kawasaki disease

One of the phenomena observed during the COVID-19 pandemic is the emergence of Multisystem Inflammatory Syndrome in children and adolescents (MIS-C). Patients with this condition exhibit some overlapping symptoms with those of Kawasaki Disease (KD). Both Kawasaki Disease and Multisystem Inflammatory Syndrome are pathological conditions associated with vascular inflammation and cardiac complications. This study aims to assess and compare laboratory findings and cardiac complications in children diagnosed with both conditions.

In this study, after the clinical diagnosis of MIS-C and Kawasaki Disease, demographic, clinical, laboratory, and echocardiographic data of the patients were entered into a checklist for evaluation. Ultimately, all information was entered into SPSS version 26 and analyzed using T-test and Chi-square tests. A significance level of 0.05 was considered for all tests.

A total of 115 patients were examined in this study. Among these, 92 patients were in the MIS-C group and 24 patients were in the Kawasaki group. No statistically significant differences were found between the two groups concerning age, gender, thrombocytopenia, and fibrinogen levels (P>0.005). However, statistically significant differences were observed between the two groups regarding white blood cell counts, lymphopenia, platelets, hemoglobin, ESR, cardiac output, pericardial effusion, vascular ectasia, and mild mitral regurgitation (MR) (P<0.005). No statistically significant differences were noted regarding moderate to severe vascular aneurysms, as well as moderate tricuspid regurgitation (TR) between the two groups (P>0.005).

Early diagnosis and proper management of cardiac complications in children with KD and MIS-C are essential to prevent long-term complications. Further studies are needed to deepen our understanding of the disease mechanisms and cardiac complications in these two clinical conditions.

Kawasaki disease (KD) is an acute, self-limiting vasculitis that predominantly affects children under five years of age. It is recognized as the leading cause of acquired coronary artery disease in this age group.

This manuscript presents two cases of KD complicated by significant coronary artery abnormalities, which ultimately necessitated heart transplantation.

Emphasizing the importance of early diagnosis and treatment, this study underscores the critical need for continued research to improve outcomes for affected patients.

One of the observed phenomena during the COVID-19 pandemic is the occurrence of Multisystem Inflammatory Syndrome in Children and Adolescents (MIS-C). Patients with this disease have some overlapping symptoms and signs with Kawasaki Disease (KD). This disease is associated with cardiac complications. This study was conducted with the aim of comparing the results of echocardiography and cardiac complications in patients with Kawasaki and Kawasaki-like inflammatory syndrome affected by COVID-19 referred to Mofid Hospital from 2020 to 2022.

This cross-sectional analytical study was conducted on the research population of children hospitalized at Mofid Hospital with a diagnosis of COVID-19 during the years 2020-2021. The clinical findings, echocardiographic results of patients, and cardiac complications were compared between the two groups of patients with Kawasaki and MIS-C.

227 patients with the final diagnosis of MIS-C or KD were investigated in this study. Among the patients, 107 (50.5%) were girls and 105 (49.5%) were boys. Of the patients, 180 were MIS-C (79.3%) and 47 were KD (20.7%), in terms of age, weight and body mass index, a significant difference was observed between the two groups (P<0.05). However, there was no statistically significant difference in the length of hospitalization between the two groups (P>0.05). In the laboratory investigations in the MIS-C and KD groups, a significant difference was observed in the levels of ESR, Uric-Asid, D-Dimer, P, Mg, WBC, PLT, MCV, BUN, Albumin and Total protein (p<0.05 ). The clinical manifestations of gastrointestinal and pulmonary involvement were significantly higher in the MIS-C group compared to KD (p<0.05). No statistical difference was observed in neurological, cardiac and skin involvement between the two groups (p>0.05). Also, no statistically significant difference was observed in the diagnostic indices between the two groups (P<0.05), in the results of the first echocardiography, pleural effusion and vascular involvement, in the second echocardiography, mild MR and pleural effusion were significantly different between the two groups, and other echocardiography results It was not significant in all three occasions (P>0.05).

MIS-C likely represents a new systemic inflammatory syndrome associated with SARS-CoV-2 infection in children, and MIS-C patients have cardiac complications. Further international studies are necessary to confirm these findings and better understand the pathophysiology of MIS-C.

