Diagnosis Of Kawasaki Disease Research Articles

Accuracy of Machine Learning in Discriminating Kawasaki Disease and Other Febrile Illnesses: Systematic Review and Meta-Analysis

Background Kawasaki disease (KD) is an acute pediatric vasculitis that can lead to coronary artery aneurysms and severe cardiovascular complications, often presenting with obvious fever in the early stages. In current clinical practice, distinguishing KD from other febrile illnesses remains a significant challenge. In recent years, some researchers have explored the potential of machine learning (ML) methods for the differential diagnosis of KD versus other febrile illnesses, as well as for predicting coronary artery lesions (CALs) in people with KD. However, there is still a lack of systematic evidence to validate their effectiveness. Therefore, we have conducted the first systematic review and meta-analysis to evaluate the accuracy of ML in differentiating KD from other febrile illnesses and in predicting CALs in people with KD, so as to provide evidence-based support for the application of ML in the diagnosis and treatment of KD. Objective This study aimed to summarize the accuracy of ML in differentiating KD from other febrile illnesses and predicting CALs in people with KD. Methods PubMed, Cochrane Library, Embase, and Web of Science were systematically searched until September 26, 2023. The risk of bias in the included original studies was appraised using the Prediction Model Risk of Bias Assessment Tool (PROBAST). Stata (version 15.0; StataCorp) was used for the statistical analysis. Results A total of 29 studies were incorporated. Of them, 20 used ML to differentiate KD from other febrile illnesses. These studies involved a total of 103,882 participants, including 12,541 people with KD. In the validation set, the pooled concordance index, sensitivity, and specificity were 0.898 (95% CI 0.874-0.922), 0.91 (95% CI 0.83-0.95), and 0.86 (95% CI 0.80-0.90), respectively. Meanwhile, 9 studies used ML for early prediction of the risk of CALs in children with KD. These studies involved a total of 6503 people with KD, of whom 986 had CALs. The pooled concordance index in the validation set was 0.787 (95% CI 0.738-0.835). Conclusions The diagnostic and predictive factors used in the studies we included were primarily derived from common clinical data. The ML models constructed based on these clinical data demonstrated promising effectiveness in differentiating KD from other febrile illnesses and in predicting coronary artery lesions. Therefore, in future research, we can explore the use of ML methods to identify more efficient predictors and develop tools that can be applied on a broader scale for the differentiation of KD and the prediction of CALs.

Abstract 4138603: Adult-onset Kawasaki Disease: An Updated Review of the English-Language Literature from 1980 Through 2023

Introduction: Kawasaki disease (KD) is a pan-vasculitis that primarily affects children and rarely adults. Available data on adult-onset KD are based on case reports and case series, both of which are limited by small sizes. We aimed to characterize the epidemiology, presentation, hospital course, and outcomes of adult-onset KD published in the English literature from 1980 through 2023. Methods: We retrospectively reviewed and included published articles with a diagnosis of KD in patients age ≥18 years. We searched PubMed and Google scholar for case reports/series published in English using the keywords “adult”, “Kawasaki disease”, and “mucocutaneous lymph node syndrome”. We extracted data from individual articles onto an Excel spreadsheet for analysis. Outcomes of interest were the demographic characteristics of patients, clinical presentation and hospital course, management, and complications. Descriptive statistics were used to analyze the data. Results: A total of 137 patients from 29 countries were included in this study. The majority were male (90/137, 64.9%; M:F ratio 1.8) and the median age was 25 years (range, 18-68). The median time to clinical presentation was 5 days (range, 4-60). The main signs and symptoms were fever (95.7%), skin rash (92.9%), conjunctivitis (89.3%), extremity changes (84.3%), oral changes (85.7%), and cervical lymphadenopathy (73.7%). The majority of the patients had no co-morbidities (125/137, 91.2%). EKG changes were not reported in 48 patients (35%). In the remaining 89, 26 (29.2%) had normal EKG or sinus tachycardia, 9 (10.1%) had ST segment elevation, and 9 (10.1%) had arrythmias or abnormal EKGs. Pharmacotherapy included IVIG (80/137, 58.4%), Aspirin (105/137, 76.6%), steroids (31/137, 22.6%) and antibiotics (68/137, 49.6%). For complications, 18.2% (25/137) had aneurysms, 8% (11/137) had myocardial infarction, 10.9% (15/137) had myocarditis, 10% had pericarditis/pericardial effusion, and 10.9% (15/137) had KD shock syndrome. The median length of stay was 14 days (range, 1-60 days). Three patients (2%) died from KD. Conclusion: Adult-onset KD is rare and is associated with significant cardiovascular complications. Clinicians should have high index of suspicion for prompt diagnoses and initiate appropriate treatment to improve outcomes.

