scholarly journals Concurrent Adrenal Neuroblastoma and Kawasaki Disease: A Report of a Rare Case

2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Samin Alavi ◽  
Alireza Fahimzad ◽  
Farzaneh Jadali ◽  
Farid Ghazizadeh ◽  
Armin Rashidi
Keyword(s):  
Heart Disease ◽  
Immune System ◽  
Kawasaki Disease ◽  
Rare Case ◽  
Systemic Vasculitis ◽  
Sympathetic Ganglia ◽  
Unknown Etiology ◽  
Susceptible Host ◽  
Acquired Heart Disease ◽  
Made In

Kawasaki disease (KD) is a systemic vasculitis of unknown etiology and a leading cause of acquired heart disease. It is assumed that there is an activation of the immune system by an infectious trigger in a genetically susceptible host. Neuroblastoma is the most common extracranial solid tumor in young children. It mainly originates from primordial neural crest cells that generate the adrenal medulla and sympathetic ganglia. A diagnosis of concurrent KD and neuroblastoma in a living child has been made in only one previous report. We report the second case and review the literature.

scholarly journals MicroRNA-223 Regulates the Development of Cardiovascular Lesions in LCWE-Induced Murine Kawasaki Disease Vasculitis by Repressing the NLRP3 Inflammasome

2021 ◽  
Vol 9 ◽  
Author(s):  
Daisuke Maruyama ◽  
Begüm Kocatürk ◽  
Youngho Lee ◽  
Masanori Abe ◽  
Malcolm Lane ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Nlrp3 Inflammasome ◽  
Systemic Vasculitis ◽  
Interleukin 1 ◽  
Negative Regulator ◽  
Unknown Etiology ◽  
Transcriptomics Data ◽  
Acquired Heart Disease ◽  
Inflammasome Activity

Kawasaki disease (KD), an acute febrile childhood illness and systemic vasculitis of unknown etiology, is the leading cause of acquired heart disease among children. Experimental data from murine models of KD vasculitis and transcriptomics data generated from whole blood of KD patients indicate the involvement of the NLRP3 inflammasome and interleukin-1 (IL-1) signaling in KD pathogenesis. MicroRNA-223 (miR-223) is a negative regulator of NLRP3 activity and IL-1β production, and its expression has been reported to be upregulated during acute human KD; however, the specific role of miR-223 during KD vasculitis remains unknown. Here, using the Lactobacillus casei cell wall extract (LCWE) murine model of KD vasculitis, we demonstrate increased miR-223 expression in LCWE-induced cardiovascular lesions. Compared with control WT mice, LCWE-injected miR-223-deficient mice (miR223−/y) developed more severe coronary arteritis and aortitis, as well as more pronounced abdominal aorta aneurysms and dilations. The enhanced cardiovascular lesions and KD vasculitis observed in LCWE-injected miR223−/y mice correlated with increased NLRP3 inflammasome activity and elevated IL-1β production, indicating that miR-223 limits cardiovascular lesion development by downmodulating NLRP3 inflammasome activity. Collectively, our data reveal a previously unappreciated role of miR-223 in regulating innate immune responses and in limiting KD vasculitis and its cardiovascular lesions by constraining the NLRP3 inflammasome and the IL-1β pathway. These data also suggest that miR-223 expression may be used as a marker for KD vasculitis pathogenesis and provide a novel therapeutic target.

scholarly journals Kawasaki Disease - a Case Report

2003 ◽  
Vol 42 (148) ◽  
Author(s):  
S Shrestha ◽  
N Adhikari
Keyword(s):  
Heart Disease ◽  
Case Report ◽  
Kawasaki Disease ◽  
Immune Activation ◽  
Systemic Vasculitis ◽  
Age Group ◽  
Female Ratio ◽  
Male Female Ratio ◽  
Acquired Heart Disease ◽  
Exact Etiology

Kawasaki disease, first described by Tomisaku Kawasaki in1967, is an acute systemic vasculitis of infancy and childhood.It is now the leading cause of acquired heart disease indeveloped countries. The most common age group affected isbetween 6 months to 5 years and the peak incidence is inchildren aged 9 to 11 months. The disease has malepreponderance with the male female ratio of 1.5:1. Thoughthe exact etiology is not known the disease is known to beassociated with unusual degree of immune activation andimmunoregulatory abnormality.

scholarly journals Kawasaki disease with bronchopneumonia: A case report

2012 ◽  
Vol 1 (8) ◽  
pp. 226-228
Author(s):  
Dayana Nicholas ◽  
Kenneth Nelson ◽  
Azmi Sarriff
Keyword(s):  
Heart Disease ◽  
Case Report ◽  
Coronary Artery ◽  
Kawasaki Disease ◽  
Systemic Vasculitis ◽  
Cervical Lymphadenopathy ◽  
Pharmaceutical Journal ◽  
Infectious Agents ◽  
School Aged Children ◽  
Acquired Heart Disease

disease (KD) is an acute systemic vasculitis that predominantly affects pre-school aged children. It is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy, and irritation and inflammation of the mouth, lips, and throat. Serious complications of KS include coronary artery dilatations and aneurysms, and KS is a leading cause of acquired heart disease. It has a predilection to coronary arteries, and its precise etiology is still unknown. Many infectious agents, including viruses and bacteria, have been suggested as potential causes of the disease.DOI: http://dx.doi.org/10.3329/icpj.v1i8.11256 International Current Pharmaceutical Journal 2012, 1(8): 226-228 

scholarly journals Is macrophage activation syndrome in Kawasaki disease underrecognized?

