Diagnosis Of Kawasaki Disease Research Articles

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.

Identification of hub genes and pathogenesis in Kawasaki disease based on bioinformatics analysis.

The aim of this study was to explore new biomarkers of Kawasaki disease (KD) and provide evidence for clinical diagnosis and treatment. Gene Expression Omnibus (GEO) datasets GSE68004 and GSE73461 were downloaded, and the differentially expressed genes (DGEs) were taken, along with DEGs enrichment analysis and protein interaction network. Finally, five algorithms in CytoHubba plug-in were applied to obtain hub genes. In this study, 32 Co-DEGs were identified, and these genes mainly participated in neutrophil degranulation, neutrophil activation involved in immune response, and negative regulation of cytokine production involved in immune response; meanwhile, they were primarily enriched in starch and sucrose metabolism, fatty acid metabolism, autophagy and apoptosis, ferroptosis, and other pathways. Combined with the results of PPI and CytoHubba, 13 key genes were selected as follows: S100A12, HK3, HP, MMP9, MCEMP1, PYGL, ARG1, HIST2H2AA, ANXA3, HIST2H2AC, HIST2H2AA3, GYG1, DYSF. These 13 key genes may mediate the occurrence and development of KD through various processes such as immune regulation, inflammatory response, glucose metabolism, autophagy, and apoptosis, which provide valuable references for the diagnosis and treatment of KD.

Determination of Serum Procalcitonin Level in Kawasaki Disease

Background: 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. Objectives: 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). Methods: 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. Results: 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. Conclusions: 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.

Values of prognostic nutritional index for predicting Kawasaki disease: a systematic review and meta-analysis.

This systematic review and meta-analysis aimed to evaluate the relationship between the prognostic nutritional index (PNI) and intravenous immunoglobulin (IVIG) resistance and coronary artery lesion (CAL) in Kawasaki disease (KD). The relevant literature was searched on PubMed, Embase, Cochrane Library, Web of Science, and Google Scholar up to August 5, 2023. A pooled sensitivity, specificity, positive likelihood ratio (PLR), negative likelihood ratio (NLR), diagnostic odds ratio (DOR), and area under curve (AUC) were calculated to assess the predicted values of PNI in KD patients with IVIG resistance and CAL. A total of 8 articles containing 10 studies involving 7,047 participants were included. The pooled results revealed a pooled sensitivity of 0.44 (0.25-0.65), a pooled specificity of 0.87 (0.73-0.94), a pooled PLR of 3.4 (2.0-5.9), a pooled NLR of 0.65 (0.48-0.87), a pooled DOR of 5.26 (2.76-10.02), and a pooled AUC of 0.75 (0.71-0.78) in the diagnosis of KD with CAL. The pooled results suggested that a pooled sensitivity was 0.69 (0.60-0.77), specificity was 0.76 (0.69-0.82), PLR was 2.9 (2.1-4.1), NLR was 0.40 (0.29-0.56), DOR was 7.27 (3.89-13.59), and AUC was 0.79 (0.75-0.82) in the diagnosis of KD with IVIG resistance. The combined results revealed the pooled sensitivity was 0.63 (0.58-0.67), specificity was 0.82 (0.80-0.83), PLR was 3.09 (1.06-8.98), NLR was 0.38 (0.07-2.02), DOR was 8.23 (0.81-83.16) in differentiating KD from febrile patients. These findings demonstrated low sensitivity and relatively high specificity of PNI for KD, KD-CAL, and IVIG-resistant KD. In conclusion, this study was the first systematic review and meta-analysis of the diagnostic value of PNI in KD with IVIG resistance and CAL. The results suggested that PNI could be used as biomarkers for distinguish KD, KD with CAL, and KD with IVIG resistance.