STEMI in an Adolescent: A Critical Case of Kawasaki Disease.

Acute coronary syndrome (ACS) is a rare cause of chest pain in adolescents and young adults. Kawasaki disease (KD) is an uncommon illness, but coronary artery aneurysms secondary to KD are implicated in approximately 5% of cases of ACS in young adults. Patients with KD may present with catastrophic coronary disease at a young age and thus early recognition of this disease process and lifelong follow-up is essential. Here we discuss a 19-year-old active duty male with no previously recognized medical history who presented with crushing chest pain while deployed. He was diagnosed with an ST elevation myocardial infarction and was found to have a giant aneurysm of the left main coronary artery with obstructive thrombus treated with emergent thrombectomy and balloon angioplasty. He developed cardiogenic shock requiring mechanical circulatory and ventilatory support. After a prolonged recovery and more in-depth history-taking, he was discovered to have been diagnosed with KD as a child without follow-up. Diagnosing ACS in young adults can be challenging, but a history of KD should increase suspicion for coronary disease. This case demonstrates the importance of screening after a childhood diagnosis of KD, specifically when caring for the tactical athlete. It also highlights the impact that social determinants of health can have on patient outcomes. Finally, it demonstrates the importance of communication and coordination between military and civilian medical centers across the globe driving the care of our deployed injured and ill active-duty military personnel.

High eosinophil counts aid in diagnosing Kawasaki disease in febrile children

Abstract Background Several recent studies have validated the newfound significance of elevated eosinophils in Kawasaki disease (KD). However, this finding was not incorporated into the suggested laboratory criteria outlined by the Kawasaki Disease Committee of the American Heart Association for assessing children with incomplete KD. Purpose We aimed to determine the potential benefits of including eosinophil levels in the laboratory evaluation of KD. Methods A retrospective cohort study involved 77,934 febrile children (FC, not KD) and 1,970 subjects diagnosed with KD. Participants were recruited from four hospitals, comprising two medical centers and two regional hospitals. The AHA recommends specific laboratory tests—white blood cell count, hemoglobin, platelet count, ALT levels, albumin, and urinalysis—for evaluating incomplete KD in children with a higher CRP than 30mg/dL. The first four items are standard blood assays. Additionally, we introduced high eosinophil counts as a new diagnostic criterion for identifying children with KD. Results All these five laboratory findings (anemia for age defined as below the 5th percentile), platelet count >450,000/mm3, white blood cell>15,000/mm3, ALT>40 U/L, and eosinophil count >190/mm3) are significantly different between KD children and FC. The proportion of having these features is higher in KD children than in FC (19.3% vs. 8.0% in anemia for age, 20.5% vs. 10.3% in thrombocytosis, 37.0% vs. 18.1% in leukocytosis, 45.7% vs. 5.8% in elevated ALT, and 62.6% vs. 29.3% in high eosinophils). Elevated eosinophil counts are most commonly associated with KD among these five parameters. Furthermore, when incorporating the parameter of elevated eosinophil counts, the proportion of KD children with at least two abnormalities rose from 35.4% to 60.7%. In a subgroup analysis of children with KD presenting at least two abnormalities, the proportion rose from 38.7% to 63.5% in the high CRP group (≧ 3.0 mg/L) and 21.6% to 49.0% in the low CRP group (< 3.0 mg/L), respectively. Conclusions Higher eosinophil counts are vital markers in KD children. This aids in the identification of additional potential KD cases, especially in those exhibiting low CRP levels.

Клиническое наблюдение ретроспективной диагностики болезни Кавасаки

This research was aimed to demonstrating to the pediatric practitioners of a wide range the clinical case of retrospective diagnosis of Kawasaki disease (KD) based on detection of coronary artery (CA) aneurysms and anamnestic data analysis. Mucocutaneous lymphonodular syndrome (KD) is a systemic vasculitis of childhood that was characterized in the observed clinical case by fever, inflammatory changes in the mucous membranes, skin, lymph nodes and CA damage. Features of this clinical case were as follows: 1. the diagnosis was established retrospectively 5 years later than the KD was suffered based on accidental detection of CA aneurysms, 2. the lack of adequate KD treatment had led to the formation of CA aneurysms, including a single one of a giant size, 3. despite the long-term absence of generally accepted thrombosis prophylaxis in a patient with a giant CA aneurysm, only 11 years later did a thrombus in the aneurysm formed for the first time, which had then regressed rapidly despite it is known from the bibliographical data and those of the Authors’ own that the risk for thrombosis is very high in patients with giant aneurysms. Conclusion: late KD diagnosis entails the development of the cardiovascular system complications, in particular, coronary aneurysms, which in its turn in the reported clinical case were an accidental finding during the patient’s checkup.

