceride, HDL-C, apolipoprotein A, and hs-CRP did not differ among the three groups. However, the levels of total cholesterol (P=0.018), LDL-C (P=0.0003), and apolipoprotein B (P=0.029) were significantly higher in group A than in group C. Further, the level of homocysteine and the aortic pulse wave velocity were significantly higher in groups A and B than in group C (P=0.0001). School-aged children after KD have high lipid profiles and arterial stiffness indicating an increased risk for early atherosclerosis.

  9. CFD-based Thrombotic Risk Assessment in Kawasaki Disease Patients with Coronary Artery Aneurysms

    NASA Astrophysics Data System (ADS)

    Sengupta, Dibyendu; Kung, Ethan; Kahn, Andrew; Burns, Jane; Marsden, Alison

    2012-11-01

    Coronary aneurysms occur in 25% of untreated Kawasaki Disease (KD) patients and put patients at increased risk for myocardial infarction and sudden death. Clinical guidelines recommend using aneurysm diameter >8 mm as the arbitrary criterion for treating with anti-coagulation therapy. This study uses patient-specific modeling to non-invasively determine hemodynamic parameters and quantify thrombotic risk. Anatomic models were constructed from CT angiographic image data from 5 KD aneurysm patients and one normal control. CFD simulations were performed to obtain hemodynamic data including WSS and particle residence times (PRT). Thrombosis was clinically observed in 4/9 aneurysmal coronaries. Thrombosed vessels required twice as many cardiac cycles (mean 8.2 vs. 4.2) for particles to exit, and had lower mean WSS (1.3 compared to 2.8 dynes/cm2) compared to vessels with non-thrombosed aneurysms of similar max diameter. 1 KD patient in the cohort with acute thrombosis had diameter < 8 mm. Regions of low WSS and high PRT predicted by simulations correlated with regions of subsequent thrombus formation. Thrombotic risk stratification for KD aneurysms may be improved by incorporating both hemodynamic and geometric quantities. Current clinical guidelines to assess patient risk based only on aneurysm diameter may be misleading. Further prospective study is warranted to evaluate the utility of patient-specific modeling in risk stratifying KD patients with coronary aneurysms. NIH R21.

  10. Sudden death as a late sequel of Kawasaki disease: postmortem CT demonstration of coronary artery aneurysm.

    PubMed

    Okura, Naoki; Okuda, Takahisa; Shiotani, Seiji; Kohno, Mototsugu; Hayakawa, Hideyuki; Suzuki, Atsuko; Kawasaki, Tomisaku

    2013-02-10

    Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that primarily affects the coronary artery (CA) and presents during childhood. The characteristic coronary arterial lesion of KD is an aneurysm. Ischemic heart disease derived from a CA aneurysm is experienced approximately two decades after the onset of acute KD. In recent years, the primary issue of concern has been asymptomatic adults with a CA aneurysm caused by undiagnosed KD. We present a case of sudden death as a late KD sequel in a young adult. A postmortem CT scan revealed a coarse calcification of a left anterior descending CA aneurysm, which was confirmed at the time of autopsy. A postmortem CT scan is useful in cases of sudden death where the detection of a calcified CA aneurysm would suggest to the forensic pathologist that the deceased suffered from a late sequel of KD. The use of screening postmortem CT scans for young people may detect cases of unsuspected CA aneurysms, raising the possibility of untreated KD.

  11. Characterization of the gut microbiota of Kawasaki disease patients by metagenomic analysis

    PubMed Central

    Kinumaki, Akiko; Sekizuka, Tsuyoshi; Hamada, Hiromichi; Kato, Kengo; Yamashita, Akifumi; Kuroda, Makoto

    2015-01-01

    Kawasaki disease (KD) is an acute febrile illness of early childhood. Previous reports have suggested that genetic disease susceptibility factors, together with a triggering infectious agent, could be involved in KD pathogenesis; however, the precise etiology of this disease remains unknown. Additionally, previous culture-based studies have suggested a possible role of intestinal microbiota in KD pathogenesis. In this study, we performed metagenomic analysis to comprehensively assess the longitudinal variation in the intestinal microbiota of 28 KD patients. Several notable bacterial genera were commonly extracted during the acute phase, whereas a relative increase in the number of Ruminococcus bacteria was observed during the non-acute phase of KD. The metagenomic analysis results based on bacterial species classification suggested that the number of sequencing reads with similarity to five Streptococcus spp. (S. pneumonia, pseudopneumoniae, oralis, gordonii, and sanguinis), in addition to patient-derived Streptococcus isolates, markedly increased during the acute phase in most patients. Streptococci include a variety of pathogenic bacteria and probiotic bacteria that promote human health; therefore, this further species discrimination could comprehensively illuminate the KD-associated microbiota. The findings of this study suggest that KD-related Streptococci might be involved in the pathogenesis of this disease. PMID:26322033

  12. [Association of TIAM1 gene polymorphisms with Kawasaki disease and its clinical characteristics].

    PubMed

    Wang, Xian; Zhu, Tian-Jiao; Zhou, Xiong-Fei; Wan, Zhi-Ting

    2015-11-01

    To investigate the association of single nucleotide polymorphisms (SNP) rs22833188 and rs2833195 in TIAM1 gene with the susceptibility to Kawasaki disease (KD) and its clinical characteristic in children. A case-control study was performed in this study. One hundred and eighty-eight children with KD and 197 normal children served as controls were enrolled. The genotypes of two SNPs rs22833188 and rs2833195 in TIAM1 gene were detected using PCR-RFLP. There were no significant differences in the genotype (AA, AG and GG) and allele frequencies of SNP rs2833188 between the KD and control groups. Significant differences in the genotype (CC, GC and GG) frequency of SNP rs2833195 were noted between the KD and control groups (P=0.017). The frequency of C allele in the KD group was higher than in the control group (P=0.015). The polymorphism of SNP rs2833188 was associated with the occurrence of rash (P=0.011), and the polymorphism of SNP rs2833195 was associated with the occurrence of conjunctival hyperemia (P=0.021). The polymorphism of rs2833195 in TIAM1 gene is associated with the susceptibility to KD. The polymorphisms rs2833188 and rs2833195 in TIAM1 gene may be associated with some clinical characteristics in children with KD.

  13. GM-CSF primes cardiac inflammation in a mouse model of Kawasaki disease

    PubMed Central

    McKenzie, Brent S.

    2016-01-01

    Kawasaki disease (KD) is the leading cause of pediatric heart disease in developed countries. KD patients develop cardiac inflammation, characterized by an early infiltrate of neutrophils and monocytes that precipitates coronary arteritis. Although the early inflammatory processes are linked to cardiac pathology, the factors that regulate cardiac inflammation and immune cell recruitment to the heart remain obscure. In this study, using a mouse model of KD (induced by a cell wall Candida albicans water-soluble fraction [CAWS]), we identify an essential role for granulocyte/macrophage colony-stimulating factor (GM-CSF) in orchestrating these events. GM-CSF is rapidly produced by cardiac fibroblasts after CAWS challenge, precipitating cardiac inflammation. Mechanistically, GM-CSF acts upon the local macrophage compartment, driving the expression of inflammatory cytokines and chemokines, whereas therapeutically, GM-CSF blockade markedly reduces cardiac disease. Our findings describe a novel role for GM-CSF as an essential initiating cytokine in cardiac inflammation and implicate GM-CSF as a potential target for therapeutic intervention in KD. PMID:27595596

  14. Acute myocardial ischemia in adults secondary to missed Kawasaki disease in childhood.

    PubMed

    Rizk, Sherif R Y; El Said, Galal; Daniels, Lori B; Burns, Jane C; El Said, Howaida; Sorour, Khaled A; Gharib, Soliman; Gordon, John B

    2015-02-15

    Coronary artery aneurysms that occur in 25% of untreated Kawasaki disease (KD) patients may remain clinically silent for decades and then thrombose resulting in myocardial infarction. Although KD is now the most common cause of acquired heart disease in children in Asia, the United States, and Western Europe, the incidence of KD in Egypt is unknown. We tested the hypothesis that young adults in Egypt presenting with acute myocardial ischemia may have coronary artery lesions because of KD in childhood. We reviewed a total of 580 angiograms of patients ≤40 years presenting with symptoms of myocardial ischemia. Coronary artery aneurysms were noted in 46 patients (7.9%), of whom 9 presented with myocardial infarction. The likelihood of antecedent KD as the cause of the aneurysms was classified as definite (n = 10), probable (n = 29), or equivocal (n = 7). Compared with the definite and probable groups, the equivocal group had more traditional cardiovascular risk factors, smaller sized aneurysms, and fewer coronary arteries affected. In conclusion, in a major metropolitan center in Egypt, 6.7% of adults aged ≤40 years who underwent angiography for evaluation of possible myocardial ischemia had lesions consistent with antecedent KD. Because of the unique therapeutic challenges associated with these lesions, adult cardiologists should be aware that coronary artery aneurysms in young adults may be because of missed KD in childhood.

  15. Adolescent Kawasaki disease: usefulness of 64-slice CT coronary angiography for follow-up investigation.

    PubMed

    Carbone, Iacopo; Cannata, David; Algeri, Emanuela; Galea, Nicola; Napoli, Alessandro; De Zorzi, Andrea; Bosco, Giovanna; D'Agostino, Rita; Menezes, Leon; Catalano, Carlo; Passariello, Roberto; Francone, Marco

    2011-09-01

    Kawasaki disease (KD) is a systemic vasculitis that mainly affects coronary arteries in children, and requires regular follow-up from the time of diagnosis. To evaluate the feasibility of 64-slice CT angiography (CTA) for follow-up of patients with KD using previously performed invasive catheter coronary angiography (CCA) as reference standard. The study group comprised 12 patients (age 17.6 ± 2.9 years, mean ± SD) with a diagnosis of KD and a previously performed CCA (interval, 32.6 ± 13.5 months) who underwent 64-slice cardiac CTA. The quality of the images for establishing the presence of coronary abnormalities was determined by two observers. The CTA findings were compared with those from the prior CCA. Adequate image quality was obtained in all patients. Mean effective dose for CTA was 6.56 ± 0.95 mSv. CTA allowed accurate identification, characterization and measurement of all coronary aneurysms (n = 32), stenoses (n = 3) and occlusions (n = 9) previously demonstrated by CCA. One patient with disease progression went on to have percutaneous coronary intervention. Coronary lesions were reliably evaluated by 64-slice CTA in the follow-up of compliant patients with KD, reducing the need for repeated diagnostic invasive CCA. Hence, in an adequately selected patient population, the role of CCA could be limited almost only to therapeutic procedures.

  16. Identification of candidate diagnostic serum biomarkers for Kawasaki disease using proteomic analysis

    PubMed Central

    Kimura, Yayoi; Yanagimachi, Masakatsu; Ino, Yoko; Aketagawa, Mao; Matsuo, Michie; Okayama, Akiko; Shimizu, Hiroyuki; Oba, Kunihiro; Morioka, Ichiro; Imagawa, Tomoyuki; Kaneko, Tetsuji; Yokota, Shumpei; Hirano, Hisashi; Mori, Masaaki

    2017-01-01

    Kawasaki disease (KD) is a systemic vasculitis and childhood febrile disease that can lead to cardiovascular complications. The diagnosis of KD depends on its clinical features, and thus it is sometimes difficult to make a definitive diagnosis. In order to identify diagnostic serum biomarkers for KD, we explored serum KD-related proteins, which differentially expressed during the acute and recovery phases of two patients by mass spectrometry (MS). We identified a total of 1,879 proteins by MS-based proteomic analysis. The levels of three of these proteins, namely lipopolysaccharide-binding protein (LBP), leucine-rich alpha-2-glycoprotein (LRG1), and angiotensinogen (AGT), were higher in acute phase patients. In contrast, the level of retinol-binding protein 4 (RBP4) was decreased. To confirm the usefulness of these proteins as biomarkers, we analyzed a total of 270 samples, including those collected from 55 patients with acute phase KD, by using western blot analysis and microarray enzyme-linked immunosorbent assays (ELISAs). Over the course of this experiment, we determined that the expression level of these proteins changes specifically in the acute phase of KD, rather than the recovery phase of KD or other febrile illness. Thus, LRG1 could be used as biomarkers to facilitate KD diagnosis based on clinical features. PMID:28262744

  17. Decreased nitric oxide production after intravenous immunoglobulin treatment in patients with Kawasaki disease.

    PubMed

    Wang, Chih-Lu; Wu, Yu-Tsun; Lee, Chia-Jung; Liu, Hsiu-Chin; Huang, Li-Tung; Yang, Kuender D

    2002-10-01

    To assess nitric oxide (NO) production and different displays of NO synthase (NOS) isoforms in patients with Kawasaki disease (KD) before and after intravenous immunoglobulin (IVIG) treatment. Blood and urine samples were collected from patients with KD before IVIG treatment and 3 days afterward. Age-matched febrile patients with various diseases served as control patients. Plasma NOx (NO3- + NO2-) levels were measured as NO production. Real-time reverse transcriptase-polymerase chain reaction and immunohistochemical staining were used to detect differential NOS isoform expression in mononuclear leukocytes (MNCs). Patients with KD (n = 40) had higher levels of NOx than the non-KD febrile control patients. The elevated NOx levels in patients with KD were significantly associated with the occurrence of coronary artery dilation (>3 mm). These elevated NOx levels significantly decreased after IVIG treatment (157.8 +/- 15.9 micromol/L vs 57.0 +/- 4.27 micromol/L, P <.01). However, excretion of urinary NOx levels was not changed after IVIG treatment. Inducible but not constitutive NOS mRNA and protein in MNCs were prominently expressed but significantly decreased after IVIG treatment. IVIG may suppress iNOS expression of MNCs in patients with KD, thus decreasing NO-mediated inflammatory responses and coronary artery dilation.

  18. Effect and Safety of TNF Inhibitors in Immunoglobulin-Resistant Kawasaki Disease: a Meta-analysis.

    PubMed

    Xue, Li-Jun; Wu, Rong; Du, Gui-Lian; Xu, Yan; Yuan, Kang-Yan; Feng, Zhi-Chun; Pan, Yu-Lin; Hu, Guang-Yu

    2016-08-23

    Previous studies showed that tumor necrosis factor (TNF) inhibitors might decrease the rate of coronary artery abnormalities in pediatrics with intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD). Therefore, we aimed to evaluate the effect and safety of TNF inhibitors in IVIG-resistant KD. We undertook a meta-analysis of clinical trials identified in systematic searches of PubMed, EMBASE, Cochrane Database, and Google scholar through May 2016. Five studies were included. Overall, rate of coronary artery aneurysm was comparable between groups (relative risk (RR), 1.05; 95 % confidence interval (95 % CI), 0.60 to 1.81; P = 0.87). No significant differences were recorded between groups in coronary artery Z scores (standardized mean difference (SMD), 0.27; 95 % CI, -0.30 to 0.85; P = 0.35). Meanwhile, TNF inhibitors were not associated with a significant decreased risk of treatment resistance compared with IVIG treatment (RR, 0.65; 95 % CI, 0.37 to 0.15; P = 0.14). However, days of fever was significantly reduced in the TNF inhibitor group (SMD, -0.66; 95 % CI, -0.90 to -0.41; P < 0.001). Additionally, risk of serious adverse events was similar between groups. Therefore, TNF inhibitors could shorten the duration of fever in IVIG-resistant KD. However, TNF inhibitors appear to have no cardioprotective effect in patients with IVIG-resistant KD.

  19. High dose anakinra for treatment of severe neonatal Kawasaki disease: a case report

    PubMed Central

    2014-01-01

    We report an 11-week-old female who presented with Kawasaki disease (KD) complicated by macrophage activation syndrome (MAS). The infant presented to the hospital with persistent fever, cough, diarrhea, and emesis, among other symptoms. Her condition quickly began to decompensate, and she developed classic features (conjunctivitis, rash, cracked lips, distal extremity edema) prompting a diagnosis of acute KD. The patient was treated with standard therapy for KD including three doses of intravenous immunoglobulin (IVIG), aspirin, and high dose glucocorticoids with no change in her condition. Due to a high suspicion for MAS, high dose anakinra therapy was initiated resulting in dramatic clinical improvements. She also received one dose of infliximab for concern for coronary artery changes, and over the course of several months, anakinra and high dose glucocorticoids were tapered. Nearly complete reversal of echocardiogram changes were observed after 8 months, and the infant is now off all immunosuppressive therapy. In this case report, we briefly review the importance of early recognition of MAS in pediatric patient populations with rheumatic diseases, and we suggest early initiation of anakinra therapy as a rapid and effective treatment option. PMID:25045337

  20. [Kawasaki disease is more prevalent in rural areas of Catalonia (Spain)].

    PubMed

    Sánchez-Manubens, Judith; Antón, Jordi; Bou, Rosa; Iglesias, Estibaliz; Calzada-Hernandez, Joan; Rodó, Xavier; Morguí, Josep-Antón

    2017-02-23

    Kawasaki disease (KD) is an acute self-limited systemic vasculitis relatively common in childhood. The etiology of KD is still unknown, although clinical, laboratory and epidemiological features suggest an infectious origin or trigger. Differences on incidence between countries have been related to specific genetic factors, ethnicity, country of birth and some other sociocultural and environmental factors. We present a population-based study on incidence of KD in Catalonia (Spain), focusing on differences between patients in rural and non-rural areas of the region. Observational population-based study including all Pediatric Units in Catalan hospitals, between 2004 and 2014. A 12-month (March 2013-March 2014) prospective collection of new cases of KD was carried out to determine the incidence of KD. The rest of the data was retrieved retrospectively. Data from 399 patients over the 10-year study period was analyzed. Among the total KD patients, 353 (88.5%) lived in non-rural areas and 46 (11.5%) in rural areas. It was found that there is a significant difference (P<.001) between the percentage of rural population observed in patients with KD (11.5%), and the expected 5% of the Catalan population. This is the first population-based study showing significant differences on KD incidence rates between rural and non-rural areas. Copyright © 2017. Publicado por Elsevier España, S.L.U.

  1. Hounsfield unit values of retropharyngeal abscess-like lesions seen in Kawasaki disease.

    PubMed

    Sasaki, Toru; Miyata, Rie; Hatai, Yoshiho; Makita, Kohzoh; Tsunoda, Koichi

    2014-04-01

    Retropharyngeal abscess-like lesions are occasionally seen in computed tomography (CT) imaging of patients with Kawasaki disease (KD) and these patients often undergo unnecessary surgery. We could distinguish the lesions from true abscesses by measuring their Hounsfield unit values (HUs). To distinguish the retropharyngeal abscess-like lesions from true abscesses without any surgical procedure. We investigated six cases of KD showing such lesions on CTs, both with and without contrast enhancement (CE). We measured the HUs of those lesions and compared them with those of 10 true abscesses as controls. Abscess-like lesions of KD were well enhanced by CE, whereas abscesses showed virtually no enhancement. The mean HU in the six KD cases was 20.0 ± 4.65 (mean ± SD) on plain CTs and 35.6 ± 4.49 on contrast CTs. In abscesses, it was 30.3 ± 4.42 on plain CTs and 30.3 ± 3.57 on contrast CTs. The difference in HU values [(HU on contrast CT) - (HU on plain CT)] was defined as ΔHU. The mean ΔHU was 15.6 ± 5.36 in the six KD lesions and 0.0 ± 2.93 in abscesses, with statistical significance of p < 0.0001 by Student's t test. Thus, ΔHU value may potentially be a useful parameter for their distinction.

  2. The Clinical Profile of Kawasaki Disease in Algerian Children: A Single Institution Experience.

    PubMed

    Boudiaf, Houda; Achir, Moussa

    2016-04-01

    Kawasaki disease (KD) in an acute vasculitis of unknown etiology. The epidemiological data available for Algerian patients remains insufficient. To describe the demographic, clinical features of children with KD and to identify the risk factors for developing coronary artery lesions (CAL). This retrospective study included children admitted with KD at the pediatric hospital in Algiers from January 2005 to December 2014. One hundred thirty-three patients (82 boys and 51 girls) with a mean age of 31 months were identified. The most common sign was fever, rash, oral changes and conjunctivitis. The cardiac complications were CAL (22.5%), pericarditis (2%) and myocarditis (1.5%). The independent variable for prediction of CAL was duration of fever >10 days, male gender and platelet count >450,000/mm3 CONCLUSION: The incidence of cardiovascular complications is high. Knowledge of KD among Algerian pediatricians should be enhanced to guarantee appropriate treatment of this disease. © The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  3. Modelling seasonal variations in the age and incidence of Kawasaki disease to explore possible infectious aetiologies

    PubMed Central

    Pitzer, Virginia E.; Burgner, David; Viboud, Cécile; Simonsen, Lone; Andreasen, Viggo; Steiner, Claudia A.; Lipsitch, Marc

    2012-01-01

    The average age of infection is expected to vary during seasonal epidemics in a way that is predictable from the epidemiological features, such as the duration of infectiousness and the nature of population mixing. However, it is not known whether such changes can be detected and verified using routinely collected data. We examined the correlation between the weekly number and average age of cases using data on pre-vaccination measles and rotavirus. We show that age–incidence patterns can be observed and predicted for these childhood infections. Incorporating additional information about important features of the transmission dynamics improves the correspondence between model predictions and empirical data. We then explored whether knowledge of the age–incidence pattern can shed light on the epidemiological features of diseases of unknown aetiology, such as Kawasaki disease (KD). Our results indicate KD is unlikely to be triggered by a single acute immunizing infection, but is consistent with an infection of longer duration, a non-immunizing infection or co-infection with an acute agent and one with longer duration. Age–incidence patterns can lend insight into important epidemiological features of infections, providing information on transmission-relevant population mixing for known infections and clues about the aetiology of complex paediatric diseases. PMID:22398170

  4. Significance of serum 25-hydroxyvitamin D3 and interleukin-6 levels in immunoglobulin treatment of Kawasaki disease in children.

    PubMed

    An, Xinjiang; Fu, Mingyu; Tian, Jing; Xue, Ying; Xu, Hui

    2016-09-01

    The aim of the study was to investigate the significance of the level of serum 25-hydroxyvitamin D3 [25-(OH)D3] and interleukin (IL)-6 in serum prior to and after immunoglobulin treatment in children suffering from Kawasaki disease in order to provide a reference for the successful treatment of Kawasaki disease in children. From February, 2013 to February, 2015, 45 patients with Kawasaki disease were enrolled in the observation group. The normal control group comprised 43 healthy volunteers and the feverish control group 46 patients with respiratory infection and fever. Venous blood was collected from each case before and after immunoglobulin treatment and the level of 25-(OH)D3 and IL-6 in the serum were measured using fluorescent quantitative PCR, enzyme-linked immunosorbent assay and western blotting. Before treatment, the level of 25-(OH)D3 in the feverish control group was significantly lower than that of the normal control group, while the level of 25-(OH)D3 in the observation group was significantly higher than that of the normal control group. The level of 25-(OH)D3 in the feverish control group was lower than the IL-6 level in the normal children, but the difference was not statistically significant (P>0.05). The level 25-(OH)D3 in the observation group was significantly higher than the IL-6 level in the normal control group. The serum content of 25-(OH)D3 was significantly higher after the treatment compared to before treatment levels and after treatment IL-6 level was only slightly lower. It was observed that the 25-(OH)D3 level in the observation group was significantly increased after immunoglobulin treatment and this was positively correlated with the effects of the treatment. The IL-6 level had no significant changes after treatment and had little correlation with the treatment effect. The results suggested that 25-(OH)D3 may be involved in the occurrence of Kawasaki disease in children and in the aggravation of the disease to some extent.

  5. A child with Epstein-Barr Virus-associated hemophagocytic lymphohistiocytosis complicated by coronary artery lesion mimicking Kawasaki disease.

    PubMed

    Kato, Shogo; Yoshimura, Ken; Tanabe, Yuko; Kimata, Takahisa; Noda, Yukihiro; Kawasaki, Hirohide; Kaneko, Kazunari

    2013-10-01

    There is considerable overlap between hemophagocytic lymphohistiocytosis (HLH) and Kawasaki disease (KD) in terms of aberrant immune response though the etiology of KD remains unknown. We present a case fulfilling the criteria of both HLH and KD complicated by coronary artery dilatation: HLH was confirmed to be triggered by Epstein-Barr virus. This case alarms us the possibility that even patients with HLH may be complicated by coronary artery lesion, which is one of the hallmarks of KD. We would like to draw attention that if features of KD become apparent in patients with HLH, echocardiographic examinations should be performed not to miss coronary artery lesion.

  6. Bull's eye dermatoscopy pattern at bacillus Calmette-Guérin inoculation site correlates with systemic involvements in patients with Kawasaki disease.

    PubMed

    Tseng, Han-Chi; Ho, Ji-Chen; Guo, Mindy Ming-Huey; Lo, Mao-Hung; Hsieh, Kai-Sheng; Tsai, Wen-Chien; Kuo, Ho-Chang; Lee, Chih-Hung

    2016-09-01

    For the past decades, although the rash at the bacillus Calmette-Guérin (BCG) inoculation site has been recognized as a diagnostic clue in Kawasaki disease, the present study is the first known one attempting to characterize BCG inoculation by dermatoscopy in Kawasaki disease and correlate the grade of BCG reaction with systemic involvement. Thirty-four patients diagnosed with Kawasaki disease by pediatric specialists were enrolled. We performed detailed history taking, laboratory examination, physical examination and dermatoscopy examinations. Based on the BCG reaction pattern by dermatoscopy, we were able to characterize three patterns: (A) Bull's eye pattern in 18 patients; (B) faint homogenous erythema in nine; and (C) central white patch in seven. Patients from group A exhibited the highest elevation of blood aspartate aminotransferase levels (P < 0.05). Notably, three patients from group A had a significantly dilated coronary artery despite i.v. immunoglobulin injection. We concluded that the bull's eye dermatoscopy sign is not only a useful diagnostic clue but also a severity biomarker in patients with Kawasaki disease.

  7. An evaluation of children with Kawasaki disease in Istanbul: a retrospective follow‐up study

    PubMed Central

    Kayiran, Sinan Mahir; Dindar, Aygün; Gurakan, Berkan

    2010-01-01

    BACKGROUND: Kawasaki disease (KD) is an acute, self‐limiting vasculitis of unknown etiology. The incidence of KD is increasing world wide. However, the epidemiological data for KD in Turkey has not been well described. OBJECTIVE: To describe the demographic, clinical, and laboratory features of children with KD who were diagnosed and managed in the American Hospital, Istanbul, Turkey. METHOD: Patients with KD were retrospectively identified from the hospital discharge records between 2002 and 2010. Atypical cases of KD were excluded. A standardized form was used to collect demographic data, clinical information, echocardiography and laboratory results. RESULTS: Thirty‐five patients with KD, with a mean age of 2.5±1.9 years, were identified. Eighty‐five point seven per cent of patients were under 5 years of age. A seasonal pattern favouring the winter months was noticed. In addition to fever and bilateral conjunctival injection, changes in the oral cavity and lips were the most commonly detected clinical signs in our cases. Coronary artery abnormalities were detected in nine patients. The majority of our patients had started treatment with intravenous immunoglobulin in the first 10 days of the onset of fever, and only one patient required systemic steroids for intravenous immunoglobulin‐resistant KD. The coronary artery abnormalities resolved in all nine patients within 8 months. CONCLUSION: This study is the most comprehensive series of children from Turkey with KD included in Medline. As adult‐onset ischemic heart disease may be due to KD in childhood, further prospective clinical investigations are needed to understand the epidemiology, management and long‐term follow‐up of the disease. PMID:21340213

  8. Pulmonary Artery Dilation and Right Ventricular Function in Acute Kawasaki Disease.

    PubMed

    Numano, Fujito; Shimizu, Chisato; Tremoulet, Adriana H; Dyar, Dan; Burns, Jane C; Printz, Beth F

    2016-03-01

    Coronary artery inflammation and aneurysm formation are the most common complications of Kawasaki disease (KD). Valvulitis and myocarditis are also well described and may lead to valvar regurgitation and left ventricular dysfunction. However, functional changes in the right heart have rarely been reported. We noted several acute KD patients with dilated pulmonary arteries (PA) and thus sought to systematically characterize PA size and right-heart function in an unselected cohort of KD patients cared for at a single clinical center. Clinical, laboratory, and echocardiographic data from 143 acute KD subjects were analyzed. PA dilation was documented in 23 subjects (16.1 %); these subjects had higher median right ventricle myocardial performance index (RV MPI), higher ratio of early tricuspid inflow velocity to tricuspid annular early diastolic velocity (TV E/e'), and lower median TV e' velocity compared to the non-PA dilation group (0.50 vs 0.38 p < 0.01, 4.2 vs 3.6 p < 0.05, and 13.5 vs 15.2 cm/s p < 0.01, respectively). Almost all subjects with PA dilation had improved PA Z-score, RV MPI, and TV E/e' in the subacute phase (p < 0.01). There were no significant differences in indices of left ventricle function between PA dilation group and non-PA dilation group. In summary, PA dilation was documented in 16 % of acute KD subjects. These subjects were more likely to have echocardiographic indices consistent with isolated RV dysfunction that improved in the subacute phase. The long-term consequence of these findings will require longitudinal studies of this patient population.

  9. Urinary Lactate Dehydrogenase Activity and Its Isozyme Patterns in Kawasaki Disease

    PubMed Central

    Kawamura, Yoichi; Kanai, Takashi; Takizawa, Mari; Yoshida, Yusuke; Tsujita, Yuki; Nonoyama, Shigeaki

    2017-01-01

    Abnormal urinary findings, such as sterile pyuria, proteinuria, and microscopic hematuria, are often seen in the acute phase of Kawasaki disease (KD). We investigated the potential significance of urinary lactate dehydrogenase (U-LDH) activity and its isozyme patterns in KD. Total U-LDH activity and its isozymes (U-LDH1-5) levels were compared among 120 patients with KD, 18 patients with viral infection (VI), and 43 patients with upper urinary tract infection (UTI) and additionally compared between intravenous immunoglobulin (IVIG) responders (n = 89) and nonresponders (n = 31) with KD. Total U-LDH activity was higher in KD (35.4 ± 4.8 IU/L, P < 0.05) and UTI patients (66.0 ± 8.0 IU/L, P < 0.01) than in VI patients (17.0 ± 6.2 IU/L). In the isozyme pattern analysis, KD patients had high levels of U-LDH1 and U-LDH2, while UTI patients had high levels of U-LDH3, U-LDH4, and U-LDH5. Furthermore, IVIG nonresponders of KD had significantly higher levels of total U-LDH activity (45.1 ± 4.7 IU/L, P < 0.05), especially U-LDH1 and U-LDH2 (P < 0.05), than IVIG responders (32.0 ± 2.8 IU/L). KD patients have increased levels of total U-LDH activity, especially U-LDH-1 and U-LDH2, indicating a unique pattern of U-LDH isozymes different from that in UTI patients. PMID:28348604

  10. Hepcidin-Induced Iron Deficiency Is Related to Transient Anemia and Hypoferremia in Kawasaki Disease Patients.

    PubMed

    Huang, Ying-Hsien; Kuo, Ho-Chang; Huang, Fu-Chen; Yu, Hong-Ren; Hsieh, Kai-Sheng; Yang, Ya-Ling; Sheen, Jiunn-Ming; Li, Sung-Chou; Kuo, Hsing-Chun

    2016-05-12

    Kawasaki disease (KD) is a type of systemic vasculitis that primarily affects children under the age of five years old. For sufferers of KD, intravenous immunoglobulin (IVIG) has been found to successfully diminish the occurrence of coronary artery lesions. Anemia is commonly found in KD patients, and we have shown that in appropriately elevated hepcidin levels are related to decreased hemoglobin levels in these patients. In this study, we investigated the time period of anemia and iron metabolism during different stages of KD. A total of 100 patients with KD and 20 control subjects were enrolled in this study for red blood cell and hemoglobin analysis. Furthermore, plasma, urine hepcidin, and plasma IL-6 levels were evaluated using enzyme-linked immunosorbent assay in 20 KD patients and controls. Changes in hemoglobin, plasma iron levels, and total iron binding capacity (TIBC) were also measured in patients with KD. Hemoglobin, iron levels, and TIBC were lower (p < 0.001, p = 0.009, and p < 0.001, respectively) while plasma IL-6 and hepcidin levels (both p < 0.001) were higher in patients with KD than in the controls prior to IVIG administration. Moreover, plasma hepcidin levels were positively and significantly correlated with urine hepcidin levels (p < 0.001) prior to IVIG administration. After IVIG treatment, plasma hepcidin and hemoglobin levels significantly decreased (both p < 0.001). Of particular note was a subsequent gradual increase in hemoglobin levels during the three weeks after IVIG treatment; nevertheless, the hemoglobin levels stayed lower in KD patients than in the controls (p = 0.045). These findings provide a longitudinal study of hemoglobin changes and among the first evidence that hepcidin induces transient anemia and hypoferremia during KD's acute inflammatory phase.

  11. Kawasaki Disease with Fever and Cervical Lymphadenopathy as the Sole Initial Presentation

    PubMed Central

    Jun, Woo Young; Ann, Yu Kyung; Kim, Ja Yeong; Kim, Soo-Jin; Yang, Hyun Suk; Bae, Sun Hwan; Chung, Sochung; Kim, Kyo Sun

    2017-01-01

    Background and Objectives Some patients with Kawasaki disease (KD) present with fever and cervical lymphadenopathy alone. The purpose of this study was to characterize the clinical features of these unusual KD patients and determine whether this is a severe form of KD associated with increased risks of intravenous immunoglobulin (IVIG) resistance and coronary artery lesions (CALs). Subjects and Methods A total of 146 children with KD were reviewed retrospectively, and classified into two groups according to initial clinical features. Those presenting with only fever and cervical lymphadenopathy (LKD) were classified as LKD patients. Other-KD patients included all except the LKD patients. Results Among 146 KD patients, 13 (8.9%) were classified as LKD patients. The LKD patients were significantly older and admitted earlier. The duration between fever onset and KD diagnosis was significantly longer in the LKD patients (5.9 days vs. 4.9 days, p=0.023). The frequency of IVIG resistance was not different between the two groups., In the LKD patients, the incidence of CALs was significantly higher in the acute phase, and without significant difference in the convalescent phase. The percentage of neutrophils and C-reactive protein, albumin, and total bilirubin levels were significantly higher in LKD patients. Conclusion Even though LKD patients were older, admitted earlier, and had higher inflammatory marker levels, they did not have a greater risk of CALs or IVIG resistance. However, echocardiography may be helpful in the acute stage if patients have only fever and cervical lymphadenopathy and are unresponsive to empirical antibiotics. PMID:28154598

  12. Predictive factors of resistance to intravenous immunoglobulin and coronary artery lesions in Kawasaki disease

    PubMed Central

    Lee, Hye Young

    2016-01-01

    Purpose We conducted a study to determine which factors may be useful as predictive markers in identifying Kawasaki disease (KD) patients with a high risk of resistance to intravenous immunoglobulin (IVIG) and developing coronary artery lesions (CAL). Methods We enrolled 287 patients in acute phase of KD at a single center. The demographic, clinical and laboratory data were collected retrospectively. Results There were 34 patients in the IVIG resistant group. The IVIG resistant group had significantly higher serum N-terminal-pro-brain natriuretic protein (NT-proBNP) levels (P<0.01) and polymorphonuclear neutrophil (PMN) percentage (P<0.01) in comparison to the IVIG responders. The results yielded sensitivity (78.8%, 60.6%), specificity (58.2%, 90%) and cutoff value (628.6 pg/mL, 80.3%) of NT-proBNP and PMN respectively, in predicting IVIG resistance. Despite IVIG administration, 13 of the 287 patients developed CAL. The patients in the CAL group had higher NT-proBNP levels (P<0.01) and higher PMN percentage (P<0.01). In these patients, the results yielded sensitivity (73.3%, 56.7%), specificity (67.9%, 88.9%) and cutoff value (853.4 pg/mL, 80.3%) of NT-proBNP and PMN respectively, for predicting CAL. The area under the curve (AUC) for predicting resistance to IVIG was NT-proBNP 0.712, PMN 0.802. The AUC for predicting CAL was NT-proBNP 0.739, and PMN 0.773. Conclusion Serum NT-proBNP levels and PMN percentage were significantly elevated in patients with KD with IVIG resistance and CAL. Thus, they may be useful predicting markers for IVIG resistance and development of CAL in KD patients. PMID:28194213

  13. Management of coronary artery aneurysms using abciximab in children with Kawasaki disease.

    PubMed

    Bachlava, Evangelia; Loukopoulou, Sophia; Karanasios, Evangelos; Chrousos, George; Michos, Athanasios

    2016-10-01

    There are limited data regarding the possible benefits of abciximab in children with Kawasaki disease (KD), who developed serious cardiac abnormalities non-responsive to standard treatment. We retrospectively identified children with KD who were treated with abciximab from 2007 to 2015. Data regarding clinical course, treatment, echocardiographic data and follow-up at 1 and 6months were retrieved. During the study period, fifteen children were identified who were diagnosed with KD and were given abciximab. The median age at onset of symptoms was 11months (range: 2months-6years). The median day of disease at admission was 10days (range: 4-26days) and the median day of administration of abciximab was 17days (range: 9-40). Twelve children were diagnosed with complete and three with incomplete KD. Aneurysms were found in 8 children: 2 had ectatic coronary arteries and 5 presented with both ectasia and aneurysms. At 1month follow-up, echocardiographic findings showed regression in the size of aneurysms in 11 children, resolution of the aneurysms or ectasia of coronary arteries in 3 children, while one child who could not take aspirin because of G6PD deficiency died. After 6months of follow-up, echocardiographic findings showed resolution of coronary abnormalities in 12 (80%) children, whereas 2 children (13.3%) presented with significant regression of aneurysms. Abciximab may have an important role in the management of severe cardiac complications of KD, although prospective randomized controlled studies are needed to fully evaluate its role. Copyright © 2016. Published by Elsevier Ireland Ltd.

  14. IL-1 Inhibition May Have an Important Role in Treating Refractory Kawasaki Disease

    PubMed Central

    Dusser, Perrine; Koné-Paut, Isabelle

    2017-01-01

    Kawasaki disease (KD) is an acute inflammatory vasculitis occurring in young children before 5 years and representing at this age, the main cause of acquired heart disease. A single infusion of 2 g/kg of intravenous immunoglobulins along with aspirin has reduced the frequency of coronary artery aneurysms from 25 to 5%. However, 10–20% of patients do not respond to standard treatment and have an increased risk of cardiac complications and death. The development of more potent therapeutic approaches of KD is an urgent need. Phenotypical and immunological similarities between KD and systemic juvenile idiopathic arthritis led to the hypothesis that KD could be considered as an autoinflammatory disease. New insights regarding KD’s pathogenesis have merged from the combination of genetic and transcriptomic data revealing the key role of interleukin-1 (IL-1) signaling in the pathogenesis of the vasculitis. Once activated, IL-1α and IL-1β trigger a local proinflammatory environment-inducing vasodilatation and attracting monocytes and neutrophils to sites causing tissue damage and stress. Both IL-1α and IL-1β have been shown to induce myocarditis and aneurysm formation in Lactobacillus casei cell-wall extract mouse model of KD; both being successfully improved with IL-1 blockade treatment such as anakinra. Treatment failure in patients with the high-risk inositol-triphosphate 3-kinase C genotype was associated with highest basal and stimulated intracellular calcium levels, increased cellular production of IL-1β, and IL-18, and higher circulating levels of both cytokines. Three clinical trials of IL-1 blockade enrolling KD patients are currently being conducted in Western Europe and in USA, they could change KD outcome. PMID:28400731

  15. Epidemiology and Clinical Features of Kawasaki Disease in South Korea, 2012-2014.

    PubMed

    Kim, Gi Beom; Park, Sohee; Eun, Lucy Youngmin; Han, Ji Whan; Lee, Soo Young; Yoon, Kyung Lim; Yu, Jeong Jin; Choi, Jong-Woon; Lee, Kyung-Yil

    2017-05-01

    To reveal the recent epidemiologic features of Kawasaki disease (KD) in South Korea based on data from a nationwide survey. We collected data between 2012 and 2014 regarding the incidence, symptoms and signs, treatment trends and coronary complications associated with acute KD by sending questionnaires to 97 hospitals with pediatric residency programs as well as 19 community hospitals without residency training. We received full and partial data from 97 and 13 hospitals, respectively (response rate: 94.8%). A total of 14,916 cases of KD were reported by these 110 hospitals (4588 in 2012, 5183 in 2013 and 5145 in 2014). The male-to-female ratio was 1.4:1, and the median age at diagnosis was 29 months. The incidence of KD per 100,000 children younger than 5 years of age were 170.9, 194.9 and 194.7 in 2012, 2013 and 2014, respectively. The recurrence rate was 4.7%. KD occurred more frequently during summer (especially June and July) and winter (December and January) seasons. Intravenous immunoglobulin was administered to 95.4% of the patients, and the nonresponder rate for the first intravenous immunoglobulin was 11.8%. Coronary aneurysm occurred in 1.7% of the patients, and giant aneurysm developed in 19 patients (0.16%) during the 3 years. One patient had myocardial infarction and 1 patient died of suspected coronary aneurysm rupture. The incidence of KD in South Korea increased to 194.7 per 100,000 children younger than 5 years in 2014; meanwhile, the coronary aneurysm rate decreased to 1.7%.

  16. Relationship between serum sodium level and coronary artery abnormality in Kawasaki disease

    PubMed Central

    Park, Sora; Kim, Ji Hong

    2017-01-01

    Purpose Kawasaki disease (KD) is an immune-related multisystemic vasculitis that occurs in children, especially ensuing from a coronary artery abnormality. Sodium level is known to be related to vascular injury, which could affect the progress of KD. The purpose of this study was to determine the serum sodium levels that could predict the occurrence of cardiac and coronary artery events in KD. Methods We conducted a retrospective review of medical records for 104 patients with KD from January 2015 to December 2015. Patients with serum Na levels of <135 mEq/L at the time of initial diagnosis were assigned to the hyponatremia group. Laboratory findings and echocardiographic data were analyzed for various aspects. Results Among the 104 patients with KD, 91 were included in the study, of whom 48 (52.7%) had hyponatremia. The degree of fever, white blood cell count, percentage of neutrophils, percentage of lymphocytes, total bilirubin level, brain natriuretic peptide level, erythrocyte sedimentation rate, and C-reactive protein level were higher in the patients with hyponatremia. They also demonstrated a trend of larger coronary artery diameters based on Z scores. Conclusion The severity of vascular inflammation in acute KD with hyponatremia might worsen the prognosis of coronary vasculature. Although no statistically significant correlation was found between the initial serum sodium levels and coronary arteriopathy in the patients with KD in this study, a long-term follow-up study with a larger number of enrolled patients should be designed in the future to elucidate the relationship between serum sodium level and coronary arteriopathy in patients with KD. PMID:28289432

  17. Usefulness of Calcium Scoring as a Screening Examination in Patients With a History of Kawasaki Disease.

    PubMed

    Kahn, Andrew M; Budoff, Matthew J; Daniels, Lori B; Oyamada, Jun; Gordon, John B; Burns, Jane C

    2017-04-01

    Subsets of patients with a remote history of Kawasaki disease (KD) have coronary artery aneurysms with associated risks of late morbidity. In a pilot study, we previously showed that computed tomography (CT) coronary artery calcium (CAC) scoring detects late CAC in patients with aneurysms and a remote history of KD. We performed CT calcium volume scoring in 166 subjects (median age 19.5 years) with a remote history of KD (median interval from KD to CT 15.1 years). Coronary arteries were classified as normal (n = 100), transiently dilated (n = 23), persistently dilated (n = 10), remodeled aneurysm (n = 9), or aneurysm (n = 24) based on echocardiography. All subjects with coronary arteries classified as normal, persistently dilated, or remodeled aneurysm had zero CAC. Of the 24 subjects with coronary aneurysms, all but 5 had CAC (median volume 542 mm(3); range 17 to 8,218 mm(3)). For subjects imaged ≥9 years after their acute KD (n = 144), the presence of CAC had a sensitivity of 95% and a specificity of 100% for detecting coronary artery abnormalities (defined as coronary artery aneurysm and/or stenosis). In conclusion, coronary calcification was not observed in subjects with a history of KD who had normal coronary arteries by echocardiography during the acute phase. Coronary calcification, which may be severe, occurs late in patients with coronary aneurysms. Therefore, CAC scanning may be a useful tool to screen patients with a remote history of KD or suspected KD and unknown coronary artery status. Copyright © 2017 Elsevier Inc. All rights reserved.

  18. Changes in clinical and laboratory features of Kawasaki disease noted over time in Daejeon, Korea.

    PubMed

    Kil, Hong-Ryang; Yu, Jae-Won; Lee, Sung-Churl; Rhim, Jung-Woo; Lee, Kyung-Yil

    2017-08-07

    Kawasaki disease (KD) becomes one of the common diseases in Korea. Changes in clinical features and laboratory findings of KD were evaluated over a period of 10 years. We reviewed the medical records of KD patients and compared the clinical and laboratory features of two KD patient groups: those admitted from 2000 to 2004 (group A, 284 cases) and those admitted from 2010 to 2014 (group B, 331 cases). There were a total of 615 KD patients (mean age: 29.7 months; male-to-female ratio = 1.6:1), including 228 incomplete KD patients. Incomplete KD patients had milder values in some laboratory indices. The preadmission and total fever durations were longer in group A than in group B. The proportion of incomplete KD was higher in group B, but incidence of coronary artery lesions (CALs) was lower. For laboratory indices, the C-reactive protein and follow-up platelet values were lower, and the hemoglobin and albumin values were higher in group B. The same clinical and laboratory findings were confirmed in the KD subgroups; those with the same fever duration of 5 or 6 days and same ages, those with complete KD, and those with incomplete KD in the two different time periods. Our findings suggest that clinical features of KD tend to be milder over time and manifest in a higher incidence of incomplete KD, lower incidence of CALs, and less severe laboratory findings in recent KD patients in Korea compared with their historic counterparts.

