Mucocutaneous Lymph Node Syndrome (Kawasaki Disease)

An acute, febrile, mucocutaneous condition accompanied by swelling of cervical lymph nodes in infants and young children. The principal symptoms are fever, congestion of the ocular conjunctivae, reddening of the lips and oral cavity, protuberance of tongue papillae, and edema or erythema of the extremities.

Also Known As:

Networked: 1628 relevant articles (97 outcomes, 185 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Aneurysm (Aneurysms)
2.	Idiopathic Thrombocytopenic Purpura (Thrombocytopenic Purpura, Autoimmune)
3.	Dermatomyositis (Dermatopolymyositis)
4.	Vasculitis (Vasculitides)
5.	Myocarditis (Carditis)

Experts

1.	Burns, Jane C: 42 articles (12/2015 - 02/2003)
2.	Shulman, Stanford T: 24 articles (08/2015 - 02/2002)
3.	Kuo, Ho-Chang: 23 articles (03/2015 - 06/2007)
4.	Newburger, Jane W: 21 articles (03/2013 - 06/2003)
5.	Yang, Kuender D: 20 articles (03/2015 - 10/2002)
6.	Tremoulet, Adriana H: 18 articles (12/2015 - 07/2008)
7.	Rowley, Anne H: 18 articles (08/2015 - 02/2002)
8.	Miura, Masaru: 16 articles (07/2015 - 01/2004)
9.	Terai, Masaru: 16 articles (01/2015 - 02/2002)
10.	Saji, Tsutomu: 16 articles (09/2014 - 12/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Mucocutaneous Lymph Node Syndrome:

