Quantitative Assessment of Myocardial Edema by MR T2 Mapping in Children With Kawasaki Disease.

BACKGROUND: Few studies assessed myocardial inflammation using Cardiovascular Magnetic Resonance Imaging in Kawasaki disease (KD) patients. PURPOSE: To quantify myocardial edema in KD patients using T2 mapping and explore the independent predictors of T2 values. STUDY TYPE: Prospective. SUBJECTS: Ninety KD patients including 40 in acute phase (26 males, 65.0%) and 50 in chronic phase (34 males, 68.0%). Thirty-one healthy volunteers (21 males, 70.0%). FIELD STRENGTH/SEQUENCE: 3.0 T T2-weighted Turbo Spin Echo-Short Time of Inversion Recovery sequence, True fast imaging with steady precession flash sequence and fast low-angle shot 3D spoiled gradient echo sequence. ASSESSMENT: T2 values were compared among KD groups and controls. STATISTICAL TEST: Student's t test and Fisher's exact test; One-way analysis of variance; Pearson correlation analysis; Receiver operating curve analysis; Multivariable linear regression. RESULTS: Global T2 value of KD patients in acute phase was the highest, followed by those of chronic-phase patients and controls (38.83 ± 2.41 msec vs. 37.55 ± 2.28 msec vs. 36.05 ± 1.64 msec). Regional T2 values showed a same trend. There were no significant differences in global and regional T2 values between KD patients with and without coronary artery (CA) dilation, no matter in acute or chronic phase (all KD patients: P = 0.51, 0.51, 0.53, 0.72; acute KD: P = 0.61, 0.37, 0.33, 0.83; chronic KD: P = 0.65, 0.79, 0.62, 0.79). No significant difference was observed in global T2 values between KD patients with Z score > 5.0 and 2.0 < Z score ≤ 5.0 (P = 0.65). Multivariate analysis demonstrated that stage of disease (ß = -0.123) and heart rate (ß = 0.280) were independently associated with global T2 values. DATA CONCLUSION: The degree of myocardial edema was more severe in acute-phase than in chronic-phase KD patients. Myocardial edema persists in patients regardless of the existence or degree of CA dilation. EVIDENCE LEVEL: 2 TECHNICAL EFFICACY: Stage 2.

Novel Predictive Scoring System for Intravenous Immunoglobulin Resistance Helps Timely Intervention in Kawasaki Disease: The Chinese Experience.

Background: Approximately 10%-20% of patients with Kawasaki disease (KD) are nonresponsive to intravenous immunoglobulin (IVIG) treatment, placing them at higher risk of developing coronary heart lesions. Early detection of nonresponsiveness is crucial to curtail this risk; however, the applicability of existing predictive scoring systems is limited to the Japanese population. Our study aimed to identify a predictive scoring system for IVIG resistance in KD specific to the Chinese population. We aimed to assess the utility of three commonly used risk-scoring systems in predicting IVIG resistance and compare them to the newly developed predictive scoring system. Methods: A total of 895 patients with KD were enrolled in this retrospective review and divided into two groups: IVIG responders and nonresponders. Clinical and laboratory variables were compared between the two groups. Multivariable logistic regression models were used to construct a new scoring system. The utility of the existing and new scoring systems was assessed and compared using the area under the receiver operating characteristic curve. Results: Albumin levels, percentage of neutrophils, and hemoglobin were independent predictors of resistance by logistic regression analysis. The new predictive scoring system was derived with improved sensitivity (60.5%) and specificity (87.8%). The area under the receiver operating characteristic curve was 0.818. Conclusion: This study developed a novel risk-scoring system for predicting resistance to IVIG treatment in KD specific to the Chinese population. Although this new model requires further validation, it may be useful for improving prognostic outcomes and reducing the risk of complications associated with KD.

Research on Early Identification Model of Intravenous Immunoglobulin Resistant Kawasaki Disease Based on Gradient Boosting Decision Tree.