  19. Clinical outcome of patients with refractory Kawasaki disease based on treatment modalities

    PubMed Central

    Kim, Hyun Jung; Lee, Hyo Eun; Yu, Jae Won

    2016-01-01

    Purpose Although a significant number of reports on new therapeutic options for refractory Kawasaki disease (KD) such as steroid, infliximab, or repeated intravenous immunoglobulin (IVIG) are available, their effectiveness in reducing the prevalence of coronary artery lesions (CAL) remains controversial. This study aimed to define the clinical characteristics of patients with refractory KD and to assess the effects of adjuvant therapy on patient outcomes. Methods We performed a retrospective study of 38 refractory KD patients from January 2012 to March 2015. We divided these patients into 2 groups: group 1 received more than 3 IVIG administration+ steroid therapy, (n=7, 18.4%), and group 2 patients were unresponsive to initial IVIG and required steroid therapy or second IVIG (n=31, 81.6%). We compared the clinical manifestations, laboratory results, and echocardiographic findings between the groups and examined the clinical utility of additional therapies in both groups. Results A significant difference was found in the total duration of fever between the groups (13.0±4.04 days in group 1 vs. 8.87±2.30 days in group 2; P=0.035). At the end of the follow-up, all cases in group 1 showed suppressed CAL. In group 2, coronary artery aneurysm occurred in 2 patients (6.4 %). All the patients treated with intravenous corticosteroids without additional IVIG developed CALs including coronary artery aneurysms. Conclusion No statistical difference was found in the development of CAL between the groups. Prospective, randomized, clinical studies are needed to elucidate the effects of adjunctive therapy in refractory KD patients. PMID:27610181

  20. Clinical outcome of patients with refractory Kawasaki disease based on treatment modalities.

    PubMed

    Kim, Hyun Jung; Lee, Hyo Eun; Yu, Jae Won; Kil, Hong Ryang

    2016-08-01

    Although a significant number of reports on new therapeutic options for refractory Kawasaki disease (KD) such as steroid, infliximab, or repeated intravenous immunoglobulin (IVIG) are available, their effectiveness in reducing the prevalence of coronary artery lesions (CAL) remains controversial. This study aimed to define the clinical characteristics of patients with refractory KD and to assess the effects of adjuvant therapy on patient outcomes. We performed a retrospective study of 38 refractory KD patients from January 2012 to March 2015. We divided these patients into 2 groups: group 1 received more than 3 IVIG administration+ steroid therapy, (n=7, 18.4%), and group 2 patients were unresponsive to initial IVIG and required steroid therapy or second IVIG (n=31, 81.6%). We compared the clinical manifestations, laboratory results, and echocardiographic findings between the groups and examined the clinical utility of additional therapies in both groups. A significant difference was found in the total duration of fever between the groups (13.0±4.04 days in group 1 vs. 8.87±2.30 days in group 2; P=0.035). At the end of the follow-up, all cases in group 1 showed suppressed CAL. In group 2, coronary artery aneurysm occurred in 2 patients (6.4 %). All the patients treated with intravenous corticosteroids without additional IVIG developed CALs including coronary artery aneurysms. No statistical difference was found in the development of CAL between the groups. Prospective, randomized, clinical studies are needed to elucidate the effects of adjunctive therapy in refractory KD patients.

  1. The Usefulness of Platelet-derived Microparticle as Biomarker of Antiplatelet Therapy in Kawasaki Disease.

    PubMed

    Kim, Hyun Jung; Choi, Eun Hye; Lim, Yeon Jung; Kil, Hong Ryang

    2017-07-01

    Little is known about platelet dynamics and the effect of antiplatelet therapy in Kawasaki disease (KD). This study sought to define platelet activation dynamics in KD patients by assaying platelet-derived microparticles (PDMPs). We measured plasma PDMPs levels in 46 patients with KD using an enzyme-linked immunosorbent assay (ELISA). Blood samples were collected before, at 2-5 days, and 9-15 days after intravenous immunoglobulin (IVIG) infusion, 2 months and 4-5 months after the onset of KD. We measured PDMP levels in 23 febrile and 10 afebrile control patients. In the acute phase of KD patients, PDMP levels increased significantly after IVIG treatment (12.04 ± 5.58 nmol before IVIG infusion vs. 19.81 ± 13.21 nmol at 2-5 days after IVIG infusion, P = 0.006). PDMP levels were negatively correlated with age and positively correlated with procalcitonin levels in the acute phase of KD. No significant difference was found in PDMP levels between KD patients with and without coronary artery lesion (CAL). Elevated PDMP levels after IVIG therapy significantly decreased below the pre-IVIG level in subacute phase (19.81 ± 13.21 nmol at 2-5 days after IVIG infusion vs. 8.33 ± 2.02 nmol at 9-15 days after IVIG infusion, P < 0.001), and PDMP levels stayed below the pre-IVIG level in the convalescent phase, during which antiplatelet therapy was given. However, PDMP levels rebounded after discontinuing aspirin in 17 patients. In conclusion, enhanced platelet activation was noted before treatment of KD and peaked immediately after IVIG treatment. Recurrent rising of PDMP levels was observed after discontinuing aspirin, although there were no significant differences between the PDMP levels at 2 months after the onset of KD and those at 4-5 months after the onset of the disease. © 2017 The Korean Academy of Medical Sciences.

  2. The Usefulness of Platelet-derived Microparticle as Biomarker of Antiplatelet Therapy in Kawasaki Disease

    PubMed Central

    2017-01-01

    Little is known about platelet dynamics and the effect of antiplatelet therapy in Kawasaki disease (KD). This study sought to define platelet activation dynamics in KD patients by assaying platelet-derived microparticles (PDMPs). We measured plasma PDMPs levels in 46 patients with KD using an enzyme-linked immunosorbent assay (ELISA). Blood samples were collected before, at 2–5 days, and 9–15 days after intravenous immunoglobulin (IVIG) infusion, 2 months and 4–5 months after the onset of KD. We measured PDMP levels in 23 febrile and 10 afebrile control patients. In the acute phase of KD patients, PDMP levels increased significantly after IVIG treatment (12.04 ± 5.58 nmol before IVIG infusion vs. 19.81 ± 13.21 nmol at 2–5 days after IVIG infusion, P = 0.006). PDMP levels were negatively correlated with age and positively correlated with procalcitonin levels in the acute phase of KD. No significant difference was found in PDMP levels between KD patients with and without coronary artery lesion (CAL). Elevated PDMP levels after IVIG therapy significantly decreased below the pre-IVIG level in subacute phase (19.81 ± 13.21 nmol at 2–5 days after IVIG infusion vs. 8.33 ± 2.02 nmol at 9–15 days after IVIG infusion, P < 0.001), and PDMP levels stayed below the pre-IVIG level in the convalescent phase, during which antiplatelet therapy was given. However, PDMP levels rebounded after discontinuing aspirin in 17 patients. In conclusion, enhanced platelet activation was noted before treatment of KD and peaked immediately after IVIG treatment. Recurrent rising of PDMP levels was observed after discontinuing aspirin, although there were no significant differences between the PDMP levels at 2 months after the onset of KD and those at 4–5 months after the onset of the disease. PMID:28581272

  3. Association between adipokines and coronary artery lesions in children with Kawasaki Disease.

    PubMed

    Kim, Hyun Jung; Choi, Eun Hye; Kil, Hong Ryang

    2014-10-01

    Body fat is an important source of adipokine, which is associated with energy balance and inflammatory and immune responses. However, the role of adipokines in coronary artery complications in Kawasaki disease (KD) has not yet been fully explained. We investigated whether serum adipokine level can be a useful marker for patients with KD who are at higher risk of developing coronary artery lesion (CAL). We measured adipokine levels and other inflammatory parameters in 40 patients with KD, 32 febrile controls, and 15 afebrile controls. Interleukin (IL)-6, tumor necrosis factor (TNF)-α and other laboratory parameters were also measured before and after intravenous immunoglobulin therapy, and in the convalescent phase. At admission, the serum resistin levels in KD children were significantly higher than those in controls (177.56 ng/mL in KD children, 76.48 ng/mL in febrile controls, and 17.95 ng/mL in afebrile controls). In patients with KD, resistin levels were significantly associated with decreased hemoglobin levels (P=0.049) and increased IL-6 levels (P=0.014). The serum IL-6 levels were significantly higher and body mass index was significantly lower in the group of KD with CALs than those without CALs (228.26 ng/mL vs. 39.18 ng/mL and 15.09 vs. 16.60, respectively). In conclusion, resistin is significantly elevated in KD patients, although it has no prognostic value of predicting coronary artery lesion in the acute stage.

  4. Urinary Colorimetric Sensor Array and Algorithm to Distinguish Kawasaki Disease from Other Febrile Illnesses

    PubMed Central

    Jin, Bo; Deng, Xiaohong; Hu, Guang; Liu, Xiaodan; Zhang, Jie; Jin, Hua; Huang, Min; Kanegaye, John T.; Tremoulet, Adriana H.; Burns, Jane C.; Wu, Jianmin; Cohen, Harvey J.; Ling, Xuefeng B.

    2016-01-01

    Objectives Kawasaki disease (KD) is an acute pediatric vasculitis of infants and young children with unknown etiology and no specific laboratory-based test to identify. A specific molecular diagnostic test is urgently needed to support the clinical decision of proper medical intervention, preventing subsequent complications of coronary artery aneurysms. We used a simple and low-cost colorimetric sensor array to address the lack of a specific diagnostic test to differentiate KD from febrile control (FC) patients with similar rash/fever illnesses. Study Design Demographic and clinical data were prospectively collected for subjects with KD and FCs under standard protocol. After screening using a genetic algorithm, eleven compounds including metalloporphyrins, pH indicators, redox indicators and solvatochromic dye categories, were selected from our chromatic compound library (n = 190) to construct a colorimetric sensor array for diagnosing KD. Quantitative color difference analysis led to a decision-tree-based KD diagnostic algorithm. Results This KD sensing array allowed the identification of 94% of KD subjects (receiver operating characteristic [ROC] area under the curve [AUC] 0.981) in the training set (33 KD, 33 FC) and 94% of KD subjects (ROC AUC: 0.873) in the testing set (16 KD, 17 FC). Color difference maps reconstructed from the digital images of the sensing compounds demonstrated distinctive patterns differentiating KD from FC patients. Conclusions The colorimetric sensor array, composed of common used chemical compounds, is an easily accessible, low-cost method to realize the discrimination of subjects with KD from other febrile illness. PMID:26859297

  5. Transmission of acute infectious illness among cases of Kawasaki disease and their household members.

    PubMed

    Tsai, Hsing-Chen; Chang, Luan-Yin; Lu, Chun-Yi; Shao, Pei-Lan; Fan, Tsui-Yen; Cheng, Ai-Ling; Hu, Jen-Jan; Yeh, Shu-Jen; Chang, Chien-Chih; Huang, Li-Min

    2015-01-01

    Kawasaki disease (KD) is a disease of unknown cause and the causative agent is most likely to be infectious in nature. To investigate the household transmission pattern of infectious illness and etiology, we thus initiated a prospective case and household study. We enrolled KD cases and their household members from February 2004 to September 2008. The KD cases and their household members accepted questionnaire-based interviews of the contact history, signs of infection, and symptoms to check whether clusters of infectious illness occurred. A total of 142 KD cases and 561 household members were enrolled. Among the 142 KD cases, 136 cases (96%) were typical KD, and six (4%) were atypical KD. Of the 561 household members, 17% were siblings, 46% were parents, 18% were grandparents, and the others were cousins or babysitters. Prior to the onset of their KD illness, 66% (94/142) KD cases had contact with ill household members. On the same day of the onset of KD cases' illness, 4% (6/142) KD cases had household members with illness. After KD cases' disease onset, 70% (100/142) KD cases had at least one other family member with illness. Overall, 61% (343/561) of all the household members had acute infectious illness during KD cases' acute stage, and 92% (130/142) of the families had clusters of infectious illness. A total of 66% KD cases had positive contact with ill household members prior to their disease onset and 92% of families had clusters of infectious illness, so KD is strongly associated with infections. Copyright © 2014. Published by Elsevier B.V.

  6. Racial/ethnic differences in the incidence of Kawasaki syndrome among children in Hawaii.

    PubMed

    Holman, Robert C; Christensen, Krista Y; Belay, Ermias D; Steiner, Claudia A; Effler, Paul V; Miyamura, Jill; Forbes, Susan; Schonberger, Lawrence B; Melish, Marian

    2010-08-01

    To describe the occurrence of Kawasaki syndrome (KS) among different racial/ethnic groups in Hawaii. Retrospective analysis of children <18 years of age, with a focus on children <5 years of age, living in Hawaii who were hospitalized with KS using the 1996-2006 Hawaii State Inpatient Data. Children <5 years of age accounted for 84% of the 528 patients <18 years of age with KS. The average annual incidence among this age group was 50.4 per 100,000 children <5 years of age, ranging from 45.5 to 56.5. Asian and Pacific Islander children accounted for 92% of the children <5 years of age with KS during the study period; the average annual incidence was 62.9 per 100,000. Within this group, Japanese children had the highest incidence (210.5), followed by Native Hawaiian children (86.9), other Asian children (84.9), and Chinese children (83.2). The incidence for white children (13.7) was lower than for these racial/ethnic groups. The median age of KS admission for children <5 years of age was 21 months overall, 24 months for Japanese children, 14.5 months for Native Hawaiian children and 26.5 months for white children. The high average annual KS incidence for children <5 years of age in Hawaii compared to the rest of the United States population reflects an increased KS incidence among Asian and Pacific Islander children, especially Japanese children. The incidence for white children was slightly higher than or similar to that generally reported nationwide. Hawaii Medical Journal Copyright 2010.

  7. Image-based modeling of hemodynamics in coronary artery aneurysms caused by Kawasaki disease.

    PubMed

    Sengupta, Dibyendu; Kahn, Andrew M; Burns, Jane C; Sankaran, Sethuraman; Shadden, Shawn C; Marsden, Alison L

    2012-07-01

    Kawasaki Disease (KD) is the leading cause of acquired pediatric heart disease. A subset of KD patients develops aneurysms in the coronary arteries, leading to increased risk of thrombosis and myocardial infarction. Currently, there are limited clinical data to guide the management of these patients, and the hemodynamic effects of these aneurysms are unknown. We applied patient-specific modeling to systematically quantify hemodynamics and wall shear stress in coronary arteries with aneurysms caused by KD. We modeled the hemodynamics in the aneurysms using anatomic data obtained by multi-detector computed tomography (CT) in a 10-year-old male subject who suffered KD at age 3 years. The altered hemodynamics were compared to that of a reconstructed normal coronary anatomy using our subject as the model. Computer simulations using a robust finite element framework were used to quantify time-varying shear stresses and particle trajectories in the coronary arteries. We accounted for the cardiac contractility and the microcirculation using physiologic downstream boundary conditions. The presence of aneurysms in the proximal coronary artery leads to flow recirculation, reduced wall shear stress within the aneurysm, and high wall shear stress gradients at the neck of the aneurysm. The wall shear stress in the KD subject (2.95-3.81 dynes/sq cm) was an order of magnitude lower than the normal control model (17.10-27.15 dynes/sq cm). Particle residence times were significantly higher, taking 5 cardiac cycles to fully clear from the aneurysmal regions in the KD subject compared to only 1.3 cardiac cycles from the corresponding regions of the normal model. In this novel quantitative study of hemodynamics in coronary aneurysms caused by KD, we documented markedly abnormal flow patterns that are associated with increased risk of thrombosis. This methodology has the potential to provide further insights into the effects of aneurysms in KD and to help risk stratify patients for

  8. Transthoracic echo-Doppler assessment of coronary microvascular function late after Kawasaki disease.

    PubMed

    Cicala, S; Galderisi, M; Grieco, M; Lamberti, A; Cosimi, R; Pellegrini, F; de Leva, F

    2008-03-01

    The goal of this study was to demonstrate that Doppler transthoracic echocardiography (TTE) may represent a valuable tool for the noninvasive demonstration of coronary microvascular dysfunction in children with previous Kawasaki disease (KD) by the measurement of coronary flow reserve (CFR) during cold pressor test (CPT). Twenty-five children with previous KD (mean follow-up, 4.6 +/- 2.6 years) were included in the study-16 with no evidence of coronary artery lesions (CALs(-)) by TTE and 9 with coronary aneurysms (CALs(+)). Seventeen age-matched healthy subjects were also recruited. Diastolic peak velocity was measured by pulsed Doppler both at rest (DPV(Rest)) and during CPT (DPV(CPT)) in the anterior descending artery. CFR was calculated as DPV(CPT)/DPV(Rest). KD patients demonstrated significantly higher values of DPV(Rest) (0.21 +/- 0.05 vs 0.13 +/- 0.01 cm/sec, p < 0.0001) and DPV(CPT) (0.33 +/- 0.07 vs 0.27 +/- 0.03 cm/sec, p < 0.005). CFR was reduced in KD compared to control subjects (1.5 +/- 0.4 vs 2.1 +/- 0.2, p < 0.0001). CFR was decreased in a similar manner in both CALs(+) patients (1.4 +/- 0.4, p = 0.002 vs controls) and CALs(-) patients (1.6 +/- 0.4, p < 0.0001 vs controls). Doppler TTE at rest and during CPT may represent a valuable modality for CFR evaluation in children with a history of KD. CFR is significantly reduced in KD patients independently of the presence of CALs.

  9. The Spectrum of Cardiovascular Lesions Requiring Intervention in Adults After Kawasaki Disease.

    PubMed

    Gordon, John B; Daniels, Lori B; Kahn, Andrew M; Jimenez-Fernandez, Susan; Vejar, Matthew; Numano, Fujito; Burns, Jane C

    2016-04-11

    The aim of this study was to characterize the range of management issues raised by adults with cardiovascular sequelae from Kawasaki disease (KD) in childhood. Aneurysms resulting from vascular inflammation associated with KD in childhood may remain clinically silent until adulthood. Adults with large aneurysms, unstable angina, or myocardial infarction following KD in childhood present unique challenges to interventional cardiologists and cardiothoracic surgeons. In an observational study of adults with histories of KD in childhood, data were collected regarding the medical histories and outcomes of 154 adult KD patients, of whom 21 underwent either percutaneous interventions or surgery. Of the 21 subjects with interventions, 11 had been diagnosed with KD in childhood, and 10 had histories of KD-compatible illnesses. Seventeen subjects were asymptomatic until experiencing acute cardiovascular symptoms: acute myocardial infarction (n = 12), angina (n = 2), end-stage congestive heart failure requiring cardiac transplantation (n = 1), and claudication (n = 2). Cardiovascular complications in these subjects illustrate the following points: 1) even small to moderate-sized aneurysms that "normalize" on echocardiography in childhood can lead to stenosis and thrombosis decades after the acute illness; 2) coronary interventions without intravascular ultrasound may result in clinically significant underestimation of vessel luminal diameter; 3) failure to assess the extent of calcification may lead to suboptimal procedural outcomes; and 4) patients with symptomatic peripheral aneurysms may benefit from endarterectomy or resection. Interventional cardiologists should be aware of the potential challenges in treating this growing population of adults. Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  10. Kawasaki Disease: Laboratory Findings and an Immunopathogenesis on the Premise of a "Protein Homeostasis System"

    PubMed Central

    Rhim, Jung-Woo; Kang, Jin-Han

    2012-01-01

    Kawasaki disease (KD) is a self-limited systemic inflammatory illness, and coronary artery lesions (CALs) are a major complication determining the prognosis of the disease. Epidemiologic studies in Asian children suggest that the etiologic agent(s) of KD may be associated with environmental changes. Laboratory findings are useful for the diagnosis of incomplete KD, and they can guide the next-step in treatment of initial intravenous immunoglobulin non-responders. CALs seem to develop in the early stages of the disease before a peak in inflammation. Therefore early treatment, before the peak in inflammation, is mandatory to reduce the risk of CAL progression and severity of CALs. The immunopathogenesis of KD is more likely that of acute rheumatic fever than scarlet fever. A hypothetical pathogenesis of KD is proposed under the premise of a "protein homeostasis system"; where innate and adaptive immune cells control pathogenic proteins that are toxic to host cells at a molecular level. After an infection of unknown KD pathogen(s), the pathogenic proteins produced from an unknown focus, spread and bind to endothelial cells of coronary arteries as main target cells. To control the action of pathogenic proteins and/or substances from the injured cells, immune cells are activated. Initially, non-specific T cells and non-specific antibodies are involved in this reaction, while hyperactivated immune cells produce various cytokines, leading to a cytokine imbalance associated with further endothelial cell injury. After the emergence of specific T cells and specific antibodies against the pathogenic proteins, tissue injury ceases and a repair reaction begins with the immune cells. PMID:22318812

  11. A national survey on the pediatric cardiologist's clinical approach for patients with Kawasaki disease.

    PubMed

    Kahwaji, I Y; Connuck, D M; Tafari, N; Dahdah, N S

    2002-01-01

    In 1994, the American Heart Association (AHA) published the most recent guidelines for long-term cardiovascular management of Kawasaki disease. Since then, recent publications have shed new light on different diagnostic, prognostic, and management issues. We sought the opinion of pediatric cardiologists practicing in U.S. fellowship programs on the subject by means of a multiple-choice survey. Two questions addressed therapy in the acute phase, each preceded by a statement from related literature. Ten duplicate questions addressed the long-term cardiovascular management in five sets of paired questions; each question was first given in reminiscence of a clinical situation and then preceded by a statement from particular publications representative of new information that has become available since the publication of the 1994 AHA guidelines. All questions were provided in the same mailing. Replies were received from 97 participants practicing at 29 institutions. For the acute illness, 21% of respondents do not use high-dose aspirin, and 50% support reassessment of current guidelines. Universal intravenous immune globulin (IVIG) administration is followed by 97%, among whom 20% agree that evaluation of selection criteria is needed. For long-term management, 60-75% advocate regular follow-up of risk level I patients, and 80% favor periodic follow-up, with stress imaging (34-40%), for risk level II. For risk level IV more respondents favor stress echocardiography as opposed to nuclear imaging, in consonance with recent literature. For risk levels III and IV, 36-40% perform coronary angiography on a regular basis, whereas 60% do so when coronary symptoms are present or when stress imaging suggests myocardial ischemia. Finally, 19-25% of respondents do not routinely advise healthy lifestyle to patients free of coronary artery lesions. In conclusion, the guidelines for conventional therapy in the acute phase and long-term cardiovascular management need to be revised.

  12. Reaction at the bacillus Calmette--Guérin inoculation site in patients with Kawasaki disease.

    PubMed

    Lai, Chi-Chieh; Lee, Pi-Chang; Wang, Chih-Chien; Hwang, Be-Tau; Meng, C C Laura; Tsai, Ming-Chih

    2013-02-01

    The bacillus Calmette-Guérin (BCG) reaction is not included in the classical clinical criteria for Kawasaki disease (KD). However, a reaction at the BCG inoculation site has been mentioned among the "other clinical findings" that are present in about 30-50% of KD patients. The objective of this study was to investigate the clinical characteristics of KD patients with reactions at the BCG inoculation site. A retrospective study of all patients diagnosed with KD between September 2000 and August 2010 was performed. The clinical presentations, laboratory results, treatment outcomes, and coronary artery abnormalities in the BCG-reactive [BCG(+)] and BCG-nonreactive [BCG(-)] groups were analyzed and compared. In total, 145 patients with KD diagnosed at our institution were included; 46 (31.7%) had a reaction at the BCG inoculation site. The BCG(+) group was younger than the BCG(-) group. Laboratory results showed higher white blood cell counts, platelet counts, and serum potassium levels, and lower low-density lipoprotein levels in the BCG(+) group. The BCG(+) group had a shorter fever duration before intravenous immunoglobulin treatment and a shorter total fever duration than the BCG(-) group. Multivariable logistic regression analysis showed that the age at diagnosis was the only factor significantly associated with a reaction at the BCG inoculation site in KD patients. In countries with a national BCG vaccination program, a reaction at the BCG inoculation site could be a useful and early diagnostic sign of KD among younger patients, especially those younger than 6 months. Copyright © 2012. Published by Elsevier B.V.

  13. Parameters to Guide Retreatment After Initial Intravenous Immunoglobulin Therapy in Kawasaki Disease

    PubMed Central

    Kim, Hyun Kwon; Oh, Jungeun; Hong, Young Mi

    2011-01-01

    Background and Objectives We sought to determine parameters to guide the decision of retreatment in patients with Kawasaki disease (KD) who remained febrile after initial intravenous immunoglobulin (IVIG). Subjects and Methods A total of 129 children with KD were studied prospectively. Patients were treated with IVIG 2 to 9 days after the onset of disease. Laboratory measures, such as white blood cell (WBC), percentage of neutrophils, C-reactive protein (CRP), and N-terminal pro-brain natriuretic peptide (NT-proBNP), were determined before and 48 to 72 hours after IVIG treatment. Patients were classified into IVIG-responsive and IVIG-resistant groups, based on the response to IVIG. Results Of a total of 129 patients, 107 patients (83%) completely responded to a single IVIG therapy and only 22 patients (17%) required retreatment: 14 had persistent fever and 8 had recrudescent fever. There was no significant difference between the groups in age, gender distribution, and duration of fever to IVIG initiation, but coronary artery lesions developed significantly more often in the resistant group than in the responsive group (31.8% vs. 2.8%, p=0.000). Compared with pre-IVIG data, post-IVIG levels of WBC, percentage of neutrophils, CRP, and NT-proBNP decreased to within the normal range in the responsive group, whereas they remained high in the resistant group. Multivariate logistic regression indicated that neutrophil counts, CRP, and NT-proBNP were independent parameters of retreatment. Conclusion Additional therapy at an early stage of the disease should be administered for febrile patients who have high values of CRP, NT-proBNP, and/or neutrophil counts after IVIG therapy. PMID:21860639

  14. Serum and urinary interleukin-6 (IL-6) levels as predicting factors of Kawasaki disease activity.

    PubMed

    Chow, Y M; Lin, C Y; Hwang, B

    1993-01-01

    From January 1988 to Autumn 1991, 60 patients suffering from Kawasaki disease (KD) were recruited in this study. Their ages ranged from 4 months to 5 years. Diagnosis was based on the criteria revised in 1984 by the KD Research Committee in Japan. Of these, 12 cases developed coronary aneurysms. First, blood samples from 60 KD patients were taken on admission before aspirin and/or intravenous immunoglobulin (IVIG) treatment. Convalescent blood samples were taken 3 months after onset of disease. The control group included (1) 10 cases of viral infection with skin rash and fever (aged 5 months to 5 years) and (2) 10 age and sex matched normal children admitted for elective pediatric surgery such as inguinal hernia. Second, urinary samples were collected from 32 cases during the acute phase of KD. Of these, 10 cases had pyuria and/or proteinuria. The results showed that the serum IL-6 levels from KD patients during the first week of acute phase were significantly increased while undetectable in the convalescent sera and controls. There was also a statistical difference between the with and without coronary aneurysm groups during the first week (336.8 +/- 95.1 vs 125.5 +/- 56.5 pg/ml, P < 0.001). Urinary IL-6 levels were significantly elevated in KD patients with pyuria and/or proteinuria (156.6 +/- 77.7 pg/mg Cr) and undetectable in the group without pyuria and proteinuria and controls during the first week. There was no difference between with and without coronary aneurysm. These results suggest that serum IL-6 level is a useful factor for predicting formation of coronary aneurysm even within one week after onset of disease.(ABSTRACT TRUNCATED AT 250 WORDS)

  15. Urinary Lactate Dehydrogenase Activity and Its Isozyme Patterns in Kawasaki Disease.

    PubMed

    Kawamura, Yoichi; Takeshita, Seiichiro; Kanai, Takashi; Takizawa, Mari; Yoshida, Yusuke; Tsujita, Yuki; Nonoyama, Shigeaki

    2017-01-01

    Abnormal urinary findings, such as sterile pyuria, proteinuria, and microscopic hematuria, are often seen in the acute phase of Kawasaki disease (KD). We investigated the potential significance of urinary lactate dehydrogenase (U-LDH) activity and its isozyme patterns in KD. Total U-LDH activity and its isozymes (U-LDH1-5) levels were compared among 120 patients with KD, 18 patients with viral infection (VI), and 43 patients with upper urinary tract infection (UTI) and additionally compared between intravenous immunoglobulin (IVIG) responders (n = 89) and nonresponders (n = 31) with KD. Total U-LDH activity was higher in KD (35.4 ± 4.8 IU/L, P < 0.05) and UTI patients (66.0 ± 8.0 IU/L, P < 0.01) than in VI patients (17.0 ± 6.2 IU/L). In the isozyme pattern analysis, KD patients had high levels of U-LDH1 and U-LDH2, while UTI patients had high levels of U-LDH3, U-LDH4, and U-LDH5. Furthermore, IVIG nonresponders of KD had significantly higher levels of total U-LDH activity (45.1 ± 4.7 IU/L, P < 0.05), especially U-LDH1 and U-LDH2 (P < 0.05), than IVIG responders (32.0 ± 2.8 IU/L). KD patients have increased levels of total U-LDH activity, especially U-LDH-1 and U-LDH2, indicating a unique pattern of U-LDH isozymes different from that in UTI patients.

  16. Hepcidin-Induced Iron Deficiency Is Related to Transient Anemia and Hypoferremia in Kawasaki Disease Patients

    PubMed Central

    Huang, Ying-Hsien; Kuo, Ho-Chang; Huang, Fu-Chen; Yu, Hong-Ren; Hsieh, Kai-Sheng; Yang, Ya-Ling; Sheen, Jiunn-Ming; Li, Sung-Chou; Kuo, Hsing-Chun

    2016-01-01

    Kawasaki disease (KD) is a type of systemic vasculitis that primarily affects children under the age of five years old. For sufferers of KD, intravenous immunoglobulin (IVIG) has been found to successfully diminish the occurrence of coronary artery lesions. Anemia is commonly found in KD patients, and we have shown that in appropriately elevated hepcidin levels are related to decreased hemoglobin levels in these patients. In this study, we investigated the time period of anemia and iron metabolism during different stages of KD. A total of 100 patients with KD and 20 control subjects were enrolled in this study for red blood cell and hemoglobin analysis. Furthermore, plasma, urine hepcidin, and plasma IL-6 levels were evaluated using enzyme-linked immunosorbent assay in 20 KD patients and controls. Changes in hemoglobin, plasma iron levels, and total iron binding capacity (TIBC) were also measured in patients with KD. Hemoglobin, iron levels, and TIBC were lower (p < 0.001, p = 0.009, and p < 0.001, respectively) while plasma IL-6 and hepcidin levels (both p < 0.001) were higher in patients with KD than in the controls prior to IVIG administration. Moreover, plasma hepcidin levels were positively and significantly correlated with urine hepcidin levels (p < 0.001) prior to IVIG administration. After IVIG treatment, plasma hepcidin and hemoglobin levels significantly decreased (both p < 0.001). Of particular note was a subsequent gradual increase in hemoglobin levels during the three weeks after IVIG treatment; nevertheless, the hemoglobin levels stayed lower in KD patients than in the controls (p = 0.045). These findings provide a longitudinal study of hemoglobin changes and among the first evidence that hepcidin induces transient anemia and hypoferremia during KD’s acute inflammatory phase. PMID:27187366

  17. Kawasaki Disease in Mongolia: Results From 2 Nationwide Retrospective Surveys, 1996–2008

    PubMed Central

    Davaalkham, Dambadarjaa; Nakamura, Yosikazu; Baigalmaa, Davaakhuu; Davaa, Gombojav; Chimedsuren, Ochir; Sumberzul, Nyamjav; Lkhagvasuren, Tserenkhuu; Uehara, Ritei; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2011-01-01

    Background Kawasaki disease (KD) has been reported in many countries. However, the incidence of KD in Mongolia is not known. This is the first report of incident cases of KD in Mongolia, which were identified using data from 2 nationwide surveys. Methods Two nationwide retrospective surveys were conducted: medical histories were collected from patients aged 0 to 16 years who were hospitalized countrywide between 1996 and 2008. Hospital records for these patients were also reviewed. Nationwide training seminars on KD were conducted before each survey. Results For the nationwide surveys, the participation rates among all hospitals with pediatric wards were 97% and 94%. Inpatient medical histories from 1996 through 2008 were reviewed, and, among children younger than 16 years, 9 patients with KD were investigated. The age of KD patients ranged from 1.4 to 14 years; 7 of 9 patients were male. Six (67%) patients fulfilled all 6 clinical diagnostic criteria; the other 3 (33%) were defined as having KD based on the presence of 5 such criteria. Fever persisting 5 or more days, bilateral conjunctival congestion, and changes of the lips and oral cavity were the most common symptoms, and cervical lymphadenopathy was the least common symptom. Cardiac sequelae developed in 5 of the patients, 4 of whom were older than 10 years. Conclusions The results of these nationwide surveys reveal that KD cases do exist in Mongolia. However, knowledge of KD among Mongolian pediatricians is likely to be poor. Thus, there is a need to augment their understanding to improve management of KD patients. Further studies are crucial to clarify the epidemiologic characteristics of KD in Mongolia. PMID:21691035

  18. Kawasaki disease in Mongolia: results from 2 nationwide retrospective surveys, 1996-2008.

    PubMed

    Davaalkham, Dambadarjaa; Nakamura, Yosikazu; Baigalmaa, Davaakhuu; Davaa, Gombojav; Chimedsuren, Ochir; Sumberzul, Nyamjav; Lkhagvasuren, Tserenkhuu; Uehara, Ritei; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2011-01-01

    Kawasaki disease (KD) has been reported in many countries. However, the incidence of KD in Mongolia is not known. This is the first report of incident cases of KD in Mongolia, which were identified using data from 2 nationwide surveys. Two nationwide retrospective surveys were conducted: medical histories were collected from patients aged 0 to 16 years who were hospitalized countrywide between 1996 and 2008. Hospital records for these patients were also reviewed. Nationwide training seminars on KD were conducted before each survey. For the nationwide surveys, the participation rates among all hospitals with pediatric wards were 97% and 94%. Inpatient medical histories from 1996 through 2008 were reviewed, and, among children younger than 16 years, 9 patients with KD were investigated. The age of KD patients ranged from 1.4 to 14 years; 7 of 9 patients were male. Six (67%) patients fulfilled all 6 clinical diagnostic criteria; the other 3 (33%) were defined as having KD based on the presence of 5 such criteria. Fever persisting 5 or more days, bilateral conjunctival congestion, and changes of the lips and oral cavity were the most common symptoms, and cervical lymphadenopathy was the least common symptom. Cardiac sequelae developed in 5 of the patients, 4 of whom were older than 10 years. The results of these nationwide surveys reveal that KD cases do exist in Mongolia. However, knowledge of KD among Mongolian pediatricians is likely to be poor. Thus, there is a need to augment their understanding to improve management of KD patients. Further studies are crucial to clarify the epidemiologic characteristics of KD in Mongolia.

  19. Decreased levels of inflammatory cytokines in immunoglobulin-resistant Kawasaki disease after plasma exchange.

    PubMed

    Fujimaru, Takuya; Ito, Shuichi; Masuda, Hiroshi; Oana, Shinji; Kamei, Koichi; Ishiguro, Akira; Kato, Hitoshi; Abe, Jun

    2014-12-01

    The pathogenesis of coronary artery aneurysm (CAA) formation in Kawasaki disease (KD) remains unknown. However, inflammatory cytokines are thought to play an important role in KD. Patients with intravenous immunoglobulin (IVIG)-resistant KD are more likely to develop CAA. For such refractory patients, steroids and emerging infliximab (IFX) are used; however, further verification is required for their efficacy and safety. Plasma exchange (PE), which removes various inflammatory cytokines, has been used in Japan for over 15 years to prevent CAA in IVIG-resistant KD patients. The sequential change in inflammatory cytokines during the time course of PE has yet to be investigated. In this study, we measured plasma levels of 13 cytokines in nine children with IVIG-resistant KD before the start of PE (day 0: D0), as well as at 1 or 2 days (D1/2), and 4 or 5 days (D4/5) after starting PE. The median age of onset was 8 months (range: 3-53 months). Before PE, patients were treated with IVIG (median dose: 4 g/kg, range: 3-4 g/kg). The median starting period of PE was 8 days after the onset of fever (range: 6-21 days), while its duration was 3 days (range: 2-5 days). Among the 13 cytokines, interleukin-6, tumor necrosis factor-α, tumor necrosis factor receptor I (TNFR1), TNFR2, granulocyte colony-stimulating factor, and IL-17 were significantly lower at D4/5 compared with D0 and/or D1/2, reflecting the potential central efficacy of PE. While three patients developed moderate CAA, their condition regressed within 1 year. The removal of inflammatory cytokines could be the central efficacy of PE against refractory KD.

  20. Tissue Doppler Imaging as a Predictor of Immunoglobulin Resistance in Kawasaki Disease.

    PubMed

    Phadke, Daniel; Patel, Sonali S; Dominguez, Samuel R; Heizer, Heather; Anderson, Marsha S; Glode, Mary P; Jone, Pei-Ni

    2015-12-01

    Kawasaki disease (KD) is characterized by myocarditis and left ventricular dysfunction during the acute phase of the illness. Despite treatment with intravenous immunoglobulin (IVIG), a significant number of patients are IVIG resistant. We evaluated KD patients in the acute phase of illness using tissue Doppler imaging (TDI) to assess whether myocardial dysfunction may predict IVIG resistance. All patients with acute KD presenting to Children's Hospital Colorado from February 2007 through March 2014 were included in this study and underwent echocardiograms with TDI evaluation at diagnosis. Patients were divided into two groups: IVIG resistant and IVIG responder. Group differences were assessed using Wilcoxon-Mann-Whitney and Chi-square testing. Receiver operating characteristic (ROC) curve analysis was utilized to determine threshold values of TDI measurements associated with IVIG resistance. Fifty-one age-matched IVIG resistant patients were compared to 51 IVIG responder patients [median age, IQR 44.57 (20.13-77.07) vs. 33.49 (17.30-62.89) months, p < 0.44]. There were significant differences in the septal and mitral early diastolic velocities (E') (p < 0.001 and p < 0.01), respectively. ROC analysis demonstrated that tricuspid E' <0.15 cm/s, septal E' <0.12 cm/s, and mitral E' <0.16 cm/s were good predictors of IVIG unresponsiveness (AUC = 0.66, 0.66, and 0.70, respectively). There were no differences between the systolic velocities and late diastolic velocities (A'). IVIG resistant KD patients present with significantly greater diastolic dysfunction compared to responders in patients with KD. TDI may be a useful tool to differentiate KD patients at higher risk of IVIG resistance.

  1. Involvement of innate and adaptive immunity in a murine model of coronary arteritis mimicking Kawasaki disease.

    PubMed

    Schulte, Danica J; Yilmaz, Atilla; Shimada, Kenichi; Fishbein, Michael C; Lowe, Emily L; Chen, Shuang; Wong, Michelle; Doherty, Terence M; Lehman, Thomas; Crother, Timothy R; Sorrentino, Rosalinda; Arditi, Moshe

    2009-10-15

    Kawasaki disease (KD) is the most common cause of acquired cardiac disease and acute vasculitis in children in the developed world. Injection of a cell wall extract isolated from Lactobacillus casei (LCCWE) into mice causes a focal coronary arteritis that histopathologically mimics the coronary lesions observed in KD patients. In this study we used this model to investigate the participation of T cells, B cells, and dendritic cells (DC) in the development of coronary arteritis. RAG1(-/-), B cell(null), and wild-type (WT) mice were injected with a single dose of LCCWE (500 microg/mouse i.p.). None of the RAG1(-/-) mice developed coronary arteritis, whereas 70% of WT and 100% of B cell(null) mice developed coronary lesions, indicating that T cells were required for lesion formation. When splenocytes isolated from LCCWE-treated mice were restimulated with LCCWE, we observed significant IFN-gamma secretion in WT but not in RAG1(-/-) mice. Immunohistochemical staining showed F4/80(+) macrophages, activated MIDC-8(+) myeloid DCs (mDC), plasmacytoid DCs, and colocalization of CD3(+) T cells with mDCs in coronary artery lesions, suggesting an Ag-driven process. T cells but not B cells are required for LCCWE-induced coronary arteritis. Similar to human lesions, the coronary lesions contain macrophages, activated mDCs, and plaslmacytoid DCs all in close proximity to T cells, further strengthening the relevance of this mouse model to the immunopathology of coronary disease in KD. These studies are consistent with the interpretation that macrophages and DCs may collaborate with T cells in the pathological mechanisms of coronary arteritis.

  2. Steroid pulse therapy for Kawasaki disease unresponsive to additional immunoglobulin therapy

    PubMed Central

    Miura, Masaru; Tamame, Takuya; Naganuma, Takashi; Chinen, Shino; Matsuoka, Megumi; Ohki, Hirotaka

    2011-01-01

    BACKGROUND: The optimal management of Kawasaki disease (KD) unresponsive to intravenous immunoglobulin (IVIG) therapy remains unclear. OBJECTIVE: To prospectively evaluate the efficacy and safety of intravenous methylprednisolone pulse (IVMP) therapy in KD cases unresponsive to additional IVIG. METHODS: KD patients who initially received IVIG (2 g/kg/24 h) and acetylsalicylic acid within nine days after disease onset were studied. Patients who did not respond received additional IVIG (2 g/kg/24 h), and those who still did not respond were given IVMP (30 mg/kg/day) for three days, followed by oral prednisolone. The response to treatment, echocardiographic findings and adverse effects were evaluated. RESULTS: Among 412 KD cases, 74 (18.0%) were treated with additional IVIG; 21 (28.4%) of the latter cases subsequently received IVMP followed by prednisolone. All cases became afebrile soon after IVMP infusion and did not have a high-grade fever during treatment with prednisolone for two to six weeks. Four weeks after disease onset, coronary artery lesions (CAL) were diagnosed according to the Japanese Ministry of Health and Welfare or the American Heart Association criteria in two of the 21 cases treated with IVMP plus prednisolone; among all 412 cases, three (0.7%) and eight (1.9%) had CAL according to each criteria, respectively. All CAL regressed completely one year after disease onset. Adverse effects of IVMP, such as hypothermia and sinus bradycardia, resolved spontaneously. CONCLUSIONS: In KD patients unresponsive to additional IVIG, IVMP promptly induced defervescence, and subsequent oral prednisolone suppressed recurrence of fever. IVMP followed by prednisolone therapy may prevent CAL, without severe adverse effects. PMID:23024586

  3. Transforming Growth Factor-β Signaling Pathway in Patients with Kawasaki Disease

    PubMed Central

    Shimizu, Chisato; Jain, Sonia; Lin, Kevin O.; Molkara, Delaram; Frazer, Jeffrey R.; Sun, Shelly; Baker, Annette L.; Newburger, Jane W.; Rowley, Anne H.; Shulman, Stanford T.; Davila, Sonia; Hibberd, Martin L.; Burgner, David; Breunis, Willemijn B.; Kuijpers, Taco W.; Wright, Victoria J.; Levin, Michael; Eleftherohorinou, Hariklia; Coin, Lachlan; Popper, Stephen J.; Relman, David A.; Fury, Wen; Lin, Calvin; Mellis, Scott; Tremoulet, Adriana H.; Burns, Jane C.