One of the most prevalent childhood vasculitis is kawasaki disease (KD), which mainly affects medium-sized arteries throughout the body. This disease usually does not cause many neurological symptoms and only rarely results in facial nerve palsy (FNP).

A 4.6-month-old iranian girl presented due to a prolonged fever. Lab tests showed increased CRP, ESR, ferritin, WBC, and platelet counts, and echocardiography revealed dilated right and left main coronary arteries, without any vegetations, pericardial effusion, and atrioventricular valve regurgitation. The patient was diagnosed with incomplete KD. At the same time, she developed left-sided peripheral FNP. Patient treated with intravenous immunoglobulin and aspirin. The fever resolved after about 36 hours, and the patient was discharged on the 16th day of hospital admission. Follow-up echocardiography demonstrated gradually resolution of her coronary arteries dilation. FNP was recovered completely after about 2 months.

FNP is an uncommon neurological symptom of KD. However, it typically resolves on its own and without any lasting effects in those who survive the illness. Nevertheless, it may indicate a higher likelihood of coronary artery involvement, and as a result, additional anti-inflammatory treatments and more closely monitored echocardiography may be necessary.

Multisystem Inflammatory syndrome in Children (MIS-C) is a common diagnosis among children in the post Covid-19 era, which usually presents with fever and varied systemic manifestations. BCG vaccination site reaction as a manifestation of MIS-C is barely ever documented. In this series, we present a rare such case report along with the other six other cases.

Seven cases with MIS-C are presented with four male and three female children. The median age of presentation was 3 (range: 0.4-14) years. Fever was the most common (100%) symptom followed by muco-cutaneous manifestation (50%, 4/7), as well as gastrointestinal (37.5%, 3/7), and cardiovascular symptoms (37.5%, 3/7). The clinical features which led to the diagnosis include BCG site vaccination, erythema multiforme, periungual and perineal peeling, unilateral knee joint effusion, skin scalding syndrome, urticaria, and acute glomerulonephritis. The initial work-up for fever did not yield any cause, except for a non-specific elevation of inflammatory markers. 6 cases had elevated anti-SARS-Co-V2 antibody titres with a negative RTPCR/RAT test and 1 case yielded positive RTPCR for covid status for acute infection. 2D Echocardiography showed Coronary artery dilatation in two patients which resolved on follow-up. All the patients responded well to intravenous immunoglobulin and methylprednisolone combination therapy with antiplatelet drugs.

BCG vaccination site reaction could occur due to antigen-homology. Cardiovascular manifestations (coronary artery dilatation) without hemodynamic instability may also be a manifestation of MIS-C, thereby placing 2D-Echo as an important step in diagnostic workup.

We present the case of a 6.5-year-old boy who presented to our center with complaints of pain in both hip joints that had started 10 days prior to admission. Following the diagnosis and treatment of septic arthritis, he developed a fever that did not respond to antibiotics. Subsequently, he was diagnosed with Kawasaki disease triggered by septic arthritis.

 Kawasaki disease is an idiopathic febrile systemic vasculitis, recognized as the leading cause of acquired heart disease in pediatric patients. Gastrointestinal manifestations can lead to delayed treatment, underscoring the importance of identifying and predicting factors associated with these manifestations to enable timely intervention.

 The primary objective of this study was to identify the aforementioned factors.

 This retrospective study collected demographic information, medical history, physical examination findings, and laboratory results of 359 children suspected of having Kawasaki disease and referred to Mofid Children’s Hospital between 2013 and 2022. The study assessed the frequency of gastrointestinal manifestations and their association with physical examination findings and laboratory data.

 A total of 359 patients were included in the study, comprising 153 males and 206 females. The mean age of participants was 2.8 ± 2.6 years. Abdominal pain was the most common clinical manifestation, reported in 39% of patients. Elevated levels of aspartate aminotransferase (AST) were observed in 38.1% of patients, while 30.2% showed elevated alanine aminotransferase (ALT) levels, and 10.5% exhibited direct hyperbilirubinemia. Patients with elevated ALT and AST levels had a higher incidence of abdominal pain, nausea/vomiting, and anorexia. Moreover, patients with positive findings of red blood cell (RBC) and white blood cell (WBC) in their stool examination had a higher incidence of fever and abdominal pain.

 Based on our statistical analysis, elevated AST and ALT levels, hyperbilirubinemia, and positive stool findings of RBC and WBC appear to be predictive factors for gastrointestinal manifestations in Kawasaki disease.