BACKGROUND: To construct a predictive model for intravenous immunoglobulin (IVIG) resistant Kawasaki disease (KD) based on the gradient boosting decision tree (GBDT), so as to early identify children with IVIG resistance and actively take additional treatment to prevent adverse events. METHODS: The case data of KD children hospitalized in the Pediatric Department of Lanzhou University Second Hospital from October 2015 to July 2020 were collected. All KD patients were divided into IVIG responsive group and IVIG resistant group. GBDT was used to explore the influencing factors of IVIG-resistant KD and to construct a prediction model. Then compared with previous models, the optimal model was selected. RESULTS: In the process of GBDT model construction, 80% of the data were used as the test set, and 20% of the data were used as the validation set. Among them, the verification set was used to adjust the hyperparameters in GDBT learning. The model performed best with a hyperparameter tree depth of 5. The area under the curve of the GBDT model constructed based on the best parameters was 0.87 (95% CI: 0.85-0.90), the sensitivity was 72.62%, the specificity was 89.04%, and the accuracy was 61.65%. The contribution degree of each feature value to the model was total bilirubin, albumin, C-reactive protein, fever time, and Na in order. CONCLUSION: The GBDT model is more suitable for the prediction of IVIG-resistant KD in this study area.

Risk-prediction models for intravenous immunoglobulin resistance in Kawasaki disease: Risk-of-Bias Assessment using PROBAST.

BACKGROUND: The prediction model of intravenous immunoglobulin (IVIG) resistance in Kawasaki disease can calculate the probability of IVIG resistance and provide a basis for clinical decision-making. We aim to assess the quality of these models developed in the children with Kawasaki disease. METHODS: Studies of prediction models for IVIG-resistant Kawasaki disease were identified through searches in the PubMed, Web of Science, and Embase databases. Two investigators independently performed literature screening, data extraction, quality evaluation, and discrepancies were settled by a statistician. The checklist for critical appraisal and data extraction for systematic reviews of prediction modeling studies (CHARMS) was used for data extraction, and the prediction models were evaluated using the Prediction Model Risk of Bias Assessment Tool (PROBAST). RESULTS: Seventeen studies meeting the selection criteria were included in the qualitative analysis. The top three predictors were neutrophil measurements (peripheral neutrophil count and neutrophil %), serum albumin level, and C-reactive protein (CRP) level. The reported area under the curve (AUC) values for the developed models ranged from 0.672 (95% confidence interval [CI]: 0.631-0.712) to 0.891 (95% CI: 0.837-0.945); The studies showed a high risk of bias (ROB) for modeling techniques, yielding a high overall ROB. CONCLUSION: IVIG resistance models for Kawasaki disease showed high ROB. An emphasis on improving their quality can provide high-quality evidence for clinical practice. IMPACT STATEMENT: This study systematically evaluated the risk of bias (ROB) of existing prediction models for intravenous immunoglobulin (IVIG) resistance in Kawasaki disease to provide guidance for future model development meeting clinical expectations. This is the first study to systematically evaluate the ROB of IVIG resistance in Kawasaki disease by using PROBAST. ROB may reduce model performance in different populations. Future prediction models should account for this problem, and PROBAST can help improve the methodological quality and applicability of prediction model development.

Síndrome inflamatorio multisistémico asociado a COVID-19 en pediatría: serie de casos en servicios públicos de salud de Neuquén / Multisystem inflammatory syndrome associated with COVID-19 in children: case series in Neuquén public health services

INTRODUCCIÓN: Durante la pandemia por SARS-CoV-2 se reportaron casos de un síndrome de inflamación multisistémica similar a la enfermedad de Kawasaki con antecedente de infección reciente o contacto con casos de COVID-19, generando una relación temporal con dicha enfermedad (SIM-C). El objetivo de este trabajo fue caracterizar los aspectos clínicos y epidemiológicos de los casos de SIM-C en menores de 18 años. MÉTODOS: Se realizó un estudio transversal, observacional y descriptivo de casos de SIM-C en menores de 18 años asistidos entre marzo de 2020 y junio de 2022 en el sistema público de la provincia de Neuquén. RESULTADOS: Serie de casos: Se incluyó a 9 pacientes con SIM-C: 55,5% de sexo femenino, con una media de edad de 6,1 años. El 77,7% de los casos de COVID-19 fueron confirmados por nexo epidemiológico. Todos los pacientes presentaron fiebre previa a la internación, el 88,8% tuvo manifestaciones mucocutáneas y compromiso abdominal. Otras manifestaciones frecuentes fueron compromiso ocular y edema de manos. El 33,3% de los pacientes requirieron internación en unidades de cuidados intensivos pediátricos. Solo 1 necesitó asistencia respiratoria mecánica por 48 horas por shock. Todos los pacientes fueron tratados con inmunoglobulina intravenosa (IGIV) 2 g/kg, y 3 pacientes recibieron corticoterapia. No hubo fallecimientos ni complicaciones en el seguimiento. DISCUSIÓN: Aunque el pronóstico es favorable, se sugiere realizar estudios que monitoreen los efectos a largo plazo de SIM-C.