Serum levels of PDGF-CC as a potential biomarker for the diagnosis of Kawasaki disease

BackgroundKawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that predominantly affects children, and no specific diagnostic biomarkers for KD are available. Platelet-derived growth factor CC (PDGF-CC) is a peptide with angiogenic properties that has been amply demonstrated to play a critical role in the cardiovascular system. This study aimed to investigate the serum expression of PDGF-CC in children with KD and to evaluate the ability of PDGF-CC to diagnose KD.MethodsA total of 96 subjects, including 59 KD patients, 17 febrile controls (FC), and 20 healthy controls (HC), were enrolled. Serum levels of PDGF-CC were measured via enzyme-linked immunosorbent assay. The associations between PDGF-CC and clinical laboratory parameters were investigated by correlation analysis. The diagnostic performance was assessed by receiver operating characteristic (ROC) curve analysis.ResultsSerum PDGF-CC levels in the KD group were significantly higher than in the FC and HC groups. Serum PDGF-CC levels in the KD group were positively correlated with white blood cell counts, percentage of neutrophils, IL-2, IL-12p70, TNF-α, and IL-1β levels, and negatively correlated with the percentage of lymphocytes. In the analysis of ROC curves, the area under the curve was 0.796 (95% confidence interval 0.688–0.880; P < 0.0001) for PDGF-CC and increased to 0.900 (95% confidence interval 0.808–0.957; P < 0.0001) in combination with white blood cell counts and C-reactive protein.ConclusionsPDGF-CC is a potential biomarker for KD diagnosis, and the combination with white blood cell counts and C-reactive protein can further improve diagnostic performance.

Kawasaki Disease in Children: A Brief Update

Kawasaki disease (KD) is a common acute vasculitis of childhood that may lead to coronary artery abnormalities in about 25% of patients if left untreated. KD is reported as the leading cause of acquired heart disease in children replacing rheumatic feverin developed countries. Strong epidemiological data are not available from developing countries, but it is increasingly recognized in rapidly industrializing countries of Asia including India,where it might has replaced rheumatic heart disease as the most common cause of acquired heart disease. This increasing recognition could be due to actual rise in number of KD cases or due to more diagnosis following increased awareness. The status of KD in Bangladesh is not known. But unpublished data from Bangabandhu Sheikh Mujib Medical University (BSMMU) show a sustained and significant increase in the number of KD cases over the years. The reasons for this increase may be similar to our neighbouring country India. Aetiology of KD still remains unknown. But there have been many hypotheses including infection, immune dysregulation, super-antigen and genetic factors. The diagnosis of KD is based on the presence of ≥ 5 days of fever and the presence of ≥4 of the 5 principal clinical features (1.Extremity Changes, 2. Conjunctival injection, 3.Oral changes 4.Exanthem/Rash, and 5. Lymphadenopathy). The diagnosis of KD may be very challenging for even a very experienced paediatrician. It must be considered in a child with prolonged fever, undue irritability and sequential appearance of principal clinical features. KD patients should be managed timely with intravenous immunoglobulin (IVIG) and aspirin in proper dose. It is to be remembered that KD is no longer considered as a one-time disease of childhood, as it can be associated with significant long term sequelae. J Bangladesh Coll Phys Surg 2024; 42: 68-77

The impact of inappropriate steroid exposure before the diagnosis of Kawasaki disease

BackgroundKawasaki disease (KD) is a systemic inflammatory disease characterized by vasculitis. In South Korea, some pediatric doctors empirically prescribe steroids to control febrile pediatric patients. This study aimed to evaluate the clinical characteristics of patients with KD after steroid exposure. MethodsThis was a single-center, retrospective, observational study. This study included patients (aged ≤15 years) between January 2020 and July 2022. We compared two groups, one group exposed to steroids and the other group who were not, using the Student's t-test or analysis of variance; otherwise, the Mann–Whitney U test or Kruskal–Wallis test was conducted. Statistical significance was set at p < 0.05. ResultsIn total, 190 patients with KD were enrolled; of these, 64 (33.7 %) had a history of steroid exposure, and 126 (66.3 %) had no history of steroid exposure. In the steroid exposure group, prolonged fever duration (6.72 ± 1.72 versus 5.61 ± 1.19, p-value = <0.001), a lower proportion of complete KD (29.69 % vs. 88.10 %, p-value = <0.001), and a significantly lower level of C-reactive protein were observed. However, no significant correlations were observed between the Transthoracic Echocardiography (TTE) results (coronary artery aneurysm, existence of pericardial effusion) and prognostic factors (days of hospitalization, the number of intravenous immunoglobulin administrations, and Kobayashi score) between the two groups. ConclusionsPatients with KD and previous steroid exposure may exhibit an incomplete KD phenotype with prolonged fever. Although previous steroid exposure does not affect the prognosis of KD, including coronary artery aneurysms, it may mask the classic features of KD, resulting in a delayed diagnosis.