    2011-01-01

    Background Transforming growth factor (TGF)-β is a multifunctional peptide that is important in T-cell activation and cardiovascular remodeling, both of which are important features of Kawasaki disease (KD). We postulated that variation in TGF-β signaling might be important in KD susceptibility and disease outcome. Methods and Results We investigated genetic variation in 15 genes belonging to the TGF-β pathway in a total 771 KD subjects of mainly European descendent from the US, UK, Australia and the Netherlands. We analyzed transcript abundance patterns using microarray and RT-PCR for these same genes and measured TGF-β2 protein levels in plasma. Genetic variants in TGFB2, TGFBR2 and SMAD3 and their haplotypes were consistently and reproducibly associated with KD susceptibility, coronary artery aneurysm formation, aortic root dilatation, and intravenous immunoglobulin treatment response in different cohorts. A SMAD3 haplotype associated with KD susceptibility replicated in two independent cohorts and an intronic SNP in a separate haplotype block was also strongly associated (A/G, rs4776338) (p=0.000022, OR 1.50, 95% CI 1.25-1.81). Pathway analysis using all 15 genes further confirmed the importance of the TGF-β pathway in KD pathogenesis. Whole blood transcript abundance for these genes and TGF-β2 plasma protein levels changed dynamically over the course of the illness. Conclusions These studies suggest that genetic variation in the TGF-β pathway influences KD susceptibility, disease outcome, and response to therapy and that aortic root and coronary artery Z scores can be used for phenotype/genotype analyses. Analysis of transcript abundance and protein levels further support the importance of this pathway in KD pathogenesis. PMID:21127203

  4. Racial/Ethnic Differences in the Incidence of Kawasaki Syndrome among Children in Hawai‘i

    PubMed Central

    Christensen, Krista Y; Belay, Ermias D; Steiner, Claudia A; Effler, Paul V; Miyamura, Jill; Forbes, Susan; Schonberger, Lawrence B; Melish, Marian

    2010-01-01

    Objective To describe the occurrence of Kawasaki syndrome (KS) among different racial/ethnic groups in Hawai‘i. Methods Retrospective analysis of children <18 years of age, with a focus on children <5 years of age, living in Hawai‘i who were hospitalized with KS using the 1996–2006 Hawai‘i State Inpatient Data. Results Children <5 years of age accounted for 84% of the 528 patients <18 years of age with KS. The average annual incidence among this age group was 50.4 per 100,000 children <5 years of age, ranging from 45.5 to 56.5. Asian and Pacific Islander children accounted for 92% of the children <5 years of age with KS during the study period; the average annual incidence was 62.9 per 100,000. Within this group, Japanese children had the highest incidence (210.5), followed by Native Hawaiian children (86.9), other Asian children (84.9), and Chinese children (83.2). The incidence for white children (13.7) was lower than for these racial/ethnic groups. The median age of KS admission for children <5 years of age was 21 months overall, 24 months for Japanese children, 14.5 months for Native Hawaiian children and 26.5 months for white children. Conclusions The high average annual KS incidence for children <5 years of age in Hawai‘i compared to the rest of the United States population reflects an increased KS incidence among Asian and Pacific Islander children, especially Japanese children. The incidence for white children was slightly higher than or similar to that generally reported nationwide. PMID:20845285

  5. Detection of coronary artery stenosis in children with Kawasaki disease. Usefulness of pharmacologic stress sup 201 Tl myocardial tomography

    SciTech Connect

    Kondo, C.; Hiroe, M.; Nakanishi, T.; Takao, A. )

    1989-09-01

    This study determined the feasibility and accuracy of quantitative 201Tl myocardial single-photon emission computed tomography (SPECT) after dipyridamole infusion to detect coronary obstructive lesions in children with Kawasaki disease. 201Tl distribution after dipyridamole infusion was measured in 23 normal children, and with these normal values, quantitative analysis of SPECT was performed in 49 patients. Thirty-four patients had coronary stenosis 90% or greater on angiograms. Side effects resulting from systemic vasodilation were observed in about 70%. Angina pectoris and ischemic ST changes were observed only in patients with coronary stenosis. These symptoms disappeared after aminophylline infusion. Results of visual and quantitative analysis of SPECT were compared. SPECT data were shown on two-dimensional polar maps, and the extent and severity scores were calculated. The sensitivity of SPECT for detection of overall coronary stenosis was 91% (visual analysis) and 88% (quantitative analysis). The specificity of SPECT was 60% visually and 93% quantitatively. The sensitivity of quantitative analysis to detect individual coronary stenosis was similar to that of visual analysis. However, the specificity of visual analysis to detect individual coronary artery stenosis was significantly less than that of quantitative analysis. From these data, we conclude that quantitative analysis of myocardial SPECT after dipyridamole infusion is a safe and accurate diagnostic method for identifying coronary stenosis in children with Kawasaki disease.

  6. [Clinical features and course of Kawasaki disease in central Tunisia: a study about 14 cases collected over a period of three years (2000-2002)].

    PubMed

    Chemli, Jalel; Kchaou, Habib; Amri, Fethi; Belkadhi, Adel; Essoussi, Ahmed Sahloul; Gueddiche, Neji; Harbi, Abdelaziz

    2005-08-01

    To analyze the clinical features and course of Kawasaki disease in central Tunisia. We studied retrospectively 14 cases of children with Kawasaki disease collected in tunisian center during three years (2000-2002). The study is about 11 boys and 3 girls (sex - ratio: 3.6/1) aged from 6 months to 8 years (mean age : 4 years). Twelve patients had at least 5 diagnostic criteria of the illness, the two others had an incomplete form. We noted cardiac complications in seven patients treated belatedly, beyond 10 days of progression, because of atypical clinical presentations. All patients had all a middle caliber coronary aneurysm that was complicated by a thrombus in three cases, associated with pericarditis and minimal mitral insufficiency in a case and with a cardiac rhythm disturbance (block of branch) in another case. Besides the cardiac complications, several other visceral manifestation could be noted: joint symptoms in five cases, GI tract symptomes in three cases, neuro-meningeal in two cases and urinary trad symptomes in two other cases. Specific treatment (aspirin with antiinflammatory dose and intravenous immune globulin (IVIG)) has been instituted in all patients. The course was favorable for 12 patients with fast regression of clinical manifestation and progressive normalisation of biologic values. Two patients did not respond to the initial IVIG treatment, and had to recense received an additional course of IGIV but without clinical nor biological improvement. These two patients were treated with corticosteroids. Cardiac lesions disappeared completely in all patients even for those with thrombosis and in patients with IVIG-resistant Kawasaki disease. Only one patient had kept neurologic sequellae: aphasia, bevavioral problemes and partial epilepsy. Kawasaki disease is not rare in our region. Incomplete or atypical presentations are frequent and are a source of diagnostic delay. Coronary aneurysm due to the delay of treatment often regresses even in patients

  7. Detection of Norovirus GII.17 Kawasaki 2014 in Shellfish, Marine Water and Underwater Sewage Discharges in Italy.

    PubMed

    La Rosa, G; Della Libera, S; Iaconelli, M; Proroga, Y T R; De Medici, D; Martella, V; Suffredini, E

    2017-03-03

    Norovirus (NoV) is a major cause of non-bacterial acute gastroenteritis worldwide, and the variants of genotype GII.4 are currently the predominant human strains. Recently, a novel variant of NoV GII.17 (GII.P17_GII.17 NoV), termed Kawasaki 2014, has been reported as the cause of gastroenteritis outbreaks in Asia, replacing the pandemic strain GII.4 Sydney 2012. The GII.17 Kawasaki 2014 variant has also been reported sporadically in patients with gastroenteritis outside of Asia, including Italy. In this study, 384 shellfish samples were subjected to screening for human NoVs using real-time PCR and 259 (67.4%) tested positive for Genogroup II (GII) NoV. Of these, 52 samples, selected as representative of different areas and sampling dates, were further amplified by conventional PCR targeting the capsid gene, using broad-range primers. Forty shellfish samples were characterized by amplicon sequencing as GII.4 (n = 29), GII.2 (n = 4), GII.6 (n = 2), GII.12 (n = 2), and GII.17 (n = 3). Sixty-eight water samples (39 seawater samples from the corresponding shellfish production areas and 29 water samples from nearby underwater sewage discharge points) were also tested using the above broad-range assay: eight NoV-positive samples were characterized as GII.1 (n = 3), GII.2 (n = 1), GII.4 (n = 2), and GII.6 (n = 2). Based on full genome sequences available in public databases, a novel RT-PCR nested assay specific for GII.17 NoVs was designed and used to re-test the characterized shellfish (40) and water (8) samples. In this second screening, the RNA of GII.17 NoV was identified in 17 additional shellfish samples and in one water sample. Upon phylogenetic analysis, these GII.17 NoV isolates were closely related to the novel GII.17 Kawasaki 2014. Interestingly, our findings chronologically matched the emergence of the Kawasaki 2014 variant in the Italian population (early 2015), as reported by hospital-based NoV surveillance. These results, showing GII.17 No

  8. Infliximab for intensification of primary therapy for Kawasaki disease: a phase 3 randomised, double-blind, placebo-controlled trial.

    PubMed

    Tremoulet, Adriana H; Jain, Sonia; Jaggi, Preeti; Jimenez-Fernandez, Susan; Pancheri, Joan M; Sun, Xiaoying; Kanegaye, John T; Kovalchin, John P; Printz, Beth F; Ramilo, Octavio; Burns, Jane C

    2014-05-17

    Kawasaki disease, the most common cause of acquired heart disease in developed countries, is a self-limited vasculitis that is treated with high doses of intravenous immunoglobulin. Resistance to intravenous immunoglobulin in Kawasaki disease increases the risk of coronary artery aneurysms. We assessed whether the addition of infliximab to standard therapy (intravenous immunoglobulin and aspirin) in acute Kawasaki disease reduces the rate of treatment resistance. We undertook a phase 3, randomised, double-blind, placebo-controlled trial in two children's hospitals in the USA to assess the addition of infliximab (5 mg per kg) to standard therapy. Eligible participants were children aged 4 weeks-17 years who had a fever (temperature ≥38·0°C) for 3-10 days and met American Heart Association criteria for Kawasaki disease. Participants were randomly allocated in 1:1 ratio to two treatment groups: infliximab 5 mg/kg at 1 mg/mL intravenously over 2 h or placebo (normal saline 5 mL/kg, administered intravenously). Randomisation was based on a randomly permuted block design (block sizes 2 and 4), stratified by age, sex, and centre. Patients, treating physicians and staff, study team members, and echocardiographers were all masked to treament assignment. The primary outcome was the difference between the groups in treatment resistance defined as a temperature of 38·0°C or higher at 36 h to 7 days after completion of the infusion of intravenous immunoglobulin. Analysis was by intention to treat. This trial is registered with ClinicalTrials.gov, NCT00760435. 196 patients were enrolled and randomised: 98 to the infliximab group and 98 to placebo. One patient in the placebo group was withdrawn from the study because of hypotension before receiving treatment. Treatment resistance rate did not differ significantly (11 [11·2%] for infliximab and 11 [11·3%] for placebo; p=0·81). Compared with the placebo group, participants given infliximab had fewer days of fever (median 1

  9. Percepcion de los profesores universitarios acerca del concepto cultura cientifica y de sus implicaciones en el nuevo bachillerato del Recinto de Rio Piedras de la Universidad de Puerto Rico

    NASA Astrophysics Data System (ADS)

    Ramos Pastrana, Nilsa

    El Senado Academico del Recinto de Rio Piedras de la Universidad de Puerto Rico aprobo en el ano academico 2005-2006 la Certificacion 46, que contiene los lineamientos de un nuevo bachillerato. Este nuevo bachillerato introdujo cambios significativos en el curriculo tradicional. Entre ellos se encuentra la reduccion del componente de educacion general y el de Ciencias Biologicas en particular. La reduccion de creditos en el componente de Ciencias Biologicas ha obligado a reevaluar el concepto de cultura cientifica que desarrollan esos cursos. El proposito del estudio consistio en auscultar las percepciones de los profesores de las Facultades de Administracion de Empresas, Humanidades, Ciencias Sociales, Ciencias Naturales, Educacion y Estudios Generales del Recinto de Rio Piedras de la Universidad de Puerto Rico en torno al concepto de cultura cientifica, los contenidos disciplinares del curso de Ciencias Biologicas y la reduccion de creditos en el nuevo bachillerato. Las preguntas que guiaron la investigacion fueron: ¿cuales son las percepciones que tienen los profesores de las Facultades de Administracion de Empresas, Ciencias Sociales, Estudios Generales, Ciencias Naturales, Humanidades y Educacion, en torno al concepto de cultura cientifica y los contenidos disciplinares del curso de Ciencias Biologicas? ¿cuales son las percepciones que tienen los profesores de Ciencias Biologicas en torno al concepto cultura cientifica y los contenidos disciplinares del curso de Ciencias Biologicas? ¿existen diferencias significativas por facultad, genero, experiencia, rango y nombramiento en las percepciones que tienen los profesores del Recinto de Rio Piedras de la Universidad de Puerto Rico sobre los elementos que caracterizan la cultura cientifica y los contenidos biologicos que deben tener los egresados del Recinto? ¿que implicaciones curriculares tienen estos testimonios en el desarrollo del concepto de cultura cientifica en el nuevo bachillerato? Para realizar la

  10. Male-specific association of the FCGR2A His167Arg polymorphism with Kawasaki disease

    PubMed Central

    Kwon, Young-Chang; Kim, Jae-Jung; Yun, Sin Weon; Yu, Jeong Jin; Yoon, Kyung Lim; Lee, Kyung-Yil; Kil, Hong-Ryang; Kim, Gi Beom; Han, Myung-Ki; Song, Min Seob; Lee, Hyoung Doo; Ha, Kee-Soo; Sohn, Sejung; Ebata, Ryota; Hamada, Hiromichi; Suzuki, Hiroyuki; Ito, Kaoru; Onouchi, Yoshihiro

    2017-01-01

    Kawasaki disease (KD) is an acute systemic vasculitis that can potentially cause coronary artery aneurysms in some children. KD occurs approximately 1.5 times more frequently in males than in females. To identify sex-specific genetic variants that are involved in KD pathogenesis in children, we performed a sex-stratified genome-wide association study (GWAS), using the Illumina HumanOmni1-Quad BeadChip data (249 cases and 1,000 controls) and a replication study for the 34 sex-specific candidate SNPs in an independent sample set (671 cases and 3,553 controls). Male-specific associations were detected in three common variants: rs1801274 in FCGR2A [odds ratio (OR) = 1.40, P = 9.31 × 10−5], rs12516652 in SEMA6A (OR = 1.87, P = 3.12 × 10−4), and rs5771303 near IL17REL (OR = 1.57, P = 2.53 × 10−5). The male-specific association of FCGR2A, but not SEMA6A and IL17REL, was also replicated in a Japanese population (OR = 1.74, P = 1.04 × 10−4 in males vs. OR = 1.22, P = 0.191 in females). In a meta-analysis with 1,461 cases and 5,302 controls, a very strong association of KD with the nonsynonymous SNP rs1801274 (p.His167Arg, previously assigned as p.His131Arg) in FCGR2A was confirmed in males (OR = 1.48, P = 1.43 × 10−7), but not in the females (OR = 1.17, P = 0.055). The present study demonstrates that p.His167Arg, a KD-associated FCGR2A variant, acts as a susceptibility gene in males only. Overall, the gender differences associated with FCGR2A in KD provide a new insight into KD susceptibility. PMID:28886140

  11. Cardiopulmonary Function, Exercise Capacity, and Echocardiography Finding of Pediatric Patients With Kawasaki Disease: An Observational Study.

    PubMed

    Tuan, Sheng-Hui; Li, Min-Hui; Hsu, Miao-Ju; Tsai, Yun-Jeng; Chen, Yin-Han; Liao, Tin-Yun; Lin, Ko-Long

    2016-01-01

    Coronary artery (CA) abnormalities influence exercise capacity (EC) of patients with Kawasaki disease (KD), and Z-score of CA is a well established method for detecting CA aneurysm. We studied the influence of KD on cardiopulmonary function and EC; meanwhile we analyzed echocardiographic findings of KD patients. We also assessed the correlation between CA Z-score and EC of KD patients to see if CA Z-score of KD patients could reflect EC during exercise.Sixty-three KD patients were recruited as KD group 1 from children (aged 5-18 y) who received transthoracic echocardiographic examinations and symptom-limited treadmill exercise test for regular follow-up of KD from January 2010 to October 2014 in 1 medical center. We then divided KD group 1 into KD group 2 (<5 y, n = 12) and KD group 3 (≥5 y, n = 51) according to time interval between KD onset to when patients received test. Control groups were matched by age, sex, and body mass index. Max-Z of CA was defined as the maximal Z-score of the proximal LCA or RCA by Dalliarre equation or Fuse calculator.All routine parameters measured during standard exercise test were similar between KD and control groups, except that peak rate pressure products (PRPPs) in KD group 1 to 3 were all lower than corresponding control groups significantly (P = 0.010, 0.020, and 0.049, respectively). PRPPs correlated with Max-Z of CA by both equations modest inversely (by Dallaire, P = 0.017, Spearman rho = -0.301; by Fuse, P = 0.014, Spearman rho = -0.309).Our study recruited larger number of KD patients and provided a newer data of EC of KD patients. Our finding suggests that after acute stage of KD, patients could maintain normal cardiorespiratory fitness. Therefore, we believe that it is important to promote cardiovascular health to KD patients and KD patients should exercise as normal peers. However, since KD patients might still have compromised coronary perfusion during exercise, it remains crucial to

  12. Three Linked Vasculopathic Processes Characterize Kawasaki Disease: A Light and Transmission Electron Microscopic Study

    PubMed Central

    Orenstein, Jan Marc; Shulman, Stanford T.; Fox, Linda M.; Baker, Susan C.; Takahashi, Masato; Bhatti, Tricia R.; Russo, Pierre A.; Mierau, Gary W.; de Chadarévian, Jean Pierre; Perlman, Elizabeth J.; Trevenen, Cynthia; Rotta, Alexandre T.; Kalelkar, Mitra B.; Rowley, Anne H.

    2012-01-01

    Background Kawasaki disease is recognized as the most common cause of acquired heart disease in children in the developed world. Clinical, epidemiologic, and pathologic evidence supports an infectious agent, likely entering through the lung. Pathologic studies proposing an acute coronary arteritis followed by healing fail to account for the complex vasculopathy and clinical course. Methodology/Principal Findings Specimens from 32 autopsies, 8 cardiac transplants, and an excised coronary aneurysm were studied by light (n=41) and transmission electron microscopy (n=7). Three characteristic vasculopathic processes were identified in coronary (CA) and non-coronary arteries: acute self-limited necrotizing arteritis (NA), subacute/chronic (SA/C) vasculitis, and luminal myofibroblastic proliferation (LMP). NA is a synchronous neutrophilic process of the endothelium, beginning and ending within the first two weeks of fever onset, and progressively destroying the wall into the adventitia causing saccular aneurysms, which can thrombose or rupture. SA/C vasculitis is an asynchronous process that can commence within the first two weeks onward, starting in the adventitia/perivascular tissue and variably inflaming/damaging the wall during progression to the lumen. Besides fusiform and saccular aneurysms that can thrombose, SA/C vasculitis likely causes the transition of medial and adventitial smooth muscle cells (SMC) into classic myofibroblasts, which combined with their matrix products and inflammation create progressive stenosing luminal lesions (SA/C-LMP). Remote LMP apparently results from circulating factors. Veins, pulmonary arteries, and aorta can develop subclinical SA/C vasculitis and SA/C-LMP, but not NA. The earliest death (day 10) had both CA SA/C vasculitis and SA/C-LMP, and an “eosinophilic-type” myocarditis. Conclusions/Significance NA is the only self-limiting process of the three, is responsible for the earliest morbidity/mortality, and is consistent with

  13. Kawasaki disease in Sicily: clinical description and markers of disease severity.

    PubMed

    Maggio, Maria Cristina; Corsello, Giovanni; Prinzi, Eugenia; Cimaz, Rolando

    2016-11-02

    Kawasaki disease (KD) is an acute systemic vasculitis of small and middle size arteries; 15-25 % of untreated patients and 5 % of patients treated with intravenous immunoglobulin (IVIG) develop coronary artery lesions (CAL). Many studies tried to find the most effective treatment in the management of resistant KD and to select the risk factors for CAL. Our data are assessed on children from west Sicily, characterized by a genetic heterogeneity. We studied the clinical data of 70 KD Sicilian children (36 males: 51 %; 34 females: 49 %), analysed retrospectively, including: demographic and laboratory parameters; echocardiographic findings at diagnosis, at 2, 6 and 8 weeks, and at 1 year after the onset of the illness. Forty-seven had Typical KD, three Atypical KD and twenty Incomplete KD. Age at the disease onset ranged from 0.1 to 8.9 years. IVIG were administered 5 ± 2 days after the fever started. Defervescence occurred 39 ± 26 hours after the first IVIG infusion. Fifty-six patients (80 %) received 1 dose of IVIG (responders); 14 patients (20 %) had a resistant KD, with persistent fever after the first IVIG dose (non responders). Ten (14 %) non responders responded to the second dose, 4 (5 %) responded to three doses; one needed treatment with high doses of steroids and Infliximab. Cardiac involvement was documented in twenty-two cases (eighteen with transient dilatation/ectasia, fifteen with aneurysms). Pericardial effusion, documented in eleven, was associated with coronaritis and aneurysms, and was present earlier than coronary involvement in seven. Hypoalbuminemia, D-dimer pre-IVIG, gamma-GT pre-IVIG showed a statistically significant direct correlation with IVIG doses, highlighting the role of these parameters as predictor markers of refractory disease. The persistence of elevated CRP, AST, ALT levels, a persistent hyponatremia and hypoalbuminemia after IVIG therapy, also had a statistical significant correlation with IVIG doses. Non responders

  14. Epidemiology, Clinical Presentation, and Outcomes of Kawasaki Disease Among Hospitalized Children in an Inner City Hospital Before and After Publication of the American Academy of Pediatrics/American Heart Association Guidelines for Treatment of Kawasaki Disease: An 11-Year Period.

    PubMed

    Sehgal, Swati; Chen, Xinguang; Ang, Jocelyn Y

    2015-11-01

    The effect of 2004 Kawasaki disease (KD) guidelines on diagnosis and outcome of KD is lacking. We studied the epidemiology of KD in our region and compared the incidence, presentation, and outcome of KD before and after publication of the guidelines. A retrospective chart review was conducted for patients admitted with a diagnosis of KD. Demographics, laboratory data, and clinical data were collected. Comparison was made between 2 groups: prepublication (2000-2004) and postpublication (2005-2009) of guidelines. A total of 312 children were included; 64% were African American, 23% White, and 2% Asian; 61% were boys; 79% were complete KD, and 66% were in winter/spring. There was a significant increase in KD cases over the 11 years. There was no significant difference in clinical findings and outcome between the 2 groups. KD admissions in our region significantly increased during the postpublication period. There was no difference in clinical presentation, laboratory findings, or outcome between the 2 groups.

  15. Early combined treatment with steroid and immunoglobulin is effective for serious Kawasaki disease complicated by myocarditis and encephalopathy.

    PubMed

    Suga, Kenichi; Inoue, Miki; Ono, Akemi; Terada, Tomomasa; Kawahito, Masami; Mori, Kazuhiro

    2016-01-01

    Severe-type Kawasaki disease (KD) complicated by serious myocarditis and encephalopathy can be successfully treated without abnormality of the coronary arteries by steroid pulse treatment and intravenous immunoglobulin (IVIg). A 4-year-old Japanese girl was diagnosed with KD due to a 6-day history of fever, rash, flushed lips, conjunctival hyperemia, palmar edema, and cervical lymphadenopathy. The day after initiation of IVIg and aspirin, cardiac gallop rhythm was identified. Cardiac ultrasonography revealed severe left ventricular dysfunction. Disturbance of consciousness, hallucinations, and slurred speech were also observed. Magnetic resonance imaging showed no abnormalities, but electroencephalography revealed high-voltage slow waves. Despite this serious disease, cardiac function and neurological abnormalities showed complete recovery without dilatation of the coronary arteries by steroid pulse treatment and additional IVIg. Follow-up at 15 months revealed no abnormality of the coronary arteries. In conclusion, we suggest that early combined treatment with steroid and IVIg is effective for serious KD complicated by myocarditis and encephalopathy.

  16. Human coronavirus NL63 is not detected in the respiratory tracts of children with acute Kawasaki disease.

    PubMed

    Shimizu, Chisato; Shike, Hiroko; Baker, Susan C; Garcia, Francesca; van der Hoek, Lia; Kuijpers, Taco W; Reed, Sharon L; Rowley, Anne H; Shulman, Stanford T; Talbot, Helen K B; Williams, John V; Burns, Jane C

    2005-11-15

    Kawasaki disease (KD) is a self-limited, systemic vasculitis of children for which an infectious trigger is suspected. Recently, an association between KD and human coronavirus (HCoV)-New Haven (NH) was reported, on the basis of polymerase chain reaction (PCR) with primers that also amplified HCoV-NL63. We investigated the possible association between these HCoVs in the respiratory tract and KD by reverse-transcriptase (RT) PCR and viral culture in a geographically and ethnically diverse population. Only 1 (2%) of 48 patients with acute KD was positive by RT-PCR for HCoV-NL63/NH in a nasopharyngeal swab. These data do not support an association between these HCoVs and KD.

  17. Ulinastatin, a urinary trypsin inhibitor, for the initial treatment of patients with Kawasaki disease: a retrospective study.

    PubMed

    Kanai, Takashi; Ishiwata, Takahiro; Kobayashi, Tohru; Sato, Hiroki; Takizawa, Mari; Kawamura, Yoichi; Tsujimoto, Hiroshi; Nakatani, Keigo; Ishibashi, Naoko; Nishiyama, Mitsunori; Hatai, Yoshiho; Asano, Yuh; Kobayashi, Tomio; Takeshita, Seiichiro; Nonoyama, Shigeaki

    2011-12-20

    Markedly activated neutrophils or higher plasma levels of neutrophil elastase are involved in the poor response to intravenous immunoglobulin (IVIG) and the formation of coronary artery lesions (CAL) in patients with acute Kawasaki disease. We hypothesized that ulinastatin (UTI), by both direct and indirect suppression of neutrophils, would reduce the occurrence of CAL. We retrospectively analyzed the clinical records of patients with Kawasaki disease between 1998 and 2009. Three hundred sixty-nine patients were treated with a combination of UTI, aspirin, and IVIG as an initial treatment (UTI group), and 1178 were treated with a conventional initial treatment, and IVIG with aspirin (control group). The baseline characteristics did not demonstrate notable differences between the two groups. The occurrence of CAL was significantly lower in the UTI group than in the control group (3% versus 7%; crude odds ratio [OR], 0.46; 95% confidence interval [CI], 0.25-0.86; P=0.01). The OR adjusted for sex, Gunma score (the predictive score for IVIG unresponsiveness), and dosage of initial IVIG (1 or 2 g/kg) was 0.32 (95% CI, 0.17-0.60; P<0.001). In addition, most CAL occurred in patients requiring additional rescue treatment and the proportion of those patients was significantly lower in the UTI group than in the control group (13% versus 22%; crude OR, 0.52; 95% CI, 0.38-0.73; P<0.001). The adjusted OR was 0.30 (95% CI, 0.20-0.44; P<0.001). UTI was associated with fewer patients requiring additional rescue treatment and reduction of CAL in this retrospective study.

  18. The Γ-Limit of the Two-Dimensional Ohta-Kawasaki Energy. Droplet Arrangement via the Renormalized Energy

    NASA Astrophysics Data System (ADS)

    Goldman, Dorian; Muratov, Cyrill B.; Serfaty, Sylvia

    2014-05-01

    This is the second in a series of papers in which we derive a Γ-expansion for the two-dimensional non-local Ginzburg-Landau energy with Coulomb repulsion known as the Ohta-Kawasaki model in connection with diblock copolymer systems. In this model, two phases appear, which interact via a nonlocal Coulomb type energy. Here we focus on the sharp interface version of this energy in the regime where one of the phases has very small volume fraction, thus creating small "droplets" of the minority phase in a "sea" of the majority phase. In our previous paper, we computed the Γ-limit of the leading order energy, which yields the averaged behavior for almost minimizers, namely that the density of droplets should be uniform. Here we go to the next order and derive a next order Γ-limit energy, which is exactly the Coulombian renormalized energy obtained by Sandier and Serfaty as a limiting interaction energy for vortices in the magnetic Ginzburg-Landau model. The derivation is based on the abstract scheme of Sandier-Serfaty that serves to obtain lower bounds for 2-scale energies and express them through some probabilities on patterns via the multiparameter ergodic theorem. Thus, without appealing to the Euler-Lagrange equation, we establish for all configurations which have "almost minimal energy" the asymptotic roundness and radius of the droplets, and the fact that they asymptotically shrink to points whose arrangement minimizes the renormalized energy in some averaged sense. Via a kind of Γ-equivalence, the obtained results also yield an expansion of the minimal energy and a characterization of the zero super-level sets of the minimizers for the original Ohta-Kawasaki energy. This leads to the expectation of seeing triangular lattices of droplets as energy minimizers.

  19. A 2-month-old boy with hemolytic anemia and reticulocytopenia following intravenous immunoglobulin therapy for Kawasaki disease: a case report and literature review

    PubMed Central

    Kim, Na Yeon; Kim, Joon Hwan; Park, Jin Suk; Kim, Soo Hyun; Cho, Yeon Kyung; Cha, Dong Hyun; Kim, Ki Eun; Kang, Myung Suh; Lim, Kyung Ah

    2016-01-01

    Herein, we report a rare case of hemolytic anemia with reticulocytopenia following intravenous immunoglobulin therapy in a young infant treated for Kawasaki disease. A 2-month-old boy presented with fever lasting 3 days, conjunctival injection, strawberry tongue, erythematous edema of the hands, and macular rash, symptoms and signs suggestive of incomplete Kawasaki disease. His fever resolved 8 days after treatment with aspirin and high dose infusion of intravenous immunoglobulin. The hemoglobin and hematocrit decreased from 9.7 g/dL and 27.1% to 7.4 g/dL and 21.3%, respectively. The patient had normocytic hypochromic anemia with anisocytosis, poikilocytosis, immature neutrophils, and nucleated red blood cells. The direct antiglobulin test result was positive, and the reticulocyte count was 1.39%. The patient had an uneventful recovery. However, reticulocytopenia persisted 1 month after discharge. PMID:28018448

  20. Pharmacokinetic and Tolerability Assessment of a Pediatric Oral Formulation of Pentoxifylline in Kawasaki Disease☆

    PubMed Central

    Best, Brookie M.; Burns, Jane C.; DeVincenzo, John; Phelps, Stephanie J.; Blumer, Jeffrey L.; Wilson, John T.; Capparelli, Edmund V.; Connor, James D.

    2003-01-01

    Background: In infants and children, treatment of Kawasaki disease (KD) with high-dose intravenous immunoglobulin (IVIG) and acetylsalicylic acid ([ASA] aspirin) diminishes inflammatory response and reduces the risk for coronary artery abnormalities. However, patients with high serum concentrations of tumor necrosis factor (TNF)-alpha, which is associated with vascular damage, may develop coronary artery lesions even with treatment. The hemorheologic agent pentoxifylline blocks the production of TNF-alpha and may be an appropriate adjunctive therapy to IVIG and ASA. Objective: The objective of this study was to assess the pharmacokinetic characteristics and tolerability of a new oral syrup formulation of pentoxifylline as an adjunct to IVIG and ASA in the treatment of KD in children. Methods: Hospitalized boys and girls aged 6 months to 5 years and who were diagnosed with KD within the first 10 days of illness were eligible. Patients were assigned to 1 of 4 pentoxifylline treatment groups, by dose level (dose levels 1, 2, 3, and 4: 10, 15, 20, and 25 mg/kg daily, respectively, divided into 3 doses). Six plasma samples collected at the time the first dose was administered, and 4 samples collected after administration of the last dose on study day 6, were assessed by high-performance liquid chromatography using noncompartmental and 1-compartment pharmacokinetic analyses for pentoxifylline and its active metabolite (M-1). TNF-alpha levels on days 1 and 6 were assessed using electroimmunoassay. Results: Fourteen boys and 10 girls were enrolled. The mean age, body weight, and illness day at study entry were 34.5 months, 13.8 kg, and 6, respectively. Pentoxifylline exhibited nonlinear kinetic characteristics, with median area under the plasma concentration–time curve from time 0 to infinity(AUC0–∞) values of 622, 3428, 8416, and 10,347 ng/mL · h for dose levels 1 to 4, respectively, on study day 1. Pentoxifylline noncompartmental oral clearance and volume of

  1. Evidence of correlation between TGFBR2 gene expression mediated by NF-kB signaling pathways and Kawasaki disease in children.

    PubMed

    Gao, Qinling; Yuan, Shuhua; Yuan, Dawei

    2017-09-15

    We explored the correlation between the TGFBR2 gene that is mediated by NF-kb signaling pathways and the pathogenesis of Kawasaki disease in children. In this study, 43 children with Kawasaki disease from April 2014 to January 2016 at our hospital were selected as the observation group, and 42 healthy children were selected as the control group. The mRNA expression levels of NF-kb gene and TGFBR2 gene in different groups were detected using fluorescence quantitative PCR. The protein expression levels of the NF-kb and TGFBR2 were detected using enzyme-linked immunosorbent assay (ELISA) in different groups. The expression levels of NF-kb and TGFBR2 in the observation group and the control group were detected using immunohistochemistry. Compared to the control group, the mRNA expression levels of NF-kb and TGFBR2 were 12.3 times and 27.5 times as high as those in the control group respectively and there were significant differences between the two groups (p<0.05). ELISA results showed that there were significant differences between the protein expression levels of NF-kb and TGFBR2 in the control group (0.87±0.12, 1.25±0.18) ug/l and those in the observation group (3.27±0.17, 8.16±0.22) ug/l (p<0.05). Western-blotting results showed that the expression levels of the NF-kB and TGFBR2 in children with Kawasaki disease were significantly higher than those in healthy subjects (p<0.05). Immunohistochemistry results showed that the positive cell rate of TGFBR2 (89.7%) was significantly higher in children with Kawasaki disease than that in healthy children (4.5%); there was significant difference between the two groups (p<0.05). The TGFBR2 may be involved in the pathogenesis of Kawasaki disease in children through NF-kB signaling pathways.

  2. Ambient Air Pollutant Exposures and Hospitalization for Kawasaki Disease in Taiwan: A Case-Crossover Study (2000–2010)

    PubMed Central

    Jung, Chau-Ren; Chen, Wei-Ting; Lin, Yu-Ting; Hwang, Bing-Fang

    2016-01-01

    Background: Kawasaki disease (KD) is an acute and multi-systemic vasculitis that occurs predominantly in infants and young children. Although the etiological agent of KD remains unclear, limited studies have reported that windborne environmental factors may trigger KD. Objectives: We conducted a time-stratified case-crossover study to assess the associations between air pollutants and KD in Taiwan. Methods: We identified children < 5 years old with a diagnosis of KD from the Longitudinal Health Insurance Database 2000 (LHID2000) between 2000 and 2010. We obtained data regarding carbon monoxide (CO), nitrogen dioxide (NO2), ozone (O3), particulate matter with aerodynamic diameter < 10 μm (PM10), and sulfate dioxide (SO2) from 70 monitoring stations and used inverse distance weighting to calculate average daily exposures for the residential postal code of each case. We performed conditional logistic regression to estimate associations between KD and each air pollutant according to interquartile range (IQR) increases and quartiles of exposure on the day of hospitalization versus 3–4 reference days during the same month for each case. Additionally, we estimated associations with single-day exposures lagged 1–2 days. Results: We identified 695 KD hospital admissions during the study period. An IQR increase (28.73 ppb) of O3 was positively associated with KD after adjusting for temperature, humidity, northward wind, and eastward wind [adjusted odds ratio = 1.21; 95% confidence interval (CI): 1.01, 1.44]. There were no significant associations between KD and CO, NO2, PM10, or SO2. The association with O3 was limited to exposure on the day of hospitalization and to exposure during the summer months (June–August). Conclusions: Our results provide new evidence that exposure to O3 may increase the risk of KD in children. However, further investigation is needed to confirm the association and identify a potential biological mechanism. Citation: Jung CR, Chen WT, Lin YT

  3. N-terminal pro-brain natriuretic peptide in acute Kawasaki disease correlates with coronary artery involvement.

    PubMed

    Adjagba, Philippe M; Desjardins, Laurent; Fournier, Anne; Spigelblatt, Linda; Montigny, Martine; Dahdah, Nagib

    2015-10-01

    We have lately documented the importance of N-terminal pro-brain natriuretic peptide in aiding the diagnosis of Kawasaki disease. We sought to investigate the potential value of N-terminal pro-brain natriuretic peptide pertaining to the prediction of coronary artery dilatation (Z-score>2.5) and/or of resistance to intravenous immunoglobulin therapy. We hypothesised that increased serum N-terminal pro-brain natriuretic peptide level correlates with increased coronary artery dilatation and/or resistance to intravenous immunoglobulin. We carried out a prospective study involving newly diagnosed patients treated with 2 g/kg intravenous immunoglobulin within 5-10 days of onset of fever. Echocardiography was performed in all patients at onset, then weekly for 3 weeks, then at month 2, and month 3. Coronary arteries were measured at each visit, and coronary artery Z-score was calculated. All the patients had N-terminal pro-brain natriuretic peptide serum level measured at onset, and the Z-score calculated. There were 109 patients enrolled at 6.58±2.82 days of fever, age 3.79±2.92 years. High N-terminal pro-brain natriuretic peptide level was associated with coronary artery dilatation at onset in 22.2 versus 5.6% for normal N-terminal pro-brain natriuretic peptide levels (odds ratio 4.8 [95% confidence interval 1.05-22.4]; p=0.031). This was predictive of cumulative coronary artery dilatation for the first 3 months (p=0.04-0.02), but not during convalescence at 2-3 months (odds ratio 1.28 [95% confidence interval 0.23-7.3]; p=non-significant). Elevated N-terminal pro-brain natriuretic peptide levels did not predict intravenous immunoglobulin resistance, 15.3 versus 13.5% (p=1). Elevated N-terminal pro-brain natriuretic peptide level correlates with acute coronary artery dilatation in treated Kawasaki disease, but not with intravenous immunoglobulin resistance.

  4. Kawasaki disease: a rare pediatric pathology in Mexico. Twenty cases report from the Hospital Infantil del Estado de Sonora.

    PubMed

    Sotelo, Norberto; González, Luis Antonio

    2007-01-01

    Kawasaki disease (KD) is an etiological illness that is relatively unknown and scarcely identified in Mexico; it affects children mainly aged 1-4 years, evolves with fever, vasculitis in diverse organs, and in the heart the disease mainly affects the coronary arteries. Our aim was to inform the clinical findings and evolution of 20 patients diagnosed with KD. We reviewed the patient clinical files retrospectively and descriptively to obtain information with regard to age, sex, clinical signs, laboratory and consultory results, echocardiography findings, complications, evolution during hospitalization, followup, and out-patient ambulatory consultations. Eighteen patients were male, two were female, six developed coronary damage, two aortic mitral-valve insufficiency, one pericardial shedding, and one, myocarditis. All patients received gamma globulin treatment with aspirin, and 16 were controlled during 6-8 months after the acute medical profile. The opportune clinical diagnostic it is fundamental to establish an early treatment with gammmaglobuline to avoid injuries in the arterial coronary level. This injury may cause eventualy ischemia or myocardial infarct

  5. Notch4 Signaling Pathway of Endothelial Progenitor Cells in a Kawasaki Disease Model Induced by Lactobacillus casei Cell Wall Extract.

    PubMed

    Wang, Huafeng; Xia, Yuanyuan; Fu, Songling; Wang, Wei; Xie, Chunhong; Zhang, Yiying; Gong, Fangqi

    2016-01-01

    The Notch4 signaling pathway of endothelial progenitor cells (EPCs) may play a crucial role in Kawasaki disease (KD). We investigated the proliferation, adhesion, migration, angiogenesis, and expression levels of Notch4, recombination signal-binding protein-Jκ (RBP-Jκ), P-selectin, and vascular cell adhesion molecule-1 (VCAM-1) of bone marrow (BM) EPCs in a KD model induced by Lactobacillus casei cell wall extract. The numbers of BM EPCs decreased significantly in the KD models. The Notch4 expression level on the EPC surface was higher in the KD models than in the controls. The proliferative, adhesive, migratory, and angiogenic properties, and double immunofluorescence-binding rate of BM EPCs were significantly impaired in the KD models. The levels of Notch4 and P-selectin mRNA were lower in the KD models than in the controls on day 3. The RBP-Jκ mRNA levels were lower in the KD models than in the controls on days 3 and 7. The levels of RBP-Jκ and vascular endothelial growth factor receptor-2 proteins decreased in the early stage. In conclusion, the BM EPC functions and bioactivities in the KD models were impaired, and the Notch4 signaling pathway is associated with KD. © 2016 S. Karger AG, Basel.

  6. Predicting the characteristics of the aetiological agent for Kawasaki disease from other paediatric infectious diseases in Japan.

    PubMed

    Nagao, Y; Urabe, C; Nakamura, H; Hatano, N

    2016-02-01

    Although Kawasaki disease (KD), which was first reported in the 1960s, is assumed to be infectious, its aetiological agent(s) remains unknown. We compared the geographical distribution of the force of infection and the super-annual periodicity of KD and seven other paediatric infectious diseases in Japan. The geographical distribution of the force of infection, which was estimated as the inverse of the mean patient age, was similar in KD and other paediatric viral infections. This similarity was due to the fact that the force of infection was determined largely by the total fertility rate. This finding suggests that KD shares a transmission route, i.e. sibling-to-sibling infection, with other paediatric infections. The super-annual periodicity, which is positively associated with the sum of an infectious disease's incubation period and infectious period, was much longer for KD and exanthema subitum than other paediatric infectious diseases. The virus for exanthema subitum is known to persist across the host's lifespan, which suggests that the aetiological agent for KD may also be capable of persistent infection. Taken together, these findings suggest that the aetiological agent for KD is transmitted through close contact and persists asymptomatically in most hosts.

  7. Epidemiologic Study on Kawasaki Disease in Korea, 2007-2014: Based on Health Insurance Review & Assessment Service Claims.

    PubMed

    Ha, Sangmi; Seo, Gi Hyeon; Kim, Kyu Yeun; Kim, Dong Soo

    2016-09-01

    The aim of this study is to assess the actual epidemiologic features of Kawasaki disease (KD) in Korea using the data from Health Insurance Review & Assessment Service (HIRA) claims from 2007 to 2014. We investigated HIRA claims of patients who had KD (International Classification of Diseases-10, M30.3) as a major diagnosis and were given intravenous immunoglobulin (IVIG) from 2007 to 2014. A total of 39,082 patients were reported during the period. The male-to-female ratio was 1.42 and the median age was 28 months. The incidence rates were 168.3 per 100,000 population aged 0 to 4 years in 2007, 159.1 in 2008, 167.3 in 2009, 190.4 in 2010, 188.2 in 2011, 190.2 in 2012, 210.4 in 2013 and 217.2 in 2014. These rates were much higher than those in the previous studies in Korea. KD occurred more often in early summer (May, June and July) and winter (December and January). The annual incidence rate of KD had been increasing every year, reaching 217.2 per 100,000 population aged 0 to 4 years in 2014. It is the second highest incidence rate of KD in the world after Japan.

  8. A molecular epidemiologic study of human adenovirus type 8 isolates causing epidemic keratoconjunctivitis in Kawasaki City, Japan in 2011.

    PubMed

    Fujimoto, Tsuguto; Matsushima, Yuki; Shimizu, Hideaki; Ishimaru, Yoko; Kano, Atsuko; Nakajima, Etsuko; Adhikary, Arun Kumar; Hanaoka, Nozomu; Okabe, Nobuhiko

    2012-01-01

    A local outbreak of epidemic keratoconjunctivitis (EKC) caused by human adenovirus type 8 (HAdV-D8) occurred in Kawasaki city, Japan in July-August 2011. Since the cases were sporadic in nature, the source of the infection could not be identified. The results of PCR analysis and the appearance of cytopathic effects in the samples indicated that 22 patients were positive for HAdV. The mean age of the patients (10 men and 12 women) was 64.3 ± 17.3 years (median, 68 years; range, 11-86 years). The sequences of hexon, which included hypervariable loop 1; the penton, which included RGD loops; and the fiber, which included the knob-coding regions, were identical in all the HAdV-positive cases. Phylogenetic analysis of the major capsid protein-encoding regions of HAdV confirmed that the isolates were HAdV-D8. Although the incidence of HAdV-D8 outbreaks has decreased in Japan since 1997, the results of our study imply that HAdV-D8 is still a causative agent for EKC outbreaks in Japan.

  9. Disruption of Endothelial Cell Homeostasis Plays a Key Role in the Early Pathogenesis of Coronary Artery Abnormalities in Kawasaki Disease

    PubMed Central

    Ueno, Kentaro; Ninomiya, Yumiko; Hazeki, Daisuke; Masuda, Kiminori; Nomura, Yuichi; Kawano, Yoshifumi

    2017-01-01

    Disruption of endothelial cell homeostasis may be associated with the pathogenesis of coronary artery abnormalities (CAA) in Kawasaki disease (KD). We sought to clarify the poorly understood pathogenic role of endothelial cell survival and death in KD vasculitis. Human umbilical vein endothelial cells (HUVECs) stimulated with sera from KD patients, compared with sera from patients with bacterial infections, exhibited significant increases in cytotoxicity, high mobility group box protein 1 (HMGB-1), and caspase-3/7 and a decrease in phosphorylated Akt/Akt (pAkt/Akt) ratios. HUVECs stimulated with sera from KD patients treated with immunoglobulin (IG) showed significantly decreased cytotoxicity, HMGB-1, and caspase-3/7 levels and increased pAkt/Akt ratios, as compared with results for untreated HUVECs (P < 0.001, P = 0.008, P = 0.040, and P < 0.001, respectively). In HUVECs stimulated with sera from KD patients, the increased cytotoxicity levels and the suppression of increased pAkt/Akt ratios after subsequent IG treatment were closely related to the development of CAA (P = 0.002 and P = 0.035). Our data reveal that shifting the balance toward cell death rather than survival appears to perturb endothelial cell homeostasis and is closely related to the development of CAA. The cytoprotective effects of IG treatment appear to ameliorate endothelial cell homeostasis. PMID:28255175

  10. On What Day of Illness Does the Dilatation of Coronary Arteries in Patients With Kawasaki Disease Begin?

    PubMed

    Fuse, Shigeto; Mori, Toshihiko; Kuroiwa, Yuki; Hirakawa, Satoshi

    2017-08-25

    In the present study we used echocardiography to investigate coronary artery diameter at the time of diagnosis of Kawasaki disease (KD), before the start of treatment.Methods and Results:Diameters of the right, left main, left anterior descending, and left circumflex coronary arteries were determined in 410 patients before KD treatment commenced. The maximum Z-score was considered to be the pretreatment, maximum coronary artery Z-score (preZmax). The cumulative probability of coronary arterial dilatation was analyzed using the Kaplan-Meier method. In the present study, 31 patients (7.6%) had a preZmax ≥3.0, 56 (13.7%) had a preZmax ≥2.5, and 96 (23.4%) had a preZmax ≥2.0. The cumulative probability of a preZmax ≥2.0 was >20% on Day 5 of illness, 40% on Day 7, and 70% on Day 10. The positive predictive value (PPV) of a preZmax of 2.0 was approximately 0.9 on Day 5 of illness. The present study demonstrates that the coronary arteries may dilate before Day 5 of illness, and that the rate of dilatation increases gradually until Day 10. Because preZmax 2.0 has high PPV after Day 5 of illness, it is a useful marker of coronary artery dilatation in the early phase of KD.

