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Jane C Burns - One of the best experts on this subject based on the ideXlab platform.
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seasonality of Kawasaki Disease a global perspective
PLOS ONE, 2013Co-Authors: Jane C Burns, David Burgner, Adriana H Tremoulet, Lauren Herzog, Olivia Fabri, Xavier Rodo, Ritei Uehara, Emelia Bainto, David W Pierce, Mary TyreeAbstract: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.
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Kawasaki Disease late cardiovascular sequelae
Current Opinion in Cardiology, 2012Co-Authors: Lori B Daniels, John B Gordon, Jane C BurnsAbstract:Purpose of reviewKawasaki Disease was first described in Japanese in 1967, and the first English language report appeared in 1974. Consequently, only recently have Kawasaki Disease patients reached adulthood and come to the attention of adult cardiologists. As children with Kawasaki Disease grow up,
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a genome wide association study identifies three new risk loci for Kawasaki Disease
Nature Genetics, 2012Co-Authors: Yoshihiro Onouchi, Jane C Burns, Chisato Shimizu, Kouichi Ozaki, Masaru Terai, Hiromichi Hamada, Takafumi Honda, Hiroyuki SuzukiAbstract:We performed a genome-wide association study (GWAS) of Kawasaki Disease in Japanese subjects using data from 428 individuals with Kawasaki Disease (cases) and 3,379 controls genotyped at 473,803 SNPs. We validated the association results in two independent replication panels totaling 754 cases and 947 controls. We observed significant associations in the FAM167A-BLK region at 8p22-23 (rs2254546, P = 8.2 × 10(-21)), in the human leukocyte antigen (HLA) region at 6p21.3 (rs2857151, P = 4.6 × 10(-11)) and in the CD40 region at 20q13 (rs4813003, P = 4.8 × 10(-8)). We also replicated the association of a functional SNP of FCGR2A (rs1801274, P = 1.6 × 10(-6)) identified in a recently reported GWAS of Kawasaki Disease. Our findings provide new insights into the pathogenesis and pathophysiology of Kawasaki Disease.
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recognition of a Kawasaki Disease shock syndrome
Pediatrics, 2009Co-Authors: John T Kanegaye, Matthew S Wilder, Delaram Molkara, Jeffrey R Frazer, Joan Pancheri, Adriana H Tremoulet, Virginia E Watson, Brookie M Best, Jane C BurnsAbstract: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 CONCLUSIONS. Kawasaki Disease shock syndrome is associated with more-severe laboratory markers of inflammation and greater risk of coronary artery abnormalities, mitral regurgitation, and prolonged myocardial dysfunction. These patients may be resistant to immunoglobulin therapy and require additional antiinflammatory treatment.
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itpkc functional polymorphism associated with Kawasaki Disease susceptibility and formation of coronary artery aneurysms
Nature Genetics, 2008Co-Authors: Yoshihiro Onouchi, Jane C Burns, Chisato Shimizu, Jane W Newburger, Tomohiko Gunji, Mayumi Yashiro, Yoshikazu Nakamura, Hiroshi YanagawaAbstract: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.
Jane W Newburger - One of the best experts on this subject based on the ideXlab platform.
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treatment of Kawasaki Disease analysis of 27 us pediatric hospitals from 2001 to 2006
Pediatrics, 2009Co-Authors: Mary Beth F Son, Robert P Sundel, Annette L Baker, Kimberlee Gauvreau, David Fulton, Jane W NewburgerAbstract:OBJECTIVES: We sought to analyze trends in admissions and to describe therapies used for acute Kawasaki Disease over a 6-year period. METHODS: The Pediatric Health Information System provides patient data including demographic variables, International Classification of Diseases, Ninth Revision codes, and services billed to patients. Patient identifiers enable tracking of medication use in and across multiple admissions within a center. We analyzed data for patients with (1) a diagnosis code for Kawasaki Disease, (2) intravenously administered immunoglobulin treatment during hospitalization, and (3) discharge between January 1, 2001, and December 30, 2006, from 27 hospitals contributing complete data over the study period. RESULTS: During the study period, 5197 Kawasaki Disease admissions were identified for 4811 patients; numbers increased 32.6% from 2001 (n = 678) to 2006 (n = 899). Retreatment with intravenous immunoglobulin was administered to 712 patients (14.8%) over the study period. Other antiinflammatory therapies included intravenously administered methylprednisolone (5.8%), orally administered prednisone (2.8%), and infliximab (1%). Use of infliximab steadily increased from 0.0% (0 of 678 patients) in 2001 to 2.3% (21 of 899 patients) in 2006. Coronary artery aneurysms were coded for 3.3% of patients. Male patients, patients CONCLUSIONS: Our report provides the first large multicenter description of agents used in the treatment of intravenously administered immunoglobulin-resistant Kawasaki Disease in the United States. Trends include increased numbers of admissions attributable to Kawasaki Disease and increased usage of infliximab.