Kawasaki disease (KD) is a systematic vasculitis that mainly affects children under five years old. Diagnosis is made based on clinical criteria.

This study aimed to determine various atypical presentations of KD to prevent misdiagnosis.

A comprehensive systematic search in PubMed, Web of Science, and Scopus databases was performed based on the PRISMA guidelines from 2012 to January 2023. Case reports and case series studies reporting the atypical presentations of KD, only in the English language, were included, and the Joanna Briggs Institute (JBI) checklist was utilized for case report studies as a quality assessment tool.

Our findings revealed that patients with KD can present with gastrointestinal and hepatobiliary, cardiovascular, mucocutaneous, neurological, musculoskeletal, respiratory, retropharyngeal, and renal complications. Macrophage activation syndrome and normal inflammatory biomarkers were rare manifestations. The most common cause of the patient’s death was cardiovascular manifestations, especially myocardial infarction. Cardiovascular and gastrointestinal involvement were the most prevalent.

Focusing on atypical and rare manifestations facilitates the correct diagnosis, leading to timely and appropriate management, improving outcomes, and reducing complications.

 The most frequent cause of coronary artery aneurysm in children is Kawasaki disease (KD). Recently, limited studies on procalcitonin (PCT) were performed to find a biomarker for the diagnosis or prognosis of children with KD.

 This study aimed to compare serum PCT levels between complete and incomplete KD and testify to the predictive validity of PCT for intravenous immunoglobulin (IVIG) resistance and predicting coronary artery lesions (CALs).

 This cross-sectional study was conducted at Namazi hospital in Shiraz, Iran, in 2019. All KD patients admitted to the hospital were included, with parental consent obtained. Kawasaki disease patients were categorized as complete KD (cKD) or incomplete KD (iKD). Two-dimensional echocardiography was performed, and peripheral venous blood was examined for PCT levels and other markers. All patients received IVIG and aspirin as standard treatment. The presence of coronary artery abnormalities (CAAs) was determined based on coronary artery size and morphology. We used Stata software version 14.0 for data analysis. Logistic regression models identified predictive factors for coronary complications. This study determined the optimal PCT cut-off point for predicted coronary complications using receiver operating characteristic (ROC) curve analysis. Approval was obtained from the Research Ethics Committees of Shiraz University of Medical Sciences.

 Procalcitonin values in 38 children hospitalized with acute KD ranged from 0.2 ng/mL to 10 ng/mL, with a mean of 2.65 ng/m. There was no correlation of PCT with patients’ age or gender, incomplete KD, or IVIG resistance. The serum PCT concentration was higher in patients with CAL (P = 0.009). The best PCT cut-off value for CAL prediction was 2.5 ng/mL (corresponding sensitivity = 81.8% and specificity = 68.7%), considering CAL prevalence as the studied group.

 Supplementary research is needed to determine the sensitivity and specificity of PCT for the diagnosis and prognosis of KD. Procalcitonin might be of value in predicting which children are at increased risk for CALs to intensify therapy.

Kawasaki disease is an acute self-limiting systemic vasculitis in childhood, resulting in arterial swelling or inflammation and eventually leading to cardiovascular problems, such as coronary artery aneurysms. Based on previous studies, serum sodium ≤133 mmol/L, albumin ≤3.2 g/dL, alanine transaminase ≥80 U/L, and neutrophil percentage ≥80% at diagnosis are risk factors for intravenous immunoglobulin (IVIg). However, the prevalence of resistance to Ig among children with Kawasaki disease varies among different countries due to diversity in evaluation, treatment, and diagnosis. Approximately, 10% to 20% of patients have IVIg-resistant Kawasaki disease. As the probability of coronary artery damage associated with IVIg-resistant Kawasaki disease is higher than that with IVIg-sensitive Kawasaki disease, the early detection and appropriate treatment of IVIg-resistant Kawasaki disease can decrease the probability of damage to coronary arteries and hospital lengths of stay and cost. Kawasaki disease in early infancy is uncommon, and sometimes it occurs with thrombosis and peripheral gangrene. A positive genetic background may play a role in susceptibility to thrombosis. We herein describe a patient suffering from an IVIg-resistant Kawasaki disease with severe coronary artery thrombosis and positive genetic mutation. Medical treatment resolved the thrombosis, but the coronary arteries remained dilated.