  11. Marked Acceleration of Atherosclerosis following Lactobacillus casei induced Coronary Arteritis in a Mouse Model of Kawasaki Disease

    PubMed Central

    Chen, Shuang; Lee, Young Ho; Crother, Timothy R.; Fishbein, Michael; Zhang, Wenxuan; Yilmaz, Atilla; Shimada, Kenichi; Schulte, Danica J; Lehman, Thomas J.A.; Shah, Prediman K.; Arditi, Moshe

    2012-01-01

    Objective To investigate if Lactobacillus casei cell wall extract (LCWE)-induced Kawasaki Disease (KD) accelerates atherosclerosis in hypercholesterolemic mice. Method and Resuslts Apoe−/− or Ldlr−/− mice were injected with LCWE (KD mice) or PBS, fed high fat diet for 8 weeks, and atherosclerotic lesions in aortic sinuses (AS), arch (AC) and whole aorta were assessed. KD mice had larger, more complex aortic lesions with abundant collagen, and both extracellular and intracellular lipid and foam cells, compared to lesions in control mice despite similar cholesterol levels. Both Apoe−/− KD and Ldlr−/− KD mice showed dramatic acceleration in atherosclerosis vs. controls, with increases in en face aortic atherosclerosis and plaque size in both the AS and AC plaques. Accelerated atherosclerosis was associated with increased circulating IL-12p40, IFN-γ, TNF-α, and increased macrophage, DC, and T cell recruitment in lesions. Furthermore, daily injections of the IL-1Ra, which inhibits LCWE induced KD vasculitis, prevented the acceleration of atherosclerosis. Conclusions Our results suggest an important pathophysiologic link between coronary arteritis/vasculitis in the KD mouse model and subsequent atherosclerotic acceleration, supporting the concept that a similar relation may also be present in KD patients. These results also suggest that KD in childhood may predispose to accelerated and early atherosclerosis as adults. PMID:22628430

  12. [Phosphatase and tensin homolog deleted on chromosome ten/phosphatidyl Inositol 3-kinase/vascular endothelial growth factor signaling pathway changes in the rabbit Kawasaki disease model].

    PubMed

    Niu, L; Fu, M Y; Tian, J; He, X H; Zhang, H N; Wang, Q W; Wang, Y; Li, C L; Wang, Z Z; An, X J

    2016-03-01

    To observe the changes of phosphatase and tensin homolog deleted on chromosome ten(PTEN)/ phosphatidyl Inositol 3-kinase(PI3K)/ vascular endothelial growth factor(VEGF)signaling pathway in a rabbit Kawasaki disease model. Model of Kawasaki disease was established in weanling Japanese big-eared rabbits with 10% bovine serum venous injection (2.5 ml/kg, 2 times, and 2 week's interval) through the ear. Twenty four rabbits were divided into 4 groups: control group (without injection of 10% bovine serum albumin, six rabbits); 1 day group (sacrificed a the second day after the establishment of Kawasaki disease models, six rabbits); 7 day group (sacrificed at the seventh day after establishment of Kawasaki disease model, six rabbits); 30 day group (sacrificed at the thirtieth day after establishment of Kawasaki disease model, six rabbits). Pathological analysis was performed on coronary artery tissue samples. The express of PTEN and PI3K were detected by immunohistochemistry. The levels of VEGF and CK were also examined with ELISA and white blood cells were counted. (1) Coronary artery of model groups was thinner, distorted and had enlarged lumen. (2) PTEN expression in 1 d group, 7 d group and 30 d group were 58.5 ± 12.9, 73.2±9.9 and 109.6 ± 24.4, respectively, significantly higher than in the control group (25.5 ± 6.9, P<0.01 or 0.05). (3) The express of PI3K was significantly upregulated in 1 d group(57.2±11.1)and 7 d group(39.9±4.8) compared to control group(19.1±3.5, P<0.01 or 0.05). The expression level of PI3K in 30 d group was 18.8 ± 7.5, which was similar as control group (P>0.05) and significantly lower than 1 d and 7 d group (both P<0.05). (4) Similarly, the level of VEGF in 1 d group, 7 d group ((89.1 ± 15.5) ng/L, (76.9±9.9) ng/L) were significantly higher while it was significantly lower in 30 d group ((19.8 ± 4.4)ng/L) compared with the control group ((33.9 ± 6.7) ng/L, P<0.01 or 0.05). The level of VEGF in 7 d group was significantly lower

  13. Assessment of the ability of myocardial contrast echocardiography with harmonic power Doppler imaging to identify perfusion abnormalities in patients with Kawasaki disease at rest and during dipyridamole stress.

    PubMed

    Ishii, M; Himeno, W; Sawa, M; Iemura, M; Furui, J; Muta, H; Sugahara, Y; Egami, K; Akagi, T; Ishibashi, M; Kato, H

    2002-01-01

    The aim of our study was to assess the ability of myocardial contrast echocardiography (MCE) with harmonic power Doppler imaging (HPDI) to identify perfusion abnormalities in patients with Kawasaki disease at rest and during pharmacological stress imaging with dipyridamole. Results were compared with those of 99mTc-tetrofosmin single-photon emission computed tomography (SPECT) imaging as the clinical reference standard. MCE with HPDI was performed on 20 patients with a history of Kawasaki disease. Images were obtained at baseline and during dipyridamole infusion (0.56 mg x kg(-1)) in the apical two- and four-chamber views. Myocardial opacification suitable for the analysis was obtained in all patients. Nine patients with stenotic lesions had a reversible defect after dipyridamole infusion detected by both MCE with HPDI and SPECT, and 3 patients with a history of myocardial infarction had a partially or completely irreversible defect detected by both methods. Three patients with coronary aneurysm without stenotic lesion, 4 patients with regressed coronary aneurysm, and 2 patients with normal coronary artery in acute phase also had normal perfusion at rest and after pharmacological stress by both methods. A 96% concordance (kappa = 0.87) was obtained when comparing the respective segmental perfusion scores using the two methods at baseline, and an 86% concordance (kappa = 0.81) was obtained at postdipyridamole infusion. After combining baseline and postdipyridamole images, each segment was labeled as having normal perfusion, irreversible defects, or reversible defects. Using these classifications, concordance for the two methods was 92% (kappa = 0.87). MCE with HPDI is a safe and feasible method by which to detect asymptomatic ischemia due to severe stenotic lesion, and it may be an important addition to the modalities used to identify patients at risk for myocardial infarction as a complication of Kawasaki disease.

  14. Clarithromycin Plus Intravenous Immunoglobulin Therapy Can Reduce the Relapse Rate of Kawasaki Disease: A Phase 2, Open-Label, Randomized Control Study.

    PubMed

    Nanishi, Etsuro; Nishio, Hisanori; Takada, Hidetoshi; Yamamura, Kenichiro; Fukazawa, Mitsuharu; Furuno, Kenji; Mizuno, Yumi; Saigo, Kenjiro; Kadoya, Ryo; Ohbuchi, Noriko; Onoe, Yasuhiro; Yamashita, Hironori; Nakayama, Hideki; Hara, Takuya; Ohno, Takuro; Takahashi, Yasuhiko; Hatae, Ken; Harada, Tatsuo; Shimose, Takayuki; Kishimoto, Junji; Ohga, Shouichi; Hara, Toshiro

    2017-07-06

    We previously reported that biofilms and innate immunity contribute to the pathogenesis of Kawasaki disease. Therefore, we aimed to assess the efficacy of clarithromycin, an antibiofilm agent, in patients with Kawasaki disease. We conducted an open-label, multicenter, randomized, phase 2 trial at 8 hospitals in Japan. Eligible patients included children aged between 4 months and 5 years who were enrolled between days 4 and 8 of illness. Participants were randomly allocated to receive either intravenous immunoglobulin (IVIG) or IVIG plus clarithromycin. The primary end point was the duration of fever after the initiation of IVIG treatment. Eighty-one eligible patients were randomized. The duration of the fever did not differ between the 2 groups (mean±SD, 34.3±32.4 and 31.1±31.1 hours in the IVIG plus clarithromycin group and the IVIG group, respectively [P=0.66]). The relapse rate of patients in the IVIG plus clarithromycin group was significantly lower than that in the IVIG group (12.5% versus 30.8%, P=0.046). No serious adverse events occurred during the study period. In a post hoc analysis, the patients in the IVIG plus clarithromycin group required significantly shorter mean lengths of hospital stays than those in the IVIG group (8.9 days versus 10.3 days, P=0.049). Although IVIG plus clarithromycin therapy failed to shorten the duration of fever, it reduced the relapse rate and shortened the duration of hospitalization in patients with Kawasaki disease. URL: http://www.umin.ac.jp/ctr/index.htm. Unique identifier: UMIN000015437. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

  15. Role of Antioxidants in Horse Serum-mediated Vasculitis in Swine: Potential Relevance to Early Treatment in Mitigation of Coronary Arteritis in Kawasaki Disease.

    PubMed

    Philip, Saji; Lee, Wen-Chuan; Cherian, Kotturathu Mammen; Wu, Mei-Hwan; Lue, Hung-Chi

    2017-08-01

    Horse serum-induced immune complex coronary vasculitis in swine is the first experimental model to mimic most of the pictures of Kawasaki disease. Immune complex mechanism has been implicated as one of the possible mechanisms in the pathogenesis of vasculitis in Kawasaki disease. Antioxidants have a significant role in the reduction of cardiovascular diseases in both human and animal studies. We tried giving vitamins A, E, and C to treat immune complex vasculitis, in the hope of mitigating coronary vasculitis in Kawasaki disease. Our study group consisted of 30 pure bred male piglets of 2-3 months of age, and they were divided into test and control groups. The test (AEC) group (n = 20) received two doses of horse serum, 10 mL (0.65 g protein)/kg body weight at 5-day intervals, and oral vitamins A, E, and C once daily for 14 days. The control group (n = 10) was further divided into the saline group (n = 3) receiving two doses of normal saline and the horse serum group (n = 7) receiving two doses of horse serum at 5-day intervals. Piglets were observed for the rashes and coronary artery dimensions. Both the AEC and the control horse serum group developed rashes after horse serum infusions, but the AEC group developed significantly fewer rashes, and no rashes were seen in the saline group. The control horse serum group (mean ± standard deviation = 2.13 ± 0.72) showed significant coronary artery dilatation, whereas there was no significant dilatation in the AEC group (mean ± standard deviation = 0.81 ± 0.58) or the control saline group (p = 0.002). Serum sickness is a prototype of immune complex vasculitis, and the severity can be ameliorated with antioxidants. A trial of therapeutic dosages of vitamins A, E, and C in acute phase of Kawasaki disease, may be effective in mitigation of coronary artery lesion in addition to intravenous immunoglobulin and aspirin. Copyright © 2017. Published by Elsevier B.V.

  16. Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial.

    PubMed

    Kobayashi, Tohru; Saji, Tsutomu; Otani, Tetsuya; Takeuchi, Kazuo; Nakamura, Tetsuya; Arakawa, Hirokazu; Kato, Taichi; Hara, Toshiro; Hamaoka, Kenji; Ogawa, Shunichi; Miura, Masaru; Nomura, Yuichi; Fuse, Shigeto; Ichida, Fukiko; Seki, Mitsuru; Fukazawa, Ryuji; Ogawa, Chitose; Furuno, Kenji; Tokunaga, Hirohide; Takatsuki, Shinichi; Hara, Shinya; Morikawa, Akihiro

    2012-04-28

    Evidence indicates that corticosteroid therapy might be beneficial for the primary treatment of severe Kawasaki disease. We assessed whether addition of prednisolone to intravenous immunoglobulin with aspirin would reduce the incidence of coronary artery abnormalities in patients with severe Kawasaki disease. We did a multicentre, prospective, randomised, open-label, blinded-endpoints trial at 74 hospitals in Japan between Sept 29, 2008, and Dec 2, 2010. Patients with severe Kawasaki disease were randomly assigned by a minimisation method to receive either intravenous immunoglobulin (2 g/kg for 24 h and aspirin 30 mg/kg per day) or intravenous immunoglobulin plus prednisolone (the same intravenous immunoglobulin regimen as the intravenous immunoglobulin group plus prednisolone 2 mg/kg per day given over 15 days after concentrations of C-reactive protein normalised). Patients and treating physicians were unmasked to group allocation. The primary endpoint was incidence of coronary artery abnormalities during the study period. Analysis was by intention to treat. This trial is registered with the University Hospital Medical Information Network clinical trials registry, number UMIN000000940. We randomly assigned 125 patients to the intravenous immunoglobulin plus prednisolone group and 123 to the intravenous immunoglobulin group. Incidence of coronary artery abnormalities was significantly lower in the intravenous immunoglobulin plus prednisolone group than in the intravenous immunoglobulin group during the study period (four patients [3%] vs 28 patients [23%]; risk difference 0·20, 95% CI 0·12-0·28, p<0·0001). Serious adverse events were similar between both groups: two patients had high total cholesterol and one neutropenia in the intravenous immunoglobulin plus prednisolone group, and one had high total cholesterol and another non-occlusive thrombus in the intravenous immunoglobulin group. Addition of prednisolone to the standard regimen of intravenous

  17. Elevated D-dimer level is a risk factor for coronary artery lesions accompanying intravenous immunoglobulin-unresponsive Kawasaki disease.

    PubMed

    Masuzawa, Yuko; Mori, Masaaki; Hara, Takuma; Inaba, Aya; Oba, Mari S; Yokota, Shumpei

    2015-04-01

    Although there are many reports on the resistance of Kawasaki disease (KD) to initial intravenous immunoglobulin (IVIg) therapy, risk factors for coronary artery lesions in such cases remain to be established. The objective of this study was to explore when additional therapies should be administered and to identify factors helpful for selecting a therapeutic option. Based on their written clinical records, we performed a retrospective review of KD patients who did not respond to initial IVIg therapy and who therefore then underwent plasma exchange (PE) therapy. This was a case-control study to compare the presence or absence of acute coronary lesions in patients treated by PE for IVIg-unresponsive KD at Yokohama City University Hospital or at Yokohama City University Medical Center. Fifteen of 44 patients had acute coronary artery lesions (CAL) correlating with high levels of white blood cells (WBC) (P = 0.045), D-dimer (P = 0.008), and fibrin/fibrinogen degradation products (P = 0.009) and lower levels of fibrinogen (P = 0.013) prior to PE therapy. There was a strong correlation between pre-PE levels of albumin and D-dimer (Pearson's correlation coefficient of 0.610). Multivariate analyses revealed that the odds ratio for CAL when D-dimer was ≥ 4.5 μg/mL was 25.06 (95% CI, 2.56-244.91, P = 0.006). D-dimer elevation and albumin decline in IVIg-unresponsive KD patients could be risk factors for acute CAL, suggesting the possibility that angitis has spread throughout the arterial system, as far as the coronary artery.

  18. Ambient Air Pollutant Exposures and Hospitalization for Kawasaki Disease in Taiwan: A Case-Crossover Study (2000-2010).

    PubMed

    Jung, Chau-Ren; Chen, Wei-Ting; Lin, Yu-Ting; Hwang, Bing-Fang

    2017-04-01

    Kawasaki disease (KD) is an acute and multi-systemic vasculitis that occurs predominantly in infants and young children. Although the etiological agent of KD remains unclear, limited studies have reported that windborne environmental factors may trigger KD. We conducted a time-stratified case-crossover study to assess the associations between air pollutants and KD in Taiwan. We identified children < 5 years old with a diagnosis of KD from the Longitudinal Health Insurance Database 2000 (LHID2000) between 2000 and 2010. We obtained data regarding carbon monoxide (CO), nitrogen dioxide (NO2), ozone (O3), particulate matter with aerodynamic diameter < 10 μm (PM10), and sulfate dioxide (SO2) from 70 monitoring stations and used inverse distance weighting to calculate average daily exposures for the residential postal code of each case. We performed conditional logistic regression to estimate associations between KD and each air pollutant according to interquartile range (IQR) increases and quartiles of exposure on the day of hospitalization versus 3-4 reference days during the same month for each case. Additionally, we estimated associations with single-day exposures lagged 1-2 days. We identified 695 KD hospital admissions during the study period. An IQR increase (28.73 ppb) of O3 was positively associated with KD after adjusting for temperature, humidity, northward wind, and eastward wind [adjusted odds ratio = 1.21; 95% confidence interval (CI): 1.01, 1.44]. There were no significant associations between KD and CO, NO2, PM10, or SO2. The association with O3 was limited to exposure on the day of hospitalization and to exposure during the summer months (June-August). Our results provide new evidence that exposure to O3 may increase the risk of KD in children. However, further investigation is needed to confirm the association and identify a potential biological mechanism.

  19. Cardiovascular Response to Exercise Testing in Children and Adolescents Late After Kawasaki Disease According to Coronary Condition Upon Onset.

    PubMed

    Gravel, Hugo; Curnier, Daniel; Dallaire, Frédéric; Fournier, Anne; Portman, Michael; Dahdah, Nagib

    2015-10-01

    Multiple cardiovascular sequelae have been reported late after Kawasaki disease (KD), especially in patients with coronary artery lesions. In this perspective, we hypothesized that exercise response was altered after KD in patients with coronary aneurysms (CAA-KD) compared to those without history of coronary aneurysms (NS-KD). This study is a post hoc analysis of exercise data from an international multicenter trial. A group of 133 CAA-KD subjects was compared to a group of 117 NS-KD subjects. Subjects underwent a Bruce treadmill test followed to maximal exertion. Heart rate (HR), systolic blood pressure (SBP), and diastolic blood pressure (DBP) were assessed at each stage of the test including recovery. Myocardial perfusion was evaluated by stress and rest Tc-99m sestamibi SPECT imaging. Endurance time was similar between NS-KD and CAA-KD (11.3 ± 2.6 vs. 11.0 ± 2.6 min; p = 0.343). HR, SBP, and DBP responses to exercise were similar between groups (p = 0.075-0.942). Myocardial perfusion defects were present in 16.5 % CAA-KD versus 22.2 % NS-KD (p = 0.255). Analysis based on myocardial perfusion status identified a lower heart rate at 1 min into recovery as well as lower DBP at 1 and 5 min into recovery in patients with abnormal SPECT imaging (p = 0.017-0.042). Compared to patients without CA involvement, the presence of coronary aneurysms at the subacute phase of KD does not induce a differential effect on exercise parameters. In contrast, exercise-induced myocardial perfusion defect late after the onset of KD correlates with abnormal recovery parameters.

  20. Role of the Egami score to predict immunoglobulin resistance in Kawasaki disease among a Western Mediterranean population.

    PubMed

    Sánchez-Manubens, Judith; Antón, Jordi; Bou, Rosa; Iglesias, Estíbaliz; Calzada-Hernandez, Joan; Borlan, Sergi; Gimenez-Roca, Clara; Rivera, Josefa

    2016-07-01

    Kawasaki disease is an acute self-limited systemic vasculitis common in childhood. Intravenous immunoglobulin (IVIG) is an effective treatment, and it reduces the incidence of cardiac complications. Egami score has been validated to identify IVIG non-responder patients in Japanese population, and it has shown high sensitivity and specificity to identify these non-responder patients. Although its effectiveness in Japan, Egami score has shown to be ineffective in non-Japanese populations. The aim of this study was to apply the Egami score in a Western Mediterranean population in Catalonia (Spain). Observational population-based study that includes patients from all Pediatric Units in 33 Catalan hospitals, both public and private management, between January 2004 and March 2014. Sensitivity and specificity for the Egami score was calculated, and a logistic regression analysis of predictors of overall response to IVIG was also developed. Predicting IVIG resistance with a cutoff for Egami score ≥3 obtained 26 % sensitivity and 82 % specificity. Negative predictive value was 85 % and positive predictive value 22 %. This low sensitivity implies that three out of four non-responders will not be identified by the Egami score. Besides, logistic regression models did not found significance for the use of the Egami score to predict IVIG resistance in Catalan population although having an area under the ROC curve of 0.618 (IC 95 % 0.538-0.698, p < 0.001). Although regression models found an area under the ROC curve >0.5 to predict IVIG resistance, the low sensitivity excludes the Egami score as a useful tool to predict IVIG resistance in Catalan population.

  1. Unique Molecular Patterns Uncovered in Kawasaki Disease Patients with Elevated Serum Gamma Glutamyl Transferase Levels: Implications for Intravenous Immunoglobulin Responsiveness

    PubMed Central

    Hao, Shiying; Deng, Xiaohong; Huang, Min; Ren, Miao; Jiang, Xiyuan; Kanegaye, John T.; Ha, Kee-Soo; Lee, JungHwa; Li, Xiaofeng; Jiang, Xuejun; Yu, Yunxian; Tremoulet, Adriana H.; Burns, Jane C.; Whitin, John C.; Shin, Andrew Y.; Sylvester, Karl G.; McElhinney, Doff B.; Cohen, Harvey J.; Ling, Xuefeng B.

    2016-01-01

    Background Resistance to intravenous immunoglobulin (IVIG) occurs in 10–20% of patients with Kawasaki disease (KD). The risk of resistance is about two-fold higher in patients with elevated gamma glutamyl transferase (GGT) levels. We sought to understand the biological mechanisms underlying IVIG resistance in patients with elevated GGT levels. Method We explored the association between elevated GGT levels and IVIG-resistance with a cohort of 686 KD patients (Cohort I). Gene expression data from 130 children with acute KD (Cohort II) were analyzed using the R square statistic and false discovery analysis to identify genes that were differentially represented in patients with elevated GGT levels with regard to IVIG responsiveness. Two additional KD cohorts (Cohort III and IV) were used to test the hypothesis that sialylation and GGT may be involved in IVIG resistance through neutrophil apoptosis. Results Thirty-six genes were identified that significantly explained the variations of both GGT levels and IVIG responsiveness in KD patients. After Bonferroni correction, significant associations with IVIG resistance persisted for 12 out of 36 genes among patients with elevated GGT levels and none among patients with normal GGT levels. With the discovery of ST6GALNAC3, a sialyltransferase, as the most differentially expressed gene, we hypothesized that sialylation and GGT are involved in IVIG resistance through neutrophil apoptosis. We then confirmed that in Cohort III and IV there was significantly less reduction in neutrophil count in IVIG non-responders. Conclusions Gene expression analyses combining molecular and clinical datasets support the hypotheses that: (1) neutrophil apoptosis induced by IVIG may be a mechanism of action of IVIG in KD; (2) changes in sialylation and GGT level in KD patients may contribute synergistically to IVIG resistance through blocking IVIG-induced neutrophil apoptosis. These findings have implications for understanding the mechanism of

  2. Association of Kawasaki disease with tropospheric winds in Central Chile: is wind-borne desert dust a risk factor?

    PubMed

    Jorquera, Héctor; Borzutzky, Arturo; Hoyos-Bachiloglu, Rodrigo; García, Alvaro

    2015-05-01

    It has been found that Kawasaki disease (KD) cases diagnosed in Japan, Hawaii and San Diego, USA increase when tropospheric wind patterns arrive from central Asia, suggesting a common, wind-borne causal agent. We analyzed KD cases hospitalized in Santiago, Chile to look for associations with local, regional and large scale meteorological variables. We compiled monthly data of KD incidence rates, local meteorological variables, large scale wind patterns and several El Niño Southern Oscillation (ENSO) indices for 2001-2010; we considered standardized anomalies in all analyses and used linear time series models to account for data autocorrelation. We found that meteorological variables explain 38% of variance in KD rates. A unit increase in northerly wind at 3 lagged months, temperature at 1 and 3 lagged months and monthly change of ENSO 4 index are associated with changes in KD rates of 0.203 (95% CI 0.049-0.358), 0.181 (95% CI 0.014-0.347), 0.192 (95% CI 0.030-0.353) and -0.307 (95% CI -0.458-0.156), respectively. These results are robust when northerly wind level is changed or when a shorter period (2005-2010) is used to estimate model parameters. We found a statistical association of KD at Santiago, Chile with tropospheric, northerly wind patterns suggesting that dust transported from the Atacama Desert could include a causative agent. A novel result is that ENSO dynamics also explain part of KD variability with a decrease in KD when La Niña is dissipating or El Niño is on the rise; hence climate scale dynamics might be taken into account in future studies worldwide - at least as a potential explanatory variable that may confound KD seasonality on a global scale.

  3. Noninvasive Evaluation of Myocardial Systolic Dysfunction in the Early Stage of Kawasaki Disease: A Speckle-Tracking Echocardiography Study.

    PubMed

    Hematian, Mohammad-Nasir; Torabi, Shirin; MalaKan-Rad, Elaheh; Sayadpour-Zanjani, Keyhan; Ziaee, Vahid; Lotfi-Tolkaldany, Masoumeh

    2015-06-01

    Evaluation of myocardial function by speckle-tracking echocardiography is a new method for the early diagnosis of systolic dysfunction. We aimed to determine myocardial speckle-tracking echocardiography indices in Kawasaki Disease (KD) patients and compare them with the same indices in control subjects. Thirty-two patients (65.5% males) with KD and 19 control subjects with normal echocardiography participated in this study. After their demographic characteristics and clinical findings were recorded, all the participants underwent transthoracic echocardiography. Strain (S), Strain Rate (SR), Time to Peak Strain (TPS), and Strain Rate (TPSR), longitudinal velocity and view point velocity images in the two, three, and four-chamber views were semi-automatically obtained via speckle-tracking echocardiography. Among the patients, Twenty-four cases (75%) were younger than 4 years. Mean global S and SR was significantly reduced in the KD patients compared to controls (17.03 ± 1.28 vs. 20.22 ± 2.14% and 1.66 ± 0.16 vs. 1.97 ± 0.25 1/second, respectively), while there were no significant differences regarding mean TPS, TPSR, longitudinal velocity and view point velocity. Using repeated measure of analysis of variances, we observed that S and SR decreased from base to apical level in both groups. The change in the pattern of age adjusted mean S and SR across levels was significantly different between the groups (P < 0.001 for both parameters). We showed changes in S and SR assessed in KD patients versus control subjects in the acute phase of KD. However, we suggest that further studies be undertaken to compare S and SR in the acute phase and thereafter in KD patients.

  4. Tropospheric winds from northeastern China carry the etiologic agent of Kawasaki disease from its source to Japan.

    PubMed

    Rodó, Xavier; Curcoll, Roger; Robinson, Marguerite; Ballester, Joan; Burns, Jane C; Cayan, Daniel R; Lipkin, W Ian; Williams, Brent L; Couto-Rodriguez, Mara; Nakamura, Yosikazu; Uehara, Ritei; Tanimoto, Hiroshi; Morguí, Josep-Anton

    2014-06-03

    Evidence indicates that the densely cultivated region of northeastern China acts as a source for the wind-borne agent of Kawasaki disease (KD). KD is an acute, coronary artery vasculitis of young children, and still a medical mystery after more than 40 y. We used residence times from simulations with the flexible particle dispersion model to pinpoint the source region for KD. Simulations were generated from locations spanning Japan from days with either high or low KD incidence. The postepidemic interval (1987-2010) and the extreme epidemics (1979, 1982, and 1986) pointed to the same source region. Results suggest a very short incubation period (<24 h) from exposure, thus making an infectious agent unlikely. Sampling campaigns over Japan during the KD season detected major differences in the microbiota of the tropospheric aerosols compared with ground aerosols, with the unexpected finding of the Candida species as the dominant fungus from aloft samples (54% of all fungal strains). These results, consistent with the Candida animal model for KD, provide support for the concept and feasibility of a windborne pathogen. A fungal toxin could be pursued as a possible etiologic agent of KD, consistent with an agricultural source, a short incubation time and synchronized outbreaks. Our study suggests that the causative agent of KD is a preformed toxin or environmental agent rather than an organism requiring replication. We propose a new paradigm whereby an idiosyncratic immune response, influenced by host genetics triggered by an environmental exposure carried on winds, results in the clinical syndrome known as acute KD.

  5. Common variants in the CRP promoter are associated with a high C-reactive protein level in Kawasaki disease.

    PubMed

    Kim, Jae-Jung; Yun, Sin Weon; Yu, Jeong Jin; Yoon, Kyung Lim; Lee, Kyung-Yil; Kil, Hong-Ryang; Kim, Gi Beom; Han, Myung Ki; Song, Min Seob; Lee, Hyoung Doo; Byeon, Jung Hye; Sohn, Saejung; Hong, Young Mi; Jang, Gi Young; Lee, Jong-Keuk

    2015-02-01

    Kawasaki disease (KD) is an acute self-limiting form of vasculitis that afflicts infants and children and manifests as fever and signs of mucocutaneous inflammation. Children with KD show various laboratory inflammatory abnormalities, such as elevations in their white blood cell (WBC) count, C-reactive protein (CRP) level, and erythrocyte sedimentation rate (ESR). We here performed a genome-wide association study (GWAS) of 178 KD patients to identify the genetic loci that influence 10 important KD laboratory markers: WBC count, neutrophil count, platelet count, CRP, ESR, hemoglobin, aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, and total protein. A total of 165 loci passed our arbitrary stage 1 threshold for replication (p < 1 × 10(-5)). Of these, only 2 SNPs (rs12068753 and rs4786091) demonstrated a significant association with the CRP level in replication study of 473 KD patients (p < 0.05). The SNP located at the CRP locus (rs12068753) demonstrated the most significant association with CRP in KD patients (beta = 4.73 and p = 1.20 × 10(-6) according to the stage 1 GWAS; beta = 3.65 and p = 1.35 × 10(-8) according to the replication study; beta = 3.97 and p = 1.11 × 10(-13) according to combined analysis) and explained 8.1% of the phenotypic variation observed. However, this SNP did not demonstrate any significant association with CRP in the general population (beta = 0.37 and p = 0.1732) and only explained 0.1% of the phenotypic variation in this instance. Furthermore, rs12068753 did not affect the development of coronary artery lesions or intravenous immunoglobulin resistance in KD patients. These results indicate that common variants in the CRP promoter can play an important role in the CRP levels in KD.

  6. The outcome of short-term low-dose aspirin treatment in Kawasaki disease based on inflammatory markers

    PubMed Central

    Yoo, Jae Won; Kim, Ji Mok

    2017-01-01

    Purpose Previously, Kawasaki disease (KD) treatment with low-dose aspirin was administered for 6–8 weeks after the acute phase. However, inflammatory marker levels normalize before 6–8 weeks. In this study, we aimed to investigate the clinical outcome of short-term low-dose aspirin treatment based on inflammatory and thrombotic marker levels. Methods We performed a retrospective review of the medical records of patients with KD who were hospitalized at Chungnam National University Hospital between September 2012 and May 2014. When fever subsided, low-dose aspirin treatment was started. Inflammatory (white blood cell count, erythrocyte sedimentation rate, and C-reactive protein) and thrombotic markers (D-dimer) were monitored at follow-ups conducted in 1- to 2-week intervals. The low-dose aspirin administration was terminated when both markers were normalized and no cardiovascular complications were observed. Results Eighty-four patients with KD (complete KD, n=49; incomplete KD, n=35) were enrolled. The inflammatory and thrombotic marker levels were normalized within 3–4 weeks on average. At the beginning the low-dose aspirin treatment, 9 patients had coronary artery lesions but 75 did not. When the low-dose aspirin administration was terminated at the time the inflammatory marker levels were normalized, no new CALs developed during the follow-up at 6–8 weeks. Conclusion Most of the inflammatory marker levels were normalized within 3–4 weeks after the acute phase of KD. New cardiovascular complications did not develop during the course of the short-term aspirin treatment based on the inflammatory marker levels, clinical findings, and echocardiography. PMID:28203257

  7. A genome-wide association analysis identifies NMNAT2 and HCP5 as susceptibility loci for Kawasaki disease.

    PubMed

    Kim, Jae-Jung; Yun, Sin Weon; Yu, Jeong Jin; Yoon, Kyung Lim; Lee, Kyung-Yil; Kil, Hong-Ryang; Kim, Gi Beom; Han, Myung-Ki; Song, Min Seob; Lee, Hyoung Doo; Ha, Kee Soo; Sohn, Sejung; Johnson, Todd A; Takahashi, Atsushi; Kubo, Michiaki; Tsunoda, Tatsuhiko; Ito, Kaoru; Onouchi, Yoshihiro; Hong, Young Mi; Jang, Gi Young; Lee, Jong-Keuk

    2017-08-31

    Kawasaki disease (KD), a systemic vasculitis of infants and children, manifests as fever and mucocutaneous inflammation. Although its etiology is largely unknown, the epidemiological data suggest that genetic factors are important in KD susceptibility. To identify genetic variants influencing KD susceptibility, we performed a genome-wide association study (GWAS) and replication study using a total of 915 children with KD and 4553 controls in the Korean population. Six single-nucleotide polymorphisms (SNPs) in three loci were associated significantly with KD susceptibility (P<1.0 × 10(-5)), including the previously reported BLK locus (rs6993775, odds ratio (OR)=1.52, P=2.52 × 10(-11)). The other two loci were newly identified: NMNAT2 on chromosome 1q25.3 (rs2078087, OR=1.33, P=1.15 × 10(-6)) and the human leukocyte antigen (HLA) region on chromosome 6p21.3 (HLA-C, HLA-B, MICA and HCP5) (rs9380242, rs9378199, rs9266669 and rs6938467; OR=1.33-1.51, P=8.93 × 10(-6) to 5.24 × 10(-8)). Additionally, SNP rs17280682 in NLRP14 was associated significantly with KD with a family history (18 cases vs 4553 controls, OR=6.76, P=5.46 × 10(-6)). These results provide new insights into the pathogenesis and pathophysiology of KD.Journal of Human Genetics advance online publication, 31 August 2017; doi:10.1038/jhg.2017.87.

  8. The outcome of short-term low-dose aspirin treatment in Kawasaki disease based on inflammatory markers.

    PubMed

    Yoo, Jae Won; Kim, Ji Mok; Kil, Hong Ryang

    2017-01-01

    Previously, Kawasaki disease (KD) treatment with low-dose aspirin was administered for 6-8 weeks after the acute phase. However, inflammatory marker levels normalize before 6-8 weeks. In this study, we aimed to investigate the clinical outcome of short-term low-dose aspirin treatment based on inflammatory and thrombotic marker levels. We performed a retrospective review of the medical records of patients with KD who were hospitalized at Chungnam National University Hospital between September 2012 and May 2014. When fever subsided, low-dose aspirin treatment was started. Inflammatory (white blood cell count, erythrocyte sedimentation rate, and C-reactive protein) and thrombotic markers (D-dimer) were monitored at follow-ups conducted in 1- to 2-week intervals. The low-dose aspirin administration was terminated when both markers were normalized and no cardiovascular complications were observed. Eighty-four patients with KD (complete KD, n=49; incomplete KD, n=35) were enrolled. The inflammatory and thrombotic marker levels were normalized within 3-4 weeks on average. At the beginning the low-dose aspirin treatment, 9 patients had coronary artery lesions but 75 did not. When the low-dose aspirin administration was terminated at the time the inflammatory marker levels were normalized, no new CALs developed during the follow-up at 6-8 weeks. Most of the inflammatory marker levels were normalized within 3-4 weeks after the acute phase of KD. New cardiovascular complications did not develop during the course of the short-term aspirin treatment based on the inflammatory marker levels, clinical findings, and echocardiography.

  9. Whole genome sequencing of an African American family highlights toll like receptor 6 variants in Kawasaki disease susceptibility

    PubMed Central

    Veeraraghavan, Narayanan; Levy, Eric; Ribeiro dos Santos, Andre M.; Yang, Hai; Hibberd, Martin L.; Tremoulet, Adriana H.; Harismendy, Olivier; Ohno-Machado, Lucila; Burns, Jane C.

    2017-01-01

    Kawasaki disease (KD) is the most common acquired pediatric heart disease. We analyzed Whole Genome Sequences (WGS) from a 6-member African American family in which KD affected two of four children. We sought rare, potentially causative genotypes by sequentially applying the following WGS filters: sequence quality scores, inheritance model (recessive homozygous and compound heterozygous), predicted deleteriousness, allele frequency, genes in KD-associated pathways or with significant associations in published KD genome-wide association studies (GWAS), and with differential expression in KD blood transcriptomes. Biologically plausible genotypes were identified in twelve variants in six genes in the two affected children. The affected siblings were compound heterozygous for the rare variants p.Leu194Pro and p.Arg247Lys in Toll-like receptor 6 (TLR6), which affect TLR6 signaling. The affected children were also homozygous for three common, linked (r2 = 1) intronic single nucleotide variants (SNVs) in TLR6 (rs56245262, rs56083757 and rs7669329), that have previously shown association with KD in cohorts of European descent. Using transcriptome data from pre-treatment whole blood of KD subjects (n = 146), expression quantitative trait loci (eQTL) analyses were performed. Subjects homozygous for the intronic risk allele (A allele of TLR6 rs56245262) had differential expression of Interleukin-6 (IL-6) as a function of genotype (p = 0.0007) and a higher erythrocyte sedimentation rate at diagnosis. TLR6 plays an important role in pathogen-associated molecular pattern recognition, and sequence variations may affect binding affinities that in turn influence KD susceptibility. This integrative genomic approach illustrates how the analysis of WGS in multiplex families with a complex genetic disease allows examination of both the common disease–common variant and common disease–rare variant hypotheses. PMID:28151979

  10. Factors Associated With Resource Utilization and Coronary Artery Dilation in Refractory Kawasaki Disease (from the Pediatric Health Information System Database).

    PubMed

    Lo, Jennifer Y; Minich, L LuAnn; Tani, Lloyd Y; Wilkes, Jacob; Ding, Qian; Menon, Shaji C

    2016-12-01

    Management guidelines for refractory Kawasaki disease (KD) are vague. We sought to assess practice variation and identify factors associated with large/complex coronary artery aneurysms (LCAA) and resource utilization in refractory KD. This retrospective cohort study identified patients aged ≤18 years with KD (2004 to 2014) using the Pediatric Health Information System. Refractory KD was defined as receiving >1 dose of intravenous immunoglobulin. Demographics, medications, concomitant infections, length of stay (LOS), and charges were collected. Antithrombotic therapy was a surrogate for LCAA. LOS and hospital charges assessed resource utilization. Multivariate regression identified factors associated with LOS, charges, and LCAA. Of 14,194 patients with KD, 2,974 (21%) had refractory KD and 203 of those 2,974 (7%) had LCAA. Additional intravenous immunoglobulin was the sole medication in 77%. Other medications added were steroids (18%), infliximab (2%), and both (3%). Warfarin, low-molecular-weight heparin, tissue plasminogen activator, and clopidogrel were prescribed with equal frequency (2%). Male gender (adjusted relative risk 1.52, 95% confidence interval [CI] 1.08 to 2.16, p <0.01), admission to an intensive care unit (4.79, 95% CI 3.40 to 6.74, p <0.001), arrhythmia (3.00, 95% CI 1.94 to 4.65, p <0.001), and concomitant viral infection (2.29, 95% CI 1.49 to 3.52, p <0.001) were associated with LCAA. Severe illness, race, region, and payer were independently associated with increased charges (p <0.05 for all). In conclusion, treatment for refractory KD varies widely. Concomitant viral infection was associated with a greater risk of LCAA in refractory KD. Better understanding of optimal management may improve outcomes and decrease both variability in management and resource utilization for refractory KD. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Association of vascular endothelial growth factor (VEGF) and VEGF receptor gene polymorphisms with coronary artery lesions of Kawasaki disease.

    PubMed

    Kariyazono, Hidehiko; Ohno, Takuro; Khajoee, Vahid; Ihara, Kenji; Kusuhara, Koichi; Kinukawa, Naoko; Mizuno, Yumi; Hara, Toshiro

    2004-12-01

    We analyzed the genetic polymorphisms of vascular endothelial growth factor (VEGF) and its receptors [Fms-related tyrosine kinase-1, kinase insert domain receptor (KDR)] in Japanese patients with Kawasaki disease (KD) and normal control subjects to examine whether these genes would contribute to the KD occurrence and/or the development of coronary artery lesion (CAL) in KD. We found that the frequency of G allele of VEGF g.-634 G>C single-nucleotide polymorphism in the promoter region was significantly higher in KD patients with CAL than in those without CAL (p = 0.012) or control subjects (p = 0.021) because of a significantly higher frequency of the GG genotype in KD patients with CAL. In addition, the frequency of the A1 allele with 11 AC repeats of KDR g.+4422(AC)11-14 dinucleotide repeat polymorphism in intron 2 was significantly higher in KD patients with CAL than in those without CAL (p = 0.013) or control subjects (p = 0.040) as a result of a significantly higher frequency of the A1A1 genotype in KD with CAL patients. The multivariate analysis of clinical features and genotypes of the two polymorphisms showed that the A1A1 genotype of KDR g.+4422(AC)11-14 polymorphism was an independent risk factor for the development of CAL with the highest odds ratio among several clinical parameters (odds ratio 6.76; 95% confidence interval 1.05-43.48). Dual luciferase assay demonstrated that the A1 allele with KDR g.+4422(AC)11 repeats showed a weaker silencer function than the A2 allele with 12 AC repeats. These findings suggested that VEGF and its receptor, KDR, genes contributed to the development of CAL in KD patients.

  12. Tropospheric winds from northeastern China carry the etiologic agent of Kawasaki disease from its source to Japan

    PubMed Central

    Rodó, Xavier; Curcoll, Roger; Robinson, Marguerite; Ballester, Joan; Burns, Jane C.; Cayan, Daniel R.; Lipkin, W. Ian; Williams, Brent L.; Couto-Rodriguez, Mara; Nakamura, Yosikazu; Uehara, Ritei; Tanimoto, Hiroshi; Morguí, Josep-Anton

    2014-01-01

    Evidence indicates that the densely cultivated region of northeastern China acts as a source for the wind-borne agent of Kawasaki disease (KD). KD is an acute, coronary artery vasculitis of young children, and still a medical mystery after more than 40 y. We used residence times from simulations with the flexible particle dispersion model to pinpoint the source region for KD. Simulations were generated from locations spanning Japan from days with either high or low KD incidence. The postepidemic interval (1987–2010) and the extreme epidemics (1979, 1982, and 1986) pointed to the same source region. Results suggest a very short incubation period (<24 h) from exposure, thus making an infectious agent unlikely. Sampling campaigns over Japan during the KD season detected major differences in the microbiota of the tropospheric aerosols compared with ground aerosols, with the unexpected finding of the Candida species as the dominant fungus from aloft samples (54% of all fungal strains). These results, consistent with the Candida animal model for KD, provide support for the concept and feasibility of a windborne pathogen. A fungal toxin could be pursued as a possible etiologic agent of KD, consistent with an agricultural source, a short incubation time and synchronized outbreaks. Our study suggests that the causative agent of KD is a preformed toxin or environmental agent rather than an organism requiring replication. We propose a new paradigm whereby an idiosyncratic immune response, influenced by host genetics triggered by an environmental exposure carried on winds, results in the clinical syndrome known as acute KD. PMID:24843117

  13. Critical Overview of the Risk Scoring Systems to Predict Non-Responsiveness to Intravenous Immunoglobulin in Kawasaki Syndrome.

    PubMed

    Rigante, Donato; Andreozzi, Laura; Fastiggi, Michele; Bracci, Benedetta; Natale, Marco Francesco; Esposito, Susanna

    2016-02-24

    Kawasaki syndrome (KS) is the most relevant cause of heart disease in children living in developed countries. Intravenous immunoglobulin (IVIG) has a preventive function in the formation of coronary artery abnormalities and a poor strictly-curative action in established coronary damage. More than two decades ago, the Harada score was set to assess which children with KS should be subject to administration of IVIG, evaluating retrospectively a large cohort of patients with regard to age, sex and laboratory data. Nowadays, high dose IVIG is administered to all children with a confirmed diagnosis of KS, but a tool for predicting non-responsiveness to the initial infusion of IVIG has not been found. The prediction of IVIG resistance is a crucial issue, as recognising these high-risk patients should consent the administration of an intensified initial treatment in combination with IVIG in order to prevent coronary injuries. Few reports have focused on factors, referring to both clinical parameters and laboratory data at the onset of KS, in order to predict which patients might be IVIG non-responsive. We have analysed three different risk scores which were formulated to predict IVIG resistance in Japanese children with typical KS, but their application in non-Japanese patients or in those with incomplete and atypical patterns of the disease has been studied in a fragmentary way. Overall, our analysis showed that early and definite ascertainment of likely IVIG non-responders who require additional therapies reducing the development of coronary artery involvement in children with KS is still a challenge.