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itpkc functional polymorphism associated with Kawasaki Disease susceptibility and formation of coronary artery aneurysms
Nature Genetics, 2008Co-Authors: Yoshihiro Onouchi, Jane C Burns, Chisato Shimizu, Jane W Newburger, Tomohiko Gunji, Mayumi Yashiro, Yoshikazu Nakamura, Hiroshi YanagawaAbstract: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.
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diagnosis treatment and long term management of Kawasaki Disease a statement for health professionals from the committee on rheumatic fever endocarditis and Kawasaki Disease council on cardiovascular Disease in the young american heart association
Pediatrics, 2004Co-Authors: Jane W Newburger, Stanford T Shulman, Jane C Burns, Masato Takahashi, Michael A Gerber, Michael H Gewitz, Lloyd Y Tani, Ann F Bolger, Patricia Ferrieri, Robert S BaltimoreAbstract:Background. Kawasaki Disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in ∼15% to 25% of untreated children and may lead to ischemic heart Disease or sudden death. Methods and Results. A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki Disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for ≥5 days and ≤4 classic criteria should undergo electrocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki Disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki Disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor-α antagonists, and abciximab. Long-term management of patients with Kawasaki Disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac Disease are classified according to risk strata. Conclusions. Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki Disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki Disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients.
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diagnosis treatment and long term management of Kawasaki Disease a statement for health professionals from the committee on rheumatic fever endocarditis and Kawasaki Disease council on cardiovascular Disease in the young american heart association
Pediatrics, 2004Co-Authors: Jane W Newburger, Stanford T Shulman, Jane C Burns, Masato Takahashi, Michael A Gerber, Michael H Gewitz, Lloyd Y Tani, Ann F Bolger, Patricia Ferrieri, Robert S BaltimoreAbstract:Background—Kawasaki Disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in 15% to 25% of untreated children and may lead to ischemic heart Disease or sudden death. Methods and Results—A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki Disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for 5 days and 4 classic criteria should undergo echocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki Disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki Disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor- antagonists, and abciximab. Long-term management of patients with Kawasaki Disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac Disease are classified according to risk strata. Conclusions—Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki Disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki Disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients. (Circulation. 2004;110:2747-2771.)
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coronary magnetic resonance angiography in adolescents and young adults with Kawasaki Disease
Circulation, 2002Co-Authors: Gerald F Greil, Rene M Botnar, Jane W Newburger, Matthias Stuber, Kraig V Kissinger, Tal Geva, Warren J Manning, Andrew J PowellAbstract:Background— In patients with Kawasaki Disease, serial evaluation of the distribution and size of coronary artery aneurysms (CAA) is necessary for risk stratification and therapeutic management. Although transthoracic echocardiography is often sufficient for this purpose initially, visualization of the coronary arteries becomes progressively more difficult as children grow. We sought to prospectively compare coronary magnetic resonance angiography (MRA) and x-ray coronary angiography findings in patients with CAA caused by Kawasaki Disease. Methods and Results— Six subjects (age 10 to 25 years) with known CAA from Kawasaki Disease underwent coronary MRA using a free-breathing T2-prepared 3D bright blood segmented k-space gradient echo sequence with navigator gating and tracking. All patients underwent x-ray coronary angiography within a median of 75 days (range, 1 to 359 days) of coronary MRA. There was complete agreement between MRA and x-ray angiography in the detection of CAA (n=11), coronary artery ste...
Brian W Mccrindle - One of the best experts on this subject based on the ideXlab platform.
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Kawasaki Disease shock syndrome versus septic shock early differentiating features despite overlapping clinical profiles
The Journal of Pediatrics, 2021Co-Authors: Alyssa Power, Kyle Runeckles, Cedric Manlhiot, Andreea Dragulescu, Annemarie Guerguerian, Brian W MccrindleAbstract:OBJECTIVES To compare the clinical features and resuscitative measures of children with Kawasaki Disease shock syndrome vs septic shock. STUDY DESIGN In this retrospective case-control study, children with Kawasaki Disease shock syndrome admitted to the intensive care unit from 2007 to 2017 were identified and compared with age-matched controls with septic shock. We studied 9 children with Kawasaki Disease shock syndrome and 18 children with septic shock. Clinical characteristics were abstracted and between-group differences were compared. RESULTS Compared with septic shock controls, children with Kawasaki Disease shock syndrome were less likely to have an underlying comorbid illness (1/9 [11%] vs 11/18 [61%]; P = .02) and were more likely to have at least 1 of the 5 classic diagnostic signs of Kawasaki Disease at presentation (9/9 [100%] vs 0/18 [0%]; P < .001), a longer duration of illness before admission (9 days [IQR, 7-14 days] vs 3 days [IQR, 1-5 days]; P = .004), and a lower platelet count at presentation (140 [IQR 73, 167]) vs 258 [IQR, 137-334]; P = .02). Among patients who underwent echocardiography, abnormalities such as ventricular dysfunction, valvulitis, and coronary artery dilation were more common in the Kawasaki Disease shock syndrome cohort (5/9 [56%] vs 0/7 [0%]; P = .03). There were no differences in volume of fluid resuscitation, vasoactive-inotropic scores, duration of inotropic therapy, or biochemical markers of illness severity (other than platelet count) between the matched groups. CONCLUSIONS A longer duration of illness before admission, lack of any significant underlying medical comorbidities, a lower platelet count, echocardiographic abnormalities, and the presence of classic diagnostic signs of Kawasaki Disease at presentation may be useful early features to differentiate Kawasaki Disease shock syndrome from septic shock.