1.	Intravenous Immunoglobulins (IVIG)FDA Link 05/01/2002 - "Although the exact mode of action is still poorly understood, the efficacy of IVIg in several diseases, such as thrombocytopenic pupura and Kawasaki disease, has been proven in clinical trials. " 08/01/2003 - "The efficacy of a high dose (2 g/kg) of Thai Red-Cross IVIG in Kawasaki disease is excellent. " 10/01/1989 - "High-dose intravenous immune globulin treatment is effective in preventing the occurrence of coronary artery abnormalities in Kawasaki disease. " 01/01/2013 - "Studies that compare the efficacy of IVIG plus corticosteroid with that of IVIG in treating Kawasaki disease were included. " 12/01/2014 - "High-density genotyping of immune loci in Kawasaki disease and IVIG treatment response in European-American case-parent trio study."
2.	Immunoglobulins (Immunoglobulin)IBA 02/01/2008 - "High-dose intravenous immunoglobulin (VIG) treatment for the acute stage of Kawasaki disease (KD) has shown to be effective and safe. " 08/01/2015 - "Glucocorticosteroids are more effective in controlling body temperature compared with intravenous immunoglobulin re-treatment in intravenous immunoglobulin-resistant Kawasaki disease children; however, glucocorticosteroids and intravenous immunoglobulin re-treatment showed no difference in the prevention of coronary artery lesions." 07/01/2010 - "In spite of the efficacy of intravenous immunoglobulin (IGIV), the absence of a specific diagnostic test and due to there being IGIV-refractory patients, Kawasaki disease is a major cause of coronary artery abnormalities (CAA). " 06/01/2009 - "More than half of the patients with acute Kawasaki disease, nonetheless, can be cured by giving only 1 gram per kilogram of immunoglobulin, thus reducing this aspect of the cost of treatment by half. " 11/01/1994 - "The mechanism of efficacy of intravenous immunoglobulin in Kawasaki disease."
3.	gamma-Globulins (gamma-Globulin)IBA 04/01/2003 - "Following the standard treatment with intravenous gamma globulin, the initial symptoms and signs of Kawasaki disease resolved completely. " 03/01/1995 - "However, high-dose intravenous gamma-globulin (IVGG) is a safe and effective treatment for Kawasaki disease. " 08/07/1986 - "We conclude that high-dose intravenous gamma globulin is safe and effective in reducing the prevalence of coronary-artery abnormalities when administered early in the course of Kawasaki syndrome." 08/01/2000 - "We evaluated the efficacy of intravenous gamma-globulin (IVGG) administration for children with Kawasaki disease to establish whether additional, more advanced therapy is needed in intractable cases. " 06/06/1991 - "In children with acute Kawasaki disease, a single large dose of intravenous gamma globulin is more effective than the conventional regimen of four smaller daily doses and is equally safe."
4.	Aspirin (Acetylsalicylic Acid)FDA LinkGeneric 11/01/1990 - "The effects of intravenous gammaglobulin (IVGG) on changes in the peripheral blood mononuclear cell subsets during acute Kawasaki disease (KD) were studied by a random selection trial of IVGG plus Aspirin (group G) compared to Aspirin alone (group A). " 02/01/1988 - "Studies on the effect of long-term use of low dose aspirin in Kawasaki disease." 05/01/1987 - "To evaluate the effectiveness of gammaglobulin in decreasing the incidence of coronary artery lesions in Kawasaki disease, a randomized controlled study in 136 patients was conducted using high doses of gammaglobulin 400 mg/kg/d for 3 days plus aspirin 30 mg/kg/d (gammaglobulin group) and aspirin alone at the same dosage (aspirin group). " 02/01/1987 - "The effect of intravenous gammaglobulin (IVGG) on the immunoregulatory abnormalities found during acute Kawasaki syndrome (KS) was studied in a randomized trial of IVGG plus aspirin (ASA) versus ASA alone. " 11/10/1984 - "The ability of high-dose intravenous gammaglobulin (IVGG) to prevent the coronary artery lesion of Kawasaki disease has been studied in a multicentre controlled trial of IVGG plus aspirin versus aspirin alone, aspirin being the conventional treatment for Kawasaki disease. "
5.	Prednisolone (Predate)FDA LinkGeneric 11/01/2012 - "Adding prednisolone to standard immunoglobulin therapy is beneficial for patients with severe Kawasaki disease." 09/01/2014 - "The present paper reviews the roles of prednisolone therapy for acute phase Kawasaki disease patients." 09/01/2014 - "Prednisolone therapy for acute phase Kawasaki disease had been considered to be controversial for several decades. " 09/01/2014 - "[Prednisolone therapy for Kawasaki disease]." 04/28/2012 - "Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial."
6.	Antineutrophil Cytoplasmic Antibodies (ANCA)IBA 01/01/2012 - "To study the value of anti-neutrophil cytoplasmic antibody (ANCA) in diagnosis of Kawasaki disease (KD). " 01/01/2012 - "Serum ANCA was detected in 30 children with typical Kawasaki disease (TKD) and in 16 with incomplete Kawasaki disease (IKD) in the acute and the recovery phases respectively. " 01/01/2012 - "[Value of anti-neutrophil cytoplasmic antibody in diagnosis of Kawasaki disease]." 05/01/1993 - "Newer investigative procedures and therapeutic approaches have led to more accurate diagnoses and improved outcome but further advances await a better understanding of the vasculitides: polyarteritis; Kawasaki disease; Wagener's granulomatosis; and ANCA in childhood." 01/01/2007 - "We screened the sera of 148 MPO-ANCA positive patients from six vasculitic syndromes: rapidly progressive gromerulonephritis (RPGN), microscopic polyangiitis (MPA), idiopathic crescentic glomerulonephritis (I-CrGN), classic polyangiitis nodosa (cPAN), Churg-Strauss syndrome (CSS), Kawasaki disease (KD); and from patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). "
7.	CytokinesIBA 05/01/2010 - "Urinary cytokines and renal Doppler study in Kawasaki disease." 03/01/1995 - "This study provides evidence to support the involvement of activated mononuclear cells, both T cells and monocytes and their secreted soluble products, cytokines, in the pathogenesis of Kawasaki disease." 11/01/2015 - "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. " 11/01/2014 - "Downregulation of Th17 cells and the related cytokines with treatment in Kawasaki disease." 09/01/2014 - "[Cytokines in Kawasaki disease]."
8.	Adrenal Cortex Hormones (Corticosteroids)IBA 07/01/2009 - "We included randomised trials comparing the addition of corticosteroids to conventional primary therapy for Kawasaki disease. " 09/01/2006 - "A multicenter prospective randomized trial of corticosteroids in primary therapy for Kawasaki disease: clinical course and coronary artery outcome." 06/01/2003 - "Corticosteroids in the initial treatment of Kawasaki disease: report of a randomized trial." 10/01/1999 - "Corticosteroids are considered to be contraindicated during the acute phase of Kawasaki disease (KD) based on unfavorable results in early studies. " 11/08/2015 - "Three options of using corticosteroids for the treatment of patients during the acute phase of Kawasaki disease, have been considered. "
9.	infliximab (Remicade)FDA Link 11/01/2010 - "Infliximab administration effective in the treatment of refractory Kawasaki disease." 07/01/2006 - "Efficacy of infliximab in long-lasting refractory Kawasaki disease." 05/17/2014 - "Infliximab for intensification of primary therapy for Kawasaki disease: a phase 3 randomised, double-blind, placebo-controlled trial." 05/17/2014 - "The addition of infliximab to primary treatment in acute Kawasaki disease did not reduce treatment resistance. " 01/01/2014 - "Infliximab as rescue therapy in refractory Kawasaki disease."
10.	Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA 08/01/2010 - "There have been genetic studies assessing the association between tumor necrosis factor (TNF)-α-308 and Kawasaki disease (KD) but the results have been conflicting due to lack of power. " 05/15/2006 - "In this study, we report rapid production of TNF-alpha in the peripheral immune system after disease induction in a murine model of Kawasaki disease. " 01/01/2015 - "[Recent advance in tumor necrosis factor-α blockade treatment for Kawasaki disease]." 08/01/2010 - "A systematic review and meta-analysis of tumor necrosis factor α-308 polymorphism and Kawasaki disease." 09/01/2008 - "Our findings suggest that CRP +1444 C-->T and TNF-alpha -308 G-->A polymorphisms are associated with predisposition to Kawasaki disease and, in patients with Kawasaki disease, increased carotid arterial stiffness and intima-media thickness in the long-term."