  14. High sensitivity C-reactive protein and endothelial function in Chilean patients with history of Kawasaki disease.

    PubMed

    Borzutzky, Arturo; Gutiérrez, Miguel; Talesnik, Eduardo; Godoy, Iván; Kraus, Jonathan; Hoyos, Rodrigo; Arnaiz, Pilar; Acevedo, Mónica

    2008-07-01

    Kawasaki disease (KD) produces endothelial inflammation, which may lead to dilatation and aneurysms of coronary and peripheral arteries. Previous studies have suggested that these patients can present endothelial dysfunction that can predispose to coronary vascular events late after KD. The purpose of this study was to determine the cardiovascular risk profile and endothelial function of Chilean children with history of KD. In a prospective case-control study, 11 patients with history of KD (age 10.6 +/- 2.0 years, interval from initial episode 8.1 +/- 3.6 years) and 11 healthy, age-, gender-, and BMI z score-matched controls were evaluated with blood pressure (BP), a fasting lipid profile, high sensitivity C-reactive protein (hsCRP), and flow-mediated dilatation of the brachial artery (FMD). One KD patient (9.1%) had persistent coronary aneurysms. There was a significant difference of mean and log-transformed concentrations of hsCRP between case and control groups (2.3 +/- 3.0 vs 0.5 +/- 0.3 mg/l, P = 0.045). None of the patients with elevated hsCRP had persistent coronary arterial lesions. No difference was found in systolic BP z score between the case and control groups. Diastolic BP z score was significantly higher in cases than controls (P = 0.039). There were no significant differences of FMD between cases and controls. Mean fasting total cholesterol, high-density and low-density lipoprotein, and triglycerides in cases were normal, with no significant difference vs controls. This study shows that Chilean children with history of KD have increased levels of hsCRP, possibly reflecting persistent low-grade inflammation. The prognostic value of hsCRP in KD patients deserves further investigation.

  15. Association of Toll-like receptor 2-positive monocytes with coronary artery lesions and treatment nonresponse in Kawasaki disease

    PubMed Central

    Kim, Nam Su

    2017-01-01

    Purpose Activation of Toll-like receptor 2 (TLR2) present on circulating monocytes in patients with Kawasaki disease (KD) can lead to the production of proinflammatory cytokines and interleukin-10 (IL-10). We aimed to determine the association of the frequency of circulating TLR2+/CD14+ monocytes (FTLR2%) with the outcomes of KD, as well as to compare FTLR2% to the usefulness of sIL-10. Methods The FTLR2% in patients with KD was measured by flow cytometry. Serum levels of IL-10 (sIL-10) were determined in 31 patients with KD before the initial treatment with intravenous immunoglobulin (IVIG) and in 21 febrile controls by using enzyme-linked immunosorbent assay. Patients were classified as having coronary artery lesions (CALs) based on the maximal internal diameters of the proximal right coronary artery and proximal left anterior descending coronary artery one month after the initial diagnosis. Results We found that FTLR2% greater than 92.62% predicted CALs with 80% sensitivity and 68.4% specificity, whereas FTLR2% more than 94.61% predicted IVIG resistance with 66.7% sensitivity and 71.4% specificity. Moreover, sIL-10 more than 15.52 pg/mL predicted CALs and IVIG resistance with 40% and 66.7% sensitivity, respectively, and 73.7% and 76.2% specificity, respectively. Conclusion We showed that measuring FTLR2% before the initial treatment could be useful in predicting CAL development with better sensitivity than sIL-10 and with results comparable to sIL-10 results for the prediction of IVIG resistance in patients with KD. However, further studies are necessary to validate FTLR2% as a marker of prognosis and severity of KD. PMID:28861111

  16. Acute and late coronary outcomes in 1073 patients with Kawasaki disease with and without intravenous γ-immunoglobulin therapy.

    PubMed

    Lin, Ming-Tai; Sun, Li-Chuan; Wu, En-Ting; Wang, Jou-Kou; Lue, Hung-Chi; Wu, Mei-Hwan

    2015-06-01

    To explore acute and late coronary outcomes and their risk/modifiers in patients with Kawasaki disease (KD). Retrospective study. 1073 patients with KD identified from a tertiary care medical centre (1980-2012; 8677 patient-years). The acute coronary severities and late outcomes (survival free of coronary aneurysm persistence and ischaemia) were assessed. Coronary arterial lesions occurred in 40.6% of cases at their acute febrile stages, and persisted beyond 1 month in 196 (18.3%, M/F=138/58) patients: 125 (11.6%) had small aneurysms, 44 (4.1%) had medium aneurysms, and 27 (2.5%) had giant aneurysms. At follow-up (1-46 years), coronary aneurysms persisted in all with giant aneurysms, in 55% of those with medium aneurysms (18% with stenosis), and in 9% of those with small aneurysms. Ischaemia events occurred in 14 patients (M/F=13/1) and caused four deaths. Among the patients with KD with coronary aneurysms, 10-year ischaemia event-free and aneurysm persistence probability was 87.5% and 20.6%, respectively. The only independent risk for aneurysm persistence was the aneurysm severity 1 month after KD onset (χ(2)=80.73, p<10(-3)). Male patients and intravenous γ-immunoglobulin (IVIG) therapy were independent risk factors of initial coronary severity but were not associated with the late coronary outcomes, even in severity stratified subgroups. The coronary severity 1 month after KD onset is most crucial to the late coronary outcomes. Although IVIG use improves the initial severity of coronary lesions, it does not further modify the long-term fate of coronary aneurysms. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  17. Augmented TLR2 expression on monocytes in both human Kawasaki disease and a mouse model of coronary arteritis.

    PubMed

    Lin, I-Chun; Kuo, Ho-Chang; Lin, Ying-Jui; Wang, Feng-Shen; Wang, Lin; Huang, Shun-Chen; Chien, Shao-Ju; Huang, Chien-Fu; Wang, Chih-Lu; Yu, Hong-Ren; Chen, Rong-Fu; Yang, Kuender D

    2012-01-01

    Kawasaki disease (KD) of unknown immunopathogenesis is an acute febrile systemic vasculitis and the leading cause of acquired heart diseases in childhood. To search for a better strategy for the prevention and treatment of KD, this study compared and validated human KD immunopathogenesis in a mouse model of Lactobacillus casei cell wall extract (LCWE)-induced coronary arteritis. Recruited subjects fulfilled the criteria of KD and were admitted for intravenous gamma globulin (IVIG) treatment at the Kaohsiung Chang Gung Memorial Hospital from 2001 to 2009. Blood samples from KD patients were collected before and after IVIG treatment, and cardiovascular abnormalities were examined by transthoracic echocardiography. Wild-type male BALB/c mice (4-week-old) were intraperitoneally injected with LCWE (1 mg/mL) to induce coronary arteritis. The induced immune response in mice was examined on days 1, 3, 7, and 14 post injections, and histopathology studies were performed on days 7 and 14. Both human KD patients and LCWE-treated mice developed coronary arteritis, myocarditis, valvulitis, and pericarditis, as well as elevated plasma levels of interleukin (IL)-2, IL-6, IL-10, monocyte chemoattractant protein (MCP)-1, and tumor necrosis factor (TNF)-α in acute phase. Most of these proinflammatory cytokines declined to normal levels in mice, whereas normal levels were achieved in patients only after IVIG treatment, with a few exceptions. Toll-like receptor (TLR)-2, but not TLR4 surface enhancement on circulating CD14+ monocytes, was augmented in KD patients before IVIG treatment and in LCWE-treated mice, which declined in patients after IVIG treatment. This result suggests that that not only TLR2 augmentation on CD14+ monocytes might be an inflammatory marker for both human KD patients and LCWE-induced CAL mouse model but also this model is feasible for studying therapeutic strategies of coronary arteritis in human KD by modulating TLR2-mediated immune activation on CD14

  18. Augmented TLR2 Expression on Monocytes in both Human Kawasaki Disease and a Mouse Model of Coronary Arteritis

    PubMed Central

    Lin, I-Chun; Kuo, Ho-Chang; Lin, Ying-Jui; Wang, Feng-Shen; Wang, Lin; Huang, Shun-Chen; Chien, Shao-Ju; Huang, Chien-Fu; Wang, Chih-Lu; Yu, Hong-Ren; Chen, Rong-Fu; Yang, Kuender D.

    2012-01-01

    Background Kawasaki disease (KD) of unknown immunopathogenesis is an acute febrile systemic vasculitis and the leading cause of acquired heart diseases in childhood. To search for a better strategy for the prevention and treatment of KD, this study compared and validated human KD immunopathogenesis in a mouse model of Lactobacillus casei cell wall extract (LCWE)-induced coronary arteritis. Methods Recruited subjects fulfilled the criteria of KD and were admitted for intravenous gamma globulin (IVIG) treatment at the Kaohsiung Chang Gung Memorial Hospital from 2001 to 2009. Blood samples from KD patients were collected before and after IVIG treatment, and cardiovascular abnormalities were examined by transthoracic echocardiography. Wild-type male BALB/c mice (4-week-old) were intraperitoneally injected with LCWE (1 mg/mL) to induce coronary arteritis. The induced immune response in mice was examined on days 1, 3, 7, and 14 post injections, and histopathology studies were performed on days 7 and 14. Results Both human KD patients and LCWE-treated mice developed coronary arteritis, myocarditis, valvulitis, and pericarditis, as well as elevated plasma levels of interleukin (IL)-2, IL-6, IL-10, monocyte chemoattractant protein (MCP)-1, and tumor necrosis factor (TNF)-α in acute phase. Most of these proinflammatory cytokines declined to normal levels in mice, whereas normal levels were achieved in patients only after IVIG treatment, with a few exceptions. Toll-like receptor (TLR)-2, but not TLR4 surface enhancement on circulating CD14+ monocytes, was augmented in KD patients before IVIG treatment and in LCWE-treated mice, which declined in patients after IVIG treatment. Conclusion This result suggests that that not only TLR2 augmentation on CD14+ monocytes might be an inflammatory marker for both human KD patients and LCWE-induced CAL mouse model but also this model is feasible for studying therapeutic strategies of coronary arteritis in human KD by modulating TLR2

  19. Association between the FCGR2A gene H131R polymorphism and risk of Kawasaki disease: a meta-analysis.

    PubMed

    Lin, L; Wang, S Y; Yang, S B; Xiao, F C

    2015-06-11

    Several previous studies have investigated whether the FCGR2A gene H131R polymorphism confers an increased risk of Kawasaki disease (KD), but conflicting results have been reported. To further explore the association of this polymorphism with KD susceptibility, we performed an extensive search of relevant studies and conducted a meta-analysis to obtain a more precise estimate of risk. Systematic searches of the electronic databases Embase, PubMed, and Google Scholar were performed to identify relevant studies. Odds ratios (ORs) and their 95% confidence intervals (CIs) were used for statistical analysis. Six studies were included in the meta-analysis, involving 1709 patients with KD and 3207 controls. Significant association was found between the FCGR2A gene H131R polymorphism and KD risk in analysis of the total population (HH vs RR: OR = 1.97, 95%CI = 1.55-2.50; HH vs HR: OR = 1.38, 95%CI = 1.21-1.57; the dominant model: OR = 0.69, 95%CI = 0.60-0.78; and the recessive model: OR = 1.65, 95%CI = 1.32-2.07). In subgroup analysis by ethnicity, significant association was found between the H131R polymorphism and KD risk in Asians, but not in Caucasians. In addition, we found no significant association between the FCGR2A gene H131R polymorphism and risk of KD-associated coronary artery lesions. In conclusion, this meta-analysis suggested that the H131R polymorphism in the FCGR2A gene might be associated with susceptibility to KD in Asians.

  20. IL-1β is Crucial for Induction of Coronary Artery Inflammation in a Mouse Model of Kawasaki Disease

    PubMed Central

    Lee, Young Ho; Schulte, Danica J.; Shimada, Kenichi; Chen, Shuang; Crother, Timothy R.; Chiba, Norika; Fishbein, Michael C.; Lehman, Thomas J.A.; Arditi, Moshe

    2012-01-01

    Background Kawasaki disease (KD) is the most common cause of acute vasculitis and acquired cardiac disease in US children. Untreated, children may develop coronary artery aneurysms, myocardial infarction and sudden death as a result of the illness. Up to a third of KD patients fail to respond to intravenous gammaglobulin (IVIG), the standard therapy, and alternative treatments are being investigated. Genetic studies have indicated a possible role for IL-1β in KD. We therefore explored the role of IL-1β in a murine model of KD. Methods and Results Using an established mouse model of KD that involves injection of Lactobacillus casei cell wall extract (LCWE), we investigated the role of IL- 1β and caspase-1 (activated by the inflammasome and required for IL-1β maturation) in coronary arteritis, and evaluated the efficacy of IL-1 receptor antagonist (IL-1Ra) as a potential treatment. LCWE-induced IL-1β maturation and secretion was dependent on the NLRP3 inflammasome in macrophages. Both caspase1-deficient and IL-1R-deficient mice were protected from LCWE-induced coronary lesions. Injection of recombinant IL-1β to caspase-1-deficient mice restored the ability of LCWE to cause coronary lesions in response to LCWE. Furthermore, daily injections of the IL-1Ra prevented LCWE-mediated coronary lesions, up to three days after LCWE injection. Conclusions Our results strongly suggest that caspase-1 and IL-1β play critical roles in the development of coronary lesions in this KD mouse model, blocked by IL-1Ra. Therefore, anti-IL-1β treatment strategies may constitute an effective, more targeted treatment of KD to prevent coronary lesions. PMID:22361326

  1. Long-Term Prognosis for Patients with Kawasaki Disease Complicated by Large Coronary Aneurysm (diameter ≥6 mm)

    PubMed Central

    Bang, Ji Seok; Kwon, Bo Sang; Song, Mi Kyung; An, Hyo Soon; Song, Young Whan; Bae, Eun Jung; Noh, Chung Il

    2017-01-01

    Background and Objectives Some patients with Kawasaki disease (KD) develop large coronary aneurysms and subsequent coronary stenosis or obstruction, leading to ischemic heart disease. This study examined the long-term outcomes of patients with KD complicated by large coronary aneurysms. Subjects and Methods The medical records of 71 patients (53 men and 18 women) diagnosed with large coronary aneurysms (diameter ≥6 mm) between December 1986 and December 2013 were retrospectively reviewed from our institutional database. Results The mean age at onset was 4.6±3.3 years, and the mean follow-up duration was 12.5±6.9 years. Maximum coronary artery internal diameter ranged from 6.1 to 25 mm. Giant coronary aneurysms occurred in 48 patients and coronary aneurysms 6-8 mm in diameter developed in 23 patients. Coronary stenosis and/or complete occlusion occurred in 30 patients (42.3%). Catheter and/or surgical interventions (mean: 1.5 interventions, range: 1-5 interventions) were performed in 20 patients (28.2%), 9 months to 18 years after KD onset, resulting in 33.7% cumulative coronary intervention rates at 20 years after onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (6-8 mm vs. ≥8 mm). Myocardial infarction occurred in 7 patients with a giant aneurysm and there was one death. Conclusions Long-term survival of patients with KD complicated by large coronary aneurysm was good even though 28.2% of patients underwent multiple catheter or surgical interventions. Careful follow-up is also necessary in KD patients with coronary aneurysms 6-8 mm in diameter, such as those with giant aneurysms. PMID:28765744

  2. Noninvasive quantification of coronary endothelial function by SPECT imaging in children with a history of Kawasaki disease.

    PubMed

    Cicala, Silvana; Pellegrino, Teresa; Storto, Giovanni; Caprio, Maria Grazia; Paladini, Rodolfo; Mainolfi, Ciro; de Leva, Francesco; Cuocolo, Alberto

    2010-12-01

    The feasibility of coronary function estimation by single photon emission computed tomography (SPECT) has been recently demonstrated. The aim of this study was to apply SPECT imaging in patients with previous Kawasaki disease (KD) to assess the coronary functional status at long-term follow-up of the acute phase of the disease. Sixteen children with a history of KD underwent 99mTc-sestamibi imaging at rest and during the cold pressor test (CPT). Myocardial blood flow (MBF) was estimated by measuring first transit counts in the pulmonary artery and myocardial counts from SPECT images. Coronary endothelial function was expressed as the ratio of the CPT to rest MBF. Six KD patients without coronary artery lesions served as controls and ten with coronary artery aneurysms during the acute phase of the disease were separated into two groups: group 1 (n=4) with regressed and group 2 (n=6) with persistent aneurysm at follow-up. The estimated coronary endothelial function was higher in controls compared to patients with coronary artery aneurysms (2.5±0.3 vs 1.7±0.7, p<0.05). A significant difference in coronary endothelial function among groups was found (F=5.21, p<0.02). Coronary endothelial function was higher in patients of group 1 than in those of group 2 (1.9±0.6 vs 1.4±0.7, p<0.02). SPECT may be applied as a noninvasive method for assessing coronary vascular function in children with a history of KD, demonstrating an impaired response to the CPT, an endothelial-dependent vasodilator stimulus. These findings reinforce the concept that coronary endothelial dysfunction may represent a long-term sequela of KD.

  3. Incidence Rate and Epidemiological and Clinical Aspects of Kawasaki Disease in Children of Maghrebi Origin in the Province of Quebec, Canada, Compared to the Country of Origin

    PubMed Central

    Gorrab, Arbia Abir; Fournier, Anne; Bouaziz, Asma Abed; Spigelblatt, Linda; Scuccimarri, Rosie; Mrabet, Ali; Dahdah, Nagib

    2016-01-01

    The incidence of Kawasaki disease in Maghreb countries is apparently low, unlike those living in the province of Quebec, Canada. This retrospective study compared Maghrebi children living in Quebec to the countries of origin, Morocco, Algeria, and Tunisia. The annualized incidence rate in Quebec (18.49/year/100 000 children under 5 years of age) was 4 to 12 times higher than in Tunisia, Morocco, and Algeria (0.95, 4.52, and 3.15, respectively). The prevalence of incomplete diagnostic criteria was higher in Quebec at 39%, Morocco 43%, and Tunisia 39% compared to Algeria at 8%, with minimal delayed diagnosis (7%) only in Quebec compared to 30%, 35%, and 62%, respectively (P < .001). The rate of coronary aneurysms was comparable however (11% in Quebec vs 4%, 10%, and 25%, in Tunisia, Morocco, and Algeria, respectively; P = .31). The higher incidence of Kawasaki disease in the Maghreb community in Quebec versus the countries of origin seems due to underdiagnosis, which represents a public health concern in those countries. PMID:27336001

  4. Diffuse mesangial sclerosis associated with Kawasaki disease: an analysis of alpha chains (alpha 1-alpha 6) of human type IV collagen in the renal basement membrane.

    PubMed

    Joh, K; Kanetsuna, Y; Ishikawa, Y; Aizawa, S; Naito, I; Sado, Y

    1997-06-01

    A case of diffuse mesangial sclerosis (DMS) associated with Kawasaki disease is reported. A previously healthy Japanese girl, aged 4 months, presented with clinical features of Kawasaki disease. At week 10 of the illness, she developed the nephrotic syndrome, which was refractory to steroid therapy. Renal biopsy demonstrated a diffuse mesangial proliferative glomerulonephritis with microcystic tubular dilatation and, ultrastructurally, marked thinning of the lamina densa in the glomerular basement membrane (GBM) and the tubular basement membrane (TBM) of the proximal tubule. She went into chronic renal failure and died at the age of 11 months. At autopsy, the kidney revealed DMS. Histologically, we found Finnish microcystic disease in its early stages in the biopsy. Using a newly developed monoclonal antibody, we analysed the alpha chains (alpha 1-alpha 6) of type IV collagen in the GBM and TBM. There was no defective constitution of alpha chains on the thin GBM, but the thin TBM of the microcystic proximal tubule showed a weak or discontinuous reactivity for alpha 1 and alpha 2 chains, suggesting faulty formation of the basement membrane. The sclerosing glomeruli of the DMS did not depend on collapse of the GBM, which was positive for alpha 3-alpha 5 chains, but mainly on the proliferation of mesangial matrix, which was positive for alpha 1 and alpha 2 chains.

  5. Incidence Rate and Epidemiological and Clinical Aspects of Kawasaki Disease in Children of Maghrebi Origin in the Province of Quebec, Canada, Compared to the Country of Origin.

    PubMed

    Gorrab, Arbia Abir; Fournier, Anne; Bouaziz, Asma Abed; Spigelblatt, Linda; Scuccimarri, Rosie; Mrabet, Ali; Dahdah, Nagib

    2016-01-01

    The incidence of Kawasaki disease in Maghreb countries is apparently low, unlike those living in the province of Quebec, Canada. This retrospective study compared Maghrebi children living in Quebec to the countries of origin, Morocco, Algeria, and Tunisia. The annualized incidence rate in Quebec (18.49/year/100 000 children under 5 years of age) was 4 to 12 times higher than in Tunisia, Morocco, and Algeria (0.95, 4.52, and 3.15, respectively). The prevalence of incomplete diagnostic criteria was higher in Quebec at 39%, Morocco 43%, and Tunisia 39% compared to Algeria at 8%, with minimal delayed diagnosis (7%) only in Quebec compared to 30%, 35%, and 62%, respectively (P < .001). The rate of coronary aneurysms was comparable however (11% in Quebec vs 4%, 10%, and 25%, in Tunisia, Morocco, and Algeria, respectively; P = .31). The higher incidence of Kawasaki disease in the Maghreb community in Quebec versus the countries of origin seems due to underdiagnosis, which represents a public health concern in those countries.

  6. A Simple Method for Assessment of Human Anti-Neu5Gc Antibodies Applied to Kawasaki Disease

    PubMed Central

    Padler-Karavani, Vered; Tremoulet, Adriana H.; Yu, Hai; Chen, Xi; Burns, Jane C.; Varki, Ajit

    2013-01-01

    N-Glycolylneuraminic acid (Neu5Gc) is an immunogenic sugar of dietary origin that metabolically incorporates into diverse native glycoconjugates in humans. Anti-Neu5Gc antibodies are detected in all human sera, though with variable levels and epitope-recognition profiles. These antibodies likely play a role in several inflammation-mediated pathologies including cardiovascular diseases and cancer. In cancer, they have dualistic and opposing roles, either stimulating or repressing disease, as a function of their dose, and some of these antibodies serve as carcinoma biomarkers. Thus, anti-Neu5Gc antibodies may signify risk of inflammation-mediated diseases, and changes in their levels could potentially be used to monitor disease progression and/or response to therapy. Currently, it is difficult to determine levels of anti-Neu5Gc antibodies in individual human samples because these antibodies recognize multiple Neu5Gc-epitopes. Here we describe a simple and specific method for detection and overall estimation of human anti-Neu5Gc antibodies. We exploit the difference between two mouse models that differ only by Neu5Gc-presence (wild-type) or Neu5Gc-absence (Cmah−/− knockout). We characterize mouse serum from both strains by HPLC, lectin and mass-spectrometry analysis and show the target Neu5Gc-epitopes. We then use Cmah−/− knockout sera to inhibit all non-Neu5Gc-reactivity followed by binding to wild-type sera to detect overall anti-Neu5Gc response in a single assay. We applied this methodology to characterize and quantify anti-Neu5Gc IgG and IgA in sera of patients with Kawasaki disease (KD) at various stages compared to controls. KD is an acute childhood febrile disease characterized by inflammation of coronary arteries that untreated may lead to coronary artery aneurysms with risk of thrombosis and myocardial infarction. This estimated response is comparable to the average of detailed anti-Neu5Gc IgG profile analyzed by a sialoglycan microarray. Both assays

  7. Treatment response in Kawasaki disease is associated with sialylation levels of endogenous but not therapeutic intravenous immunoglobulin G.

    PubMed

    Ogata, Shohei; Shimizu, Chisato; Franco, Alessandra; Touma, Ranim; Kanegaye, John T; Choudhury, Biswa P; Naidu, Natasha N; Kanda, Yutaka; Hoang, Long T; Hibberd, Martin L; Tremoulet, Adriana H; Varki, Ajit; Burns, Jane C

    2013-01-01

    Although intravenous immunoglobulin (IVIG) is highly effective in Kawasaki disease (KD), mechanisms are not understood and 10-20% of patients are treatment-resistant, manifesting a higher rate of coronary artery aneurysms. Murine models suggest that α2-6-linked sialic acid (α2-6Sia) content of IVIG is critical for suppressing inflammation. However, pro-inflammatory states also up-regulate endogenous levels of β-galactoside:α2-6 sialyltransferase-I (ST6Gal-I), the enzyme that catalyzes addition of α2-6Sias to N-glycans. We asked whether IVIG failures correlated with levels of α2-6Sia on infused IVIG or on the patient's own endogenous IgG. We quantified levels of α2-6Sia in infused IVIG and endogenous IgG from 10 IVIG-responsive and 10 resistant KD subjects using multiple approaches. Transcript levels of ST6GAL1, in patient whole blood and B cell lines were evaluated by RT-PCR. Plasma soluble (s)ST6Gal-I levels were measured by ELISA. There was no consistent difference in median sialylation levels of infused IVIG between groups. However, α2-6Sia levels in endogenous IgG, ST6GAL1 transcript levels, and ST6Gal-I protein in serum from IVIG-resistant KD subjects were lower than in responsive subjects at both pre-treatment and one-year time points (p <0.001, respectively). Our data indicate sialylation levels of therapeutic IVIG are unrelated to treatment response in KD. Rather, lower sialylation of endogenous IgG and lower blood levels of ST6GALI mRNA and ST6Gal-I enzyme predict therapy resistance. These differences were stable over time, suggesting a genetic basis. Because IVIG-resistance increases risk of coronary artery aneurysms, our findings have important implications for the identification and treatment of such individuals.

  8. [Changes in peripheral blood T helper 9 cells and interleukin-9 in children in the acute stage of Kawasaki disease].

    PubMed

    Sun, Rui-Li; Zhu, Shu-Xia; Zhang, Yan-Yan; Wu, Yi-Fei; Wang, Xing-Jian

    2016-08-01

    To investigate the changes in the expression levels of peripheral blood T helper 9 (Th9) cells and cytokine interleukin-9 (IL-9) in children in the acute stage of Kawasaki disease (KD) and their clinical significance. A total of 45 children in the acute stage of KD who were treated from April 2014 to July 2015 were enrolled, and the children were followed up in the recovery stage. Another 45 healthy children who underwent physical examination were enrolled as the control group. Flow cytometry was used to measure the percentage of peripheral blood Th9 cells, and ELISA was used to measure the serum level of IL-9. The children in the acute stage of KD showed a significantly higher percentage of Th9 cells and a significantly higher serum level of IL-9 compared with those in the recovery stage and the control group (P<0.05). The percentage of Th9 cells and serum level of IL-9 showed no significant differences between the children in the recovery stage and those in the control group (P>0.05). In the acute stage, the percentage of Th9 cells was positively correlated with the levels of IL-9, C-reactive protein (CRP), procalcitonin (PCT), erythrocyte sedimentation rate (ESR), platelet count (PLT), and globulin (r=0.624, 0.324, 0.402, 0.382, 0.467, and 0.386 respectively, all P<0.05), but negatively correlated with serum albumin (r=-0.306, P<0.05). The serum level of IL-9 was positively correlated with the levels of CRP, PCT, ESR, PLT, and globulin (r=0.365, 0.456, 0.403, 0.423, and 0.453 respectively, all P<0.05), but negatively correlated with serum albumin (r=-0.343, P<0.05). The children in the acute stage of KD show significant increases in the percentage of peripheral Th9 cells and serum cytokine IL-9 level, which return to normal in the recovery stage. In the acute stage of KD, the expression levels of Th9 and IL-9 are closely correlated with laboratory markers. The results suggest that Th9 cells and IL-9 play important roles in the pathogenesis and outcome of KD.

  9. Kawasaki Disease-Specific Molecules in the Sera Are Linked to Microbe-Associated Molecular Patterns in the Biofilms

    PubMed Central

    Murata, Kenji; Kanno, Shunsuke; Nishio, Hisanori; Saito, Mitsumasa; Tanaka, Tamami; Yamamura, Kenichiro; Sakai, Yasunari; Takada, Hidetoshi; Miyamoto, Tomofumi; Mizuno, Yumi; Ouchi, Kazunobu; Waki, Kenji; Hara, Toshiro

    2014-01-01

    Background Kawasaki disease (KD) is a systemic vasculitis of unknown etiology. The innate immune system is involved in its pathophysiology at the acute phase. We have recently established a novel murine model of KD coronary arteritis by oral administration of a synthetic microbe-associated molecular pattern (MAMP). On the hypothesis that specific MAMPs exist in KD sera, we have searched them to identify KD-specific molecules and to assess the pathogenesis. Methods We performed liquid chromatography-mass spectrometry (LC-MS) analysis of fractionated serum samples from 117 patients with KD and 106 controls. Microbiological and LC-MS evaluation of biofilm samples were also performed. Results KD samples elicited proinflammatory cytokine responses from human coronary artery endothelial cells (HCAECs). By LC-MS analysis of KD serum samples collected at 3 different periods, we detected a variety of KD-specific molecules in the lipophilic fractions that showed distinct m/z and MS/MS fragmentation patterns in each cluster. Serum KD-specific molecules showed m/z and MS/MS fragmentation patterns almost identical to those of MAMPs obtained from the biofilms formed in vitro (common MAMPs from Bacillus cereus, Yersinia pseudotuberculosis and Staphylococcus aureus) at the 1st study period, and from the biofilms formed in vivo (common MAMPs from Bacillus cereus, Bacillus subtilis/Bacillus cereus/Yersinia pseudotuberculosis and Staphylococcus aureus) at the 2nd and 3rd periods. The biofilm extracts from Bacillus cereus, Bacillus subtilis, Yersinia pseudotuberculosis and Staphylococcus aureus also induced proinflammatory cytokines by HCAECs. By the experiments with IgG affinity chromatography, some of these serum KD-specific molecules bound to IgG. Conclusions We herein conclude that serum KD-specific molecules were mostly derived from biofilms and possessed molecular structures common to MAMPs from Bacillus cereus, Bacillus subtilis, Yersinia pseudotuberculosis and Staphylococcus

  10. Kawasaki disease patients with redness or crust formation at the Bacille Calmette-Guérin inoculation site.

    PubMed

    Uehara, Ritei; Igarashi, Hiroshi; Yashiro, Mayumi; Nakamura, Yosikazu; Yanagawa, Hiroshi

    2010-05-01

    A specific diagnostic test for Kawasaki disease (KD) is currently unavailable. Redness or crust formation at the Bacille Calmette-Guérin (BCG) inoculation site is listed as a positive sign in the diagnostic guidelines of KD. The purpose of this study was to investigate the epidemiologic features of KD patients with such changes at the BCG inoculation site and to evaluate the specificity of this sign in KD diagnosis. Data on KD patients who received BCG vaccination were analyzed from a Japanese nationwide epidemiologic survey on KD conducted in 2007. Patients who had 5 or 6 principal signs (complete cases) with redness or crust formation at the BCG inoculation site were compared by sex, year of hospital visit, day of first hospital visit, recurrent status, and presence of KD in siblings. To evaluate the specificity of the sign for KD diagnosis, patients aged 2 years or younger who were diagnosed as having respiratory syncytial virus or rotavirus infection using a commercial rapid test and who required hospitalization were observed. Of the 15,524 KD patients with a history of BCG vaccination, 7745 (49.9%) had redness or crust formation at the BCG inoculation site. This was observed in more than 70% of complete KD patients aged 3 to 20 months. Of these patients, the proportion with this sign in the group whose first day of hospital visit was within 1 to 4 days from the onset was significantly larger than that of the other patients groups (5-9 or 10+ days) (52.1%, P < 0.001). Among the patients with respiratory syncytial virus or rotavirus infection, none showed these changes at BCG inoculation site. Redness or crust formation at the BCG inoculation site is a useful diagnostic sign for KD among children aged 3 to 20 months in countries with a BCG vaccination program. Even if patients have 4 or fewer signs of the clinical criteria for KD, physicians should consider that patients with redness or crust formation at the BCG inoculation site could suffer from KD.

  11. Application of prospective ECG-triggered dual-source CT coronary angiography for infants and children with coronary artery aneurysms due to Kawasaki disease

    PubMed Central

    Duan, Y; Wang, X; Cheng, Z; Wu, D; Wu, L

    2012-01-01

    Objectives The aim of this study was to prospectively evaluate the initial application and value of prospective electrocardiogram (ECG)-triggered dual-source CT coronary angiography (DSCTCA) in the diagnosis of infants and children with coronary artery aneurysms due to Kawasaki disease. Methods 19 children [12 males; mean age 13.47 months, range 3 months to 5 years; mean heart rate 112 beats per minute (bpm), range 83–141 bpm] underwent prospective ECG-triggered DSCTCA with free breathing. Subjective image quality was assessed on a five-point scale (1, excellent; 5, non-diagnostic) by two blinded observers. The location, number and size of each aneurysm were observed and compared with those of transthoracic echocardiography (TTE) performed within 1 week. Interobserver agreement concerning the subjective image quality was evaluated with Cohen's κ-test. Bland–Altman analysis was used to evaluate the agreement on measurements (diameter and length of aneurysms) between DSCTCA and TTE. The average effective dose required for DSCTCA was calculated for all children. Results All interobserver agreement for subjective image quality assessment was excellent (κ=0.87). The mean±standard deviation (SD) aneurysm diameter with DSCTCA was 0.76±0.36 cm and with TTE was 0.76±0.39 cm. The mean±SD aneurysm length with DSCTCA was 2.06±1.35 cm and with TTE was 2.00±1.22 cm. The Bland–Altman plot for agreement between DSCTCA and TTE measurements showed good agreement. The mean effective dose was 0.36±0.06 mSv. Conclusion As an alternative diagnostic modality, prospective ECG-triggered DSCTCA with excellent image quality and low radiation exposure has been proved useful for diagnosing infants and children with coronary artery aneurysms due to Kawasaki disease. Advances in knowledge Prospective ECG–triggered DSCTCA for infants and children allows rapid, accurate assessment of coronary aneurysms due to Kawasaki diseases, compared with TTE. PMID:22932064

  12. Use of Lagrangian transport models and Sterilized High Volume Sampling to pinpoint the source region of Kawasaki disease and determine the etiologic agent

    NASA Astrophysics Data System (ADS)

    Curcoll Masanes, Roger; Rodó, Xavier; Anton, Jordi; Ballester, Joan; Jornet, Albert; Nofuentes, Manel; Sanchez-Manubens, Judith; Morguí, Josep-Anton

    2015-04-01

    Kawasaki disease (KD) is an acute, coronary artery vasculitis of young children, and still a medical mystery after more than 40 years. A former study [Rodó et al. 2011] demonstrated that certain patterns of winds in the troposphere above the earth's surface flowing from Asia were associated with the times of the annual peak in KD cases and with days having anomalously high numbers of KD patients. In a later study [Rodó et al. 2014], we used residence times from an Air Transport Model to pinpoint the source region for KD. Simulations were generated from locations spanning Japan from days with either high or low KD incidence. In order to cope with stationarity of synoptic situations, only trajectories for the winter months, when there is the maximum in KD cases, were considered. Trajectories traced back in time 10 days for each dataset and location were generated using the flexible particle Lagrangian dispersion model (FLEXPART Version 8.23 [Stohl et al. 2005]) run in backward mode. The particles modeled were air tracers, with 10,000 particles used on each model run. The model output used was residence time, with an output grid of 0.5° latitude × longitude and a time resolution of 3 h. The data input used for the FLEXPART model was gridded atmospheric wind velocity from the European Center for Medium-Range Weather Forecasts Re-Analysis (ERA-Interim at 1°). Aggregates of winter period back-trajectories were calculated for three different regions of Japan. A common source of wind air masses was located for periods with High Kawasaki disease. Knowing the trajectories of winds from the air transport models, a sampling methodology was developed in order to capture the possible etiological agent or other tracers that could have been released together. This methodology is based on the sterilized filtering of high volumes of the transported air at medium tropospheric levels by aircraft sampling and a later analyze these filters with adequate techniques. High purity

  13. Catheter-based anatomic and functional assessment of coronary arteries in anomalous aortic origin of a coronary artery, myocardial bridges and Kawasaki disease.

    PubMed

    Qureshi, Athar M; Agrawal, Hitesh

    2017-06-13

    Most diagnostic testing in patients with anomalous aortic origins of coronary arteries, myocardial bridges, and coronary artery changes after Kawasaki disease are performed with the use of noninvasive techniques. In some cases, however, further diagnostic information is needed to guide the clinician in treating these patients. In such instances, cardiac catheterization with invasive anatomic and functional testing is an invaluable tool. Moreover, interventional treatment in the cardiac catheterization laboratory may be performed in a small subset of these patients. As the diagnosis of these conditions is now becoming more common, it is important for pediatric interventional cardiologists to be familiar with these techniques. In this article, the role of angiography, intravascular ultrasound, fractional flow reserve, and optical coherence tomography in these patients is reviewed. © 2017 Wiley Periodicals, Inc.

  14. High regression rate of coronary aneurysms developed in patients with immune globulin-resistant Kawasaki disease treated with steroid pulse therapy.

    PubMed

    Adachi, Shinya; Sakaguchi, Heima; Kuwahara, Takashi; Uchida, Yasushi; Fukao, Toshiyuki; Kondo, Naomi

    2010-04-01

    Kawasaki disease is an acute vasculitis syndrome of unknown etiology that mainly affects small and medium-size arteries, particularly the coronary artery. Coronary artery lesions may develop into aneurismal formation and thrombotic occlusion, and progress to ischemic heart disease. The aim of the present study was to investigate the effect of steroid pulse therapy following intravenous immune globulin (IVIG) treatment on the regression rate of aneurysms in Kawasaki disease. Among 93 sequential patients referred to us, because of coronary artery lesions in the acute phase, we found 23 aneurysms in 12 patients during the period from January 1997 to January 2008. We divided them into two groups: a non-steroid group, 7 patients (13 aneurysms) treated with single or multiple IVIG but no steroid pulse therapy; and a steroid group, 5 patients (10 aneurysms) treated with multiple IVIG followed by steroid pulse therapy. We compared the regression rate of the aneurysms between the two groups, retrospectively. The regression rates of the aneurysms in the steroid group were significantly higher than those in the non-steroid group when we analyzed 1) all aneurysms (p = 0.007), 2) giant aneurysms (aneurismal diameter was 4 or more x normal, or > 8 mm) (p = 0.018), and 3) aneurysms in IVIG-resistant patients who were resistant to initial IVIG therapy (p = 0.035). All aneurysms, including the giant aneurysms in the steroid group, regressed, and the regression rate of the aneurysms in the non-steroid group was about 46%(6/13). Steroid pulse therapy may be beneficial for IVIG-resistant patients. Our data suggest that steroid pulse therapy may lead to regression of aneurysms.

  15. Estimation of the incidence of Kawasaki disease in Taiwan. A comparison of two data sources: nationwide hospital survey and national health insurance claims.

    PubMed

    Lue, Hung-Chi; Chen, Lei-Ru; Lin, Ming-Tai; Chang, Luan-Yin; Wang, Jou-Kou; Lee, Chin-Yun; Wu, Mei-Hwan

    2014-04-01

    Kawasaki disease (KD), first described by Dr. Tomisaku Kawasaki in 1967, was found for the first time in Taiwan in 1976. It continued to occur in increased numbers. For the study of incidence rates and epidemiological features of KD, we conducted five nationwide hospital surveys (NHS) in 1987, 1992, 1994, 2001 and 2008, respectively. We estimated also the annual incidence rates of KD during 1996-2007, based on the National Health Insurance (NHI) database, which had been implemented since 1995, covering 98% of the population in Taiwan. The annual incidence rates of KD during the twelve years, from1996 to 2007, estimated by the NHS and the NHI claims were compared, analyzed and discussed. During 1996-2007, a total of 9,938 cases of KD were reported by the Departments of Pediatrics of all hospitals surveyed, and a total of 11,849 cases of KD were claimed in the NHI database. The annual number of cases and incidence rates of KD based on NHI claims constantly surpassed those by the NHS. The ratio of the two incidence rates varied from 1.10 to 1.33. They were well correlated (r = 0.902, p < 0.001) with a linear equation, NHI = 16.07 + 0.93*NHS. The changes in annual incidence rate by the NHI were mean 1.149, p = 0.07, 95% CI -0.082 - 2.382, and those by the NHS were mean 1.562, p <0.001, CI 0.656 - 2.468. The annual incidence rates of KD can be estimated by the NHI claims and by the classic NHS. The values estimated by the NHI claims constantly outnumbered those by the NHS. Some pitfalls involved in the NHI claims are discussed. Copyright © 2013. Published by Elsevier B.V.

  16. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association.

    PubMed

    McCrindle, Brian W; Rowley, Anne H; Newburger, Jane W; Burns, Jane C; Bolger, Anne F; Gewitz, Michael; Baker, Annette L; Jackson, Mary Anne; Takahashi, Masato; Shah, Pinak B; Kobayashi, Tohru; Wu, Mei-Hwan; Saji, Tsutomu T; Pahl, Elfriede

    2017-04-25

    Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries. To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and long-term outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up. These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances. © 2017 American Heart Association, Inc.

  17. Borrowing Basics. FDIC Money Smart Financial Education Curriculum = Conceptos Basicos sobre Prestamos. FDIC Money Smart Plan de Educacion para Capacitacion en Finanzas.

    ERIC Educational Resources Information Center

    Federal Deposit Insurance Corp., Washington, DC.

    This module, an introduction to credit, is one of ten in the Money Smart curriculum, and includes an instructor guide and a take-home guide. It was developed to help adults outside the financial mainstream enhance their money skills and create positive banking relationships. It is designed to enable participants to decide when and how to use…

  18. Check It Out. FDIC Money Smart Financial Education Curriculum = Conceptos Basicos sobre Cuentas Corientes. FDIC Money Smart Plan de Educacion para Capacitacion en Finanzas.

    ERIC Educational Resources Information Center

    Federal Deposit Insurance Corp., Washington, DC.

    This module on how to choose and keep a checking account is one of ten in the Money Smart curriculum, and includes an instructor guide and a take-home guide. It was developed to help adults outside the financial mainstream enhance their money skills and create positive banking relationships. It is designed to enable participants to open and keep a…

  19. Sobre la terapia génica para enfermedades de la retina.

    PubMed

    Fischer, M Dominik

    2017-07-11

    Las mutaciones en un gran número de genes provocan degeneración de la retina y ceguera sin que exista actualmente cura alguna. En las últimas décadas, la terapia génica para enfermedades de la retina ha evolucionado y se ha convertido en un nuevo y prometedor paradigma terapéutico para estas enfermedades poco comunes. Este artículo refleja las ideas y los conceptos que parten de la ciencia básica hacia la aplicabilidad de la terapia génica en el ámbito clínico. Se describen los avances y las reflexiones actuales sobre la eficacia de los ensayos clínicos en la actualidad y se discuten los posibles obstáculos y soluciones de cara al futuro de la terapia génica para enfermedades de la retina. © 2017 S. Karger AG, Basel.

  20. Effect of dalteparin, a low-molecular-weight heparin, as adjunctive therapy in patients with Kawasaki disease: a retrospective study

    PubMed Central

    2014-01-01

    Background Dalteparin, a low-molecular-weight heparin, has anticoagulant and anti-angiogenic activity. This study investigated whether dalteparin reduced coronary artery lesion (CAL) prevalence, and resistance to intravenous immunoglobulin (IVIG) therapy in Kawasaki disease (KD). Methods This retrospective study comprised two parts. In the first cohort, 126 patients with KD (68 male, 58 female; median age: 22 months, range: 1–67 months) admitted to Nihon University Nerima-Hikarigaoka Hospital from January 2004 to June 2008, received either dalteparin 75 IU/kg/day, IVIG 400 mg/kg/day for 5 consecutive days, and aspirin 30 mg/kg/day, or dalteparin 75 IU/kg/day and aspirin 30 mg/kg/day, until clinical improvement. Control data came from the 2005–6 Nationwide KD survey. In the second cohort, 112 patients with KD (59 male, 53 female; median age: 19 months, range: 1–66 months) admitted from June 2010 to February 2012, received either dalteparin 75 IU/kg/day, IVIG 2.0 g/kg over 12 h, and aspirin 30 mg/kg/day, or dalteparin 75 IU/kg/day and aspirin 30 mg/kg/day. Control data came from the 2009–10 Nationwide KD survey. No patients enrolled in the nationwide surveys received dalteparin. All patients at our institution were given dalteparin in their combination therapy. Results A comparison of the first cohort with controls in the nationwide survey showed that the prevalence of initial administration of IVIG was 80.2% versus 86.0%; the rate of additional IVIG administration was 7.1% versus 14.0% (p = 0.03); CAL prevalence in the acute period was 4.8% versus 11.9% (p < 0.01); and the prevalence of cardiovascular sequelae was 0% versus 3.8% (p < 0.05). A comparison of the second cohort with controls in the nationwide survey showed that the rate of initial administration of IVIG was 92.9% versus 89.5%; the rate of additional IVIG administration was 8.9% versus 17.1% (p = 0.02); the prevalence of resistance to IVIG was 3.6% versus 14.9% (p

  1. Echocardiographic Evaluation of Coronary Abnormalities and Cardiac Function in a Murine Model of Kawasaki Disease Using High-frequency Ultrasound.