The symptoms and laboratory findings of Kawasaki disease (KD) and systemic-onset juvenile idiopathic arthritis (SoJIA) may overlap in the early phases. Coronary artery lesions are common complications seen in KD.

Cases Presentation: 

In this article, we report three cases of SoJIA (two males and one female) with prolonged relapsing fever and coronary artery involvement. Initially, all three cases were presumed to have KD and were treated with IVIG. All three cases had arthritis and lymphadenopathy, and one of them had a skin rash. After 3-8 weeks, fever and main clinical symptoms returned. In the second evaluation, they met the criteria for SoJIA and were treated with methylprednisolone, ibuprofen, and methotrexate. High ferritin levels were observed in all three cases (mean=6024 ng/ml).

Coronary artery involvement may rarely be seen in the early phases of SoJIA. Continuing or relapsing fever, late-onset arthritis, and increased serum ferritin levels may help distinguish SoJIA from KD.

During the Coronavirus disease 2019 (COVID-19) pandemic, children are less affected than adults, and underlying conditions and older age are associated with severe disease. Following the SARS-CoV-2 pandemic, outbreaks of cases with Kawasaki-like disease and a few others with the multisystem inflammatory syndrome in children related to COVID-19 (MIS-C) have been reported. Here, we report a two-year-old girl initially admitted to urgent pediatric care and was diagnosed with Kawasaki disease associated with COVID-19 infection to highlight the clinical features and signs. Physical examination showed a fussy infant with swelling of the hands, feet, and lips, red lips, restlessness, anorexia, and fever. According to the clinical and laboratory findings, Kawasaki disease was diagnosed. Laboratory tests for SARS-CoV-2 were positive. The patient's clinical symptoms improved after receiving medications such as IVIG and ascorbic acetyl acid.

Kawasaki disease is an acute febrile disease with the vasculitis of small and medium vessels that mainly affects children under the age of five, usually occurring after a viral respiratory diseases. During the coronavirus disease-19 (COVID-19) pandemic, a significant increase was reported in the number of children with symptoms similar to Kawasaki disease, which is known as a multi-system inflammatory syndrome in children (MIS-C). A Kawasaki-like disease is rare in adults, especially in the context of COVID-19. We hereby present the case of A 41 year old female patient presented with complaints of bleeding from the gums and red and watery eyes, high fever, sore throat, weakness, and lethargy. She mentioned a recent contact with a COVID-19 patient. Covid-19 was confirmed by a positive reverse transcription polymerase chain reaction (RT-PCR) test. Other lab tests and clinical manifestations revealed inflammatory phenomena that fully complied with multi-system inflammatory syndrome in adults (MIS-A) criteria. The patient was treated with a possible diagnosis of MIS-A in the context of COVID-19 and was discharged in good general condition. COVID-19 rarely presents in adults without clear respiratory symptoms and in the form of multisystem inflammatory syndrome (MIS-A). Due to the possibility of irreversible complications,MIS-A requires special attention and early diagnosis and treatment.

 Studies investigating the association between ITPKC rs28493229 polymorphisms and Kawasaki disease (KD) risk found inconsistent data. Thus, we performed this meta-analysis to combine and analyze the available studies to get a precise estimation of the association.

 Relevant studies identified in the PubMed, Web of Science, Scopus, and CNKI databases were used to perform a meta-analysis. Pooled odds ratios (OR) with a 95% confidence interval (95% CI) were calculated under fixed- and random-effects models to appraise the association.

 A total of eight case-control studies with 2,721 KD cases and 5,307 controls were selected. The results showed a statistically significant association between ITPKC rs28493229 polymorphism and an increased risk of KD under all five genetic models, i.e., allele (C vs. G: OR = 1.434, 95% CI 1.209-1.700, P ≤ 0.001), homozygote (CC vs. GG: OR = 2.085, 95% CI 1.423-3.055, P ≤ 0.001), heterozygote (CG vs. GG: OR = 1.530, 95% CI 1.359-1.722, P ≤ 0.001), dominant (CC+CG vs. GG: OR = 1.490, 95% CI 1.229-1.806, P ≤ 0.001), and recessive (CC vs. CG + GG: OR = 1.799, 95% CI 1.231-2.629, P = 0.002) in the overall population. When stratified by country, there was a significant association among Taiwanese.

