Clinical Manifestations Vary with Different Age Spectrums in Infants with Kawasaki Disease

Background. Kawasaki disease (KD) is an acute systemic vasculitis with unknown etiology. The diagnosis of KD depends on clinical manifestations. The prevalence of coronary artery abnormality (CAA) is 11.0% and results in cardiac sequelae, such as myocardial infarction or coronary aneurysm, which are the most serious complications in KD.Methods. We divided KD's children into different age groups: ≤6 months old, 7 months to 1 year old, and >1 year old, respectively. Different parameters were compared in each group.Results. Infants ≤6 months old are less likely to fulfill KD's major diagnostic criteria within 10 days, are prone to develop incomplete KD with the lowest cholesterol level, and have the greatest chance to have CAA and the laboratory features associated with CAA, such as the longest time needed to confirm CA diagnosis, lower hemoglobin level, lower albumin level, and higher platelet count. Infants <1 year old develop higher percentage of leukocytosis and sterile pyuria. But this group has fewer patients with neck lymphadenopathy.

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Abstract 210: Kawasaki Disease in Patients Older Than 10-years Old in a Children’s Hospital In Mexico City

Circulation ◽

10.1161/circ.131.suppl_2.210 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

ITZEL E RIOS-OLIVARES ◽

LUIS M GARRIDO-GARCIA

Keyword(s):

Kawasaki Disease ◽

Mexico City ◽

Systemic Vasculitis ◽

Clinical Manifestations ◽

Cervical Lymphadenopathy ◽

Skin Lesions ◽

Clinical Feature ◽

Unknown Etiology ◽

Cardiac Complications ◽

Increased Risk

Background: Kawasaki Disease (KD) is an acute febrile illness characterized by systemic vasculitis of unknown etiology. Cardiac sequelae, such as coronary artery aneurysms (CAA), are one of the most important aspects of this disease. Actually KD is most frequently presented in children younger than 5-years old. Objective: To describe the clinical and laboratory features, cardiac sequelae and outcome in children older than 10-years old with KD who were attended at the Instituto Nacional de Pediatría in Mexico City. Methods: An observational, descriptive, retrospective and transversal case study. We reviewed the medical records of patients older than 10-years diagnosed with KD from August 1995 to May 2014, and analyzed gender, age, clinical manifestations, hemoglobin, leucocyte count, platelet count, ESR, CRP, albumin, sodium, potassium, AST, ASL, time from the onset of the symptoms to diagnosis, treatment used, the development of CAA and outcome in the acute phase of the disease. Results: We studied 18 cases of KD in patients older than 10-years old, 72.2% (13 of 18) were male with a mean age of 154 months (range 120 to 200). The time from the onset of the fever to diagnosis was 10.6 ± 5.8 days, (range 3 to 21 days). Skin lesions were the most common manifestation of KD and cervical lymphadenopathy was the least common clinical feature. 2 patients presented with KD shock syndrome. Complete KD was diagnosed in only 50% (9 of 18) of our cases. 16 patients received IVGG, 2 patients required a second GGIV dose and 10 patients also received steroids. 6 of 18 patients (30%) developed CAA. There were no deaths in our group. Conclusions: KD in patients older than 10-years old represent a clinical challenge because in the majority of the cases they presented with an atypical clinical picture which contribute to a delayed diagnosis. Also there is an increased risk of developing cardiac complications and CAA in this group of patients.

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Predictive value of brachial artery flow-mediated dilation on coronary artery abnormality in acute stage of Kawasaki disease

Scientific Reports ◽

10.1038/s41598-021-87704-y ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Yizhou Wen ◽

Xianmin Wang ◽

Yonghong Guo ◽

Mei Jin ◽

Jimei Xi ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Brachial Artery ◽

Characteristic Curve ◽

Coronary Aneurysm ◽

Acute Stage ◽

Flow Mediated Dilation ◽

Strong Negative Correlation ◽

Coronary Artery Abnormality ◽

Artery Flow

AbstractCoronary artery abnormalities (CAAs) are a severe complication of Kawasaki disease (KD) that may lead to cardiovascular events. Given the evidence that brachial artery flow-mediated dilation (FMD) decreases in children after the onset of KD, we hypothesized that it could be an early marker of CAA development in the acute stage and investigated its relationship with variation in the coronary artery diameter. A total of 326 sex- and age-matched children were enrolled, including 120 with KD, 109 febrile children and 97 healthy controls. In this study, FMD was significantly decreased in the KD group compared with the febrile and healthy groups. FMD was lower in the CAA group than in the no coronary artery abnormality group. The comparison of FMD showed an obvious difference among the CAA subgroups. The FMD in the coronary aneurysm (CA) group showed a strong negative correlation with the pretreatment maximum coronary artery Z-score (preZmax). While preZmax was 2.5, the receiver operating characteristic curve indicated an optimal cutoff point of 3.44% for FMD. FMD ≤ 3.44% could be considered as a signal of coronary lesions in acute stage of KD.