Evidence-based guidelines for the diagnosis and treatment of Kawasaki disease in children in China (2023)

Kawasaki disease (KD) is an acute self-limiting vasculitis, and it is the most common cause of acquired heart disease in children under 5 years old. One of the improvement goals in pediatric quality control work for the year 2023, as announced by the National Health Commission, is to reduce the incidence of cardiac events and KD-related mortality in children with KD. In order to standardize the diagnosis, treatment, and long-term management practices of KD in China, and effectively prevent and reduce the incidence of coronary artery lesions and long-term adverse effects, the guideline working group followed the principles and methods outlined by the World Health Organization and referenced existing evidence and experiences to develop the "Evidence-based guidelines for the diagnosis and treatment of Kawasaki disease in children in China (2023)". The guidelines address the clinical questions regarding the classification and definition of KD, diagnosis of different types of KD, treatment during the acute phase of KD, application of echocardiography in identifying complications of KD, and management of KD combined with macrophage activation syndrome. Based on the best evidence and expert consensus, 20 recommendations were formulated, aiming to provide guidance and decision-making basis for healthcare professionals in the diagnosis and treatment of KD in children.

Research advances in genetic polymorphisms in Kawasaki disease

Kawasaki disease (KD) is a systemic inflammatory vascular disorder that predominantly affects children and is the leading cause of acquired heart disease in children. Although the etiology of this disease remains unclear, genome-wide association and genome-wide linkage studies have shown that some susceptible genes and chromosomal regions are associated with the development and progression of KD. With the advancement of high-throughput DNA sequencing techniques, more and more genomic information related to KD is being discovered. Understanding the genes involved in the pathogenesis of KD may provide novel insights into the diagnosis and treatment of KD. By analyzing related articles and summarizing related research advances, this article mainly discusses the T cell activation-enhancing genes that have been confirmed to be closely associated with the development and progression of KD and reveals their association with the pathogenesis of KD and coronary artery lesions.

Abstract 15148: How Reassuring is an Initial Echocardiogram With Normal Coronary Arteries for Patients Presenting With Acute Kawasaki Disease?