    PubMed

    Zhang, Xin-Xin; Du, Zhong-Dong; Wen, Shang-Guan; Sun, Xiu-Ping

    2017-06-20

    Murine model of coronary arterial inflammation has been widely accepted as an animal model of and used in Kawasaki disease (KD). This study sought to evaluate the developmental changes of coronary arteries and cardiac function in a murine model of KD with a high-frequency ultrasound system and to provide evidence for the preparation of the model of KD. Lactobacillus casei cell wall extract was prepared and injected into C57BL/6 mice intraperitoneally (i.p.) to induce KD. A total of 120 mice were grouped into three groups. The intravenous immunoglobulin (IVIG) treatment group was i.p. injected with IVIG (2 g/kg), while the KD model and normal control groups were i.p. injected with 0.5 ml of phosphate buffered solution on day 5. All high-resolution echocardiography detection of mouse heart was performed by the same senior technician. Animal echocardiography was performed by measuring the coronary artery dimensions and cardiac function on days 0, 7, 14, 28, and 56 (high-resolution small animal ultrasound [Vevo770 pattern; VisualSonic, Canada] with broadband probe [RMVTM707B; frequency, 30 mHz; depth of focus, 1.2 cm]) which were measured and analyzed with Vevo770 software. Pathological studies revealed focal inflammatory infiltrate asymmetrically distributed around the coronary artery trunk in the KD model group. Echocardiographic study including coronary dimension and cardiac function measurements was successfully performed in all subjects. The KD model and IVIG treatment groups showed left coronary artery dilation on days 7, 14, 28, and 56. The diameter of left coronary artery in the KD model group (0.53 ± 0.09 mm; 0.36 ± 0.07 mm; 0.34 ± 0.05 mm; 0.34 ± 0.04 mm) was significantly larger than those of IVIG treatment group (0.22 ± 0.02 mm; 0.28 ± 0.03 mm; 0.26 ± 0.03 mm; 0.27 ± 0.05 mm; 0.26 ± 0.03 mm; all P < 0.01) and the normal control group (0.21 ± 0.02 mm; 0.22 ± 0.03 mm; 0.22 ± 0.02 mm; 0.23 ± 0.02 mm; 0.27 ± 0.04 mm; all P< 0.01) on days 7, 14, 28

  2. Kawasaki Disease (For Parents)

    MedlinePlus

    ... dry, cracked lips swollen tongue with a white coating and big red bumps (called "strawberry tongue") sore, ... take you to a site outside of KidsHealth's control. About KidsHealth Nemours.org Reading BrightStart! Contact Us ...

  3. Elevated Inducible Nitric Oxide Levels and Decreased Hydrogen Sulfide Levels Can Predict the Risk of Coronary Artery Ectasia in Kawasaki Disease.

    PubMed

    Song, Ruixia; Liu, Guiying; Li, Xiaohui; Xu, Wenya; Liu, Jia; Jin, Hongfang

    2016-02-01

    Kawasaki disease (KD) is a vasculitis disease in children that is associated with coronary artery ectasia (CAE). We investigated whether inducible nitric oxide synthase (i-NOS) and hydrogen sulfide (H2S) could be used to predict CAE secondary to KD. We enrolled 65 children with KD (35 cases with CAE and 30 cases without CAE), 33 healthy children, and 32 children with fever but without vasculitis disease (febrile group). We measured plasma nitric oxide (NO), total nitric oxide synthase (Total-NOS), i-NOS, constructive nitric oxide synthase (c-NOS) levels, and H2S content in all patients. Plasma NO, Total-NOS, i-NOS, and H2S were higher in KD children than in healthy and febrile children (P < 0.05). The i-NOS level was higher in KD children with CAE compared to those without CAE, while the H2S was lower (both P < 0.05). Using a combination of i-NOS (higher than 10 U/mL) and H2S (lower than 3.31 μmol/L) to predict CAE had 80 % sensitivity and 81 % specificity (P < 0.05). Elevated plasma i-NOS and decreased plasma H2S levels in the acute phase of KD have good predictive value for CAE and may be used to guide appropriate clinical treatment and prevent future cardiovascular complications.

  4. Genetic variants in PLCB4/PLCB1 as susceptibility loci for coronary artery aneurysm formation in Kawasaki disease in Han Chinese in Taiwan.

    PubMed

    Lin, Ying-Ju; Chang, Jeng-Sheng; Liu, Xiang; Tsang, Hsinyi; Chien, Wen-Kuei; Chen, Jin-Hua; Hsieh, Hsin-Yang; Hsueh, Kai-Chung; Shiao, Yi-Tzone; Li, Ju-Pi; Lin, Cheng-Wen; Lai, Chih-Ho; Wu, Jer-Yuarn; Chen, Chien-Hsiun; Lin, Jaung-Geng; Lin, Ting-Hsu; Liao, Chiu-Chu; Huang, Shao-Mei; Lan, Yu-Ching; Ho, Tsung-Jung; Liang, Wen-Miin; Yeh, Yi-Chun; Lin, Jung-Chun; Tsai, Fuu-Jen

    2015-10-05

    Kawasaki disease (KD) is an acute, inflammatory, and self-limited vasculitis affecting infants and young children. Coronary artery aneurysm (CAA) formation is the major complication of KD and the leading cause of acquired cardiovascular disease among children. To identify susceptible loci that might predispose patients with KD to CAA formation, a genome-wide association screen was performed in a Taiwanese KD cohort. Patients with both KD and CAA had longer fever duration and delayed intravenous immunoglobulin treatment time. After adjusting for these factors, 100 susceptibility loci were identified. Four genes were identified from a single cluster of 35 using the Ingenuity Pathway Analysis (IPA) Knowledge Base. Silencing KCNQ5, PLCB1, PLCB4, and PLCL1 inhibited the effect of lipopolysaccharide-induced endothelial cell inflammation with varying degrees of proinflammatory cytokine expression. PLCB1 showed the most significant inhibition. Endothelial cell inflammation was also inhibited by using a phospholipase C (PLC) inhibitor. The single nucleotide polymorphism rs6140791 was identified between PLCB4 and PLCB1. Plasma PLC levels were higher in patients with KD and CC+CG rs6140791genotypes, and these genotypes were more prevalent in patients with KD who also had CAA. Our results suggest that polymorphism of the PLCB4/B1 genes might be involved in the CAA pathogenesis of KD.

  5. Genetic variants in PLCB4/PLCB1 as susceptibility loci for coronary artery aneurysm formation in Kawasaki disease in Han Chinese in Taiwan

    PubMed Central

    Lin, Ying-Ju; Chang, Jeng-Sheng; Liu, Xiang; Tsang, Hsinyi; Chien, Wen-Kuei; Chen, Jin-Hua; Hsieh, Hsin-Yang; Hsueh, Kai-Chung; Shiao, Yi-Tzone; Li, Ju-Pi; Lin, Cheng-Wen; Lai, Chih-Ho; Wu, Jer-Yuarn; Chen, Chien-Hsiun; Lin, Jaung-Geng; Lin, Ting-Hsu; Liao, Chiu-Chu; Huang, Shao-Mei; Lan, Yu-Ching; Ho, Tsung-Jung; Liang, Wen-Miin; Yeh, Yi-Chun; Lin, Jung-Chun; Tsai, Fuu-Jen

    2015-01-01

    Kawasaki disease (KD) is an acute, inflammatory, and self-limited vasculitis affecting infants and young children. Coronary artery aneurysm (CAA) formation is the major complication of KD and the leading cause of acquired cardiovascular disease among children. To identify susceptible loci that might predispose patients with KD to CAA formation, a genome-wide association screen was performed in a Taiwanese KD cohort. Patients with both KD and CAA had longer fever duration and delayed intravenous immunoglobulin treatment time. After adjusting for these factors, 100 susceptibility loci were identified. Four genes were identified from a single cluster of 35 using the Ingenuity Pathway Analysis (IPA) Knowledge Base. Silencing KCNQ5, PLCB1, PLCB4, and PLCL1 inhibited the effect of lipopolysaccharide-induced endothelial cell inflammation with varying degrees of proinflammatory cytokine expression. PLCB1 showed the most significant inhibition. Endothelial cell inflammation was also inhibited by using a phospholipase C (PLC) inhibitor. The single nucleotide polymorphism rs6140791 was identified between PLCB4 and PLCB1. Plasma PLC levels were higher in patients with KD and CC+CG rs6140791genotypes, and these genotypes were more prevalent in patients with KD who also had CAA. Our results suggest that polymorphism of the PLCB4/B1 genes might be involved in the CAA pathogenesis of KD. PMID:26434682

  6. Changes of clinical characteristics and outcomes in patients with Kawasaki disease over the past 7 years in a single center study.

    PubMed

    Kang, Hye Jin; Kim, Gee Na; Kil, Hong Ryang

    2013-09-01

    This single-center study was conducted to assess the changes in epidemiological and clinical characteristics and outcomes of patients with Kawasaki disease (KD) over the past 7 years. This retrospective study included 135 children with KD, admitted to Chungnam National University Hospital, Daejeon, between 2004 and 2005 (group A, n=53) and between 2011 and 2012 (group B, n=82). Medical records were reviewed to obtain information regarding the presenting signs and symptoms, demographic characteristics, and laboratory and echocardiographic findings associated with KD. The hospital admission date after onset was significantly earlier in group B than in group A (P=0.008). The proportion of patients with incomplete KD was 45.3% and 65.9% in group A and B, respectively (P=0.018). The number of pretreatment coronary artery lesions (CALs) were significantly lesser in group B than in group A. (10/53 vs. 5/82, P=0.021). No significant differences was observed in the incidence of CALs at discharge, febrile phase duration, hospital stay duration, incidence of retreatment, and intravenous immunoglobulin dose between 2 groups. The total febrile phase was shorter in patients with incomplete KD than in those with complete KD in both groups. The proportion of incomplete KD has become higher. Furthermore, early admission and management of patients with KD may be related to increased incomplete KD and decreased CALs. Therefore, we believe that a diagnostic strategy for incomplete KD should be established regardless of the presence of coronary lesions.

  7. Enhancement of activated beta1-integrin expression by prostaglandin E2 via EP receptors in isolated human coronary arterial endothelial cells: implication for the treatment of Kawasaki disease.

    PubMed

    Kajimoto, M; Ichiyama, T; Ueno, Y; Shiraishi, M; Hasegawa, M; Furukawa, S

    2009-04-01

    Plasma prostaglandin E(2) (PGE(2)) levels are markedly elevated in acute Kawasaki disease (KD). We evaluated the function of the EP receptors in the expression of activated beta(1)-integrin stimulated by PGE(2) in human coronary arterial endothelial cells (HCAEC). We determined the mRNA expression of the PGE(2) receptors, EP receptors (EP(1-4)) in HCAEC by RT-PCR and protein expression by Western blotting. We evaluated the function of the EP receptors in the expression of activated beta(1)-integrin stimulated by PGE(2) in HCAEC, using antagonists and agonists of the EP receptors, by flow cytometry. RT-PCR revealed mRNAs for all four EP receptors in HCAEC. Western blotting demonstrated EP(1), EP(2) and EP(3) expression in HCAEC. The EP(2) and EP(3) agonists enhanced the expression of activated beta(1)-integrin in HCAEC. The potency of the EP(2) agonist was significantly greater than that of the EP(3) agonist. Pretreatment with the EP(1), EP(2) and EP(3) antagonists inhibited the expression of activated beta(1)-integrin induced by PGE(2) in HCAEC. The potency of the EP(2) antagonist was significantly greater than that of the EP(1) and EP(3) antagonists. Our results suggest that PGE(2) mainly induces the activation of beta(1)-integrins via the EP(2) receptor in HCAEC. Our results further suggest that the EP(2) antagonist modulates the inflammatory response during KD vasculitis.

  8. Evaluation of longitudinal and radial left ventricular functions using two- and three-dimensional echocardiography before and after intravenous immunoglobulin administration in patients with acute Kawasaki disease.

    PubMed

    Hashimoto, Ikuo; Saitou, Yu; Sakata, Nao; Shibata, Ko

    2017-09-11

    The relationship between left ventricular (LV) function and longitudinal or radial contraction has not yet been elucidated in patients with acute Kawasaki disease (KD), especially before and after intravenous immunoglobulin (IVIG) administration. We studied 28 KD patients without coronary aneurysms (average 3.2 years). The LV end-diastolic volume (LVEDV), end-systolic volume (LVESV), stroke volume (LVSV), and ejection fraction (LVEF) were assessed using three-dimensional echocardiography before IVIG, after IVIG administration, and in the convalescent phase. LV fractional shortening (LVFS) and the mitral annular systolic excursion (MAPSE) z-score were measured as surrogates for radial and longitudinal LV wall motions, respectively. Serum brain natriuretic peptide (BNP) was also assessed as a heart failure indicator in acute KD patients. In all KD patients, the LVEDV decreased in the acute phase with preservation of the LVESV. Both the LVSV and the MAPSE z-score were lowest before IVIG and increased after IVIG. The MAPSE z-score was highly correlated with LVEF before and after IVIG administration. Although there was a good correlation between the Log BNP and LVEF before IVIG, it was lost after IVIG. the LVEDV decreased longitudinally during the acute phase of KD with preservation of the LVESV, reducing both the LVSV and LVEF. The serum BNP level is a useful marker for evaluating LV function only prior to IVIG administration. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  9. Genetic variations in the receptor-ligand pair CCR5 and CCL3L1 are important determinants of susceptibility to Kawasaki disease.

    PubMed

    Burns, Jane C; Shimizu, Chisato; Gonzalez, Enrique; Kulkarni, Hemant; Patel, Sukeshi; Shike, Hiroko; Sundel, Robert S; Newburger, Jane W; Ahuja, Sunil K

    2005-07-15

    Kawasaki disease (KD) is an enigmatic, self-limited vasculitis of childhood that is complicated by development of coronary-artery aneurysms. The high incidence of KD in Asian versus European populations prompted a search for genetic polymorphisms that are differentially distributed among these populations and that influence KD susceptibility. Here, we demonstrate a striking, inverse relationship between the worldwide distribution of CCR5- Delta 32 allele and the incidence of KD. In 164 KD patient-parent trios, 4 CCR5 haplotypes including the CCR5- Delta 32 allele were differentially transmitted from heterozygous parents to affected children. However, the magnitude of the reduced risk of KD associated with the CCR5- Delta 32 allele and certain CCR5 haplotypes was significantly greater in individuals who also possessed a high copy number of the gene encoding CCL3L1, the most potent CCR5 ligand. These findings, derived from the largest genetic study of any systemic vasculitis, suggest a central role of CCR5-CCL3L1 gene-gene interactions in KD susceptibility and the importance of gene modifiers in infectious diseases.

  10. Correlation between brachial artery flow-mediated dilation and endothelial microparticle levels for identifying endothelial dysfunction in children with Kawasaki disease.

    PubMed

    Ding, Yue-Yue; Ren, Yan; Feng, Xing; Xu, Qiu-Qin; Sun, Ling; Zhang, Jian-Min; Dou, Ji-Juan; Lv, Hai-Tao; Yan, Wen-Hua

    2014-03-01

    We investigated vascular endothelial dysfunction by sonographic features of flow-mediated dilation (FMD) and circulating endothelial microparticles (EMPs) in Kawasaki disease (KD). Twenty-eight patients with KD were prospectively grouped according to stage of disease: acute, subacute, and convalescent. In addition, 28 healthy children and 28 febrile children were selected as controls. And cases in the convalescent phase were divided into two subgroups: coronary artery lesion (CAL) and no coronary lesion (NCAL). CD144(+)/CD42b(-), CD62E(+), and CD105(+) EMPs were measured by flow cytometry; FMD was obtained by sonography. There were significant differences in FMD among the five groups. When compared with healthy controls, there were significantly greater numbers of CD144(+)/CD42b(-), CD62E(+), and CD105(+) EMPs and a higher proportion of CD62E(+) EMPs in KD patients. The proportions and numbers of CD144(+)/CD42b(-), CD62E(+), and CD105(+) EMPs in KD patients were not statistically different than in febrile controls. There were no significant differences in FMD and EMPs between the CAL and NCAL subgroups. There were significantly negative correlations between the values of FMD and EMPs in the three phases of KD. The increased levels of EMPs have significant correlation with decreased values of FMD, both of which may reflect endothelial dysfunction in child KD.

  11. Correlation of HAMP gene polymorphisms and expression with the susceptibility and length of hospital stays in Taiwanese children with Kawasaki disease

    PubMed Central

    Lu, Hsing-Fang; Wong, Henry Sung-Ching; Yu, Hong-Ren; Kuo, Hsing-Chun; Huang, Fu-Chen; Lo, Mao-Hung; Hsieh, Kai-Sheng; Chen, Su-Fen; Chang, Wei-Chiao; Kuo, Ho-Chang

    2017-01-01

    Kawasaki disease (KD) is a form of systemic vasculitis. Regarding its pathogenesis, HAMP gene encoding hepcidin, which is significant for iron metabolism, has a vital function. In this study, we recruited a total of 381 KD patients for genotyping. Data from 997 subjects (500 subjects from cohort 1; 497 subjects from cohort 2) were used for analysis. Using TaqMan allelic discrimination, we determined five tag SNPs (rs916145, rs10421768, rs3817623, rs7251432, and rs2293689). Treatment outcome data related to such clinical phenotypes as coronary artery lesions (CAL), coronary artery aneurysms (CAA), and intravenous immunoglobulin (IVIG) effects were also collected. Furthermore, we measured plasma hepcidin levels with an enzyme-linked immunosorbent assay. We found that HAMP gene polymorphism (rs7251432, and rs2293689) was significantly correlated with KD risk and that plasma hepcidin levels both before and after IVIG treatment had a significantly positive correlation with length of hospital stays (R = 0.217, p = 0.046 and R = 0.381, p < 0.0001, respectively). In contrast, plasma hepcidin levels has a negative correlation with KD patients’ albumin levels (R = −0.27, p < 0.001) prior to IVIG treatment. This study's findings indicate that HAMP might have a role in the disease susceptibility, as well as its expressions correlated length of hospital stays, and albumin levels in Taiwanese children with KD. PMID:28881695

  12. Shape effects on the cluster spreading process of spin-crossover compounds analyzed within an elastic model with Eden and Kawasaki dynamics

    NASA Astrophysics Data System (ADS)

    Enachescu, Cristian; Nishino, Masamichi; Miyashita, Seiji; Boukheddaden, Kamel; Varret, François; Rikvold, Per Arne

    2015-03-01

    In this paper we study the growth properties of domains of low-spin molecules in a high-spin background in open-boundary elliptically shaped spin-crossover systems within the framework of a mechanoelastic model. The molecules are situated on a triangular lattice and are linked by springs, through which they interact. Elliptical shapes are chosen in order to allow an in-depth analysis of cluster shapes as a function of the local curvature at their starting point and the length of the interface between the two phases. In contrast to the case of rectangular and hexagonal shapes, where the clusters always start from corners, we find that for ellipses clusters nucleate from vertices, covertices, or any other site. We apply and compare two kinds of dynamics, Eden-like and Kawasaki, in order to determine the stable shape of the clusters and the most probable starting points. We show that the wetting angle for small clusters is somewhat higher than π /2 and approaches this value only for large clusters. The stability of clusters is analyzed by comparing the Gibbs free energy to the elastic energy in the system and is discussed as a function of the cluster size, curvature of the starting place, and temperature.

  13. Comprehensive pathogen detection associated with four recurrent episodes of Kawasaki disease in a patient during a single year using next-generation sequencing

    PubMed Central

    Sekizuka, Tsuyoshi; Oba, Kunihiro; Katano, Harutaka; Kinumaki, Akiko; Terai, Masaru; Mizutani, Tetsuya; Kuroda, Makoto

    2016-01-01

    Introduction: Kawasaki disease (KD) is the most common multisystem vasculitis in childhood. Pathogens can be associated with the onset of KD. However, a lack of consistency prevails among reports about this disease. Case presentation: For this case of a 1-year-old boy with four recurrent episodes of KD within a year, we analysed profiles of pathogen reads in his serum and pharynx specimens using next-generation sequencing. Comparative analysis of the identified bacterial reads from serum samples found significant correlation of bacteria such as Streptococcus and Haemophilus spp. with the first and fourth episodes (R2 = 0.9506) before treatment. In the first convalescent phase, the number of Streptococcus spp. was reduced remarkably (P < 0.0001). From sequencing of the pharynx specimen from the fourth episode, a similar correlation was found with serum from the fourth episode (R2 = 0.6633). Conclusion: In this case, Streptococcus spp. may have been associated with onset of KD. Further studies must be undertaken to evaluate the putative association of micro-organism infection with KD pathogenesis. PMID:28348750

  14. High-density Genotyping of Immune Loci in Kawasaki Disease and IVIG Treatment Response in European-American Case-parent Trio Study

    PubMed Central

    Shendre, Aditi; Wiener, Howard W.; Zhi, Degui; Vazquez, Ana I; Portman, Michael A.; Shrestha, Sadeep

    2014-01-01

    Kawasaki disease (KD) is a diffuse and acute small-vessel vasculitis observed in children and has genetic and autoimmune components. We genotyped 112 case-parent trios of European decent (confirmed by AIMS) using the ImmunoChip array and performed association analyses with susceptibility to KD and IVIG non-response. KD susceptibility was assessed using the transmission disequilibrium test whereas IVIG non-response was evaluated using multivariable logistic regression analysis. We replicated SNPs in three gene regions (FCGR, CD40/CDH22, and HLA-DQB2/HLA-DOB) that have been previously associated with KD and provide support to other findings of several novel SNPs in genes with potential pathway in KD pathogenesis. SNP rs838143 in the 3′ UTR of FUT1 gene (2.7×10-5) and rs9847915 in the intergenic region of LOC730109 ∣ BRD7P2 (6.81×10-7) were the top hits for KD susceptibility in additive and dominant models, respectively. The top hits for IVIG responsiveness were rs1200332 in the intergenic region of BAZ1A ∣ C14orf19 (1.4×10-4) and rs4889606 in the intron of the STX1B gene (6.95×10-5) in additive and dominant models, respectively. Our study suggests that genes and biological pathways involved in autoimmune diseases play an important role in the pathogenesis of KD and IVIG response mechanism. PMID:25101798

  15. ST-elevation myocardial infarction in a young adult secondary to giant coronary aneurysm thrombosis: an important sequela of Kawasaki disease and a management challenge.

    PubMed

    Potter, Elizabeth L; Meredith, Ian T; Psaltis, Peter James

    2016-01-20

    Thrombosis of a coronary artery aneurysm (CAA) is a rare trigger for ST-elevation myocardial infarction (STEMI) and an important cause of STEMI in young adults previously affected by Kawasaki disease. Initial management should proceed in line with standard STEMI-management guidelines advocating antiplatelet medication and emergency coronary angiography. Acute CAA thrombosis presents the interventional cardiologist with unique challenges during attempted percutaneous revascularisation. In the absence of consensus guidelines, experiential reporting can therefore be of great value. We report on a 36-year-old Vietnamese woman presenting with an inferior STEMI secondary to two giant thrombosed aneurysms of the right coronary artery. Coronary wiring and thrombus aspiration temporarily improved coronary flow but recurrent thrombus with distal embolisation resulted in ventricular fibrillation and cardiogenic shock. Emergency surgical revascularisation subsequently provided a definitive and successful outcome. We discuss the challenges of percutaneous coronary intervention in this scenario and review previous reports to give an overview of principles of decision-making and management. 2016 BMJ Publishing Group Ltd.

  16. Identification of KCNN2 as a susceptibility locus for coronary artery aneurysms in Kawasaki disease using genome-wide association analysis.

    PubMed

    Kim, Jae-Jung; Park, Young-Mi; Yoon, Dankyu; Lee, Kyung-Yil; Seob Song, Min; Doo Lee, Hyoung; Kim, Kwi-Joo; Park, In-Sook; Nam, Hyo-Kyoung; Weon Yun, Sin; Ki Han, Myung; Mi Hong, Young; Young Jang, Gi; Lee, Jong-Keuk

    2013-08-01

    Kawasaki disease (KD) is often complicated by coronary artery lesions (CALs), including aneurysms. Because of the complications associated with KD, this disorder is the leading cause of acquired heart disease in children from developed countries. To identify genetic loci that confer a higher risk of developing CALs, we performed a case-control association study using previous genome-wide association study data for samples from KD cases only (n=186) by grouping KD patients without CALs (control: n=123) vs KD patients with extremely large aneurysms (diameter>5 mm) (case: n=17). Twelve loci with one or more sequence variants were found to be significantly associated with CALs (P<1 × 10(-5)). Of these, an SNP (rs17136627) in the potassium intermediate/small conductance calcium-activated channel, subfamily N, member 2 (KCNN2) at 5q22.3 was validated in 32 KD patients with large aneurysms (diameter>5 mm) and 191 KD patients without CALs (odds ratio (OR)=12.6, P(combined)=1.96 × 10(-8)). This result indicates that the KCNN2 gene can have an important role in the development of coronary artery aneurysms in KD.

  17. Discussions about the Nature of Science in a Course on the History of Astronomy. (Spanish Title: Discusiones sobre la Naturaleza de la Ciencia en un Curso sobre Historia de la Astronomía.) Discussões sobre a Natureza da Ciência em um Curso sobre a História da Astronomia

    NASA Astrophysics Data System (ADS)

    Pires de Andrade, Victória Flório; L'Astorina, Bruno

    2010-07-01

    There are an increasing number of researches in science education that affirm the importance of discussions on the "nature of science" in basic education level as well as in teacher training. The history of science applied to education is a way to contextualize epistemological discussions, allowing both the understanding of scientific content and learning about science concepts. We present some reasonably consensual definitions on the nature of science that have been widely discussed by the academic community. We show also some episodes in the history of astronomy which can lead to discussions involving some aspects of the nature of science, and how they can do it. Hay un número creciente de investigaciones en la enseñanza de las ciencias que afirman la importancia de debates sobre la "naturaleza de la ciencia" en la educación básica y formación del profesorado. La historia de la ciencia aplicada a la educación es una manera de contextualizar los debates de la epistemología, lo que permite tanto la comprensión de los contenidos científicos como el aprendizaje de conceptos científicos. En esto trabajo, presentamos algunas definiciones bastante consensuales sobre la naturaleza de la ciencia que han sido ampliamente discutidas por la comunidad académica y mostramos cómo algunos episodios en la historia de la astronomía pueden llevar a discusiones sobre algunos aspectos de la naturaleza de la ciencia. Há um número crescente de pesquisas na área de ensino de ciências que afirmam a importância de discussões sobre a "natureza da ciência" na educação básica e na formação de professores. A história da ciência aplicada ao ensino é uma maneira de contextualizar discussões epistemológicas, permitindo tanto a compreensão de conteúdos científicos quanto o aprendizado de noções sobre as ciências. Neste trabalho apresentamos algumas definições razoavelmente consensuais sobre a natureza da ciência que foram amplamente discutidas pela

  18. Mortality among persons with a history of Kawasaki disease in Japan: can paediatricians safely discontinue follow-up of children with a history of the disease but without cardiac sequelae?

    PubMed

    Nakamura, Yosikazu; Aso, Eiko; Yashiro, Mayumi; Uehara, Ritei; Watanabe, Makoto; Tajimi, Morihiro; Oki, Izumi; Ojima, Toshiyuki; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2005-04-01

    To clarify the question of whether patients with Kawasaki disease suffer a higher mortality rate after the incidence of the disease in comparison with age-matched healthy individuals. Between July 1982 and December 1992, 52 collaborating hospitals collected data on all patients having a new, definite diagnosis of Kawasaki disease. Patients were followed up until 31 December 2001 or their death. The expected number of deaths was calculated from Japanese vital statistics data and compared with the observed number. Of 6576 patients enrolled, 29 (20 males and 9 females) died. The standardized mortality ratio (SMR: the observed number of deaths divided by the expected number of deaths based on the vital statistics in Japan) was 1.15 (95% CI: 0.77-1.66). In spite of the high SMRs during the acute phase, the mortality rate was not high after the acute phase for the entire group of patients. Although the SMR after the acute phase was 0.75 for those without cardiac sequelae, six males (but none of the females) with cardiac sequelae died during this period; and the SMR for the male group with cardiac sequelae was 1.95 (95% CI: 0.71-4.25). The mortality from congenital anomalies of the circulatory system was elevated, but no increase in cancer deaths was observed. Although it was not statistically significant, the mortality rate among males with cardiac sequelae due to Kawasaki disease appeared to be higher than in the general population. On the other hand, the mortality rates for females with the sequelae and both males and females without sequelae were not elevated.

  19. Concepciones y concepciones alternativas de estudiantes universitarios/as de biologia y futuros maestros/as de Ciencia de escuela secundaria sobre la teoria de evolucion biologica por seleccion natural

    NASA Astrophysics Data System (ADS)

    Morales Ramos, Egda M.

    La teoria de evolucion biologica (TEB) por seleccion natural es uno de los conceptos unificadores mas importantes del curriculo de Biologia. En Puerto Rico se han hecho pocas investigaciones que abunden sobre las concepciones y concepciones alternativas (CA) que tienen los estudiantes universitarios/as de Biologia y los maestros/as de Ciencia del nivel secundario sobre esta teoria. La politica publica educativa actual establece mediante documentos normativos como los Estandares de contenido y Expectativas de grado del Programa de Ciencias [Puerto Rico Core Standards] la ensenanza de esta teoria. Sin embargo, no se encontraron preguntas sobre la seleccion natural en los ejercicios de practica provistos por el Departamento de Educacion para las pruebas estandarizadas lo cual puede influir para que no se ensene adecuadamente. Las preguntas de investigacion fueron 1. ¿Cuales son las concepciones y concepciones alternativas de estudiantes universitarios/as y de los futuros maestros y maestras de Ciencia sobre la TEB? 2. ¿Cuales conceptos que seleccionan los estudiantes universitarios/as y los futuros maestros y maestras de Ciencia sobre la TEB coinciden con lo aceptado como valido por la comunidad cientifica? y 3. ¿Como comparan las respuestas de la prueba original. v. Entendiendo el cambio biologico que mide concepciones y CA sobre la TEB por seleccion natural, con las de la traducida al idioma espanol? Se utilizo el metodo cuantitativo con un diseno de investigacion transversal por encuesta. La tecnica principal para recopilar los datos fue una prueba con doce items, que formo parte de un instrumento para el cual se recopilaron diversas fuentes de evidencia acerca de su validez. Las muestras estuvieron formadas por 69 estudiantes de Ciencias Naturales y por 16 estudiantes futuros maestros y maestras del nivel secundario de la UPR-RP. Se utilizaron estadisticas descriptivas, analisis de Ji cuadrado y se calcularon los coeficientes alfa de Cronbach y de Spearman

  20. Histo-blood group gene polymorphisms as potential genetic modifiers of the development of coronary artery lesions in patients with Kawasaki disease.

    PubMed

    Yamamura, K; Ihara, K; Ikeda, K; Nagata, H; Mizuno, Y; Hara, T

    2012-04-01

    Abnormal immunological responses to certain microbial agents may play a crucial role in the pathogenesis of Kawasaki disease (KD). The association studies between histo-blood group genes (Lewis and ABO blood types) and various types of infectious diseases or vasculopathy have been carried out based on the fact that glycosylated antigens could directly mediate microbial infections. We attempted to clarify the role of blood type antigens in the development of KD and coronary artery lesions in KD patients. The subjects included 164 KD patients enrolled from 1998 to 2003 (1st group), 232 patients from 2004 to 2009 (2nd group), and 223 healthy children and 118 patients with growth hormone deficiency as controls. The genotyping of the FUT2 and FUT3 genes, and ABO genotypes, was determined with the TaqMan SNP assay and allele-specific polymerase chain reaction. No significant differences were observed in the genotypes and allele frequencies of the FUT2 and FUT3 genes between the groups. The frequency of the BB blood genotype was significantly higher in KD patients with coronary artery lesions in the 1st and 2nd groups than in the controls (17% and 14% vs. 5%, P = 0.0020). This is the first report to investigate the roles of ABO and Lewis blood types in the development of KD, and in the formation of coronary artery lesions in KD patients. These data suggest that the ABO blood type may play a role in the development of coronary artery lesions in KD patients.

  1. CD8+ T Cells Contribute to the Development of Coronary Arteritis in the Lactobacillus casei Cell Wall Extract-Induced Murine Model of Kawasaki Disease.

    PubMed

    Noval Rivas, Magali; Lee, Youngho; Wakita, Daiko; Chiba, Norika; Dagvadorj, Jargalsaikhan; Shimada, Kenichi; Chen, Shuang; Fishbein, Michael C; Lehman, Thomas J A; Crother, Timothy R; Arditi, Moshe

    2017-02-01

    Kawasaki disease (KD) is the leading cause of acquired heart disease among children in developed countries. Coronary lesions in KD in humans are characterized by an increased presence of infiltrating CD3+ T cells; however, the specific contributions of the different T cell subpopulations in coronary arteritis development remain unknown. Therefore, we sought to investigate the function of CD4+ and CD8+ T cells, Treg cells, and natural killer (NK) T cells in the pathogenesis of KD. We addressed the function of T cell subsets in KD development by using a well-established murine model of Lactobacillus casei cell wall extract (LCWE)-induced KD vasculitis. We determined which T cell subsets were required for development of KD vasculitis by using several knockout murine strains and depleting monoclonal antibodies. LCWE-injected mice developed coronary lesions characterized by the presence of inflammatory cell infiltrates. Frequently, this chronic inflammation resulted in complete occlusion of the coronary arteries due to luminal myofibroblast proliferation (LMP) as well as the development of coronary arteritis and aortitis. We found that CD8+ T cells, but not CD4+ T cells, NK T cells, or Treg cells, were required for development of KD vasculitis. The LCWE-induced murine model of KD vasculitis mimics many histologic features of the disease in humans, such as the presence of CD8+ T cells and LMP in coronary artery lesions as well as epicardial coronary arteritis. Moreover, CD8+ T cells functionally contribute to the development of KD vasculitis in this murine model. Therapeutic strategies targeting infiltrating CD8+ T cells might be useful in the management of KD in humans. © 2016, American College of Rheumatology.

  2. Changes of clinical characteristics and outcomes in patients with Kawasaki disease over the past 7 years in a single center study

    PubMed Central

    Kang, Hye Jin; Kim, Gee Na

    2013-01-01

    Purpose This single-center study was conducted to assess the changes in epidemiological and clinical characteristics and outcomes of patients with Kawasaki disease (KD) over the past 7 years. Methods This retrospective study included 135 children with KD, admitted to Chungnam National University Hospital, Daejeon, between 2004 and 2005 (group A, n=53) and between 2011 and 2012 (group B, n=82). Medical records were reviewed to obtain information regarding the presenting signs and symptoms, demographic characteristics, and laboratory and echocardiographic findings associated with KD. Results The hospital admission date after onset was significantly earlier in group B than in group A (P=0.008). The proportion of patients with incomplete KD was 45.3% and 65.9% in group A and B, respectively (P=0.018). The number of pretreatment coronary artery lesions (CALs) were significantly lesser in group B than in group A. (10/53 vs. 5/82, P=0.021). No significant differences was observed in the incidence of CALs at discharge, febrile phase duration, hospital stay duration, incidence of retreatment, and intravenous immunoglobulin dose between 2 groups. The total febrile phase was shorter in patients with incomplete KD than in those with complete KD in both groups. Conclusion The proportion of incomplete KD has become higher. Furthermore, early admission and management of patients with KD may be related to increased incomplete KD and decreased CALs. Therefore, we believe that a diagnostic strategy for incomplete KD should be established regardless of the presence of coronary lesions. PMID:24223600

  3. Increased frequency of immunoglobulin (Ig)A-secreting cells following Toll-like receptor (TLR)-9 engagement in patients with Kawasaki disease

    PubMed Central

    Giordani, L; Quaranta, M G; Marchesi, A; Straface, E; Pietraforte, D; Villani, A; Malorni, W; Del Principe, D; Viora, M

    2011-01-01

    Kawasaki disease (KD) is an acute vasculitis affecting mainly infants and children. Human B cells express Toll-like receptor (TLR)-9, whose natural ligands are unmethylated cytosine–guanine dinucleotide (CpG) motifs characteristic of bacterial DNA. The aim of this study was to clarify the pathogenesis of KD analysing the activation status of peripheral blood mononuclear cells (PBMC), focusing on B lymphocyte activation and functions. Ten patients and 10 age-matched healthy donors were recruited from the Bambino Gesù Hospital of Rome, Italy and enrolled into this study. We determined phenotype profile and immunoglobulin (Ig) production of PBMC from KD patients and age-matched controls. We found that the frequency of CD19+ B lymphocytes and CD19+/CD86+ activated B lymphocytes from KD patients during the acute phase before therapy was increased significantly. Moreover, B lymphocytes of acute-phase KD patients were more prone to CpG oligodeoxynucleotide (ODN) activation compared with the age-matched controls, as assessed by a significant increase of the number of IgA-secreting cells (SC). In the same patients we found a marked increase of IgM, IgG, interleukin (IL)-6 and tumour necrosis factor (TNF)-α production compared with the control group. In addition, in two convalescent KD patients, conventional treatment with intravenous immunoglobulin (IVIG) restored the normal frequency of CD19+ B cells, the number of IgA-, IgM- and IgG-SC and the production of IL-6 and TNF-α. Our findings indicate that the percentages of peripheral B lymphocytes of acute-phase KD patients are increased and are prone to bacterial activation in terms of increased numbers of IgA-SC and increased production of IL-6 and TNF-α inflammatory cytokines. Thus, our data support the hypothesis of an infectious triggering in KD. PMID:21175593

  4. Increased frequency of immunoglobulin (Ig)A-secreting cells following Toll-like receptor (TLR)-9 engagement in patients with Kawasaki disease.

    PubMed

    Giordani, L; Quaranta, M G; Marchesi, A; Straface, E; Pietraforte, D; Villani, A; Malorni, W; Del Principe, D; Viora, M

    2011-03-01

    Kawasaki disease (KD) is an acute vasculitis affecting mainly infants and children. Human B cells express Toll-like receptor (TLR)-9, whose natural ligands are unmethylated cytosine-guanine dinucleotide (CpG) motifs characteristic of bacterial DNA. The aim of this study was to clarify the pathogenesis of KD analysing the activation status of peripheral blood mononuclear cells (PBMC), focusing on B lymphocyte activation and functions. Ten patients and 10 age-matched healthy donors were recruited from the Bambino Gesù Hospital of Rome, Italy and enrolled into this study. We determined phenotype profile and immunoglobulin (Ig) production of PBMC from KD patients and age-matched controls. We found that the frequency of CD19(+) B lymphocytes and CD19(+) /CD86(+) activated B lymphocytes from KD patients during the acute phase before therapy was increased significantly. Moreover, B lymphocytes of acute-phase KD patients were more prone to CpG oligodeoxynucleotide (ODN) activation compared with the age-matched controls, as assessed by a significant increase of the number of IgA-secreting cells (SC). In the same patients we found a marked increase of IgM, IgG, interleukin (IL)-6 and tumour necrosis factor (TNF)-α production compared with the control group. In addition, in two convalescent KD patients, conventional treatment with intravenous immunoglobulin (IVIG) restored the normal frequency of CD19(+) B cells, the number of IgA-, IgM- and IgG-SC and the production of IL-6 and TNF-α. Our findings indicate that the percentages of peripheral B lymphocytes of acute-phase KD patients are increased and are prone to bacterial activation in terms of increased numbers of IgA-SC and increased production of IL-6 and TNF-α inflammatory cytokines. Thus, our data support the hypothesis of an infectious triggering in KD.

  5. Mycoplasma Infection as a cause of Persistent Fever after Intravenous Immunoglobulin Treatment of Patients with Kawasaki Disease: Frequency and Clinical Impact

    PubMed Central

    Yoon, Kyung Lim; Cha, Sung-Ho; Moon, Sung Kyoung; Jung, Hae Woon

    2017-01-01

    Background Mycoplasma is a common cause of respiratory infections and may require differential diagnosis from Kawasaki disease (KD). In this study, we investigated the frequency and clinical manifestations of mycoplasma infection in patients with KD. Materials and Methods Medical records of 375 in-patients admitted for treatment during the acute stage of KD, were collected, and reviewed retrospectively. Of these patients, 152 (40.5%) were also tested for recent mycoplasma infection. Patients with positive results (anti-mycoplasma IgM Ab >1:640 or cold agglutinin >1:64) were designated as the case group (n = 37, 24.3%) whereas those with negative results were designated as the control group (n = 115, 75.7%). Clinical findings of the two groups were compared. Results Patients in the case group were older than those in the control group (mean age, 48.2 ± 32.1 months, vs. 31.7 ± 21.7 months; P = 0.001). There were significant differences between the case and control groups in the changes in the extremities (78.3% vs. 57.4%, respectively; P = 0.031), and in fever duration (6.5 ± 2.5 days vs. 5.4 ± 1.5 days; P = 0.047). Of the 37 patients with positive mycoplasma testing, 7 (18.9%) had persistent fever even after the symptoms and signs of systemic inflammation (acute phase of KD) had been resolved. These patients were positive for mycoplasma infection during further evaluation of persistent fever, and all of them responded to macrolide antibiotics. Conclusions We found that mycoplasma infection is somewhat related to KD. When fever persists after resolution of the acute stage of KD, mycoplasma infection may be considered as a possible cause of fever in preschool-aged children. PMID:28271651

  6. Z-score of Mitral Annular Plane Systolic Excursion is a Useful Indicator of Evaluation of Left Ventricular Function in Patients with Acute-Phase Kawasaki Disease.

    PubMed

    Hashimoto, Ikuo; Watanabe, Kazuhiro

    2017-06-01

    We previously reported the clinical usefulness of the mitral annular plane systolic excursion (MAPSE) to evaluate the left ventricular (LV) function in patients with Kawasaki disease (KD) in the acute-phase. However, the feasibility of the MAPSE z-score has not been evaluated in patients with acute KD. We prospectively studied 60 KD patients without coronary aneurysms. The MAPSE z-scores were calculated using our standard MAPSE data. Brain natriuretic peptide (BNP) was measured as a parameter of LV function. In total, 281 healthy age- and body size-matched subjects were chosen as the control group. The MAPSE z-score decreased in the acute-phase (median value, -1.4) and increased in the convalescent phase (median value, 0.18; P < 0.0001). However, there was no significant difference in the MAPSE z-score between patients in the convalescent phase and the control patients (0.18 vs. 0.02, P = 0.199). Multivariate regression analysis revealed that BNP was an independent predictor of the MAPSE z-score (β = 0.40, P < 0.005). According to the receiver operating characteristic (ROC) analysis, the optimal cutoff value for the MAPSE z-score to judge LV dysfunction was -0.9. The MAPSE z-score is a useful index to evaluate LV function, and the cutoff value of -0.9 can be an indicator to judge LV dysfunction in the patients with acute-phase KD.

  7. Peripheral Endothelial (Dys)Function, Arterial Stiffness and Carotid Intima-Media Thickness in Patients after Kawasaki Disease: A Systematic Review and Meta-Analyses

    PubMed Central

    Dietz, Sanne M.; Tacke, Carline E. A.; Hutten, Barbara A.; Kuijpers, Taco W.

    2015-01-01

    Background Kawasaki disease (KD) is a systemic pediatric vasculitis. Its main complication is the development of coronary arterial aneurysms (CAA), causing an increased risk for ischemia and myocardial infarction. It is unclear whether KD patients, apart from the presence of CAA, have an increased cardiovascular disease (CVD) risk due to the previous systemic vasculitis. The aim of this study was to systematically review and meta-analyse the literature regarding surrogate markers for CVD risk in KD patients. Methods Medline and Embase were searched for articles comparing endothelial dysfunction (flow-mediated dilation, nitroglycerin-mediated dilation and peripheral arterial tonometry), vascular stiffness (stiffness index, pulse wave velocity) and carotid intima-media thickness (cIMT) between patients and controls. Two investigators assessed the articles for eligibility and evaluated quality. Results Thirty studies were included. For all outcomes, moderate to high heterogeneity between studies was found. Most studies reported a decreased flow-mediated dilation in the whole KD- and CAA-positive group compared to controls, while data on CAA-negative patients were conflicting. The stiffness index was increased in the majority of studies evaluating the whole KD- and CAA-positive group, but not in most studies on CAA-negative patients. Mean cIMT was neither significantly increased in the whole KD-group nor in the CAA-positive group nor in most studies studying CAA-negative patients. Studies measuring maximum cIMT were conflicting. Conclusion Literature suggests that surrogate markers for CVD risk in KD patients are increased in CAA-positive but not in CAA-negative patients. This may indicate that CAA-positive patients should be monitored for CVD in later life. The results of this review have to be interpreted with care due to substantial heterogeneity between studies and methodological limitations, as well as the lack of long-term follow-up studies. PMID:26161871

  8. Multicenter, Single-Arm, Phase IV Study of Combined Aspirin and High-Dose “IVIG-SN” Therapy for Pediatric Patients with Kawasaki Disease

    PubMed Central

    Yoon, Kyung Lim; Lee, Hae Yong; Yu, Jeong Jin; Lee, Jae Young; Han, Mi Young; Kim, Ki Yong

    2017-01-01

    Background and Objectives Intravenous immunoglobulin-SN (IVIG-SN) is a new human immunoglobulin product. Its safety is ensured by pathogen-elimination steps comprising solvent/detergent treatment and a nanofiltration process. This multicenter clinical study was designed to evaluate the efficacy and safety of combined aspirin and high-dose IVIG-SN therapy in pediatric patients with Kawasaki disease (KD). Subjects and Methods We evaluated coronary artery lesions (CALs) at 2 and 7 weeks after administering IVIG-SN; total fever duration; and variations in erythrocyte sedimentation rate, N-terminal pro B-type natriuretic peptide or B-type natriuretic peptide, and creatine kinase-myocardial band level before and after treatment with IVIG-SN (2 g/kg). Adverse events were monitored. Results Forty-five patients were enrolled, three of whom were excluded according to the exclusion criteria; the other 42 completed the study. The male:female ratio was 0.91:1, and the mean age was 29.11±17.23 months. The mean fever duration before IVIG-SN treatment was 6.45±1.30 days. Although most patients had complete KD (40 patients, 90.91%), four had atypical KD (9.09%). After IVIG-SN treatment, one patient (2.38%) had CALs, which was significantly lower than the incidence reported previously (15%) (p=0.022), but not significantly different from recent data (5%). There were no serious adverse events, though 28 patients (63.64%) had mild adverse events. Three adverse drug reactions occurred in 2 patients (eczema, anemia, and increased eosinophil count), all of which were transient. Conclusion IVIG-SN treatment in patients with KD was safe and effective. PMID:28382076

  9. Study on the relationship between mean platelet volume and platelet distribution width with coronary artery lesion in children with Kawasaki disease.