2020 ◽  
pp. jrheum.200361 ◽  
Author(s):  
Valentina Natoli ◽  
Silvia Rosina ◽  
Angelo Ravelli
Keyword(s):  
Heart Disease ◽  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Developed Countries ◽  
Unknown Etiology ◽  
Pediatric Age ◽  
Acquired Heart Disease ◽  
Activation Syndrome

Kawasaki disease (KD) is an acute vasculitis of unknown etiology that predominantly affects children < 5 years of age. It is now the leading cause of acquired heart disease in the pediatric age in developed countries1.

scholarly journals The emergence of Kawasaki disease in India and China

2018 ◽  
Vol 2017 (3) ◽  
Author(s):  
Ankur Kumar Jindal ◽  
Vingesh Pandiarajan ◽  
Raju Khubchandani ◽  
Nutan Kamath ◽  
Tapas Sabui ◽  
...  
Keyword(s):  
Developing Countries ◽  
Heart Disease ◽  
Kawasaki Disease ◽  
Developed Countries ◽  
Resource Constrained ◽  
Diagnosis And Management ◽  
China And India ◽  
The World ◽  
Acquired Heart Disease ◽  
India And China

Kawasaki disease (KD) is recognized as a leading cause of acquired heart disease in children in developed countries. Although global in distribution, Japan records the highest incidence of KD in the world. Epidemiological reports from the two most populous countries in the world, namely China and India, indicate that KD is now being increasingly recognized. Whether this increased reporting is due to increased ascertainment, or is due to a true increase in incidence, remains a matter of conjecture. The diagnosis and management of KD in developing countries is a challenging proposition. In this review we highlight some of the difficulties faced by physicians in managing children with KD in resource-constrained settings. 

scholarly journals Kawasaki Disease: A Clinician’s Update

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Nathan Jamieson ◽  
Davinder Singh-Grewal
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Reference Lists ◽  
Systemic Vasculitis ◽  
Clinical Criteria ◽  
Treatment Practices ◽  
Fever Onset ◽  
Environmental Trigger ◽  
Acquired Heart Disease ◽  
Developed World

Aims. Kawasaki disease is an acute systemic vasculitis and is the most common cause of acquired heart disease in children in the developed world. This review aims to synthesise recent insights into the disease and provide an update for clinicians on diagnostic and treatment practices.Methods. We conducted a review of the literature exploring epidemiology, aetiology, diagnosis, and management of Kawasaki disease. We searched MEDLINE, Medline In-Process, Embase, Google Scholar, and reference lists of relevant articles.Conclusions. Kawasaki disease is a febrile vasculitis which progresses to coronary artery abnormalities in 25% of untreated patients. The disease is believed to result from a genetically susceptible individual’s exposure to an environmental trigger. Incidence is rising worldwide, and varies widely across countries and within different ethnic groups. Diagnosis is based on the presence of fever in addition to four out of five other clinical criteria, but it is complicated by the quarter of the Kawasaki disease patients with “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%. Given its severe morbidity and potential mortality, Kawasaki disease should be considered as a potential diagnosis in cases of prolonged paediatric fever.

The epidemiology of Kawasaki disease: a global update

2015 ◽  
Vol 100 (11) ◽  
pp. 1084-1088 ◽  
Author(s):  
Surjit Singh ◽  
Pandiarajan Vignesh ◽  
David Burgner
Keyword(s):  
Public Health ◽  
Heart Disease ◽  
North America ◽  
Kawasaki Disease ◽  
Common Cause ◽  
China And India ◽  
Global Epidemiology ◽  
Acquired Heart Disease ◽  
Childhood Vasculitis ◽  
Rheumatic Heart

Kawasaki disease (KD) is a childhood vasculitis and the most frequent cause of paediatric acquired heart disease in North America, Europe and Japan. It is increasingly recognised in rapidly industrialising countries such as China and India where it may replace rheumatic heart disease as the most common cause of acquired heart disease in children. We review the current global epidemiology of KD and discuss some public health implications.

scholarly journals CD40Gene Polymorphisms Associated with Susceptibility and Coronary Artery Lesions of Kawasaki Disease in the Taiwanese Population