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The Mysteries That Surround Kawasaki Disease: A Literature Review

Panorama brasileiro de tungstênio (w) entre os anos de 2008 e 2014 ◽

10.32749/nucleodoconhecimento.com.br/health/kawasaki-disease ◽

2021 ◽

pp. 52-64

Author(s):

Karoline Rossi ◽

Danilo José Silva Moreira ◽

Juliana Brito da Fonseca ◽

Suzana dos Santos Vasconcelos ◽

Vinicius Faustino Lima de Oliveira ◽

...

Keyword(s):

Lymph Node ◽

Kawasaki Disease ◽

Literature Review ◽

Intravenous Immunoglobulin ◽

Clinical Picture ◽

Systemic Vasculitis ◽

Age Groups ◽

Mucocutaneous Lymph Node Syndrome ◽

Three Stages ◽

Lymph Node Syndrome

Kawasaki disease (KD) or Mucocutaneous Lymph node Syndrome is a systemic vasculitis, which mainly affects children under five years of age with Asian descent, but can also reach other age groups, as well as any other breed. The clinical picture of KD has three stages: acute febrile stage, in which conjunctival congestion, oral mucositis, erythema, flaking, polymorphic rash and laterocervical lymphadenopathy, appear as main symptoms; the subacute stage, which occurs at the end of fever, and leads to the appearance of skin flaking in the limbs, arthritis, arthralgia and thrombocytosis and finally the stage of convalescence that arises when symptoms are almost dissipating and continues until their normalization. The most used treatment occurs from the administration of intravenous immunoglobulin, which for better prognosis of the pathology should be initiated early.

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Clinical manifestations of Kawasaki disease in different age groups: retrospective data from Southwest China

Clinical Rheumatology ◽

10.1007/s10067-020-05069-5 ◽

2020 ◽

Vol 39 (10) ◽

pp. 3027-3032

Author(s):

Lianjie Shi ◽

Jianhong Li ◽

Di Qie ◽

Xintian Hua ◽

Jinyong Pan ◽

...

Keyword(s):

Kawasaki Disease ◽

Southwest China ◽

Age Groups ◽

Clinical Manifestations ◽

Retrospective Data

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

The Scientific World JOURNAL ◽

10.1100/2012/520865 ◽

2012 ◽

Vol 2012 ◽

pp. 1-5 ◽

Cited By ~ 20

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.

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

The Scientific World JOURNAL ◽

10.1100/2012/634835 ◽

2012 ◽

Vol 2012 ◽

pp. 1-5 ◽

Cited By ~ 3

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.

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Incomplete Kawasaki disease in Egypt

Global Cardiology Science and Practice ◽

10.21542/gcsp.2017.24 ◽

2018 ◽

Vol 2017 (3) ◽

Author(s):

Hala M Agha ◽

Hala S Hamza

Keyword(s):