    PubMed

    Liu, Ruixi; Gao, Fang; Huo, Junming; Yi, Qijian

    2012-01-01

    Mean platelet volume (MPV) and platelet distribution width (PDW) are correlated with platelet function and may be a more sensitive index than platelet number as a marker of clinical interest in various disorders. Therefore, this study was designed to answer the following questions: do MPV and PDW levels change in Kawasaki disease (KD), is there any relation between CAL in children with MPV and PDW and whether MPV and PDW might support a diagnosis of incomplete KD. A total of 309 KD patients and 160 sex-age matched healthy subjects were enrolled into the study. For all subjects following tests were performed: MPV, PDW, platelet count, white blood cells counts (WBC), C reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Patients with CALs were assigned to three groups depending on the extent of CALs which were visualized by echocardiography: dilatation and/or ectasia, aneurysm and giant aneurysms. We compared patients with fever and four or five of the principal criteria (complete KD, cKD) to the other patients (iKD). Compared with healthy controls a significant decrease in MPV and PDW (p < 0.001 and p < 0.05, respectively) and increase in WBC, platelet count, CRP and ESR (p all < 0.001) was noted in children with KD. There were no statistically differences in MPV and PDW between KD with CALs and KD without CALs (p > 0.05). However, MPV and PDW were significantly lower in patients with iKD than in group with cKD (p = 0.003, p = 0.014, respectively). It was first shown that patients with KD have lower MPV and PDW than control subjects. The diagnosis of iKD is challenging but can be supported by the presence of lower MPV and PDW.

  10. Activated myeloid dendritic cells accumulate and co-localize with CD3+ T cells in coronary artery lesions in patients with Kawasaki disease.

    PubMed

    Yilmaz, Atilla; Rowley, Anne; Schulte, Danica J; Doherty, Terence M; Schröder, Nicolas W J; Fishbein, Michael C; Kalelkar, Mitra; Cicha, Iwona; Schubert, Katja; Daniel, Werner G; Garlichs, Christoph D; Arditi, Moshe

    2007-08-01

    Emerging evidence implicating the participation of dendritic cells (DCs) and T cells in various vascular inflammatory diseases such as giant cell arteritis, Takayasu's arteritis, and atherosclerosis led us to hypothesize that they might also participate in the pathogenesis of coronary arteritis in Kawasaki disease (KD). Coronary artery specimens from 4 patients with KD and 6 control patients were obtained. Immunohistochemical and computer-assisted histomorphometric analyses were performed to detect all myeloid DCs (S-100(+), fascin(+)), all plasmacytoid DCs (CD123(+)) as well as specific DC subsets (mature myeloid DCs [CD83(+)], myeloid [BDCA-1(+)] and plasmacytoid DC precursors [BDCA-2(+)]), T cells (CD3(+)), and all antigen-presenting cells (HLA-DR(+)). Co-localization of DCs with T cells was assessed using double immunostaining. Significantly more myeloid DCs at a precursor, immature or mature stage were found in coronary lesions of KD patients than in controls. Myeloid DC precursors were distributed equally in the intima and adventitia. Mature myeloid DCs were particularly abundant in the adventitia. There was a significant correlation between mature DCs and HLA-DR expression. Double immunostaining demonstrated frequent contacts between myeloid DCs and T cells in the outer media and adventitia. Plasmacytoid DC precursors were rarely found in the adventitia. In conclusion, coronary artery lesions of KD patients contain increased numbers of mature myeloid DCs with high HLA-DR expression and frequent T cell contacts detected immunohistochemically. This suggests that mature arterial myeloid DCs might be activating T cells in situ and may be a significant factor in the pathogenesis of coronary arteritis in KD.

  11. IL-1 signaling is critically required in stromal cells in Kawasaki Disease Vasculitis Mouse Model. Role of both IL-1α and IL-1β

    PubMed Central

    Lee, Youngho; Wakita, Daiko; Dagvadorj, Jargalsaikhan; Shimada, Kenichi; Chen, Shuang; Huang, Ganghua; Lehman, Thomas J.A.; Fishbein, Michael C.; Hoffman, Hal M.; Crother, Timothy R.; Arditi, Moshe

    2015-01-01

    Objective Kawasaki disease (KD) is the most common cause of acute vasculitis and acquired cardiac disease among US children. We have previously shown that both TLR2/MyD88 and IL-1β signaling are required for the Lactobacillus casei cell wall extract (LCWE)-induced KD vasculitis mouse model. The objectives of this study were to investigate the cellular origins of IL-1 production, the role of CD11c+ Dendritic Cells (DCs) and macrophages and the relative contribution of hematopoietic and stromal cells for IL-1 responsive cells, as well the MyD88 signaling in LCWE-induced KD mouse model of vasculitis. Approach and Results Using mouse knockout models as well as antibody depletion, we found that both IL-1α and IL-1β were required for LCWE-induced KD. Both DCs and macrophages were necessary and we found that MyD88 signaling was required in both hematopoietic and stromal cells. However, IL-1 response and signaling was critically required in non-endothelial stromal cells, but not hematopoietic cells. Conclusions Our results suggest that IL-1α and IL-1β as well as CD11c+ DCs and macrophages are essential for the development of KD vasculitis and coronary arteritis in this mouse model. Bone marrow chimera experiments suggest that MyD88 signaling is important in both hematopoietic and stromal cells, while IL-1 signaling and response is required only in stromal cells, but not in endothelial cells. Determining the role IL-1α and IL-1β and of specific cell types in the KD vasculitis mouse model may have important implications for the design of more targeted therapies and understanding of the molecular mechanisms of KD immunopathologies. PMID:26515418

  12. Replication and meta-analysis of GWAS identified susceptibility loci in Kawasaki disease confirm the importance of B lymphoid tyrosine kinase (BLK) in disease susceptibility.

    PubMed

    Chang, Chia-Jung; Kuo, Ho-Chang; Chang, Jeng-Sheng; Lee, Jong-Keuk; Tsai, Fuu-Jen; Khor, Chiea Chuen; Chang, Li-Ching; Chen, Shih-Ping; Ko, Tai-Ming; Liu, Yi-Min; Chen, Ying-Ju; Hong, Young Mi; Jang, Gi Young; Hibberd, Martin L; Kuijpers, Taco; Burgner, David; Levin, Michael; Burns, Jane C; Davila, Sonia; Chen, Yuan-Tsong; Chen, Chien-Hsiun; Wu, Jer-Yuarn; Lee, Yi-Ching

    2013-01-01

    The BLK and CD40 loci have been associated with Kawasaki disease (KD) in two genome-wide association studies (GWAS) conducted in a Taiwanese population of Han Chinese ancestry (Taiwanese) and in Japanese cohorts. Here we build on these findings with replication studies of the BLK and CD40 loci in populations of Korean and European descent. The BLK region was significantly associated with KD susceptibility in both populations. Within the BLK gene the rs2736340-located linkage disequilibrium (LD ) comprising the promoter and first intron was strongly associated with KD, with the combined results of Asian studies including Taiwanese, Japanese, and Korean populations (2,539 KD patients and 7,021 controls) providing very compelling evidence of association (rs2736340, OR = 1.498, 1.354-1.657; P = 4.74×10(-31)). We determined the percentage of B cells present in the peripheral blood mononuclear cell (PBMC) population and the expression of BLK in the peripheral blood leukocytes (leukocytes) of KD patients during the acute and convalescent stages. The percentage of B cells in the PBMC population and the expression of BLK in leukocytes were induced in patients in the acute stage of KD. In B cell lines derived from KD patients, and in purified B cells from KD patients obtained during the acute stage, those with the risk allele of rs2736340 expressed significantly lower levels of BLK. These results suggest that peripheral B cells play a pathogenic role during the acute stage of KD. Decreased BLK expression in peripheral blood B cells may alter B cell function and predispose individuals to KD. These associative data suggest a role for B cells during acute KD. Understanding the functional implications may facilitate the development of B cell-mediated therapy for KD.

  13. Infliximab is the new kid on the block in Kawasaki disease: a single-centre study over 8 years from North India.

    PubMed

    Singh, Surjit; Sharma, Dhrubajyoti; Suri, Deepti; Gupta, Anju; Rawat, Amit; Rohit, Manoj Kumar

    2016-01-01

    This was a single-centre study to evaluate the usefulness of tumour necrosis factor-α (TNF-α) blocker, infliximab (IFX), for treatment of Kawasaki disease (KD) in children in Northern Indian. The study was carried out in the Paediatric Allergy-Immunology Unit, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh. The study period was January 2007 to March 2015. Review of records of 23 children with KD who had received IFX was carried out. Median age at presentation was 2 years (range 2 months to 12 years). Indications for using IFX were intravenous immunoglobulin (IVIg) resistance (12/23 patients); severe KD especially when coronary artery abnormalities (CAAs) had developed in spite of IVIg (9/23 patients); retinal vasculitis in association with KD (1 patient) and economic reasons (1 patient). Twenty one (21/23) patients had received IVIg (2 g/kg) as first line therapy. A dose of IFX was 5-7 mg/kg given intravenously. Screening tests for tuberculosis (chest xray, Tuberculin test, QuantiFERON-TB Gold test) were not carried out prior to IFX infusion in any patient. Duration of follow-up was 0-20 months in 13 patients; 21-40 months in 5 patients and >40 months in 6 patients. Mean follow-up was 28.78±25.49 months, range 1-84 months. Eleven of 12 patients (11/12) who had IVIg resistance showed prompt resolution with IFX. Nineteen patients (19/23) in the cohort had CAAs. Of these, 12 showed improvement over mean follow-up of 28.78±25.49 months (range 1-84 months) and 4 showed normalisation. No adverse reactions were noted during infusion of IFX. On follow-up, none of these patients has developed tuberculosis or any other significant infection over a cummulative follow-up of 662 months. IFX can be considered as a useful adjunct in treatment of children with KD.

  14. Reverse regulation of soluble receptor for advanced glycation end products and proinflammatory factor resistin and S100A12 in Kawasaki disease

    PubMed Central

    2012-01-01

    Introduction Kawasaki disease (KD), an acute febrile disease, characterized by systemic vasculitis, predominantly affects infants and children under 5 years of age. Coronary artery lesions (CALs) are its most critical complication, and the etiology remains unknown yet. In order to explore the value of resistin, S100A12 and soluble receptor for advanced glycation end products (sRAGE) in the pathophysiology of KD, we studied the serum levels of resistin, S100A12 and sRAGE in different stages of KD. Methods Serum levels of resistin, S100A12 and sRAGE were measured by enzyme-linked immunosorbent assay (ELISA) method in 15 healthy children and 40 KD patients at acute, afebrile and subacute stage. Results The resistin and S100A12 levels, including the ratio of resistin to sRAGE and S100A12 to sRAGE increased significantly in the acute stage, and decreased progressively in the afebrile and subacute stage. However, the sRAGE levels decreased significantly in the acute stage, and increased progressively in the afebrile and subacute stage. In the acute, afebrile and subacute stage, the resistin levels were higher in intravenous immunoglobulin (IVIG) non-responders (0.64 ± 0.30, 0.48 ± 0.35, 0.28 ± 0.19, × 102 ng/ml) than in IVIG responders (0.35 ± 0.24, 0.21 ± 0.19, 0.12 ± 0.05, × 102 ng/ml). In the acute and subacute stage, the S100A12 levels were higher in IVIG non-responders (7.92 ± 2.61, 4.98 ± 4.75, × 102 ng/ml) than in IVIG responders (5.05 ± 3.22, 2.35 ± 2.26, × 102 ng/ml). In the afebrile and subacute stage, the sRAGE levels were lower in IVIG non-responders (3.51 ± 2.64, 3.65 ± 3.27, × 102 pg/ml) than in IVIG responders (6.00 ± 2.78, 7.19 ± 2.88, × 102 pg/ml). The resistin levels were positively correlated with S100A12 levels. The sRAGE levels were negatively related with S100A12 and resistin levels. Conclusions Resistin, S100A12 and sRAGE are involved in the pathophysiology of KD. PMID:23171632

  15. Expression of CD40 ligand on CD4+ T-cells and platelets correlated to the coronary artery lesion and disease progress in Kawasaki disease.

    PubMed

    Wang, Chih-Lu; Wu, Yu-Tsun; Liu, Chieh-An; Lin, Mei-Wei; Lee, Chia-Jung; Huang, Li-Tung; Yang, Kuender D

    2003-02-01

    Kawasaki disease (KD) is an acute febrile vasculitic syndrome in children. CD40 ligand (CD40L) has been implicated in certain types of vasculitis. We proposed that CD40L expression might be correlated with coronary artery lesions in KD. Blood samples were collected from 43 patients with KD before intravenous immunoglobulin (IVIG) treatment and 3 days afterward. Forty-three age-matched febrile children with various diseases were studied in parallel as controls. CD40L expression on T-cells and platelets were detected by flow cytometry, and soluble CD40L (sCD40L) levels were measured by enzyme-linked immunosorbent assay. We found that CD40L expression on CD4(+) T-cells was significantly higher in patients with KD than in the febrile control (FC) group (28.69 +/- 1.17% vs 4.37 +/- 0.36%). CD40L expression decreased significantly 3 days after IVIG administration (28.69 +/- 1.17% vs 13.53 +/- 0.55%). CD40L expression on platelets from patients with KD was also significantly higher than in the FC group (8.20 +/- 0.41% vs 1.26 +/- 0.12%) and decreased after IVIG therapy. sCD40L levels were also significantly higher in KD patients with those of FC (9.69 +/- 0.45 ng/mL vs 2.25 +/- 0.19 ng/mL) but were not affected by IVIG treatment 3 days afterward (9.69 +/- 0.45 ng/mL vs 9.03 +/- 0.32 ng/mL). More interesting, we found that in KD patients, CD40L expression on CD4(+) T-cells and platelets but not on CD8(+) T-cells or sCD40L was correlated with the occurrence of coronary artery lesions. CD40L might play a role in the immunopathogenesis of KD. IVIG therapy might downregulate CD40L expression, resulting in decrease of CD40L-mediated vascular damage in KD. This implicates that modulation of CD40L expression may benefit to treat KD vasculitis.

  16. Assessment of coronary ischaemia by myocardial perfusion dipyridamole stress technetium-99 m tetrofosmin, single-photon emission computed tomography, and coronary angiography in children with Kawasaki disease: pre- and post-coronary bypass grafting.

    PubMed

    Mostafa, Mostafa S; Sayed, Ashraf O; Al Said, Yasser M

    2015-06-01

    Coronary artery lesions in Kawasaki disease invasively assessed by coronary angiography. Evaluation of myocardial perfusion by single-photon emission computed tomography may identify the haemodynamic significance of coronary lesions. To evaluate diagnostic accuracy of dipyridamole stress technetium-99 m tetrofosmin, single-photon emission computed tomography as a possible alternative to invasive coronary angiography for detection and follow-up of myocardial ischaemia in patients with Kawasaki disease, and pre- and post-coronary bypass grafting. Coronary angiography and single-photon emission computed tomography were performed on 21 patients who were classified into three groups - group I (stenosis), group II (giant aneurysms), and group III (small aneurysms). Of the 21 patients, 16 (groups I and II) patients with myocardial perfusion defects, who underwent coronary bypass grafting, were followed up with single-photon emission computed tomography. In group I, all patients had significant coronary stenosis and 100% of them had perfusion defects in the anterior and septal walls. In group II, all patients had giant aneurysms and 83% of them had inferior and inferolateral perfusion defects. In group III, all patients had small aneurysms and 100% of them had normal perfusion. Pre-coronary bypass grafting myocardial ischaemic defects disappeared in all patients after surgery. Sensitivity, specificity, and accuracy of single-photon emission computed tomography were 94, 100, and 95%, respectively. Technetium-99 m tetrofosmin single-photon emission computed tomography can be applied as an accurate non-invasive diagnostic technique for detecting myocardial perfusion defects with coronary artery lesions, and to show improved or even normalised perfusion of the myocardium in patients after surgical revascularisation.

  17. Superantigen involvement and susceptibility factors in Kawasaki disease: profiles of TCR Vβ2+ T cells and HLA-DRB1, TNF-α and ITPKC genes among Filipino patients.

    PubMed

    Natividad, Magdalena F; Torres-Villanueva, Celia Aurora T; Saloma, Cynthia P

    2013-01-01

    Superantigens and genetic factors may play roles in the etiology and susceptibility to Kawasaki disease (KD). To investigate these roles, percentages of TCR-Vβ2+ T cells were compared by flow cytometry using anti-Vβ2 monoclonal antibodies and genotyping was done on HLA-DRB1 exon 2, the -308 site of the TNF-α promoter region, and ITPKC SNP rs28493229 by polymerase chain reaction followed by direct sequencing. There were higher percentages of Vβ2+ T-cells in KD patients (9.5 ± 2.15%) compared to healthy controls (7.25 ± 1.48%) (P<0.05, Student's t-test, n=6-8/group). However, no polymorphisms were observed in exon 2 of HLA-DRB1 and in the -308 region of the TNF-α promoter. The ITPKC SNP rs28493229 G/C polymorphism was observed in 1 KD patient and 4 healthy controls. This study suggests that KD etiology may be associated with a superantigen and that HLA-DRB1 exon2, TNF-α -308 region and ITPKC SNP rs28493229 may not be associated with KD. This is the first study investigating Vβ2+ T cells and candidate genes involvement among Filipino KD patients.

  18. Superantigen involvement and susceptibility factors in Kawasaki disease: profiles of TCR Vβ2+ T cells and HLA-DRB1, TNF-α and ITPKC genes among Filipino patients

    PubMed Central

    Natividad, Magdalena F; Torres-Villanueva, Celia Aurora T; Saloma, Cynthia P

    2013-01-01

    Superantigens and genetic factors may play roles in the etiology and susceptibility to Kawasaki disease (KD). To investigate these roles, percentages of TCR-Vβ2+ T cells were compared by flow cytometry using anti-Vβ2 monoclonal antibodies and genotyping was done on HLA-DRB1 exon 2, the -308 site of the TNF-α promoter region, and ITPKC SNP rs28493229 by polymerase chain reaction followed by direct sequencing. There were higher percentages of Vβ2+ T-cells in KD patients (9.5 ± 2.15%) compared to healthy controls (7.25 ± 1.48%) (P<0.05, Student’s t-test, n=6-8/group). However, no polymorphisms were observed in exon 2 of HLA-DRB1 and in the -308 region of the TNF-α promoter. The ITPKC SNP rs28493229 G/C polymorphism was observed in 1 KD patient and 4 healthy controls. This study suggests that KD etiology may be associated with a superantigen and that HLA-DRB1 exon2, TNF-α -308 region and ITPKC SNP rs28493229 may not be associated with KD. This is the first study investigating Vβ2+ T cells and candidate genes involvement among Filipino KD patients. PMID:23565324

  19. Repeated stent thrombosis after DES implantation and localized hypersensitivity to a stent implanted in the distal portion of a coronary aneurysm thought to be a sequela of Kawasaki disease: autopsy report.

    PubMed

    Yokouchi, Yuki; Oharaseki, Toshiaki; Ihara, Fumie; Naoe, Shiro; Sugawara, Shigetada; Takahashi, Kei

    2010-02-01

    The patient was a 40-year-old Japanese woman. At 37 years of age she underwent stent implantation in LAD#7 for an acute myocardial infarction. Subsequently, coronary intervention was performed four times because of occlusion of the stent. Sudden death occurred at 40 years of age due to ventricular tachycardia. Clinically, the patient had had no history of collagen disease, anti-phospholipid antibody syndrome or coagulation disorder. The autopsy revealed only very mild atherosclerotic changes in the aorta and various other organs, but concentric thickening of the intima was observed in all three branches of the coronary arteries. Also, aneurysms accompanied by calcification were observed at each of LAD #6, LCx #11 and RCA #4PD. The stent was occluded with a thrombus, and the vascular walls showed infiltration by lymphocytes, plasma cells and numerous eosinophils. The eosinophil infiltration was confined to the site of the stent. It was surmised that the patient had experienced late stent thrombosis due to a hypersensitivity reaction to the DES on the basis of a development of a state of high susceptibility to thrombus formation because of a coronary aneurysm. The aneurysm was suspected of being a post-inflammatory change of Kawasaki disease.

  20. Study protocol for a phase III multicentre, randomised, open-label, blinded-end point trial to evaluate the efficacy and safety of immunoglobulin plus cyclosporin A in patients with severe Kawasaki disease (KAICA Trial)

    PubMed Central

    Aoyagi, Reiko; Hamada, Hiromichi; Sato, Yasunori; Suzuki, Hiroyuki; Onouchi, Yoshihiro; Ebata, Ryota; Terauchi, Moe; Terai, Masaru; Hanaoka, Hideki; Hata, Akira

    2015-01-01

    Introduction Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown aetiology that predominantly affects infants and young children. We hypothesise that cyclosporin A (CsA) may be effective in treating KD by regulating the Ca2+/NFAT signalling pathway. This trial compares the current standard therapy of intravenous immunoglobulin (IVIG) and the combined IVIG+CsA therapy in paediatric patients with severe KD. Methods and analysis This trial is a phase III, multicentre, randomised, open-label, blinded-end point trial that evaluates the efficacy and safety of IVIG+CsA therapy. Patients with severe KD who satisfy the eligibility criteria are randomised (1:1) to receive either CsA (5 mg/kg/day for 5 days; Neoral) plus high-dose IVIG (2 g/kg for 24 h and aspirin 30 mg/kg/day), or high-dose IVIG alone (2 g/kg for 24 h and aspirin 30 mg/kg/day). The primary end point is the frequency of occurrence of coronary artery abnormalities during the trial period. An independent end point review committee will be in charge of the trial assessment. Ethics and dissemination The protocol was approved by the Institutional Review Board of each institution. The trial was notified and registered at the Pharmaceutical and Medical Devices Agency, in Japan. The trial is currently on-going and is scheduled to finish in April 2017. The findings will be disseminated through peer-reviewed publications and conference presentations. Trial registration number JMA-IIA00174; Pre-results. PMID:26628527

  1. A comparison of the predictive validity of the combination of the neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio and other risk scoring systems for intravenous immunoglobulin (ivig)-resistance in Kawasaki disease

    PubMed Central

    Kanai, Takashi; Kawamura, Yoichi; Yoshida, Yusuke; Nonoyama, Shigeaki

    2017-01-01

    Background We recently reported that the combination of the neutrophil-to-lymphocyte ratio (NLR) and the platelet-to-lymphocyte ratio (PLR) is a novel and useful predictor of intravenous immunoglobulin (IVIG)-resistance in Kawasaki disease (KD). In the present study, to evaluate the effectiveness of the new risk score, we compared its predictive validity to that of previously reported risk scores. Materials and methods The laboratory records of 437 patients with KD before IVIG therapy were retrospectively analyzed, and the IVIG-responsive (n = 344) and IVIG-resistant (n = 93) patients were compared. The validity of the new score (the combination of NLR≥3.83 and PLR≥150) for predicting IVIG resistance in KD was compared to that of the Kobayashi, Egami and Sano risk scores. Results The new score and the Kobayashi score displayed high sensitivity (0.72 and 0.70 respectively) and specificity (0.67 and 0.68 respectively), while the Egami and Sano scores showed high specificity (0.71 and 0.81 respectively) but relatively low sensitivity (0.56 and 0.45 respectively). The odds ratios (ORs) for the new score, the Kobayashi score, the Egami score and the Sano score were 5.34 (95% confidence interval [CI] 3.22–8.85), 4.87 (95% CI 2.96–8.01), 3.14 (95% CI 1.96–5.03) and 3.53 (95% CI 2.17–5.77) respectively. Conclusions The predictive validity of the combination of NLR≥3.83 and PLR≥150, which is a simple and convenient indicator, was equal to or higher than that of the other risk scores. This suggests that the new score could be a widely available marker for predicting IVIG resistance in KD. PMID:28542183

  2. The Understanding of Astronomy Concepts by Students from Basic Education of a Public School. (Spanish Title: El Entendimiento de Conceptos de Aastronmía Por Los Alumnos de Educación Básica en Una Escuela Pública.) O Entendimento de Conceitos de Astronomia Por Alunos da Educação Básica: O Caso de Uma Escola Pública Brasileira

    NASA Astrophysics Data System (ADS)

    Iria Machado, Daniel; dos Santos, Carlos

    2011-07-01

    We present the results obtained in a research on the comprehension of basic astronomical concepts, in which 561 students from fifth grade middle school to third grade high school of a public school of the city of Foz do Iguaçu (Brazil) took part. A test with 20 multiple-choice questions was applied to indentify the most common conceptions expressed by the students. This test was elaborated based on the literature about misconceptions and covered the following topics: the day-night cycle; the time zones; the seasons of the year; the phases of the Moon; the movement of the Moon; the apparent movement of the Sun in the celestial sphere; the eclipses; the dimensions and distances in the Universe; the brightness of the stars and its observation from Earth. Though a small progress was verified in the proportion of scientifically acceptable answers when comparing the eighth grade of middle school to the fifth, and the third grade of high school to the first, there was an overall predominance of alternative conceptions regarding most of the explored subjects, which persisted up to the last year of secondary school. The comparison to data found in this research made in other socio-cultural contexts revealed, in many aspects, similar notions and difficulties revealed by the students. Se presentan los resultados de una investigación sobre la comprensión de conceptos astronómicos básicos, en la cual participaron 561 estudiantes que cursaban entre el quinto grado de la enseñanza primaria y el tercer año de la enseñanza secundaria de una escuela pública de la ciudad de Foz do Iguaçu (Brasil). Se utilizó un test de 20 preguntas de opción múltiple para identificar las concepciones más comunes expresadas por los estudiantes. Este instrumento de recolección de datos se desarrolló en base a la literatura sobre las concepciones alternativas y trató los siguientes temas: el ciclo día-noche, los husos horarios, las estaciones del año, las fases de la Luna, el

  3. Sobre el estado evolutivo de β Pictoris

    NASA Astrophysics Data System (ADS)

    Brunini, A.; Benvenuto, O. G.

    Desde el descubrimiento de fuertes excesos infrarrojos en β Pictoris, esta estrella ha sido muy estudiada y es considerada candidata a poseer un sistema planetario propio. β Pic está rodeada de un disco asimétrico de polvo que se observa de canto y que esta vacío a distancias <= 40 AU. Esto se considera una fuerte evidencia en favor de la presencia de (al menos) un planeta gigante. Recientemente se han observado líneas de material circunestelar que se han interpretado como consecuencia de la caída de objetos cometarios sobre esta estrella. Recientemente se ha utilizado la existencia del disco de polvo para atribuir una edad corta (pre - secuencia principal) a βPic. Sin embargo, la evaporación de estos cometas provee suficiente polvo como para explicar la presencia del disco observado sin necesidad de edades cortas. En este trabajo mostramos que la comparación entre la tasa de impactos cometarios estimada en el Sistema Solar para diferentes etapas de su evolución y los datos observados en β Pic indica edades avanzadas para β Pic. Esta estimación debe tomarse con cautela ya que depende de la estructura de los sistemas planetarios. Además mostramos que, desde el punto de vista de la evolución estelar y con las incertezas presentes en la luminosidad y la temperatura efectiva, existe un continuo de edades posible para β Pic. Sin embargo, empleando los datos provenientes de los flujos cometarios encontramos que una edad prolongada es consistente con ambos tratamientos.

  4. Modelo de accesibilidad de conceptos matematicos aplicados en el curso de Astronomia Descriptiva para estudiantes con impedimentos visuales en la UPR

    NASA Astrophysics Data System (ADS)

    Isidro Villamizar, Gloria Maria

    Este estudio utiliza metodologia de investigacion cualitativa, con el proposito de describir, analizar y evaluar los procesos de diseno y desarrollo de un modelo de accesibilidad que consiste en estrategias de ensenanza de las matematicas para estudiantes con impedimentos visuales matriculados en el curso de Astronomia Descriptiva en la UPR. Se utilizaron las siguientes estrategias para recopilar la informacion, 1) reflexiones de la investigadora en el proceso de diseno y desarrollo de las lecciones adaptadas, que se registraron en un diario reflexivo. 2) entrevista semiestructurada luego de haber trabajado las lecciones de aprendizaje adaptadas con los participantes. 3) observaciones y notas de la investigadora del trabajo de los participantes. Para obtener la informacion de los participantes se obtuvo los permisos institucionales necesarios; se seleccionaron los participantes y se validaron los instrumentos; se realizo el desarrollo de las lecciones adaptadas con los participantes; y finalmente, se analizo la informacion obtenida. El diseno de las lecciones de aprendizaje adaptadas se hizo siguiendo las recomendaciones curriculares de los temas de matematicas aplicados en el curso de Astronomia Descriptiva realizado por la investigadora durante su semestre de internado. El testimonio de las voces de los participantes se obtuvo del proceso de desarrollo de las lecciones de aprendizaje adaptadas de temas seleccionados de conceptos matematicos requeridos en el curso de Astronomia Descriptiva y de la entrevista semiestructurada con los participantes, luego de haber trabajado las lecciones de aprendizaje. Para el desarrollo de las lecciones de aprendizaje, se utilizaron materiales tactiles adaptados, materiales tactiles disenados y materiales disponibles comercialmente. Los textos de las lecciones se imprimieron en tinta y en Braille. Se exhorta a disenar y desarrollar estrategias de ensenanza accesibles, considerando como recursos para evaluar su efectividad a

  5. Actitud y conocimiento sobre donación de órganos de estudiantes de medicina de una universidad pública del noreste de México.

    PubMed

    Sebastián-Ruiz, María José; Guerra-Sáenz, Elda Karina; Vargas-Yamanaka, Anna Karen; Barboza-Quintana, Oralia; Ríos-Zambudio, Antonio; García-Cabello, Ricardo; Palacios-Saucedo, Gerardo Del Carmen

    2017-01-01

    Averiguar la actitud y el conocimiento que tienen los estudiantes de medicina de una universidad pública del noreste de México sobre la donación de órganos y tejidos. Estudio prolectivo, descriptivo, observacional y transversal. Se incluyeron 3056 encuestas de 34 reactivos realizadas a estudiantes de medicina durante el periodo 2013-2015 y se eliminaron aquellas con menos del 80% de los reactivos contestados. Análisis estadístico: frecuencias absolutas, porcentajes, medias, desviación estándar y prueba de ji al cuadrado. Se consideró significativo un valor de p < 0.05. El 74% de los estudiantes donaría sus órganos y el 41% lo haría por reciprocidad; del 26% que no donaría sus órganos, el 48% no lo haría por temor a que tomen sus órganos antes de estar muerto. Un 86% estaría dispuesto a donar los órganos de algún familiar. El 64% ha comentado el tema de la donación con su familia y el 67% con amigos. El 50% no ha recibido información alguna sobre el tema. El 68% entiende el concepto de muerte encefálica. Pocos estudiantes reciben información sobre donación de órganos al llegar a la universidad; pese a ello, la mayoría tiene una actitud positiva y estarían -dispuestos a -donar. The issue of organ donation is scarcely addressed during undergraduate medical studies. To evaluate the knowledge and attitude towards organ donation of medicine students of a Northwestern Mexico public university. A prolective, descriptive, observational, and cross-sectional study. A 34 items cross-sectional survey evaluating knowledge and attitude towards organ donation in 3056 medicine students during 2013-2015. Descriptive statistics were used as absolute frequencies, percentages, mean and standard deviation, as well as the Chi-square test. A p < 0.05 was considered significant. 74% of students would donate their own organs, mainly due to reciprocity (41%). 26% of students would not donate, 48% of them because of fear that their organs could be taken before death

  6. Evolution of the Astronomy Concepts Along Basic Education Cycle. (Breton Title: Evolução dos Conceitos de Astronomia no Decorrer da Educação Básica.) La Evolución de los Conceptos de Astronomía Durante la Educación Básica

    NASA Astrophysics Data System (ADS)

    Darroz, Luiz Marcelo; da Rosa, Cleci Werner; Becker da Rosa, Álvaro; Samudio Pèrez, Carlos Ariel

    2014-07-01

    a base para futuras discussões relacionadas à atuação dessas instâncias como meio de promover mudanças ativistas na estrutura curricular, proporcionando, mais efetivamente, a educação em astronomia no ensino básico. Si bien la astronomía es considerada una de las ciencias más antiguas de la humanidad y aunque la comprensión de sus conceptos haya traído enormes avances para la ciencia y, consecuentemente, para la sociedad, se observa que una parte significativa de personas se encuentran al margen de estos conocimientos. De acuerdo con los Parámetros Curriculares Nacionales para la Educación Básica, corresponde a la escuela la difusión de los conceptos científicamente correctos, entre ellos los relacionados al área de astronomía. Sobre esta cuestión, se presenta una investigación realizada con 140 estudiantes del noveno año de la enseñanza fundamental y con 120 estudiantes del tercer año de la enseñanza de nivel medio de cuatro escuelas de la región de Passo Fundo/RS. Se pretendía averiguar, por medio de un cuestionario compuesto de preguntas abiertas y de opción múltiple, el conocimiento de este grupo de alumnos sobre términos y fenómenos astronómicos básicos y, también, si el índice de aciertos crece en la medida que los alumnos avanzan a los diferentes y sucesivos años de la enseñanza fundamental y media. De modo general, los resultados demuestran que la enseñanza de astronomía en la educación básica muestra deficiencias. De los 20 asuntos investigados, 17 revelan índices de aciertos semejantes en las respuestas dadas por los estudiantes de nivel básico y medio, mostrando que muchas concepciones equivocadas permanecen a lo largo de la educación básica. Esto hace evidente que estos temas no son - o son poco - abordados durante esos niveles de escolarización. Así, puede concluirse que la discusión de los conceptos relacionados a la astronomía debe recibir un mayor énfasis en cuanto al abordaje de los diferentes

  7. Sobre os sistemas de referência celeste

    NASA Astrophysics Data System (ADS)

    Poppe, P. C. R.; Martin, V. A. F.

    2003-02-01

    Apresentamos neste trabalho, algumas discussões sobre os sistemas de referência utilizados em Astronomia. Claramente, não é possível esgotar todo este assunto num único texto, mas esperamos, contudo, que o presente material possa ser apreciado nos cursos de Introdução à Astronomia, que estão cada vez mais presentes nas atuais propostas curriculares das graduações de Física. As discussões pertinentes às "Bases de Referência Celeste", serão apresentadas em um outro trabalho.

  8. Infective endocarditis: diagnosis, antimicrobial therapy, and management of complications: a statement for healthcare professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, and the Councils on Clinical Cardiology, Stroke, and Cardiovascular Surgery and Anesthesia, American Heart Association: endorsed by the Infectious Diseases Society of America.

    PubMed

    Baddour, Larry M; Wilson, Walter R; Bayer, Arnold S; Fowler, Vance G; Bolger, Ann F; Levison, Matthew E; Ferrieri, Patricia; Gerber, Michael A; Tani, Lloyd Y; Gewitz, Michael H; Tong, David C; Steckelberg, James M; Baltimore, Robert S; Shulman, Stanford T; Burns, Jane C; Falace, Donald A; Newburger, Jane W; Pallasch, Thomas J; Takahashi, Masato; Taubert, Kathryn A

    2005-06-14

    Despite advances in medical, surgical, and critical care interventions, infective endocarditis remains a disease that is associated with considerable morbidity and mortality. The continuing evolution of antimicrobial resistance among common pathogens that cause infective endocarditis creates additional therapeutic issues for physicians to manage in this potentially life-threatening illness. This work represents the third iteration of an infective endocarditis "treatment" document developed by the American Heart Association under the auspices of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease of the Young. It updates recommendations for diagnosis, treatment, and management of complications of infective endocarditis. A multidisciplinary committee of experts drafted this document to assist physicians in the evolving care of patients with infective endocarditis in the new millennium. This extensive document is accompanied by an executive summary that covers the key points of the diagnosis, antimicrobial therapy, and management of infective endocarditis. For the first time, an evidence-based scoring system that is used by the American College of Cardiology and the American Heart Association was applied to treatment recommendations. Tables also have been included that provide input on the use of echocardiography during diagnosis and treatment of infective endocarditis, evaluation and treatment of culture-negative endocarditis, and short-term and long-term management of patients during and after completion of antimicrobial treatment. To assist physicians who care for children, pediatric dosing was added to each treatment regimen. The recommendations outlined in this update should assist physicians in all aspects of patient care in the diagnosis, medical and surgical treatment, and follow-up of infective endocarditis, as well as management of associated complications. Clinical variability and complexity in infective

  9. Avisos de salud sobre el PFOA y PFOS en el agua potable

    EPA Pesticide Factsheets

    La EPA estableció avisos de salud sobre el ácido perfluorooctanoico (PFOA) y el sulfonato de perfluorooctano (PFOS) para proporcionar información a los operadores de sistemas de agua potable y funcionarios estatales, tribales y locales sobre los riesgos de

  10. Lecturas sobre educacion de adultos en America latina (Readings on Adult Education in Latin America). Serie: Retablo de Papel 14.

    ERIC Educational Resources Information Center

    Latapi, Pablo, Comp.; Castillo, Alfonso, Comp.

    Twelve essays written in Spanish on the state of adult education in Latin America are presented. The essays are organized into three main sections, including: "Concepto y evolucion historica de la educacion de adultos" (Conception and Historical Evolution of Adult Education); "Aspectos particulares" (Specific Subjects); and…

  11. Lecturas sobre educacion de adultos en America latina (Readings on Adult Education in Latin America). Serie: Retablo de Papel 14.

    ERIC Educational Resources Information Center

    Latapi, Pablo, Comp.; Castillo, Alfonso, Comp.

    Twelve essays written in Spanish on the state of adult education in Latin America are presented. The essays are organized into three main sections, including: "Concepto y evolucion historica de la educacion de adultos" (Conception and Historical Evolution of Adult Education); "Aspectos particulares" (Specific Subjects); and…

  12. La utilizacion de los mapas conceptuales en la ensenanza de biologia y su efecto sobre el dominio del proceso de fotosintesis en los estudiantes universitarios

    NASA Astrophysics Data System (ADS)

    Gonzalez Rivera, Maria M.

    Se investigo el efecto de los mapas conceptuales sobre el dominio del proceso de fotosintesis en estudiantes universitarios. La investigacion utilizo dos estrategias: mapas conceptuales individuales y mapas conceptuales colaborativos, con el fin de investigar si existen diferencias significativas en el dominio del proceso de fotosintesis. El analisis de los datos incluyo aspectos cualitativos y cuantitativos. Se desprende del estudio que el 80% de los estudiantes describen la utilizacion de los mapas conceptuales como una experiencia beneficiosa. El 70% de los estudiantes expreso que los mapas conceptuales son utiles en el aprendizaje del proceso de fotosintesis y el 61% indico que facilitan la comprension de los conceptos. Los hallazgos mas importantes del analisis cuantitativo indican que los estudiantes que utilizaron los mapas conceptuales mejoraron significativamente su desempeno en la posprueba global. Se utilizo la prueba Mann-Whitney para investigar si existian diferencias significativas en la posprueba y preprueba global, el valor de W = 1945.0, para un valor p de 0.00, lo cual establece diferencias significativas. Para determinar si existian diferencias significativas entre la posprueba y preprueba del grupo individual, se realizo la prueba nuevamente. El valor de W correspondio a 490.5, que es significativo, con un valor p de 0.00. Se concluye que existen diferencias significativas entre la ejecucion de la posprueba y preprueba del grupo individual. Los datos proveen suficiente evidencia para sostener que los estudiantes que utilizaron la estrategia de mapas conceptuales individuales mejoraron el dominio del proceso de fotosintesis significativamente. Se realizo nuevamente la prueba para los resultados de posprueba y preprueba del grupo colaborativo. El valor de W correspondio a 446 con un valor p de 0.00. Se concluyo que existen diferencias significativas entre la ejecucion de la posprueba y preprueba del grupo colaborativo. Finalmente, se efectuo una

  13. Basic Concepts of Astronomy: a Methodological Proposal. (Spanish Title: Conceptos Básicos de Astronomía: Una Propuesta Metodológica.) Conceitos Básicos de Astronomia: Uma Proposta Metodológica

    NASA Astrophysics Data System (ADS)

    Darroz, Luiz Marcelo; Heineck, Renato; Samudio Pérez, Carlos Ariel

    2011-12-01

    In this report, the development of a methodological proposal which approaches basic concepts of astronomy-grounded pedagogically on Meaningful Learning is described. The proposal, which consists of four meetings, was developed by teachers and academics of the course of Professor in Physics of the University of Passo Fundo (UPF), through an extension course to a group of highschool students of a public school of the town of Passo Fundo, RS. The work was focused into basic concepts of astronomy. The signs of Meaningful Learning have been obtained by means of research and evaluation tools that were applied at the end of each meeting. The evaluation of the proposal has been conducted by means of a final questionnaire which was answered by the participants at the end ofthe development of activities. By means of the results obtained from the different instruments, and the comments made by the participants during the activities and by means of the high rates of approval obtained in the final questionnaire, we think that the proposal reached the established goals and it may be repeated with the certainty of success. En este relato se describe una propuesta de desarrollo metodológico que aborda conceptos básicos de astronomía fundamentada pedagógicamente en el Aprendizaje Significativo. La propuesta que comprende cuatro encuentros, fue desarrollada por profesores y académicos del curso de Licenciatura en Física de la Universidad de Passo Fundo (UPF), a través de un curso de extensión para un grupo de Liceo del 6º año de una Escuela Pública de la ciudad de Passo Fundo/RS. El trabajo tuvo como eje principal los "conceptos básicos de astronomía". Los indicios de Aprendizaje Significativo fueron obtenidos por instrumentos de pesquisa y evaluación, siempre aplicados después de cada encuentro. La evaluación de la propuesta fue hecha a través de un cuestionario final y contestado por los participantes al finalizar el desarrollo de actividades. Por los resultados

  14. Hoja informativa sobre la Agenda de Acción EJ 2020

    EPA Pesticide Factsheets

    Página principal de la hoja informativa sobre la Agenda de Acción EJ 2020 de la Agencia de Protección Ambiental de EE.UU. que delinea el plan estratégico de justicia ambiental de la agencia para los próximos cuatro años.

  15. Some Research-Based Issues and Recommendations Expressed at the Seminario Internacional Sobre la Educacion Bilingue.

    ERIC Educational Resources Information Center

    Bernal, Ernesto M.

    The first Seminario Internacional Sobre la Educacion Bilingue (International Seminar on Bilingual Education), under the aegis of the National Association for Bilingual Education and the Mexican secretary for public education, brought together professionals from Canada, the United States, and Mexico in Oaxtepec, Mexico in November 1986 to share…

  16. Obtenga más información sobre la etiqueta Safer Choice

    EPA Pesticide Factsheets

    Entérese más acerca del programa EPA Diseño para el medio ambiente, incluyendo información sobre su historia, proyectos anteriores y otros programas de la EPA que están relacionados con este programa.

  17. Kawasaki disease and ENSO-driven wind circulation

    NASA Astrophysics Data System (ADS)

    Ballester, Joan; Burns, Jane C.; Cayan, Dan; Nakamura, Yosikazu; Uehara, Ritei; Rodó, Xavier

    2013-05-01

    disease (KD) is the most common cause of acquired heart disease in children worldwide. Recently, a climatological study suggested that KD may be triggered by a windborne agent traveling across the north Pacific through the westerly wind flow prevailing at midlatitudes. Here we use KD records to describe the association between enhanced disease activity on opposite sides of the basin and different phases of the El Niño-Southern Oscillation (ENSO) phenomenon, via the linkage to these tropospheric winds. Results show that years with higher-than-normal KD cases in Japan preferentially occur during either El Niño Modoki or La Niña conditions, while in San Diego during the mature phase of El Niño or La Niña events. Given that ENSO offers a degree of predictability at lead times of 6 months, these modulations suggest that seasonal predictions of KD could be used to alert clinicians to periods of increased disease activity.

  18. Ultimate VHF Broadband Interferometer Zen KAWASAKI and Manabu AKITA

    NASA Astrophysics Data System (ADS)

    Kawasaki, Z.; Akita, M.

    2013-12-01

    Lightning Research Group of Osaka University (LRGOU) has been developing an interferometer for thunderstorm monitoring during these two decades. When LRGOU initiated this project, many related scientists claimed that LRGOU's system was a time of arrival and it must be a sophisticated TOA system. However the key technology of the system were broad band and digital data processing, and it is named the VHF broad band digital interferometer (BDITF), because the phase difference of Fourier components played the very important role. Then the BDITF finally has been realized as a quasi-real time lightning monitoring system, and LRGOU has been operating their BDITFs around Osaka area. Since the BDITF captures the VHF impulses associated with lightning discharges by amplitude triggering, it occasionally misses one of the bi-directional leader progressions because of relatively small amplitude VHF impulses mainly emitted by positive leader tips. All of high density of VHF pulses associated with recoil leaders may not be recorded. Then the ordinary BDITF is able to be accepted as the practical operational system, but from the aspect of science it has been sioriously expected to be improved its performance. To overcome the difficulty New Mexico Institute of Mining and Technology (NMIMT) and LRGOU have started the joint project, and a continuous recording system for digital signals is adopted [M. Stock et al, and M Akita et al,]. The field campaigns have been conducting in New Mexico and in Japan, and successful observations are accomplished. The algorithm to identify individual VHF pulses associated with lightning discharges from back ground noise and/or artificial noise, the dispersion of phase differences for all Fourier components is examined. In case by of small dispersion it is concluded that analyzed VHF pulse has high possibility to be emitted by lightning discharges. This interpretation means that the recorded VHF pulse shape might maintain without deformation during propagation, like electromagnetic wave propagation in non-dispersive medium. This procedure presented exciting results of lightning channel imaging, and the bi-directional leader propagation can be visualized in terms of azimuth and elevation as a function of time. Moreover all of the VHF radiation process for an entire lightning flash from initiation to termination can be imaged, and it is concluded that the system might be an ultimate broad band digital interferometer. The authors would like to show their appreciation to Paul Krehbiel, NMIMT, who gave a chance and suggestion of a continuous recording scheme for BDITF. M. Stock et al. : Continuous Broadband Digital Interferometry of Lightning using a Generalized Cross Correlation Algorithm M. Akita et al. : Data Processing Procedure 1 using Distribution of Slopes of Phase Differences for Broadband VHF Interferometer

  19. Construction of a state evolution for Kawasaki dynamics in continuum

    NASA Astrophysics Data System (ADS)

    Berns, Christoph; Kondratiev, Yuri; Kutoviy, Oleksandr

    2013-06-01

    We consider conservative, non-equilibrium stochastic jump dynamics of interacting particles in continuum. These dynamics have a (grand canonical) Gibbs measure as invariant measure. The problem of existence of these dynamics is studied. The corresponding time evolution of correlation functions is constructed.