Therapies and Procedures

1.	Plasma Exchange 02/01/2012 - "The aim of the present study was to investigate the long-term efficacy of plasma exchange (PE) treatment for refractory Kawasaki disease. " 06/01/2012 - "Efficacy of plasma exchange therapy for Kawasaki disease by cytokine profiling." 02/01/2012 - "Long-term efficacy of plasma exchange treatment for refractory Kawasaki disease." 08/01/2015 - "Search terms used included "Kawasaki disease" and "plasma exchange" to extract articles that described KD cases treated with PE. " 04/01/2008 - "A report of two cases of Kawasaki disease treated with plasma exchange."
2.	Transplants (Transplant) 04/01/1990 - "Thus an internal mammary artery graft has proved to be satisfactory in the management of children with coronary artery obstruction caused by Kawasaki disease; it appears that both the quality of life and the life expectancy of those patients can be improved by the surgery." 07/01/2014 - "We tested the hypothesis that OCT could identify coronary abnormalities not seen by angiography in children with a history of Kawasaki disease (KD) and pediatric heart transplant (TX) recipients. " 10/01/2013 - "Decreased portal vein flow during Kawasaki disease in a liver transplant patient." 06/01/2012 - "Histopathology of the longest-lived saphenous vein graft in a patient with Kawasaki disease." 11/01/2006 - "Redo off-pump coronary bypass grafting with arterial grafts for Kawasaki disease."
3.	Coronary Artery Bypass (Coronary Artery Bypass Surgery) 07/01/2010 - "To compare the long-term efficacy of percutaneous coronary intervention (PCI) and coronary artery bypass grafting (CABG) for stenotic lesions after Kawasaki disease. " 10/01/2001 - "The purpose of this study was to assess the long-term clinical outcome of coronary artery bypass grafting in pediatric patients with Kawasaki disease. " 09/01/2014 - "[Coronary artery bypass grafting for cardiovascular sequelae in Kawasaki disease]." 06/01/2012 - "[Coronary artery bypass after Kawasaki disease]." 09/01/2010 - "Pediatric coronary artery bypass is performed for ischemic complications of Kawasaki disease, congenital coronary anomalies, and iatrogenic pediatric coronary artery problems. "
4.	Transplantation (Transplant Recipients) 07/01/2014 - "Feasibility of optical coherence tomography in children with Kawasaki disease and pediatric heart transplant recipients." 02/01/2013 - "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. " 07/01/2000 - "Experience is reported in children with prior Kawasaki disease or heart transplant recipients, as well as patients with congenital coronary abnormalities. " 10/01/1997 - "In these 10 patients, the timing of transplantation was within 6 months after diagnosis of Kawasaki disease (4 patients), 1 to 5 years after diagnosis (3 patients), and 9 to 12 years after diagnosis (3 patients). " 02/01/2013 - "An apparent case of undiagnosed donor Kawasaki disease manifesting as coronary artery aneurysm in a pediatric heart transplant recipient."
5.	Catheters 06/25/2002 - "Catheter intervention for Kawasaki disease can be accomplished and can be effective in the short term, but the long-term efficacy should be verified by further study." 06/25/2002 - "The purpose of this study was to assess the sequential follow-up results of catheter intervention in Kawasaki disease by use of quantitative coronary angiography (QCA) and intravascular ultrasound imaging. " 06/25/2002 - "Sequential follow-up results of catheter intervention for coronary artery lesions after Kawasaki disease: quantitative coronary artery angiography and intravascular ultrasound imaging study." 10/01/2001 - "The Research Committee of Ministry of Health, Labour and Welfare 'Study of treatment and long-term management in Kawasaki disease' reported the guidelines for catheter intervention in coronary artery lesion in Kawasaki disease in this paper. " 02/01/2011 - "Rotational ablation may be the most appropriate catheter intervention technique for patients with Kawasaki disease. "