Kawasaki Disease: A Single-Center Study from a Tertiary Care hospital in Dhaka, Bangladesh

Background: Kawasaki Disease (KD) is recognized as the leading cause of acquired heart disease in children in developed countries. This is a pioneer study from Bangabandhu Sheikh Mujib Medical University (BSMMU), documenting the common epidemiological, clinical, laboratory and echocardiographic findings, including management and immediate outcome of KD children. Method: This retrospective study includes all patients with a diagnosis of KD, who attended the pediatric rheumatology division at BSMMU from January 2015 to December 2019. Data were collected from medical records at diagnosis and immediately after management. Relevant statistical tests were done and a p value < 0.05 was considered as statistically significant. Result: A total of 129 cases were diagnosed with KD, among them complete KD cases were 62% and incomplete KD was 38%. The mean age at onset was significantly higher in incomplete KD than complete KD. The majority of the cases (67%) were between 1 to 5 years of age and male (72%) children were predominant. Fever was the most common presenting feature (100%), followed by bilateral conjunctivitis, mucosal involvement, rash, changes in extremities, cervical lymphadenopathy and others. Coronary artery dilatation was present in 30.5% cases with no significant differences between complete and incomplete KD cases. Aspirin was given to all the cases and IVIG was given to only 46.6% children. Rests of the children were treated with methyl prednisolone. All the cases improved after treatment and there was no immediate mortality. Conclusion: It seems that KD is not an uncommon disease and incomplete KD cases are relatively more prevalent. About 30% KD cases had coronary artery abnormalities. Immediate outcome of management is excellent but needs long term follow-up. BANGLADESH J CHILD HEALTH 2023; VOL 47 (1) : 9-16

An incomplete form of Kawasaki disease in a 2-month-old girl. How the diagnostic algorithm works in practice

BACKGROUND. Kawasaki disease is a rare, self-limiting inflammatory disease primarily affecting children under 5 years old. In 25 % of cases, it can lead to coronary artery aneurysms if not treated promptly with intravenous human immunoglobulin. A significant portion of Kawasaki disease patients present with an incomplete form, complicating diagnosis, while the risk of coronary artery aneurysms remains unchanged. Particularly vulnerable are infants under 6 months old, who may exhibit even more limited clinical symptoms, making them especially susceptible to severe cardiac complications. OBJECTIVE. To analyze a clinical case of an incomplete form of Kawasaki disease in a 2-month-old girl, raise awareness of Kawasaki disease, including its incomplete form, among healthcare workers in Ukraine, emphasize the critical importance of proper diagnostic evaluation of coronary arteries, and underscore the necessity of early administration of immunoglobulin when Kawasaki disease is suspected, even in the absence of pathological changes in the heart. MATERIALS AND METHODS. The study involved collecting medical history, physical examination data, laboratory and instrumental studies, and their analysis. The diagnosis of Kawasaki disease, including its incomplete form, was based on the 2017 recommendations of the American Heart Association. CLINICAL CASE. A 2-month-old girl presented with symptoms initially resembling a urinary tract infection, with no therapeutic response to multiple courses of antibacterial therapy. During the second week of illness, she developed swelling in her hands and feet, which, along with persistent fever, significant inflammatory response, and lack of improvement with antibiotics, led to the suspicion and diagnosis of Kawasaki disease based on the algorithm for incomplete Kawasaki disease. After administration of an immunomodulatory dose of intravenous human immunoglobulin at 2 g/kg on the 11th day of illness, there was a rapid regression of symptoms, normalization of inflammation markers, and overall improvement in the child's condition. By the second week of illness, small aneurysms of the right and left coronary arteries had formed but regressed within a few weeks from the onset of the disease. CONCLUSIONS. Kawasaki disease requires prompt therapeutic intervention and should be suspected in any child, especially those under one year of age, with a high fever lasting at least 5 days, unexplained by a preliminary diagnosis, and no response to antibiotic treatment in the presence of “bacterial” blood tests. Serial echocardiograms assessing coronary arteries, absolute dimensions, and Z-scores are mandatory.

Kawasaki Disease Diagnosis and Treatment in over 1000 Patients: A Continuum of Dysregulated Inflammatory Responses.