Background: We sought to determine the utility of current echocardiography surveillance recommendations for coronary artery (CA) involvement for children with Kawasaki disease (KD) with normal baseline studies. Methods: The International KD Registry enrolled 1200 patients (36 sites, 7 countries) with a site diagnosis of KD from 01/2020 to 01/2023; 139 with positive/possible COVID-19 infection/exposure and 174 with no echo within 10 days of admission were excluded, leaving 887 patients for analysis of results of serial echos and associated factors. Results: An initial echo was performed within 5 days of admission for 96% and within 10 days of symptom onset for 83% of patients; the max Z score in any CA branch at initial echo was normal (Z &lt;2) 78.9%, dilation (Z 2-&lt;2.5) 6.6%, small aneurysm (CAA; Z 2.5-&lt;5) 10.5%, medium CAA (Z 5-&lt;10) 2.9% and large CAA (Z ≥10) for 1.2% of patients. Higher CA Z score/Z score category were both significantly related to greater time from symptom onset to echo (p&lt;0.001). For those with initial normal CAs, a second echo (median of 16 days after admission) was normal for 94.2%, dilation 1.9%, small CAA 2.7%, medium CAA 0.6%, and large CAA for 0.6%. For those with 2 normal echos, a third echo (median of 44 days after admission) was normal for 97.6%, dilation 1.4%, small CAA 0.5%, and 1 patient each with medium and large CAA. Additionally, after up to 6 normal echos, 3 patients had developed small CAA, 1 medium and 1 large CAA. Overall, a total of 24 (2.8%) patients had large CAA (13 admitted &gt;10 days after symptom onset). Of these, 10 had large CAA evident at initial echo performed at day 0-3 after admission, 9 had lesser involvement at initial echo that then progressed to large CAA, and 5 had normal initial and subsequent echos and then were noted to have large CAA at an echo performed 14, 17, 21, 23 and 53 days after admission (the patient with a large CAA first detected at 53 days had no interim echos performed since their first normal echo). Conclusions: Current recommendations for serial echo assessment are effective in detecting all patients with large CAA. For rare patients, despite a normal initial echo large CAA may nonetheless develop. Additionally, important CA involvement may be evident at presentation (often delayed presentation), precluding prevention.

Prediction nomogram for coronary artery aneurysms at one month in Kawasaki disease

BackgroundCoronary status at one month after Kawasaki disease (KD) onset had a great significance. The present study aimed to establish a prediction model for coronary artery aneurysms (CAA) at one month in children with KD.MethodsPatients with a diagnosis of KD between May 2017 and Dec 2018 were enrolled as the development cohort to build a prediction model. The model was validated by internal and external validation. Patients between Jan 2019 and Dec 2019 were enrolled as the validation cohort. The adaptive least absolute shrinkage and selection operator (LASSO) was used to select the possible predictors. Receiving operating characteristic curve (ROC), calibration plots, and decision curve analysis (DCA) were used to evaluate the performance of the model. The performance of the Son score was also assessed.ResultsLASSO regression demonstrated that age, sex, and CALs in the acute stage were predictors for CAA at one month. The area under the ROC (AUC) was 0.946 (95% confidence interval: 0.911–0.980) with a sensitivity of 92.5% and a specificity of 90.5%. The calibration curve and the DCA showed a favorable diagnostic performance. The internal and external validation proved the reliability of the prediction model. The AUC of our model and the Son score were 0.941 and 0.860, respectively (P < 0.001).ConclusionOur prediction model for CAA at one month after disease onset in KD had an excellent predictive utility.

Dilemma in diagnosing incomplete Kawasaki disease in a resource limited setting

Introduction and importance: Kawasaki disease (KD) is an acute febrile systemic vasculitis that predominantly affects small to medium sized vessels and mostly occurs in children below 5 years of age. The morbidity and mortality mostly occur due to cardiac involvement. Case presentation: The authors present a case of a 5-year-old male child from hilly region of Nepal who presented with fever for 7 days along with strawberry tongue and non-exudative conjunctivitis without rashes, extremity changes or lymphadenopathy. A suspicion of incomplete KD (IKD) was made. The notable investigation findings were increased erythrocyte sedimentation rate, C-reactive protein, leucocyte count and platelets. Echocardiography showed normal findings. Based on the clinical features and supplemental laboratory findings, a diagnosis of IKD was made. The patient improved after intravenous immunoglobulin and Aspirin. Clinical discussion: The main learning objective that the authors get from this case is the challenges in the diagnosis of IKD in the resource limited setting like Nepal. Whether or not to start intravenous immunoglobulin is a dilemma for the physician in most of the cases of suspected IKD, due to the high cost and poor availability of intravenous immunoglobulin in this setting. Hence, the use of inflammatory markers, supplemental laboratory findings together with the few diagnostic criteria met by the patient helps in making a diagnosis and institute timely treatment with intravenous immunoglobulin and aspirin. Conclusion: Diagnosis of KD in difficult in resource limited setting.