 Our meta-analysis results supported that the ITPKC rs28493229 polymorphism is strongly associated with susceptibility to KD.

One of the leading causes of acquired heart disease in the world today is Kawasaki disease [KD], an acute systemic vasculitis in children. The purpose of this study was to identify the clinical and laboratory manifestations of children with KD admitted to the 17th Shahrivar Educational Hospital in Rasht, Iran.

We retrospectively studied the cases of 75 children with KD who were admitted to the 17th Shahrivar Educational Hospital in Rasht between 2011 and 2018. The frequency distribution of age, sex, seasonal prevalence, clinical manifestations including fever, skin rash, changes in the lips and oral cavity, conjunctivitis, changes in the extremities, and cervical lymphadenopathy; laboratory findings including platelets and leukocyte count, erythrocyte sedimentation rate [ESR], and C-reactive protein [CRP]; and response to treatment were studied. The obtained data were analyzed using descriptive statistical methods.

Out of 75 patients, the most common clinical manifestations were fever [100%], skin rash [78.67%], alterations to the lips and mouth [74.67%], bilateral non-purulent conjunctivitis [64%], changes in the extremities [46.67%], and cervical lymphadenopathy [38.67%], respectively. Among 75 patients, 70 [93.33%] had ESR above 40 mm/h, and 63 patients [84%] had high C-reactive protein [CRP]. Sixty percent of children had incomplete Kawasaki disease. Eight of our patients [10.6%] developed coronary heart disease.

In this study, the most frequent principal clinical manifestations were fever, skin rash, and alterations in the oral cavity and lips. Cervical lymphadenopathy had the lowest prevalence. Increased ESR and CRP were also the most common laboratory findings.

Kawasaki disease is a form of vasculitis, mainly in small and medium-sized arteries of unknown origin, and often occurs in childhood. It is the leading form of heart disease acquired in childhood in developed countries. Pyelonephritis is not usually associated with Kawasaki, but hereby we represent a case of Kawasaki disease associated with pyelonephritis; however, we can’t say whether pyelonephritis had a role in stating Kawasaki and it could be a proper and valuable topic for research.

Kawasaki disease is a systemic vasculitis of unknown etiology that is common in the pediatric patients. The primary therapeutic strategy includes aspirin and Intravenous Immunoglobulin. The present study aimed to investigate the Neutrophil to Lymphocyte Ratio (NLR) in the patients with Kawasaki and its relationship with the resistance to IVIg therapy.

The present study included the patients presenting to the Motahari Hospital, Urmia, Iran, whom diagnosed with Kawasaki disease and received IVIg during 2008-2019. The authors collected the data from the patients' medical records and re-assessed the patients' data for meeting the diagnostic criteria for Kawasaki. Afterward, the data of eligible patients entered the analysis.

The data from 460 patients were obtained and assessed for meeting the diagnostic criteria for Kawasaki. Of 460 patients, 241 met the eligibility criteria, and the data for other patients that meet exclusion criteria were excluded from the study. According to the results of our study, response to IVIg therapy had a significant relationship with the variables of blood and urinary leukocyte counts (p=0.013 and p=0.01, respectively). However, we didn’t find any significant relationship between the response to IVIg therapy and the variables of age, gender, NLR, neutrophil count, lymphocyte count, C-Reactive Protein (CRP), serum albumin level, ALT, hemoglobin, platelet count, and the interval between onset of symptoms and treatment initiation.

High blood leukocyte count along with low urinary leukocyte count can predict the response to IVIg treatment and subsequent prognosis in the patients affected by the Kawasaki disease. However, the NLR did not show clinical relevance.

We present a case of funnel-shaped aneurysmal dilatation of left main coronary artery (LMCA) with aggressive stenosis of osteoproximal left circumflex coronary artery presenting with lateral wall coronary ischemia in a 6‑year‑old school‑going child who recovered from Kawasaki disease (KD) 1 month back. Although aneurysmal dilatation of LMCA during first 2 months of recovery from KD is well known, with median duration for the development of stenotic lesion in coronaries after KD is approximately 6 months and all the cases of coronary stenosis described so far have been described in the presence of large coronary artery aneurysm, our case is rare to illustrate aggressive stenosis of osteoproximal left circumflex coronary artery just after 1 month of recovery in spite of the presence of small coronary artery aneurysm.