Kawasaki disease (KD) is a pediatric vasculitis, leading to coronary artery aneurysms (CAAs) in ~4-14%. Attention to the etiology and course of KD was generated by the close mimic of a SARS-CoV-2-induced phenotype, called multisystem inflammatory syndrome in children (MIS-C). A total of 1179 cases were collected from 2012 with ~50% of cases retrospectively included. Clinical characteristics were described and risk factors for CAA (persistence) were investigated. Phenotypic patterns of the prospectively included KD patients were evaluated. These patterns were also compared to the seronegative KD and seropositive MIS-C cases identified during the SARS-CoV-2 pandemic. KD mostly affected boys and children < 5 years. IVIG resistance, CAAs, and giant CAAs occurred in 24.5%, 21.4%, and 6.6%, respectively. Giant CAAs were significantly more likely to normalize to a normal Z score in patients that were younger than 2.5 years old at the time of initial giant CAA (χ2 test p = 0.02). In our prospective (SARS-CoV-2-seronegative) KD series, there was a diminishing male predominance over time, whereas the proportions of incomplete presentations (p < 0.001) and patients with circulatory shock (p = 0.04) increased since the COVID-19 pandemic. Pre- and post-pandemic KD cases presented with different levels of C-reactive protein, thrombocyte counts, and hemoglobin levels over the years. Compared to pandemic KD, SARS-CoV-2-seropositive MIS-C patients were older (p < 0.001), and more often required intensive care admission (p < 0.001), with a gradual decrease over time between 2020 and 2022 (p = 0.04). KD carried a substantial risk of CAA development in contrast to MIS-C. the phenotypic changes seen over the last twelve years of our prospective follow-up study suggest a spectrum of hyperinflammatory states with potentially different triggering events within this clinical entity.

Expansion of a Novel Subset of L-Selectin+ Classical Monocytes in Kawasaki Disease.

Kawasaki disease (KD) is an acute systemic vasculitis that is associated with dysregulated immune responses. Monocytes play a central role in innate immunity. Our previous single-cell RNA sequencing of peripheral blood mononuclear cells (PBMC) revealed a new subset of monocytes in children with KD called L-Selectin+ classical monocytes (SELL+ CM). Therefore, we aimed to investigate the correlation between KD and SELL+ CM. Peripheral blood samples were collected from 81 KD patients, 18 febrile patients and 36 healthy children before treatment. Among them, ten KD patients were followed up, and samples were obtained before and after intravenous immunoglobulin (IVIG) treatment. Analysis of SELL+ CM was performed using flow cytometry. Additionally, ROC curve analysis was conducted to assess the diagnostic value of SELL+ CM for KD. Classical monocytes (CM) expressed the highest levels of L-selectin in children with KD. The ratio of SELL+ CM in CM was significantly higher in KD patients than in febrile and healthy children. Following IVIG treatment, the ratio of SELL+ CM in CM showed a downward trend. The receiver operating characteristic (ROC) curve analysis (the area under the curve, AUC = 0.71) indicated the potential diagnostic value of SELL+ CM in KD. The correlation analysis suggested that SELL+ CM may serve as a new clinical index for patients with KD. In KD, the ratio of SELL+ CM in CM significantly increases during the acute phase, which may become a potential biomarker and help facilitate KD diagnosis based on clinical features.

Bilateral facial nerve palsy complicating Kawasaki disease: A case report and literature review.

Kawasaki disease (KD) manifests as an acute, self-limited vasculitis disease that constitutes the primary cause of acquired heart disease in children under 5 years of age. Facial nerve palsy (FNP) is a rare complication associated with coronary artery lesions (CALs) in patients with KD. Patients with KD and FNP usually present atypically, leading to a delayed diagnosis and treatment of KD. A 4-month-old boy with fever, left FNP and bilateral conjunctival injection with spontaneous resolution, was admitted to the hospital, received a short course of intravenous dexamethasone, and experienced rapid FNP recovery on the first admission. The patient experienced a resurgence of fever, bilateral conjunctival injection, and right FNP, which led to readmission. Physical examination revealed redness at the site of Bacillus Calmette-Guérin inoculation, reddening of lips, and desquamation of the distal extremities. Echocardiography revealed right-sided CALs. The patient initially missed KD on the first admission, and was later diagnosed with complete KD with FNP on the second admission. After a short course of intravenous dexamethasone, the left FNP resolved quickly. However, right FNP recurred after corticosteroids withdrawal. Meanwhile, more typical symptoms were observed, and KD was diagnosed. Treatment ensued with intravenous immunoglobulin (IVIG), aspirin, and dexamethasone. The patient achieved rapid remission, without recurrence. Echocardiography continued to show normal findings during 1-year follow-up after discharge. The clinical symptoms of FNP complicating KD in children are atypical and can easily lead to delayed diagnosis and treatment. FNP in patients with KD may serve as a risk factor for CALs, which are more challenging to resolve than the FNP itself. Adding corticosteroids to IVIG may be recommended to reduce IVIG resistance, decrease the risk of developing CALs, and alleviate CALs.