Coronary external diameter index for assessing coronary artery involvement in Kawasaki disease

Aim: Transthoracic echocardiography is commonly used to assess coronary artery dilatation in Kawasaki disease (KD). However, existing criteria often miss early abnormalities. This study examines the utility of a new parameter, coronary external diameter index (CEDi), for early diagnosis and monitoring in KD. Methods: CEDi of left main (LM) and right coronary artery (RCA), calculated as the ratio of coronary artery external diameter (i.e., the distance between the outer coronary edges measured in the proximal segment of the artery) and the diameter of the aortic annulus, was evaluated in 34 patients (age 23 mouths ± 13 months) with KD at the hospital admission and after 2 weeks and 8 weeks of treatment. The control group consisted of 210 healthy children aged 20 months ± 13.4 months. Z-score charts for LM and RCA coronary external diameter (CED) were obtained. Results: Compared with controls, KD patients had a markedly higher mean value of LM CEDi (0.53 ± 0.06 vs. 0.33 ± 0.04; P &lt; 0.0001) and RCA CEDi (0.48 ± 0.05 vs. 0.31 ± 0.04; P &lt; 0.0001) at hospital admission. By ROC analysis, LM CEDi of 0.41, and RCA coronary artery thickness index (CATi) of 0.39 were the best cut-offs to confirm the clinical diagnosis of KD, both exhibiting 100% sensitivity and specificity. Mean LM CEDi and RCA CEDi values decreased significantly (P &lt; 0.0001) after 2 weeks of follow-up and were similar to controls (P = 0.53 and P = 0.12, respectively) 8 weeks after admission. Conclusions: In patients with KD, CEDi of LM and RCA is an accurate parameter to evaluate coronary artery involvement in the early phase of the illness and during follow-up.

Non-cardiac sequelae of Kawasaki Disease. The survey study of 90 cases and literature review

Introduction and objectiveThe best-known long-term complications of Kawasaki Disease (KD) are coronary artery aneurysms. However, there are numerous case reports about other unusual chronic complications. These data are only anecdotal, and more systematic observation is missing. We aimed to detect and describe chronic non-cardiac complications of KD.Material and methodsWe surveyed parents of children with KD diagnosed between 2014 and 2019 by phone with the use of standardized questionnaire. Clinical data about the acute phase of the disease, treatment, outcome, and symptoms and signs observed within three months since KD diagnosis were recorded from the caregivers. We recruited children from 3 children’s hospitals in Warsaw and a support group on social media.ResultsNinety children met the inclusion criteria. Parents of 30 children (33%) reported some neurologic sequelae, with persistent irritability being the most common, followed by sleep disturbances, aggression, and chronic fatigue. Seventeen children (19%) suffered either arthralgia or unspecific pain in the upper and lower extremities. In nine children (10%), either atopic or seborrheic dermatitis began shortly after KD. Five children (6%) presented with ophthalmic complications.ConclusionsVarious unspecific complications may emerge after the acute phase of KD. Although correlation does not imply causation, and possible explanations of observed abnormalities are numerous, our data may enhance clinicians’ awareness of frequent and poorly understood findings observed after KD in children. It also might help reassure parents that what they observe lies within a range of common and usually transient KD sequelae.

Clinical value of echocardiography combined with serum Cav-1, NFATc1, and PAI-1 in the diagnosis of Kawasaki disease complicated with coronary artery lesions.