Kawasaki Disease ◽

Viral Infections ◽

Systemic Vasculitis ◽

Vascular Inflammation ◽

Clinical Findings ◽

Unknown Etiology ◽

Cervical Lymphadenitis ◽

Barr Virus ◽

Childhood Illnesses ◽

Epstein Barr

[first paragraph of article]Kawasaki disease (KD) is a hybrid condition at the junction of infectious diseases, immunology, rheumatology, and cardiology.1 KD is a systemic vasculitis of unknown etiology predominately affecting medium-sized vessels such as the coronary arteries, which mainly affects infants and children2. The disease itself may be the characteristic manifestation of a common pathway of immune-mediated vascular inflammation in genetically susceptible hosts3. Untreated KD may lead to the formation of coronary artery aneurysms and sudden cardiac death in children. The diagnosis of KD is based on the clinical features of fever of at least 5 days together with at least 4 or 5 other features including rash, bilateral conjunctival injection, changes in peripheral extremities, lymphadenopathy and oropharyngeal changes4. The diseases that must be differentiated from KD because of similar clinical findings include viral infections (measles, adenovirus, enterovirus, and Epstein-Barr virus), scarlet fever, staphylococcal scaled skin syndrome, toxic shock syndrome, polyarteritis nodosa, bacterial cervical lymphadenitis, and juvenile rheumatoid arthritis5,6. Because each of the symptoms commonly occurs in other childhood illnesses, the disease can be difficult to diagnose, especially in children who present with an incomplete form of the disease. KD has not been previously reported from Egypt and there are special challenges in recognizing complete KD in a country where physicians have limited experience with the disease. The diagnosis of incomplete KD is thus even more challenging in this setting.

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Evaluation of Cardiac Coronary Artery in Children with Kawasaki Disease by Echocardiography

Advanced Emergency Medicine ◽

10.18686/aem.v6.83 ◽

2018 ◽

Vol 6 (2) ◽

Author(s):

Lin Zhu

Keyword(s):

Coronary Heart Disease ◽

Heart Disease ◽

Coronary Artery ◽

Kawasaki Disease ◽

Medical Technology ◽

Positive Impact ◽

Clinical Manifestations ◽

Clinical Treatment ◽

Unknown Etiology ◽

Effective Development

<p>Kawasaki disease is an acute, unknown etiology of vascular inflammatory response syndrome, heart coronary artery changes as one of the main clinical manifestations of Kawasaki disease. How to determine the effective changes of coronary heart disease in children has become a lot of research scholars concern. With the continuous development of medical technology in recent years, echocardiography is widely used in the clinical diagnosis of various diseases and for the effective development of clinical treatment had a positive impact. A large number of research scholars found that by echocardiography, it can effectively achieve the diagnosis of coronary heart disease in children with Kawasaki disease, in order to lay a good foundation for the development of clinical treatment.</p>

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Abstract 12638: Autophagy-mitophagy Induction Improves Cardiovascular Inflammation in a Murine Model of Kawasaki Disease Vasculitis

Circulation ◽

10.1161/circ.142.suppl_3.12638 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Stefanie Marek-iannucci ◽

Angela Gomez ◽

Debbie Moreira ◽

Malcolm Lane ◽

Rebecca Porritt ◽

...

Keyword(s):

Kawasaki Disease ◽

Excision Repair ◽

Systemic Vasculitis ◽

Unknown Etiology ◽

Recent Experimental Data ◽

Intermittent Fasting ◽

Autophagic Flux ◽

Clinical Genetic ◽

Mtdna Damage ◽

Mitochondrial Fractions

Kawasaki Disease (KD), an acute febrile illness and systemic vasculitis of unknown etiology, is the leading cause of acquired heart disease among children. Recent experimental data from mouse models, as well as clinical, genetic and transcriptome evidence from KD patients suggest a key role of the NLRP3-IL-1β pathway in the pathogenesis of KD. NLRP3 can be activated by mitochondrial (mt) DNA released in the setting of defective autophagy/mitophagy, but a potential role for autophagy/mitophagy in KD cardiovascular inflammation has not been determined. In the Lactobacillus casei cell wall extract (LCWE) mouse model of KD vasculitis, LCWE injection in WT mice results in coronary arteritis, aortitis and myocarditis, mimicking human KD. We found that expression of the autophagy/mitophagy markers Parkin and p62 was significantly higher in whole lysate and mitochondrial fractions of heart tissue of LCWE-injected mice than controls, indicating impaired autophagic flux and mitophagy. Parkin -/- mice developed significantly more LCWE-induced cardiovascular lesions than control mice, and treatment of WT mice with a GLP-1 receptor agonist, known to activate Parkin, significantly reduced LCWE-induced inflammation. LCWE-induced cardiovascular lesions were amplified in mice deficient in OGG1, a base excision repair protein sensitive to mtDNA damage. Finally, inhibiting autophagy with chloroquine exacerbated LCWE-induced KD vasculitis, whereas inducing autophagy by intermittent fasting decreased cardiovascular inflammation in the model. Altogether, our data suggest that impaired autophagy/mitophagy exacerbates KD and supports a role for mtDNA damage in the disease. These findings enhance our understanding of KD pathogenesis and may provide novel therapeutic targets.

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

Case Reports in Pediatrics ◽

10.1155/2013/931703 ◽

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.

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