Intelligent diagnosis of Kawasaki disease from real-world data using interpretable machine learning models

ObjectiveThis study aimed to leverage real-world electronic medical record data to develop interpretable machine learning models for diagnosis of Kawasaki disease while also exploring and prioritizing the significant risk factors. MethodsA comprehensive study was conducted on 4087 pediatric patients at the Children’s Hospital of Chongqing, China. The study collected demographic data, physical examination results, and laboratory findings. Statistical analyses were performed using IBM SPSS Statistics, Version 26.0. The optimal feature subset was used to develop intelligent diagnostic prediction models based on the Light Gradient Boosting Machine, Explainable Boosting Machine (EBM), Gradient Boosting Classifier (GBC), Fast Interpretable Greedy-Tree Sums, Decision Tree, AdaBoost Classifier, and Logistic Regression. Model performance was evaluated in three dimensions: discriminative ability via receiver operating characteristic curves, calibration accuracy using calibration curves, and interpretability through SHAP (SHapley Additive exPlanations) and LIME (Local Interpretable Model-Agnostic Explanations). ResultsIn this study, Kawasaki disease was diagnosed in 2971 participants. Analysis was conducted on 31 indicators, including red blood cell distribution width and erythrocyte sedimentation rate. The EBM model demonstrated superior performance relative to other models, with an area under the curve of 0.97, second only to the GBC model. Furthermore, the EBM model exhibited the highest calibration accuracy and maintained its interpretability without relying on external analytical tools such as SHAP and LIME, thus reducing interpretation biases. Platelet distribution width, total protein, and erythrocyte sedimentation rate were identified by the model as significant predictors for the diagnosis of Kawasaki disease. ConclusionThis study used diverse machine learning models for early diagnosis of Kawasaki disease. The findings demonstrated that interpretable models such as EBM outperformed traditional machine learning models in terms of both interpretability and performance. Ensuring consistency between predictive models and clinical evidence is crucial for the successful integration of artificial intelligence into real-world clinical practice.

The Future of Kawasaki Disease Diagnosis: Liquid Biopsy May Hold the Key.

Kawasaki disease (KD) is a febrile illness characterised by systemic inflammation of small- and medium-sized blood vessels, which commonly occurs in young children. Although self-limiting, there is a risk of developing coronary artery lesions as the disease progresses, with delay in diagnosis and treatment. Unfortunately, the diagnosis of KD continues to remain a clinical dilemma. Thus, this article not only summarises the key research gaps associated with KD, but also evaluates the possibility of using circulating endothelial injury biomarkers, such as circulating endothelial cells, endothelial microparticles and vascular endothelial cell-free DNA, as diagnostic and prognostic tools for KD: a "liquid biopsy" approach. The challenges of translating liquid biopsies to use in KD and the opportunities for improvement in its diagnosis and management that such translation may provide are discussed. The use of endothelial damage markers, which are easily obtained via blood collection, as diagnostic tools is promising, and we hope this will be translated to clinical applications in the near future.

Biomarkers and Related Factors for the Diagnosis, Risk of Coronary Artery Lesions, and Resistance to Intravenous Immunoglobulin in Kawasaki Disease: An Umbrella Review of Meta-Analyses.