  20. A Low Temperature Analysis of the Boundary Driven Kawasaki Process

    NASA Astrophysics Data System (ADS)

    Maes, Christian; O'Kelly de Galway, Winny

    2013-12-01

    Low temperature analysis of nonequilibrium systems requires finding the states with the longest lifetime and that are most accessible from other states. We determine these dominant states for a one-dimensional diffusive lattice gas subject to exclusion and with nearest neighbor interaction. They do not correspond to lowest energy configurations even though the particle current tends to zero as the temperature reaches zero. That is because the dynamical activity that sets the effective time scale, also goes to zero with temperature. The result is a non-trivial asymptotic phase diagram, which crucially depends on the interaction coupling and the relative chemical potentials of the reservoirs.

  1. Effects of an acute bout of exercise on serum soluble leptin receptor (sOB-R) levels.

    PubMed

    Yang, Chung Bing; Chuang, Chung-Chief; Kuo, Chung Sen; Hsu, Chin Hsing; Tsao, Te Hung

    2014-01-01

    We investigated the effects of an acute bout of exercise on serum soluble leptin receptor (sOB-R) concentrations. Eighteen male participants completed two different exercise sessions with intensities of 25% and 65% maximal aerobic capacity (VO2max). In addition to the energy expenditure during exercise sessions being measured, blood samples were collected before exercise, and immediately, at 24 h, and at 48 h post-exercise to analyse sOB-R, leptin and insulin levels. At 24 h post-exercise, sOB-R and leptin concentrations at the 65% VO2max were significantly different from those at the 25% VO2max. Leptin levels at 48 h post-exercise were also significantly lower for the 65% VO2max than for the 25% VO2max (P < 0.01). In the 65% VO2max session, the energy expenditure during exercise was significantly associated with leptin concentrations at 24 h and 48 h and sOB-R concentrations at 24 h post-exercise. However, no correlations were found between sOB-R and leptin at the three post-exercise time points. In conclusion, an acute bout of exercise with 920 kcal of output resulted in an increase in sOB-R levels at 24 h post-exercise. However, the changes in sOB-R levels due to an acute bout of exercise might not contribute to the delayed decrease observed for leptin.

  2. Información general sobre la preparación y la respuesta frente a desastres naturales

    EPA Pesticide Factsheets

    Esta página brinda información general sobre la manera en que se puede preparar para enfrentar desastres naturales potenciales y las acciones a tomar después de dichos desastres naturales y otras emergencias ambientales.

  3. Conversation with Lara about the Earth and Land. (Spanish Title: Conversando con Lara sobre la Tierra y la Teirra.) Conversando com Lara sobre a Terra e a Terra

    NASA Astrophysics Data System (ADS)

    da Conceição Barbosa-Lima, Maria

    2010-12-01

    The present article is the analysis of a conversation between the author and Lara, a four-yearold- girl, enrolled in nursery school, while she makes a drawing of the Earth. It took place outside school environment and without any other person around to avoid interference during the interview. According to Ferreira & Silva (2004), a researcher can only comprehend a child's drawing, or form, by listening to him/her while he/she is creating it. Lara presented the traditional flat drawing, picturing the sky parallel to the ground, as reported by Nardi & Carvalho (1996). However, when asked to draw the World - term used by Butterworth et al. (2002), in order to avoid unnecessary confusion - she represented it by a circle, with herself on the surface. Her drawings led to the conclusion that such girl does not know yet the World in which she lives is the Earth, and probably because of that, within her age and consequent maturity, she accurately differentiates the concepts of land and Earth. El presente artículo analiza una entrevista libre, mientras una niña de 4 años y 4 meses, matriculada en el pregrado, dibuja la Tierra. Esta entrevista se realizó fuera del ambiente escolar y sin otra persona alrededor que pudiera interferir. De acuerdo con Ferreira Silva (2004), para quien investiga es posible conocer realmente lo que un niño o una niña pone en el papel a través de grafismos y/o dibujos si se lo escucha durante el proceso de creación de la escritura con imágenes. La niña, en este caso, representa la Tierra con el tradicional dibujo plano y el "cielo" paralelo al suelo, conforme analizaron Nardi & Carvalho (1996). Pero, cuando se Le solicita dibujar el "Mundo" - palabra empleada en un trabajo de Butterworth et al. (2002), con intención de no provocar "confusiones" innecesarias a sus sujetos de investigación- lo representa de forma circular, poniéndose sobre su superficie. Sus dibujos llevan a concluir que esta niña aún no tiene conocimiento que el mundo

  4. A kinetic concepto of lipid transport in ruminants.

    PubMed

    Palmquist, D L

    1976-03-01

    Summarization of the literature shows a strong correlation between dietary fatty acid intake and total lipid concentration in plasma in lactating cows whereas total milk fat secreted is related to neither of these. In the process of plasma triglyceride removal, chylomicra and very low density lipoproteins are converted to low density lipoproteins. Limited kinetic data indicate that the fractional removal rates for chulomicra and very low density lipoproteins are rapid in lactating cows whereas fractional removal of low density lipoproteins is slower, resulting in accumulation of the latter in plasma. Under such conditions, low density lipoprotein concentrations of plasma would not be expected to reflect quantitatively the transfer of plasma triglyceride fatty acids to milk fat. Quantitative analysis or triglyceride fatty acid turnover in density less than 1.006 lipoproteins should delineate the role of plasma lipid transport in milk fat synthesis. High fat diets protected from rumen biohydrogenation have proven to be a useful approach in studying ruminant fat metabolism and may be used more extensively to elucidate the role of cholesterol in plasma lipid transport and the metabolism of essential fatty acids in ruminants.

  5. O que bilíngues bimodais têm a nos dizer sobre desenvolvimento bilíngue?

    PubMed Central

    de Quadros, Ronice Müller; Lillo-Martin, Diane; Pichler, Deborah Chen

    2013-01-01

    O objetivo deste trabalho é apresentar o que as pesquisas que estamos desenvolvendo com crianças ouvintes, filhas de pais surdos, adquirindo Língua Brasileira de Sinais (Libras) e Português e Língua de Sinais Americana (ASL) e Inglês (Lillo-Martin et al. 2010) têm a nos dizer sobre desenvolvimento bilíngue. Os dados deste estudo fazem parte de um banco de dados de interações espontâneas coletadas longitudinalmente, alternando contextos de aquisição da Libras e do português como língua alvo, no Brasil e dados coletados longitudinalmente. nos mesmos contextos, de crianças adquirindo ASL e inglês1. Além disso, há também dados do estudo experimental com testes aplicados nos dois pares de línguas que se agregam ao presente estudo. Uma visão geral dos estudos desenvolvidos sobre a aquisição bilíngue bimodal por crianças ouvintes, filhas de pais surdos, será apresentada e, então, serão expostos alguns aspectos linguísticos deste tipo de aquisição, considerando as discussões sobre aquisição bilíngue a partir da pesquisa realizada. PMID:24431480

  6. A Reappraising of Cosmography: the Interface Between Astronomical and Geographic Studies. (Breton Title: Releitura do Conceito de Cosmografia: a Interface Entre os Estudos Astronômicos e Geográficos.) Una Relectura del Concepto de Cosmografía: la Interfase Entre los Estudios Astronómicos y Geográficos

    NASA Astrophysics Data System (ADS)

    Azevedo Sobreira, Paulo Henrique

    2012-12-01

    The concept of "Cosmography" is in disuse since the 80s of the last century, but the astronomical themes previously discussed in the school subjects of Geography and Cosmography remain in current textbooks. The use of term "Cosmography" was rescued in this research, and the study of its re-signification prompted the appearance of the term Geographic Cosmography. The Geographic Cosmography is a field of studies of the Geography, whose set of knowledge and skills is predominantly scholar. It studies the interface between terrestrial and celestial knowledge, and assigns a geographic significance to them. It examines human and natural relationships with Sidereal Space and its consequences for society and nature. O conceito de "Cosmografia" está em desuso desde os anos 80 do século XX, mas os temas astronômicos anteriormente abordados nas disciplinas escolares de Cosmografia e de Geografia permanecem nos atuais livros didáticos. O uso do termo "Cosmografia" foi resgatado nesta pesquisa e o estudo de sua ressignificação proporcionou o surgimento do termo Cosmografia Geográfica. A Cosmografia Geográfica é um campo de estudos da Geografia, cujo conjunto de conhecimentos e habilidades é predominantemente escolar. Estuda a interface entre os conhecimentos terrestres e os celestes e lhes atribui significância geográfica. Analisa as relações humanas e naturais com o Espaço Sideral e suas consequências para a sociedade e a natureza.

    Aunque el concepto de "Cosmografía" no se usa desde la década de los '80 del siglo pasado, los temas astronómicos que se enseñaban anteriormente en las asignaturas escolares de Cosmografía y de Geografía permanecen en los actuales libros didácticos. El uso del término "Cosmografía" fue rescatado en esta investigación y el estudio de su resignificación proporcionó el surgimiento del término Cosmografía Geográfica. La Cosmografía Geográfica es un campo de estudio de la Geografía, donde

  7. University Students' Conceptions about the Moon Phases. (Spanish Title: Concepciones de Estudiantes Universitários sobre Las Fases de la Luna.) Concepções de Estudantes Universitários sobre as Fases da Lua

    NASA Astrophysics Data System (ADS)

    de Fátima Oliveira Saraiva, Maria; da Silveira, Fernando Lang; Steffani, Maria Helena

    2011-07-01

    In this article we describe the development of a multiple choice test about lunar phases and analyze the results of its application to ten groups of Physics students at the UFRGS. During the improvement of the test, we noticed that the percentage of right answers about some concepts increased significantly when associated with the reformulation of the question, emphasizing the importance of being careful to avoid incorrect answers generated by unclear questions, and not by ignorance on the matter. We confirm the results of other studies that show that students have great difficulty to relate the Moon's phase with its position in the sky at given time. On the other hand, our results suggest that, in general, students of Physics understand the phenomenon of lunar phases better than the average of university students. En estese artículo se describe la elaboración de una prueba de opción múltiple sobre las fases de la Luna y se analizan los resultados de su aplicación en diez grupos de estudiantes de Física de UFRGS. Durante el mejoramiento de la prueba observamos que el porcentaje de aciertos creció considerablemente cuando considerada una nueva redacción de la pregunta, destacando el cuidado que se debe tomar a fin de evitar respuestas incorrectas generadas por preguntas poco claras y no a causa de la ignorancia de los estudiantes sobre el tema. Confirmamos los resultados de otros estudios que las mayores dificultades de los alumnos sobre el tema fases de la Luna están en relacionar la fase de la Luna con su posición en el cielo en determinado momento. Por otra parte, nuestros resultados sugieren que, en general, los estudiantes de la Física comprenden mejor el fenómeno de las fases lunares que el promedio de los estudiantes universitarios. Neste artigo descrevemos a elaboração de um teste de múltipla escolha sobre as fases da Lua e analisamos os resultados de sua aplicação em dez grupos de estudantes de Física da UFRGS. Durante o aprimoramento do

  8. Sobre a largura da última superfície de espalhamento

    NASA Astrophysics Data System (ADS)

    Nobre, M. A. S.; Pires, N.; Lima, J. A. S.

    2003-08-01

    De acordo com o modelo do "Big-Bang", no universo primordial a matéria estava em equilíbrio térmico com a radiação. Com a expansão a temperatura da radiação cai. Quando a temperatura chega em torno dos 4.000K, os espalhamentos diminuem, começando a recombinação dos prótons e elétrons em Hidrogênio neutro (era conhecida como da recombinação). Ao final da recombinação, os fótons se propagam livremente sofrendo, em princípio, somente os efeitos do "redshift" cosmológico. Esses fótons nos alcançam hoje como a radiação cósmica de fundo (RCF), e parecem vir de uma superfície esférica ao nosso redor, tal que o raio dela é a distância que ele viajou desde seu último espalhamento na época da recombinação. Naturalmente, esse processo não ocorreu abruptamente, implicando na existência de uma largura no espaço dos "redshifts" que deve depender do modelo cosmológico específico e dos processos físicos considerados. Neste trabalho analisamos os efeitos de diferentes modelos - a saber, aqueles com decaimento do vácuo L(t), criação de matéria, quintessência e gás de Chaplygin - sobre a última superfície de espalhamento da RCF, em particular sua largura e a função visibilidade, que determina a probabilidade de um fóton ter tido seu último espalhamento num "redshift" z. No caso particular dos modelos com decaimento do vácuo, existe uma forte dependência da função visibilidade com L(t). Tais efeitos poderão ser testados através da análise dos resultados de experimentos mais precisos que estão atualmente em andamento, como por exemplo, o WMAP.

  9. Tendências de teses e dissertações sobre ensino de astronomia no Brasil

    NASA Astrophysics Data System (ADS)

    Bretones, P. S.; Megid Neto, J.

    2003-08-01

    Neste trabalho são apresentados os resultados de uma pesquisa do tipo estado da arte sobre teses e dissertações defendidas no Brasil e relativas ao ensino de Astronomia. Teve por objetivo identificar essa produção e conhecer as principais tendências da pesquisa nesse campo. O procedimento inicial consistiu de um levantamento bibliográfico junto ao Centro de Documentação em Ensino de Ciências (CEDOC) da Faculdade de Educação da UNICAMP e ao Banco de Teses da CAPES disponível na Internet. Foram localizadas 13 dissertações de mestrado e 3 teses de doutorado, as quais foram estudadas em função dos seguintes aspectos: instituição, ano de defesa, nível escolar abrangido no estudo, foco temático do estudo e gênero de trabalho acadêmico. Deste conjunto de pesquisas, 13 (81,3%) delas foram defendidas a partir da segunda metade dos anos 90, indicando uma preocupação mais recente com temas relativos ao ensino de Astronomia no conjunto da produção acadêmica em programas de pós-graduação no Brasil. Verificou-se que 43,7% dos trabalhos foram produzidas na USP e 18,8% na UNICAMP. Quanto ao nível escolar abrangido nos estudos, predominaram os estudos direcionados ao Ensino Fundamental de 5a a 8a séries (62,5%). No que diz respeito ao foco temático das pesquisas, as principais tendências voltaram-se: 56,3% para Conteúdo e Método; 43,8% para Concepções do Professor; 37,5% para Currículo e Programas; 37,5% para Recursos Didáticos. Quanto ao gênero de trabalho acadêmico, verificou-se que 43,8% são de Pesquisa Experimental e 31,3% de Pesquisa de Análise de Conteúdo. Estudos de revisão bibliográfica como este visam colaborar com a divulgação ampla da produção acadêmica em determinada área, traçando algumas de suas tendências. Ao mesmo tempo possibilita, a partir de investigações decorrentes, apontar as suas contribuições para o ensino e sinalizar com necessidades a serem supridas por futuras pesquisas.

  10. Desarrollo de fotonovelas para concienciar sobre trastornos de la conducta alimentaria en latinos en los Estados Unidos

    PubMed Central

    Reyes-Rodríguez, Mae Lynn; García, Marissa; Silva, Yormeri; Sala, Margarita; Quaranta, Michela; Bulik, Cynthia M.

    2016-01-01

    Resumen El objetivo de este estudio fue desarrollar fotonovelas, un tipo de novela gráfica popular en la población latina, para crear conciencia y educar sobre los trastornos de la conducta alimentaria (TCA). Cuatro caricaturas ilustradas y guiones adaptados para adultos y adolescentes de ambos sexos fueron presentados en discusiones focales y en una entrevista de profundidad. Diecisiete latinos adultos (14 mujeres; 3 hombres) y 10 adolescentes (9 féminas; 1 varón) participaron en el estudio. Los participantes encontraron las fotonovelas interesantes y que captaban más la atención que los folletos tradicionales. El uso del espanglish y la clarificación de las diferencias entre los TCA fueron sugeridos por las adolescentes femeninas. Los adultos varones sugirieron cambiar el título, que se enfocara en las consecuencias en la salud de los TCA para que llame la atención en los hombres a leer la historia. Basado en la aceptación encontrada en este estudio, la fotonovela pudiera ser una avenida prometedora para crear conciencia y educar a la comunidad latina sobre los TCA en los Estados Unidos. PMID:27313838

  11. Reflexión bioética sobre el uso de organismos genéticamente modificados

    PubMed Central

    Yunta, Eduardo Rodríguez

    2011-01-01

    El presente artículo reflexiona desde los 4 principios de la bioética el uso comercial de organismos genéticamente modificados. Se cuestiona fundamentalmente la falta de transferencia de tecnología entre el mundo desarrollado y en desarrollo y el que el presente sistema de patentamiento de organismos vivos modificados fomenta intereses comerciales y no da debida importancia al desarrollo sostenible de la agricultura y ganadería en los países en desarrollo, donde más se necesita. Se reflexiona sobre la importancia que tiene evaluar los riesgos antes de introducirse en el mercado organismos genéticamente modificados y la necesidad de regulación en los países. PMID:21927675

  12. A Proposed Activity for a Meaningful Learning about the Moon Phases. (Breton Title: Uma Proposta de Atividade Para a Aprendizagem Significativa sobre as Fases da Lua.) Una Actividad Propuesta Para EL Aprendizaje Significativo Acerca de Las Fases de la Luna

    NASA Astrophysics Data System (ADS)

    Martins, Bruno Andrade; Langhi, Rodolfo

    2012-12-01

    This paper presents one of the concepts of Astronomy and its consequent failure in teaching this topic in high school, even when the official documents point out the necessity of Astronomy teaching at this school level. Among the spontaneous conceptions in Astronomy that high school students carry with them, even after the end of the school, we emphasized in this research the Moon phases. The development of different strategies in relation to traditional methods, aimed to teaching-learning process on this topic was considered in this study. These strategies were devised based on the reference frame of the Meaningful Learning, as elaborated by Ausubel. The proposals presented here include the active participation of students in experimental activities and other didactic activities, for their continuous evaluation during the process. These activities finished with a Comics elaboration about the Moon phases. Therefore, the objective of this paper is to present a proposal for differentiated teaching activity about Moon phases supported by the theoretical principles of Meaningful Learning at Physics classes. Este texto foca um dos conteúdos de Astronomia e a consequente falha no ensino deste tema no ensino médio, apesar de os documentos oficiais apresentarem a necessidade de se trabalhar a Astronomia neste nível de ensino. Dentre as concepções alternativas em Astronomia que os alunos do ensino médio carregam consigo, mesmo após o término dos estudos, destacamos, nesta pesquisa, o fenômeno das fases da Lua. O desenvolvimento de estratégias diferenciadas em relação ao ensino tradicional, visando o processo de ensino-aprendizagem sobre este tema, foi contemplado neste trabalho como um dos resultados obtidos sob a luz dos referenciais da aprendizagem significativa, fundamentados em Ausubel. Segundo a proposta aqui apresentada, a participação ativa dos alunos na execução de uma atividade experimental e outras atividades didáticas, que visam sua cont

  13. Human breast milk and adipokines--A potential role for the soluble leptin receptor (sOb-R) in the regulation of infant energy intake and development.

    PubMed

    Zepf, F D; Rao, P; Moore, J; Stewart, R; Ladino, Yuli Martinez; Hartmann, B T

    2016-01-01

    Concentrations of different adipokines in human breast milk are thought to be able to affect energy intake of the infant. Leptin is a hormone synthesized by adipose tissue and the human placenta and favors satiety. The availability of leptin in breast milk is influenced by epithelial cells of the mammary gland that are known to be able to produce leptin, as well as leptin from maternal circulation that is transported to the breast milk, and which can thus in turn reach neonatal blood after absorption. Research so far as mainly focused on leptin concentrations in breast milk. However, evidence suggests that in addition to leptin concentrations levels of the so-called soluble leptin receptor (sOb-R), the main high-affinity binding protein for leptin in humans, are necessary in order to calculate the free leptin index (FLI) and to assess function of the leptin axis. FLI is calculated from the ratio of leptin to the sOb-R, and serves as the main parameter for assessing function of the leptin axis throughout maturation and development. Here we propose that assessing sOb-R levels in addition to leptin concentrations in breast milk could serve as a valuable tool to investigate effects of the leptin axis in breast milk because sOb-R concentrations can impact available leptin levels, and which in turn can have significant implications for infant energy intake and related development.

  14. Educación sobre sexualidad y prevención del VIH: un diagnóstico para América Latina y el Caribe

    PubMed Central

    DeMaria, Lisa M.; Galárraga, Omar; Campero, Lourdes; Walker, Dilys M.

    2016-01-01

    RESUMEN Objetivo Mostrar, a través de un diagnóstico en América Latina y el Caribe, el panorama legislativo y curricular sobre sexualidad y prevención contra el virus de inmunodeficiencia humana (VIH) en el ámbito escolar, contrastándolo con los comportamientos sexuales reportados en encuestas demográficas y de salud. Métodos En mayo de 2008 se realizó, con el apoyo del Fondo de Población de las Naciones Unidas (UNFPA), una encuesta a informantes clave en 34 países de la Región. El cuestionario autoaplicado solicitó información sustantiva de agentes de las diferentes partes interesadas, como ministerios de educación y de salud, sobre los programas de prevención contra el VIH/Sida que se están aplicando en las escuelas. Resultados Respondieron a la encuesta 27 países que representan 95,5% de la población objetivo (6 a 18 años de edad). La mayoría de los países informó tener al menos un libro de texto o un capítulo específico para enseñar los temas de educación sobre sexualidad y prevención del VIH. En la escuela secundaria se cubren la mayor parte de los temas pertinentes relevantes para la educación sobre sexualidad, pero no todos. Por ejemplo, el problema de la discriminación por orientación o preferencia sexual no se incluye en los programas escolares. Conclusiones El material educativo sobre sexualidad debe ser revisado y actualizado periódicamente de modo que refleje los avances en los temas y en la forma de tratar los contenidos. En cada país el currículo debe abordar el tema del respeto a la diversidad sobre orientación, preferencia e identidad sexuales, y en particular el manejo apropiado de la educación para prevenir infecciones de transmisión sexual (ITS) en hombres que tienen sexo con hombres. Los esfuerzos de evaluación de la efectividad de los programas deben contemplar desenlaces tales como marcadores biológicos (incidencia y prevalencia de ITS y embarazo) y no únicamente indicadores de conocimiento y

  15. Giant right coronary artery aneurysm secondary to Kawasaki disease in child: a case report.

    PubMed

    Zhang, Shanshan; Liu, Geli; Yu, Tielian; Zhou, Guiming; Zheng, Rongxiu

    2015-01-01

    Coronary artery aneurysm or ectasia was reported in approximately 15% to 25% of the affected children, particularly in the proximal end of the main blood vessel and the left anterior descending part. Rare patients have been reported with aneurysm in the distal end of the right coronary artery. In this case report, we present a rare case with aneurysm in the distal end of the right coronary artery. Multi-slice computed tomography was performed for the coronary angiography. Aspirin (10 mg/kg body weight per day) and gamma globulin (2 kg/kg body weight) was administrated via intravenous injection. The patient is currently in a healthy status with a 12-month follow up.

  16. A resolution designating January 26, 2011, as "National Kawasaki Disease Awareness Day".

    THOMAS, 112th Congress

    Sen. Webb, Jim [D-VA

    2011-01-26

    01/26/2011 Read twice and referred to the Committee on the Judiciary. (text of measure as introduced: CR S291) (All Actions) Tracker: This bill has the status IntroducedHere are the steps for Status of Legislation:

  17. Non-equilibrium steady states in two-temperature Ising models with Kawasaki dynamics

    NASA Astrophysics Data System (ADS)

    Borchers, Nick; Pleimling, Michel; Zia, R. K. P.

    2013-03-01

    From complex biological systems to a simple simmering pot, thermodynamic systems held out of equilibrium are exceedingly common in nature. Despite this, a general theory to describe these types of phenomena remains elusive. In this talk, we explore a simple modification of the venerable Ising model in hopes of shedding some light on these issues. In both one and two dimensions, systems attached to two distinct heat reservoirs exhibit many of the hallmarks of phase transition. When such systems settle into a non-equilibrium steady-state they exhibit numerous interesting phenomena, including an unexpected ``freezing by heating.'' There are striking and surprising similarities between the behavior of these systems in one and two dimensions, but also intriguing differences. These phenomena will be explored and possible approaches to understanding the behavior will be suggested. Supported by the US National Science Foundation through Grants DMR-0904999, DMR-1205309, and DMR-1244666

  18. Boundary driven Kawasaki process with long-range interaction: dynamical large deviations and steady states

    NASA Astrophysics Data System (ADS)

    Mourragui, Mustapha; Orlandi, Enza

    2013-01-01

    A particle system with a single locally-conserved field (density) in a bounded interval with different densities maintained at the two endpoints of the interval is under study here. The particles interact in the bulk through a long-range potential parametrized by β⩾0 and evolve according to an exclusion rule. It is shown that the empirical particle density under the diffusive scaling solves a quasilinear integro-differential evolution equation with Dirichlet boundary conditions. The associated dynamical large deviation principle is proved. Furthermore, when β is small enough, it is also demonstrated that the empirical particle density obeys a law of large numbers with respect to the stationary measures (hydrostatic). The macroscopic particle density solves a non-local, stationary, transport equation.

  19. Rigidity of critical points for a nonlocal Ohta–Kawasaki energy

    NASA Astrophysics Data System (ADS)

    Dipierro, Serena; Novaga, Matteo; Valdinoci, Enrico

    2017-04-01

    We investigate the shape of critical points for a free energy consisting of a nonlocal perimeter plus a nonlocal repulsive term. In particular, we prove that a volume-constrained critical point is necessarily a ball if its volume is sufficiently small with respect to its isodiametric ratio, thus extending a result previously known only for global minimizers. We also show that, at least in one-dimension, there exist critical points with arbitrarily small volume and large isodiametric ratio. This example shows that a constraint on the diameter is, in general, necessary to establish the radial symmetry of the critical points.

  20. Tendências De Teses e Dissertações Sobre Educação em Astronomia No Brasil

    NASA Astrophysics Data System (ADS)

    Bretones, Paulo Sergio; Megid Neto, Jorge

    2005-07-01

    Apresentam-se os resultados de uma pesquisa do tipo estado da arte sobre teses e dissertações defendidas no Brasil e relativas ao ensino de Astronomia, com objetivo de identificar essa produção e conhecer as principais tendências da pesquisa nesse campo. Foram localizadas 13 dissertações de mestrado e 3 teses de doutorado, as quais foram estudadas em função dos seguintes aspectos: isntituição, ano de defesa, nível escolar abrangido no estudo, foco temático do estudo e gênero de trabalho acadêmico. Pretende-se assim colaborar com a divulgação ampla da produção acadêmica na área. Ao mesmo tempo o estudo possibilita, a partir de investigações decorrentes, apontar as contribuições dessa produção para o ensino e sinalizar com necessidades a serem supridas por futuras pesquisas.

  1. Influencias socioculturales en conceptos astronómicos: el caso del horizonte en la reforma copernicana

    NASA Astrophysics Data System (ADS)

    Szapiro, A.

    2017-10-01

    I analyze the meaning given by Ptolemy to the concept of horizon; especially how it allowed him to prove, through astronomical observations, the central position of the Earth in the Universe. I review the ways in which Copernicus and Galileo gave heliocentric answers to the Ptolemaic proof. I show that Galilean solution entailed a radical conceptual change, which brought with it a new sense to the concept of horizon. I state that this change was the product of sociocultural influences of the period.

  2. Concepto de ingeniería del Laboratorio Científico de Marte

    NASA Image and Video Library

    El robot más grande y "más malo", y más nuevo para Marte es el Laboratorio Científico de Marte. Tiene el tamaño de un vehículo utilitario deportivo y está provisto de 10 instrumentos, nunca antes h...

  3. Epidemiological and Clinical Characteristics of Kawasaki Disease and Factors Associated with Coronary Artery Abnormalities in East China: Nine Years Experience

    PubMed Central

    Tang, Yunjia; Gao, Xiang; Shen, Jie; Sun, Ling

    2016-01-01

    Background: Because of the different genetic backgrounds, living environments and economic conditions, the epidemiologic, clinical characteristics and risk factors for coronary artery abnormalities (CAAs) in the Chinese population may differ among different parts of China. Methods: We did a retrospective study to explore the clinical characteristics and risk factors for CAAs in east China between 2006 and 2014. Results: There were 1016 patients during the study period. Of the 1004 patients who completed echocardiographic studies, 23.9% had CAAs. Sex, serum albumin, erythrocyte sedimentation rate (ESR), Mycoplasma pneumoniae (MP) infection, intravenous immunoglobulin (IVIG) started after the 10th day of illness and IVIG non-responders were independent risk factors associated with CAA. Conclusions: East China has a lower incidence of CAAs compared with southwest and northeast China, while similar to north China. Male gender, serum albumin, ESR, MP infection, IVIG started after the 10th day of illness and IVIG non-responders were predictive of CAA. PMID:26884440

  4. 76 FR 14116 - Notice of Receipt of Petition for Decision That Nonconforming 2002 Kawasaki Ninja ZX-6R...

    Federal Register 2010, 2011, 2012, 2013, 2014

    2011-03-15

    .... 30141(a)(1)(A), as set forth in 49 CFR part 593. Instead, the petitioner chose to establish import... to be adequate as set forth in 49 U.S.C part 30141(a)(1)(B). The petitioner contends that the 2002... Cars, 122 Motorcycle Brake Systems, and 205 Glazing Materials. The petitioner further contends that...

  5. Cambios históricos en el aporte terrígeno de la cuenca del Río de la Plata sobre la plataforma interna Uruguaya

    NASA Astrophysics Data System (ADS)

    Marrero, Analía; Tudurí, Adriana; Pérez, Laura; Cuña, Caroline; Muniz, Pablo; Lopes Figueira, Rubens; Michaelovitch de Mahiques, Michel; Alves de Lima Ferreira, Paulo; Pittauerová, Daniela; Hanebuth, Till; García Rodríguez, Felipe

    2014-12-01

    B 13813-4. También se determinó una mayor acumulación de sedimentos a través del tiempo en el cinturón de barro del Río de la Plata (plataforma continental adyacente), comparado con aquel registrado en la Barra del Indio (límite entre zona intermedia y externa del estuario). Estas diferencias podrían estar relacionadas con la influencia del Río de la Plata, el cual genera un ambiente altamente dinámico sobre la Barra del Indio y un ambiente más estable sobre el cinturón de barro en la plataforma continental.

  6. Desarrollo de la Escala sobre el Estigma Relacionado con el VIH/SIDA para Profesionales de la Salud mediante el uso de métodos mixtos123

    PubMed Central

    Varas-Díaz, Nelson; Neilands, Torsten B.; Guilamo-Ramos, Vincent; Cintrón Bou, Francheska N.

    2009-01-01

    El estigma relacionado con el VIH/SIDA continúa siendo un obstáculo para la prevención primaria y secundaria del VIH. Las consecuencias para las personas que viven con la enfermedad han sido muy documentadas y continúan siendo una gran preocupación para las personas que proveen servicios de salud y para aquellas que investigan el tema. Estas consecuencias son preocupantes cuando el estigma emana de profesionales de la salud porque se puede limitar el acceso a los servicios. Uno de los principales obstáculos para la investigación del estigma relacionado con el VIH en Puerto Rico es la falta de instrumentos cuantitativos para evaluar las manifestaciones del estigma entre profesionales de la salud. El objetivo principal de este estudio fue desarrollar y probar las propiedades psicométricas de una escala sobre el estigma relacionado con el VIH/SIDA culturalmente apropiada para personas que proveen servicios de salud puertorriqueñas y desarrollar una versión corta de la escala que pudiera usarse en escenarios clínicos con tiempo limitado. El instrumento desarrollado estuvo basado en evidencia cualitativa recopilada entre profesionales y estudiantes de profesiones de la salud puertorriqueños/as (n=80) y administrado a una muestra de 421 profesionales de la salud en adiestramiento. La escala contenía 12 dimensiones del estigma relacionado con el VIH/SIDA. El análisis cuantitativo corroboró 11 de ellas, teniendo como resultado un instrumento con validez y confiabilidad satisfactoria. Estas dimensiones, a su vez, fueron subcomponentes de un factor de estigma general superior. PMID:20333258

  7. Investigaccion-accion en la sala de clases sobre las creencias de la cultura de la ciencia de un grupo de estudiantes universitarios y su relacion reciproca con el aprendizaje de las ciencias biologicas

    NASA Astrophysics Data System (ADS)

    Cordova-Santiago, Lizzette Astrid

    La investigacion---accion que se llevo a cabo en la sala de clases tenia como punto de partida las creencias de la cultura de la ciencia de un grupo de estudiantes universitarios para luego examinar sus implicaciones en el proceso de aprendizaje de las Ciencias Biologicas. ¿Que se supone que hagan las creencias en relacion con el aprendizaje? ¿En que consiste incorporar este aspecto a la practica educativa universitaria? Utilizando el modelo de Kemmis y McTaggart (1987) la investigacion-accion se planteo como un proceso dinamico en cuatro momentos en espiral constituidos por la planificacion, la accion, la observacion y la reflexion. Cada una de las fases tuvo una intencion retrospectiva y prospectiva formando una espiral de autorreflexion del conocimiento y la accion. Se llevaron a cabo audio grabaciones en clases y analisis de documentos. Ademas, la profesora-investigadora hizo un portafolio para reflexionar sobre las creencias de la cultura de la ciencia que tienen los estudiantes y las creencias del aprendizaje que tiene la profesora y sobre como la comprension de estos elementos ayudo a mejorar su practica educativa a traves del tiempo. Los resultados obtenidos apuntan a que las creencias de la cultura de la ciencia que tiene el grupo de estudiantes son diversas. Ellos si creen que la ciencia tiene una cultura la cual describieron como: complicada y desconocida que evoluciona constantemente, que es un conjunto de metodos, que es altamente tecnologica, que resuelve problemas de salud, ayuda a interpretar la realidad del mundo que los rodea y su origen y que existen unas intersecciones entre la ciencia y el poder. Sobre las creencias del proceso de aprendizaje de la profesora-investigadora, estas senalan que el modelaje de actores, la vision de la academia que tiene ella asi como la participacion y negociacion entre todos los involucrados en el proceso educativo, son factores que inciden en el proceso de aprendizaje.

  8. How Is Vasculitis Treated?

    MedlinePlus

    ... Clinical Trials Links Related Topics Aneurysm Blood Tests Kawasaki Disease Raynaud's Smoking and Your Heart Send a link ... of vasculitis. For example, the standard treatment for Kawasaki disease is high-dose aspirin and immune globulin. Immune ...

  9. Cardiovascular Conditions of Childhood

    MedlinePlus

    ... diagnosed, treated and even cured in younger children. Kawasaki Disease This childhood illness can result in long-term ... complications. Learn the symptoms, diagnosis and treatment for Kawasaki disease. Cholesterol Raised cholesterol levels early in life may ...

  10. 78 FR 13341 - Notice of Agreements Filed

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-02-27

    ... Carriers, Inc. and Kawasaki Kisen Kaisha, Ltd. ] Filing Party: John P. Meade, Esq.; Vice-President; K-Line... geographic scope of the agreement and updates the address of Kawasaki Kisen Kaisha, Ltd. By Order of the...

  11. Distinción Empírica Entre Engagement y Trabajolismo en Enfermeras Hospitalarias de Japón: Efecto Sobre la Calidad del Sueño y el Desempeño Laboral

    PubMed Central

    Kubota, Kazumi; Shimazu, Akihito; Kawakami, Norito; Takahashi, Masaya; Nakata, Akinori; Schaufeli, Wilmar B.

    2016-01-01

    Objetivo El objetivo de este estudio es demostrar la distinción entre engagement y trabajolismo, estudiando su relación con la calidad del sueño y el desempeño laboral. Método Un total de 447 enfermeras de 3 hospitales de Japón fueron entrevistadas mediante un cuestionario autoadministrado que incluía la escala Utrecht (UWES, Utrecht Work Engagement Scale), la Escala de Adicción al Trabajo Holandesa (DUWAS, Dutch Workaholism Scale), preguntas sobre la calidad del sueño (7 ítems) con respecto a (1) dificultad para conciliar el sueño, (2) dificultad para mantener el sueño, (3) despertar temprano por la mañana, (4) dormirse o tomar siestas durante el día, (5) somnolencia diurna excesiva en el trabajo, (6) dificultad para despertarse por la mañana, y (7) despertar cansado en la mañana, y el Cuestionario sobre Salud y Desempeño (CSD) de la Organización Mundial de la Salud. Resultados Los modelos de ecuaciones estructurales demostraron que el engagement se relaciona positivamente con la calidad del sueño y el rendimiento laboral, mientras que el trabajolismo tiene una relación negativa con la calidad del sueño y el desempeño laboral. Conclusión Los resultados indican que el engagement y el trabajolismo son conceptualmente diferentes. El primero tiene una connotación positiva, mientras que el segundo se asocia de manera negativa al bienestar (buena calidad del sueño y buen rendimiento en el trabajo). PMID:26752805

  12. Modelos para la Unificacion de Conceptos, Metodos y Procedimientos Administrativos (Guidelines for Uniform Administrative Concepts, Methods, and Procedures).

    ERIC Educational Resources Information Center

    Serrano, Jorge A., Ed.

    These documents, discussed and approved during the first meeting of the university administrators affiliated with the Federation of Private Universities of Central America and Panama (FUPAC), seek to establish uniform administrative concepts, methods, and procedures, particularly with respect to budgetary matters. The documents define relevant…

  13. High School Student's Alternative Conceptions About the Phenomenon of the Formation of the Moon Phases. (Spanish Title: Concepciones Alternativas de Alumnos de Educación Media Sobre el Fenómeno de Formación de las Fases de La Luna.) Concepções Alternativas de Alunos do Ensino Médio Sobre o Fenômeno de Formação das Fases da Lua

    NASA Astrophysics Data System (ADS)

    Iachel, Gustavo; Langhi, Rodolfo; Fernandes Scalvi, Rosa Maria

    2008-07-01

    Forty students, at ages between 14 and 18 years old, from three schools in Bauru city, were questioned about their alternative conceptions concerning the phenomenon of formation of the Moon Phases. It was observed that some of the pupils confound the phenomenon of the formation of the Moon Phases with the phenomenon of the formation of the lunar eclipses, others are unaware of the reason of the phenomenon, they present incoherent alternative conceptions of the reality or incomplete conceptions. The results found here are aimed at the teachers of Elementary Education and can be used as a subsidy for future development of new pedagogical methods. Cuarenta estudiantes, con edad entre 14 y 18 años, pertenecientes a tres escuelas de la ciudad de Bauru, fueron cuestionados sobre sus concepciones alternativas acerca del fenómeno de la formación de las fases de la Luna. Fue observado que algunos alumnos confunden el fenómeno de formación de las fases de la Luna con el fenómeno de formación de los eclipses lunares, otros desconocen el motivo delfenómeno, presentan concepciones alternativas incoherentes con la realidad o bien presentan concepciones incompletas. Los resultados aquí encontrados son destinados a los profesores de la Enseñanza Básica y podrán ser usados como ayuda para el futuro desarrollo de nuevos métodos pedagógicos. Quarenta estudantes, com idades entre 14 e 18 anos, pertencentes a três escolas da cidade de Bauru, foram questionados sobre suas concepções alternativas acerca do fenômeno de formação das fases da Lua. Foi observado que alguns dos alunos confundem o fenômeno da formação das fases da Lua com o fenômeno da formação dos eclipses lunares, outros desconhecem o motivo do fenômeno, apresentam concepções alternativas incoerentes com a realidade ou então concepções incompletas. Os resultados aqui encontrados são destinados aos professores do Ensino Básico e poderão ser usados como subsídio parafuturo desenvolvimento de

  14. [The Spanish AIDS Study Group and Spanish National AIDS Plan (GESIDA/Secretaría del Plan Nacional sobre el Sida) recommendations for the treatment of tuberculosis in HIV-infected individuals (Updated January 2013)].

    PubMed

    Rivero, Antonio; Pulido, Federico; Caylá, Joan; Iribarren, José A; Miró, José M; Moreno, Santiago; Pérez-Camacho, Inés

    2013-12-01

    This consensus document was prepared by an expert panel of the Grupo de Estudio de Sida (GESIDA [Spanish AIDS Study Group]) and the Plan Nacional sobre el Sida (PNS [Spanish National AIDS Plan]). The document updates current guidelines on the treatment of tuberculosis (TB) in HIV-infected individuals contained in the guidelines on the treatment of opportunistic infections published by GESIDA and PNS in 2008. The document aims to facilitate the management and treatment of HIV-infected patients with TB in Spain, and includes specific sections and recommendations on the treatment of drug-sensitive TB, multidrug-resistant TB, and extensively drug-resistant TB, in this population. The consensus guidelines also make recommendations on the treatment of HIV-infected patients with TB in special situations, such as chronic liver disease, pregnancy, kidney failure, and transplantation. Recommendations are made on the timing and initial regimens of antiretroviral therapy in patients with TB, and on immune reconstitution syndrome in HIV-infected patients with TB who are receiving antiretroviral therapy. The document does not cover the diagnosis of TB, diagnosis/treatment of latent TB, or treatment of TB in children. The quality of the evidence was evaluated and the recommendations graded using the approach of the Grading of Recommendations Assessment, Development and Evaluation Working Group.

  15. Coronary artery aneurysm presenting as a large mass encroaching on the right atrium in an adult patient with untreated Kawasaki disease

    PubMed Central

    Wassef, Nancy

    2015-01-01

    A 26-year-old man presented with atypical chest pain and dyspnoea for several days before admission that was not related to exertion. The patient had no medical history other than a long duration of fever and rash as a child, which resolved after a few weeks. The initial blood investigations showed thrombocytopenia and raised inflammatory markers with a negative troponin level. The patient had positive antiplatelet antibodies and was diagnosed with immune thrombocytopenic purpura (ITP). Echocardiography showed a large cystic mass at the right atrium. Coronary multislice CT showed a huge aneurysm at the origin of the right coronary artery (RCA), which was confirmed by coronary angiography. The patient received intravenous immunoglobulin and platelet transfusion before coronary artery bypass surgery and he had a successful resection of the aneurysm with a saphenous vein graft to distal RCA. The patient had a good recovery and was discharged home. PMID:25819826

  16. Efectos combinados de la ampliación de la atención primaria de salud y de las transferencias condicionadas de dinero en efectivo sobre la mortalidad infantil en Brasil, 1998–2010*

    PubMed Central

    Guanais, Frederico C.

    2015-01-01

    Objetivos. Examiné los efectos combinados del acceso a la atención primaria mediante el Programa de Salud Familiar (PSF) y las transferencias condicionadas de dinero en efectivo del Programa Bolsa Familia (PBF) sobre la mortalidad infantil posneonatal (MIPN) en Brasil. Métodos. Empleé un análisis ecológico longitudinal usando datos en panel de 4 583 municipios brasileños de 1998 al 2010, con 54 253 observaciones en total. Estimé modelos de regresión de efectos fijos por mínimos cuadrados ordinarios, con la tasa de MIPN como la variable dependiente y el PSF, el PBF y sus interacciones como las principales variables independientes de interés. Resultados. La asociación de una mayor cobertura del PSF con una menor tasa de MIPN se volvió más fuerte conforme aumentaba la cobertura del PBF. En los promedios de todas las demás variables, cuando la cobertura de PBF era 25%, la MIPN predicha fue 5,24 (intervalo de confianza [IC] de 95% = 4,95, 5,53) para una cobertura del PSF de 0%, y de 3,54 (IC de 95% = 2,77, 4,31) para una cobertura del PSF de 100%. Cuando la cobertura del PBF era de 60%, la MIPN predicha fue 4,65 (IC de 95% = 4,36, 4,94) para una cobertura del PSF de 0%, y de 1,38 (IC de 95% = 0,88, 1,89) para una cobertura del PSF de 100%. Conclusiones. El efecto del PSF depende de la ampliación del PBF. Para las poblaciones empobrecidas y subatendidas, la combinación de intervenciones tanto del lado de la oferta como del lado de la demanda podría ser necesaria para mejorar los resultados en salud.

  17. High School Students' Previous Knowledge about the Stars. (Spanish Title: Conocimientos Previos de Estudiantes de Secundaria Acerca de Las Estrellas.) O Conhecimento Prévio de Alunos do Ensino Médio sobre as Estrelas

    NASA Astrophysics Data System (ADS)

    Iachel, Gustavo

    2011-12-01

    Based on the readings of articles related to Astronomy Education published in Brazil, it was noticed a lack of research on previous knowledge of students about the physical characteristics of stars, a fact that motivated us to develop this study. Previous knowledge o