To analyze the clinical value of echocardiography combined with serum lacuna protein-1 (Cav-1), activated T cell nuclear factor C1 (NFATc1), and plasminogen activator inhibitor-1 (PAI-1) in the diagnosis of Kawasaki disease (KD) complicated with coronary artery lesions (CAL). A total of 200 children with KD treated in our hospital from January 2019 to October 2021 were grouped as the KD alone group (n = 56) and the KD complicated with CAL group (n = 144) according to the results of coronary angiography. The levels of Cav-1, NFATc1, and PAI-1 were detected by enzyme-linked immunosorbent assay. Echocardiography was performed and the internal diameters of left and right coronary arteries were compared between the two groups. The area under the curve (AUC), sensitivity, and specificity of echocardiography combined with serum Cav-1, NFATc1, and PAI-1 in the diagnosis of KD complicated with CAL were analyzed with receiver operating characteristic (ROC) curve. Coronary angiography, as the gold standard, showed that the sensitivity of echocardiography in diagnosing KD with CAL was 88.19% (127/144), the specificity was 66.07% (37/56), and the accuracy was 82.00% (164/200). ROC curve analysis revealed that the AUC of KD complicated with CAL diagnosed by echocardiography, Cav-1, NFATc1, and PAI-1 was 0.819, 0.715, 0.688, and 0.663, respectively, and the AUC of combined diagnosis of the four was 0.896. The combination of echocardiography, Cav-1, NFATc1, and PAI-1 has high value in diagnosing KD complicated with CAL, which can be widely used in clinical practice.

Comparison of Demographic, Clinical, and Echocardiographic Features Between Complete and Incomplete, and Early and Late Presenters of Kawasaki Disease: A 10-Year Single-Center Experience.

The diagnosis of Kawasaki disease(KD)is based mainly on clinical findings and supported by laboratory tests. Complete KD fulfills the main clinical criteria, while incomplete KD includes patients with fewer main criteria and compatible laboratory or echocardiographic findings. The study compares the demographic, clinical, laboratory, and echocardiographic parameters between the complete and incomplete KD and early and late presenters. Moreover, it describes the coronary manifestations of the study population. A retrospective review of all patients admitted with a diagnosis of KD during the period from January 2010 to September 2020 was conducted. Clinical presentation, laboratory features, echocardiographic observations, and follow-up data were examined. Moreover, the patients were further classified as early presenters (presented within 10 days of fever onset) and late presenters (presented after 10 days of disease onset). A comparison between complete and incomplete KD and early and late presenters was performed for demographic, clinical, and echocardiographic features. A total of 76 patients were admitted with a diagnosis of KD.The median age of presentation was 28 months, with a range of five to 144months, and the median timing was seven days, with a range of one to 30days. The median follow-up period was six weeks, with a range of one to 192weeks. Complete KD was present in 38 patients (50%), and 38 (50%) had incomplete KD.Skin manifestations, oral mucosal changes, skin desquamation, conjunctivitis, and lymphadenopathy were present more in patients with complete KD than incomplete ones. Complete and incomplete diseases did not differ regarding coronary artery lesions. Of the patients,53 (70%) presented 10 days or less after the onset of fever, and 23 (30%) presented after the 10th day of disease onset. Comparison between early and late presenters revealed significantly greater mucus membrane changes and lymphadenopathy manifestations among the early presenters and coronary artery lesions among the late presenters. The clinical features of KD should prompt early referral for evaluation, echocardiography, and early administration of intravenous immunoglobulin to prevent coronary artery complications. The complete form of Kawasaki does not have more frequent coronary artery lesions than the incomplete form. Additionally, late presenters may be at increased risk for coronary artery abnormalities than early presenters.