Kawasaki disease (KD) is a self-limited febrile disease predominantly affecting infants and children under 5 years old. Coronary artery lesions (CAL) are a prevalent complication, highlighting the necessity for swift diagnosis and treatment. A comprehensive review of biomarkers applicable for the diagnosis and treatment of Kawasaki disease (KD) in clinical settings is imperative. To provide a comprehensive review and analysis of biomarkers for diagnosis of KD, incidence of CAL, and intravenous immunoglobulin (IVIG) resistance. The data included in our study were sourced from searches conducted in PubMed/MEDLINE, Embase, EBSCO, and Google Scholar until March 15, 2024. Studies investigating the association with KD or evaluating diagnostic value were included in our study. Eligibility was independently assessed by two authors, with conflicts resolved through discussion. Data extraction was performed by 2 independent authors, following Meta-analyses Of Observational Studies in Epidemiology (MOOSE) guideline. Data were pooled using a random-effects model. We assess biomarkers relevant to KD, categorizing them into three groups: diagnostic, associated with CAL incidence, and linked to IVIG resistance. For studies focusing solely on association, we present standardized mean differences (SMD). For those reporting sensitivity and specificity as diagnostic measures, we calculate the diagnostic odds ratio (DOR) to compare their efficacy. We identified 14 meta-analyses on biomarkers related to KD. 11 biomarkers exhibited diagnostic value for KD, while 21 were associated with its progression. Four biomarkers, including non-codingRNAs (DOR, 19.35 [95% CI, 13.58-27.56]), Serumferritin (DOR, 24.90 [11.67-53.12]), NterminalproBNP (DOR, 21.03 [9.03-49.00]), and microRNAs (DOR, 45.28 [6.30-325.52]), have significant diagnostic value for the diagnosis of KD. Seven biomarkers showed significant association with the incidence of CAL. Twenty biomarkers were for the prediction of IVIG resistance, including prognostic nutritional index (DOR, 7.72 [95% CI, 2.37-25.09]), non-codingRNAs (DOR, 14.63 [3.24-66.14]), neutrophil to lymphocyte ratio (DOR, 6.62 [4.05-10.81]), platelet to lymphocyte ratio (DOR, 3.30 [2.10-5.19]), and Creactive protein (DOR, 6.58 [3.69-11.74]). Based on the evidence, we have proposed various biomarkers associated with KD. Our aim is for these biomarkers to have wide applicability in both diagnostic and therapeutic settings.

Clinico-Laboratory Profile and Outcome of Kawasaki Disease in Children in a Tertiary Care Hospital in Kathmandu

Introduction: Kawasaki disease (KD) is one of the most common vasculitis of childhood. It contributes significantly to acquired heart disease in children and results in coronary artery abnormalities including aneurysm in 25% of untreated children. This study aims to retrospectively assess the clinical course, laboratory results, and presenting symptoms of patients with complete and incomplete KD treated at tertiary care hospital in Kathmandu.Method: This retrospective study was conducted in the Department of Pediatrics. Data from January 2020 to December 2020 were retrieved from medical records department of the hospital and analyzed using Microsoft excel.Result: The total number of cases evaluated in our study was 9. The median age at the time of the diagnosis of KD was 24 months (median age for incomplete KD-21 months, complete KD-37 months). Male to female ratio was 1.2:1. The peak season of KD occurrence was Spring (March – May). Mean duration of stay in the hospital was 7 days. All children presented with fever, with an mean fever duration of 7 days at the time of presentation. Fever was present in every case. The most common principal symptom was a polymorphous skin rash, observed in 88% of the cases. Elevated CRP levels (≥3 mg/dl) were noted in all 9 cases. Cardiovascular complications were present in 88% of the patients, with coronary artery aneurysm (CAA) detected in 11%, coronary artery dilation in 22%, and mild pericardial effusion documented in 66% of the cases. Eight patients (88%) received IVIG, all 9 patients (100%) received aspirin, and 2 patients received steroids. There were no fatalities.Conclusion: KD is a common vasculitis in children which do not have significant sex predilection in oppose to most of the studies. Majority of cases occurred in spring season. The most common symptom was polymorphous rash. A high level of suspicion for diagnosing KD is essential, and early treatment is crucial to prevent complications and reduce the future economic burden on families and the nation.

Pediatric inflammatory multisystem syndrome or Kawasaki disease - continuous challenges in Pediatrics - case report

Introduction. Kawasaki disease (KD) and pediatric inflammatory multisystem syndrome (PIMS) are similar vasculitis conditions affecting medium and small vessels, particularly the coronary arteries. This study aimed to highlight diagnostic and treatment challenges in a case initially diagnosed as PIMS, which evolved into atypical KD. Materials and methods. A 2-year-10-month-old male was admitted with a high fever persisting for 6 days, unresponsive to antipyretics. Results. On admission, the patient exhibited a poor general condition, pale skin, non-pruritic erythematous rash, and enlarged, non-painful lymph nodes. Laboratory tests showed elevated inflammatory markers, leukocytosis with neutrophilia, hypoalbuminemia, liver enzyme abnormalities, altered cardiac markers, and elevated D-dimers, but no initial echocardiographic changes. Positive SARS-CoV-2 antibodies led to an initial PIMS diagnosis, and treatment with intravenous methylprednisolone, antiplatelet, anticoagulant therapy, and hepatoprotective agents was started. The patient’s condition initially improved but then worsened with febrile spikes, rash, neutropenia, thrombocytopenia, anemia, and coronary artery dilation on echocardiography. This led to a diagnosis of KD with atypical onset, and treatment with immunoglobulin, antiplatelet, and anticoagulant therapy was initiated. The patient’s condition improved slowly. Conclusions. KD and PIMS share overlapping characteristics, making them difficult to distinguish. There is no definitive criterion to differentiate KD and PIMS, leading to potential diagnostic errors.