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Ho-Chang Kuo ◽  
Mei-Chyn Chao ◽  
Yu-Wen Hsu ◽  
Ying-Chi Lin ◽  
Ying-Hsien Huang ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Systemic Vasculitis ◽  
Recessive Model ◽  
Unknown Etiology ◽  
Nucleotide Polymorphisms ◽  
Taiwanese Population ◽  
Allelic Discrimination Assay ◽  
Allelic Discrimination ◽  
First Time

Background. Kawasaki disease (KD) is characterized by systemic vasculitis of unknown etiology. Our previous studies showed expression ofCD40ligand on CD4+ T cells correlated to the coronary artery lesion (CAL) and disease progress in KD. Other studies from Japan suggested the role ofCD40Lin the pathogenesis of CAL, and this might help explain the excessive number of males affected with KD but cannot be reproduced by Taiwanese population. This study was conducted to investigate theCD40polymorphism in KD and CAL formation.Methods. A total of 950 subjects (381 KD patients and 569 controls) were investigated to identify 2 tagging single-nucleotide polymorphisms (tSNPs) ofCD40(rs4810485 and rs1535045) by using the TaqMan allelic discrimination assay.Results. A significant association was noted with regards toCD40tSNPs (rs1535045) between controls and KD patients (P=0.0405, dominant model). In KD patients, polymorphisms ofCD40(rs4810485) showed significant association with CAL formation (P=0.0436, recessive model). Haplotype analysis did not yield more significant results between polymorphisms ofCD40and susceptibility/disease activity of KD.Conclusions. This study showed for the first time that polymorphisms ofCD40are associated with susceptibility to KD and CAL formation, in the Taiwanese population.

scholarly journals DC-SIGN(CD209) Promoter −336 A/G (rs4804803) Polymorphism Associated with Susceptibility of Kawasaki Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Hong-Ren Yu ◽  
Wei-Pin Chang ◽  
Lin Wang ◽  
Ying-Jui Lin ◽  
Chi-Di Liang ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Adhesion Molecule ◽  
Risk Allele ◽  
Systemic Vasculitis ◽  
Intercellular Adhesion Molecule ◽  
Unknown Etiology ◽  
High Dose ◽  
Lectin Receptor ◽  
Ivig Resistance ◽  
Intracellular Adhesion Molecule

Kawasaki disease (KD) is characterized by systemic vasculitis of unknown etiology. High-dose intravenous immunoglobulin (IVIG) is the most effective therapy for KD to reduce the prevalence of coronary artery lesion (CAL) formation. Recently, the α2, 6 sialylated IgG was reported to interact with a lectin receptor, specific intracellular adhesion molecule-3 grabbing nonintegrin homolog-related 1 (SIGN-R1) in mice and dendritic cell-specific intercellular adhesion molecule-3 grabbing nonintegrin (DC-SIGN) in human, and to trigger an anti-inflammatory cascade. This study was conducted to investigate whether the polymorphism ofDC-SIGN(CD209) promoter −336 A/G (rs4804803) is responsible for susceptibility and CAL formation in KD patients using Custom TaqMan SNP Genotyping Assays. A total of 521 subjects (278 KD patients and 243 controls) were investigated to identify an SNP of rs4804803, and they were studied and showed a significant association between the genotypes and allele frequency of rs4804803 in control subjects and KD patients (P=0.004under the dominant model). However, the promoter variant ofDC-SIGNgene was not associated with the occurrence of IVIG resistance, CAL formation in KD. The G allele ofDC-SIGNpromoter −336 (rs4804803) is a risk allele in the development of KD.

scholarly journals Methylprednisolone as an alternative therapy for Kawasaki disease: case series

2020 ◽  
Vol 60 (5) ◽  
pp. 283-6
Author(s):  
Yudha Fadhol Arafah ◽  
Sasmito Nugroho ◽  
Noormanto Noormanto ◽  
Nadya Arafuri ◽  
Indah Kartika Murni
Keyword(s):  
Kawasaki Disease ◽  
Systemic Vasculitis ◽  
Alternative Therapy ◽  
Case Series ◽  
Cervical Lymphadenopathy ◽  
Conjunctival Hyperemia ◽  
Disease Case ◽  
The Usa ◽  
Acquired Heart Disease ◽  
The Uk

Kawasaki disease (KD), or mucocutaneous syndrome, is an acute, systemic vasculitis of small- and medium-sized arteries that predominantly affects patients younger than five years.1  KD is the leading cause of childhood acquired heart disease in the developed world.2 The incidence in those aged under 5 years varies widely throughout the world, accounting for 8.4 per 100,000 in the UK, 17.5 to 20.8 per 100,000 in the USA, and 239.6 per 100,000 in Japan.2 The diagnosis of classic KD is based on the simultaneous presence of high fever for 5 or more days with at least four of five other symptoms (bilateral conjunctival hyperemia, ulcerations of the lips and inflammation of the oral cavity, polymorphous rash, edema and desquamation of the extremities, and cervical lymphadenopathy), or fever associated with less than 4 of the diagnostic criteria and echocardiographic abnormalities of the coronary arteries.3

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