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abstract o 71 thrombosis and thromboprophylaxis for patients with giant coronary artery aneurysms after Kawasaki Disease a study from the north american Kawasaki Disease registry
Circulation, 2015Co-Authors: Cedric Manlhiot, William T Mahle, Kevin D Hill, Dawn Tucker, Ashwini Kulkarni, Lillian Lai, Brett R Anderson, Aaron K Olson, Brian W MccrindleAbstract:Background: Children with giant coronary artery aneurysms (CAA) after Kawasaki Disease (KD) are at substantial risk of thrombosis. There are currently no evidence-based guidelines for optimal throm...
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delayed diagnosis of Kawasaki Disease what are the risk factors
Pediatrics, 2007Co-Authors: Luann L Minich, Lynn A Sleeper, Andrew M Atz, Brian W Mccrindle, Steven D Colan, Beth F Printz, Gloria L Klein, Robert P Sundel, Masato Takahashi, Victoria L VetterAbstract:OBJECTIVE Because late diagnosis of Kawasaki Disease increases the risk for coronary artery abnormalities, we explored the prevalence of and possible risk factors for delayed diagnosis by using the database of the Pediatric Heart Network trial of corticosteroid treatment for Kawasaki Disease. METHODS We collected sociodemographic and clinical data at presentation for all patients who were treated for presumed Kawasaki Disease at 8 centers (7 in the United States, 1 in Canada). Delayed diagnosis was evaluated by total number of illness days to diagnosis and by the percentage of patients who were treated after day 10 of illness. Independent predictors of delayed diagnosis were identified by using multivariate linear and logistic regression. RESULTS Of the 589 patients who received intravenous immunoglobulin, 27 were treated before screening for the trial and excluded; 562 patients formed the cohort for analysis. Kawasaki Disease was diagnosed at 7.9 +/- 3.9 days, 92 (16%) cases after day 10. Centers were similar with respect to patient age and gender. Centers differed in the patient percentage with incomplete Kawasaki Disease; clinical criteria of cervical adenopathy, oral changes, and conjunctivitis; and distance of residence from the center. Independent predictors of greater number of illness days at diagnosis included center, age of <6 months, incomplete Kawasaki Disease, and greater distance from the center. Independent predictors of diagnosis after day 10 were age of <6 months, incomplete Kawasaki Disease, and greater distance). Socioeconomic variables had no association with delayed diagnosis. CONCLUSIONS Even after adjustment for patient factors, illness duration at diagnosis varies by center. These findings underscore the need to maintain a high index of suspicion of Kawasaki Disease in the infant who is younger than 6 months and has prolonged fever even with incomplete criteria. Outreach educational programs may be useful in promoting earlier recognition and treatment of Kawasaki Disease.
Robert S Baltimore - One of the best experts on this subject based on the ideXlab platform.
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diagnosis treatment and long term management of Kawasaki Disease a statement for health professionals from the committee on rheumatic fever endocarditis and Kawasaki Disease council on cardiovascular Disease in the young american heart association
Pediatrics, 2004Co-Authors: Jane W Newburger, Stanford T Shulman, Jane C Burns, Masato Takahashi, Michael A Gerber, Michael H Gewitz, Lloyd Y Tani, Ann F Bolger, Patricia Ferrieri, Robert S BaltimoreAbstract:Background. Kawasaki Disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in ∼15% to 25% of untreated children and may lead to ischemic heart Disease or sudden death. Methods and Results. A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki Disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for ≥5 days and ≤4 classic criteria should undergo electrocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki Disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki Disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor-α antagonists, and abciximab. Long-term management of patients with Kawasaki Disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac Disease are classified according to risk strata. Conclusions. Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki Disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki Disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients.