Outcomes Associated with Giant Coronary Artery Aneurysms after Kawasaki Disease: A Single-Center United States Experience

To determine the long-term outcomes among a cohort of patients with Kawasaki disease (KD) and a history of giant coronary artery aneurysms (CAA) at a single US center. There were 60 patients with KD and giant CAAs identified between 1989 and 2023. The majority of patients were male (71.7%) with median age at diagnosis of 0.9 years (0.2-13.3). Patients were followed for a median of 11 years, up to 34.5 years. MACE occurred in 13 (21.7%) patients at a median of 1.4 years (0.04-22.6) after KD diagnosis. The 10-, 20-, and 30-year MACE-free rates were 75%, 75%, and 60%. Patients with maximal CA z-scores ≥20 or bilateral CAA were more likely to have MACE. During follow-up, 26.7% of CAA regressed to normal luminal diameter at a median of 3.6 years (0.6-12.0). The 10-, 20- and 30-year likelihood of CA regression to normal luminal diameter was 36%, 46%, and 46%. Over 30 years, MACE occurred in nearly 22% of patients, more often in those with bilateral CAA or CA z-scores ≥20. Despite regression to normal luminal diameter in over 25% of CAA, patients with a history of KD-associated giant CAA require ongoing surveillance for cardiac complications, even years after the initial disease.

Hypovitaminosis D and Leukocytosis to Predict Cardiovascular Abnormalities in Children with Kawasaki Disease: Insights from a Single-Center Retrospective Observational Cohort Study.

Introduction: An aberrant immune response involving yet unidentified environmental and genetic factors plays a crucial role in triggering Kawasaki disease (KD). Aims: The aim of this study was to assess general and laboratory data at the onset of KD in a single-center cohort of children managed between 2003 and 2023 and retrospectively evaluate any potential relationship with the development of KD-related cardiovascular abnormalities (CVAs). Patients and methods: We took into account a total of 65 consecutive children with KD (42 males, median age: 22 months, age range: 2-88 months) followed at the Department of Life Sciences and Public Health in our University; demographic data, clinical signs, and laboratory variables at disease onset, before IVIG infusion, including C-reactive protein, hemoglobin, white blood cell (WBC) count, neutrophil count, platelet count, aminotransferases, natremia, albumin, total bilirubin, and 25-hydroxyvitamin D were evaluated. Results: Twenty-one children (32.3% of the whole cohort) were found to have echocardiographic evidence of CVAs. Univariate analysis showed that diagnosis of KD at <1 year or >5 years was associated with CVAs (p = 0.001 and p = 0.01, respectively); patients with CVAs had a longer fever duration and mostly presented atypical or incomplete presentations. Interestingly, all patients with CVAs had lower levels of vitamin D (less than 30 mg/dL, p = 0.0001) and both higher WBC and higher neutrophil counts than those without CVAs (p = 0.0001 and p = 0.01, respectively). Moreover, blood levels of albumin were significantly lower in KD patients with CVAs compared to those without (11/21, 52% versus 13/44, 30%, p = 0.02). Multiple logistic regression with correction for sex showed that serum vitamin D < 30 ng/mL, WBC count > 20.000/mm3, and age > 60 months at KD onset were the only independent factors statistically associated with CVAs. Conclusions: Hypovitaminosis D, WBC count over 20.000/mm3, and age above 5 years at KD onset emerged as independent factors statistically associated with the occurrence of CVAs.

Kawasaki Disease in a 5-Year-Old Boy: A Rare Vasculitis Syndrome Presenting with Mucocutaneous Manifestations

Kawasaki disease (KD) is a rare, self-limiting vasculitis predominantly affecting children under the age of five. We report a case of a 5-year-old boy presenting with fever, generalized maculopapular rash, peeling of lips, erythematous oral mucosa, lymphadenopathy, and nail changes. The patient was admitted to the pediatric ward with a 3-day history of low-grade fever, rash, and vomiting episodes. Physical examination revealed bilateral lymphadenopathy in the cervical, axillary, and inguinal regions. Laboratory investigations showed elevated white blood cell count, neutrophilia, platelet count, alkaline phosphatase, aspartate aminotransferase, C-reactive protein, and erythrocyte sedimentation rate. The antistreptolysin O titer was positive, indicating recent streptococcal infection. Based on the clinical presentation and diagnostic criteria, a diagnosis of Kawasaki disease was established. The patient was treated with intravenous ceftriaxone, azithromycin, pantoprazole, and supportive therapy, including paracetamol, antihistamines, zinc, and topical calamine lotion. After 15 days of treatment, the patient showed improvement and was discharged with a planned follow-up. This case highlights the importance of early recognition and prompt treatment of Kawasaki disease to prevent potential complications, such as coronary artery aneurysms. Increased awareness among pediatricians is crucial for timely diagnosis and management of this rare vasculitis syndrome, especially in developing countries where cases may go undetected