6 Evaluation of a Standardized Kawasaki Disease Protocol for Management and Follow-Up

Abstract Background In 2017, it was recognized that there was significant variation in the management and follow-up of Kawasaki disease (KD) at our institution, resulting in inefficient use of hospital resources and patient confusion. A multidisciplinary team from cardiology, rheumatology, emergency medicine, infectious diseases (ID), and general paediatrics created a standardized protocol distributed on October 10th, 2019. With the evolution of multisystem inflammatory syndrome in children associated with SARS-COVID 19 (MISC), we expanded our protocol in December 2021 to include a 1-week follow-up echocardiogram (echo). Objectives It has been 3 years since the KD standardized protocol was implemented. As a result, we seek to evaluate its effectiveness in improving patient care, specifically in the patients with normal coronary arteries. Design/Methods We reviewed the cardiology echo database to define patients who had an echo performed for KD (January 1, 2020-August 17, 2022). These patients were reviewed in the electronic hospital record to define those with a discharge diagnosis of KD. Results Of the 138 patients defined, 47 patients were discharged with KD; normal coronary arteries (N=38) were seen in 81% (38/47). Classic KD: 47% (18/38); incomplete KD: 53% (20/38); 13% (5/38) were diagnosed with both MISC and KD. Average age: 3.4 years (3 months to 9.5 years). We observed that rheumatology was consulted in 89% (34/38), cardiology in 100%, and ID in 50% (19/38). All patients were treated with low-dose acetylsalicylic acid (38/38), and the majority were treated with intravenous immunoglobulin, 92% (35/38). Some patients were treated with steroids, 26% (10/38). Average length of stay: 4.8 days (1-18 days). Twenty-one patients were seen from when we initiated performing a 1-week echo. In addition to the existing protocol, 24% (5/21) of cases missed having a 1-week follow-up echo because the cardiologist was not aware of the practice. Eighty-nine% (34/38) had their 6-week follow-up echo and follow-up in the post-hospitalization clinic (PHC) at the appropriate time. Patients were incorrectly scheduled to see the cardiologist in 13% (5/38) and the PHC in 3% (1/38) cases. Conclusion With the implementation of a standardized management and follow-up protocol for KD, our patients are now guaranteed follow-up with access to effective patient-centred care in a streamlined manner that targets appropriate use of resources. A satisfaction survey should be performed to inquire if families are pleased with this process.

Diagnosis, Progress, and Treatment Update of Kawasaki Disease.

Kawasaki disease (KD) is an acute inflammatory disorder that primarily affects children and can lead to coronary artery lesions (CAL) if not diagnosed and treated promptly. The original clinical criteria for diagnosing KD were reported by Dr. Tomisaku Kawasaki in 1967 and have been used for decades. However, research since then has highlighted the limitations of relying solely on these criteria, as they might lead to underdiagnosis or delayed diagnosis, potentially increasing the risk of coronary artery complications. This review appears to discuss several important aspects related to KD diagnosis and management. The current diagnostic methods for KD might need updates, especially considering cases that do not fit the typical clinical criteria. Recognizing diagnostic pitfalls and distinguishing KD from other conditions that might have similar clinical presentations is essential. The differences and similarities between KD and Multisystem Inflammatory Syndrome in Children (MIS-C), another inflammatory condition that has been associated with COVID-19, were also reviewed. The review explores the potential role of eosinophil count, new biomarkers, microRNA panels, and scoring systems in aiding the diagnosis of KD. Overall, the review article provides a comprehensive overview of the evolving landscape of KD diagnosis and management, incorporating new diagnostic methods, biomarkers, and treatment approaches to improve patient outcomes and reduce the risk of complications.

Association of Yersinia Infection With Kawasaki Disease: A Prospective Multicenter Cohort Study.

Yersinia infection is known to present with Kawasaki disease (KD)-like symptoms although differentiating the 2 has been a challenge. The present study aimed to describe the clinical characteristics and prevalence of Yersinia infection presenting with KD-like symptoms. The present, prospective, multicenter study enrolled patients who received a diagnosis of KD between January 2021 and January 2022 at 2 hospitals in Tokyo. Stool samples were collected within 3 days of the start of KD treatment, and cultures were performed for Yersinia . Clinical history and symptoms suggestive of Yersinia infection were also evaluated. During the study period, 141 KD patients were screened and 117 patients with evaluable stool samples were registered. Only 1 patient was positive for Yersinia pseudotuberculosis , which was detected from both stool and blood cultures. The patient was refractory to KD treatment but improved after initiation of appropriate antibiotic therapy. Routine screening for Yersinia is not appropriate for patients with KD and should be limited to certain patients in high-risk areas and those who are refractory to the standard KD treatment.