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diagnosis treatment and long term management of Kawasaki Disease a statement for health professionals from the committee on rheumatic fever endocarditis and Kawasaki Disease council on cardiovascular Disease in the young american heart association
Pediatrics, 2004Co-Authors: Jane W Newburger, Stanford T Shulman, Jane C Burns, Masato Takahashi, Michael A Gerber, Michael H Gewitz, Lloyd Y Tani, Ann F Bolger, Patricia Ferrieri, Robert S BaltimoreAbstract:Background—Kawasaki Disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in 15% to 25% of untreated children and may lead to ischemic heart Disease or sudden death. Methods and Results—A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki Disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for 5 days and 4 classic criteria should undergo echocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki Disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki Disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor- antagonists, and abciximab. Long-term management of patients with Kawasaki Disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac Disease are classified according to risk strata. Conclusions—Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki Disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki Disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients. (Circulation. 2004;110:2747-2771.)
Stanford T Shulman - One of the best experts on this subject based on the ideXlab platform.
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Kawasaki Disease insights into pathogenesis and approaches to treatment
Nature Reviews Rheumatology, 2015Co-Authors: Stanford T Shulman, Anne H RowleyAbstract:This Review summarizes recent advances in understanding of the pathologic processes and pathophysiologic mechanisms leading to coronary arteritis in Kawasaki Disease, and describes current approaches to its treatment. Kawasaki Disease is the most common cause of acquired heart Disease among children in developed countries, in whom the resulting coronary artery abnormalities can cause myocardial ischaemia, infarction and even death. Epidemiologic data strongly suggest an infectious aetiology, although the causative agent has yet to be identified. Genetic factors also increase susceptibility to Kawasaki Disease, as indicated by its strikingly high incidence rate in children of Asian ethnicity and by an increased incidence in first-degree family members. The treatment of Kawasaki Disease is based on timely administration of intravenous immunoglobulin and aspirin. However, the management of patients who do not respond to this standard therapy remains challenging; although several options are available, comparative data on which to base treatment decisions are scarce. The added value of adjunctive therapy with corticosteroids in patients at particularly high risk of coronary complications has been demonstrated in Japanese populations, but identification of high-risk patients has proven to be difficult in ethnically diverse populations.
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Kawasaki Disease summary of the american heart association guidelines
American Family Physician, 2006Co-Authors: Alexandra F Freeman, Stanford T ShulmanAbstract:Kawasaki Disease is an acute vasculitis of childhood that predominantly affects the coronary arteries. The etiology of Kawasaki Disease remains unknown, although an infectious agent is strongly suspected based on clinical and epidemiologic features. A genetic predisposition is also likely, based on varying incidences among ethnic groups, with higher rates in Asians. Symptoms include fever, conjunctival injection, erythema of the lips and oral mucosa, rash, and cervical lymphadenopathy. Some children with Kawasaki Disease develop coronary artery aneurysms or ectasia, ischemic heart Disease, and sudden death. Kawasaki Disease is the leading cause of acquired heart Disease among children in developed countries. This article provides a summary of the diagnostic and treatment guidelines published by the American Heart Association.
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diagnosis treatment and long term management of Kawasaki Disease a statement for health professionals from the committee on rheumatic fever endocarditis and Kawasaki Disease council on cardiovascular Disease in the young american heart association
Pediatrics, 2004Co-Authors: Jane W Newburger, Stanford T Shulman, Jane C Burns, Masato Takahashi, Michael A Gerber, Michael H Gewitz, Lloyd Y Tani, Ann F Bolger, Patricia Ferrieri, Robert S BaltimoreAbstract:Background. Kawasaki Disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in ∼15% to 25% of untreated children and may lead to ischemic heart Disease or sudden death. Methods and Results. A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki Disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for ≥5 days and ≤4 classic criteria should undergo electrocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki Disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki Disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor-α antagonists, and abciximab. Long-term management of patients with Kawasaki Disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac Disease are classified according to risk strata. Conclusions. Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki Disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki Disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients.
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diagnosis treatment and long term management of Kawasaki Disease a statement for health professionals from the committee on rheumatic fever endocarditis and Kawasaki Disease council on cardiovascular Disease in the young american heart association
Pediatrics, 2004Co-Authors: Jane W Newburger, Stanford T Shulman, Jane C Burns, Masato Takahashi, Michael A Gerber, Michael H Gewitz, Lloyd Y Tani, Ann F Bolger, Patricia Ferrieri, Robert S BaltimoreAbstract:Background—Kawasaki Disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in 15% to 25% of untreated children and may lead to ischemic heart Disease or sudden death. Methods and Results—A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki Disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for 5 days and 4 classic criteria should undergo echocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki Disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki Disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor- antagonists, and abciximab. Long-term management of patients with Kawasaki Disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac Disease are classified according to risk strata. Conclusions—Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki Disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki Disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients. (Circulation. 2004;110:2747-2771.)