Kawasaki disease in Kenya and review of the African literature

BackgroundKawasaki disease has been described across the globe, although publications from Africa are limited. To our knowledge, there are no publications on Kawasaki disease from Kenya, which triggered this report.MethodsA retrospective cross-sectional study was undertaken to identify in-patients with a discharge diagnosis of Kawasaki disease, over 2 different 5-year periods, at two pediatric hospitals in Nairobi, Kenya. We reviewed the medical records of all patients and report their clinical findings, diagnostic workup and treatment. In addition, we undertook a detailed review of the literature.ResultsTwenty-three patients with Kawasaki disease were identified, of those 12 (52.2%) had incomplete disease. The mean age was 2.3 years (SD+/-2.2) (range 0.3–10.3) with a male to female ratio of 1:1. The mean duration of fever at diagnosis was 8.3 days (SD+/-4.7) (range 2–20). Oral changes were the most common clinical feature and conjunctivitis the least common. Thrombocytosis at diagnosis was seen in 52% (12/23). Twenty-one patients (91.3%) were treated with intravenous immunoglobulin and all except 1 received aspirin. Baseline echocardiograms were performed in 95.7% (22/23) and found to be abnormal in 3 (13.6%). Follow-up data was limited. Our literature review identified 79 publications with documented cases of Kawasaki disease in children from 22 countries across the African continent with a total of 1115 patients including those from this report. Only 153 reported cases, or 13.7%, are from sub-Saharan Africa.ConclusionsThis is the first publication on Kawasaki disease from Kenya and one of the largest reports from sub-Saharan Africa. It is the first to have a complete review of the number of published cases from the African continent. Challenges in the diagnosis and management of Kawasaki disease in many African countries include disease awareness, infectious confounders, access and cost of intravenous immunoglobulin, access to pediatric echocardiography and follow-up. Increasing awareness and health care resources are important for improving outcomes of Kawasaki disease in Africa.

Складність діагностики рефрактерної хвороби Кавасакі під «маскою» гемобластозу: клінічний випадок із досвіду гематолога

Kawasaki disease (KD) is an acute systemic vasculitis that is the most common cause of acquired heart disease in children under 5 years of age with hyperthermia. Diagnosis of KD is a clinical challenge, given the wide range of clinical manifestations and similarities with many viral and bacterial diseases. Purpose - to describe a clinical case of refractory КD in a three-month-old girl with an emphasis on the importance of echocardiography and coronary angiography for the final verification of the disease, urgent initiation of treatment with minimal suspicion of КD. Clinical case. The article reports on a three-month-old patient with a difficult diagnosis of refractory form of КD. The disease debuted with hyperthermia, enterocolitis, obstructive bronchitis, and hepatoliver syndrome. The manifestations of skin exanthema were initially considered as an allergic dermatitis to the use of a cephalosporin antibiotic. Multisystemic inflammatory syndrome associated with SARS-CoV-2 infection was suspected. After the use of immunosuppressive therapy with mega-doses of dexamethasone and intravenous human immunoglobulin 2 g/kg/day for 3 days, clinical improvement was achieved. Subsequently, the haemogram showed an increase in neutrophilic hyperleukocytosis to the appearance of blast cells, hyperthrombocytosis and severe anaemia. A differentiation was made between a leukemic reaction, the debut of myeloproliferative disease, juvenile myelomonocytic leukaemia. Against the background of a rapid decrease in the number of leukocytes, the girl developed hyperthermia, migratory intermittent maculopapular rash, and foots edema. Laboratory findings included thrombocytosis and an increase in acute-phase parameters. Echocardiography revealed left ventricular dilation, a small amount of excess fluid in the pericardium, dilatation of the left coronary artery evenly to the bifurcation. Computed tomography revealed giant coronary artery aneurysms, which gave grounds to diagnose KD. The study was performed in accordance with the principles of the Declaration of Helsinki. Informed consent of the parents of child was obtained for the study. No conflict of interests was declared by the authors.