Assessment of vascular and endothelial function in Kawasaki disease.

BACKGROUND: Kawasaki disease (KD) is an acute febrile vasculitis. Patients with previous KD have increased risk of coronary arterial aneurysms (CAA) and early-onset arteriosclerosis. Endothelial dysfunction is the earliest manifestation of arteriosclerosis. We aimed to explore the endothelial function and clinical characteristics of patients with previous KD. METHODS: In this case-control study, we investigated childhood KD patients, with and without CAA, and a group of healthy controls. We obtained the anthropometric measurements, metabolic markers, vascular ultrasonography evaluating arterial stiffness and flow-mediated dilatation (FMD), and clinical information obtained by reviewing the patients' charts. Continuous variables were compared using non-parametric analyses and categorical variables, using the chi-square or Fisher's exact tests. RESULTS: Seventy KD patients (median current age, 12.95 years; median follow-up duration, 10.88 years) and 14 healthy controls were recruited. FMD was significantly lower in the CAA group (n = 15) than the control group (FMDs: 5.59% [interquartile range, 3.99-6.86%] vs. 7.49% [5.96-9.42%], p = 0.049; diastolic FMD: 6.48% [4.14-7.32%] vs. 7.87% [6.19-9.98%], p = 0.042). The CAA group had a higher percentage of impaired FMD and the significantly largest coronary segments of the three groups. Other parameters including metabolic markers, carotid intima-media thickness, and arterial stiffness were not statistically different. CONCLUSION: KD patients, especially those with CAAs, may have impaired endothelial function. FMD may be a good indicator of endothelial dysfunction for use in long-term follow-up of KD patients.

Predicting immunoglobulin resistance in Kawasaki disease: an assessment of neutrophil to lymphocyte platelet ratio.

BACKGROUND: Kawasaki disease (KD) is an acute febrile illness of unknown etiology and predictors for intravenous immunoglobulin (IVIG) resistance have been widely explored in recent decades. Neutrophil to lymphocyte platelet ratio (NLPR) was reported to be associated with the outcomes in many diseases. However, its relationship with IVIG resistance has not be explored. METHODS: The medical data of patients diagnosed with KD in Children's Hospital of Soochow University between January 2019 and December 2020 were retrospectively reviewed and analyzed. Patients were trisected into three groups based on NLPR. Logistics regression was used to analyze the association between NLPR and IVIG resistance. Restricted cubic spine was used to exhibit the relationship. Sensitivity analysis and subgroup analysis were also carried out. RESULTS: A total of 803 patients were included in the present study (61.8% males; median age: 24 months). IVIG resistance occurred in 74 (9.2%) patients. Multivariable-adjusted analyses revealed higher NLPR (odds ratio [95% confidence interval]: 1.12 [1.00-1.24]) was an independent predictor of IVIG resistance, which was strengthened by sensitivity analyses. The association of NLPR and IVIG resistance was not modified by age, sex, CALs, or days of IVIG initiation ≤ 4. CONCLUSION: NLPR may be a valuable prognostic marker in KD patients with IVIG resistance.

A decision tree model for predicting intravenous immunoglobulin resistance and coronary artery involvement in Kawasaki disease.

OBJECTIVES: This study aims to develop a new algorithm for predicting intravenous immunoglobulin (IVIG) resistance and coronary artery involvement in Kawasaki disease (KD) through decision tree models. METHODS: Medical records of children hospitalized for KD were analysed retrospectively. We compared the clinical characteristics, and the laboratory data in the groups with IVIG resistance and coronary artery dilatations (CADs) in KD patients. The decision tree models were developed to predict IVIG resistance and CADs. RESULTS: A total 896 patients (511 males and 385 females; 1 month-12 years) were eligible. IVIG resistance was identified in 111 (12.3%) patients, and CADs were found in 156 (17.4%). Total bilirubin and nitrogen terminal- pro-brain natriuretic peptide (NT-proBNP) were significantly higher in IVIG resistant group than in IVIG responsive group (0.62 ± 0.8 mg/dL vs 1.38 ± 1.4 mg/dL and 1231 ± 2136 pg/mL vs 2425 ± 4459 mL, respectively, P < 0.01). Also, CADs were more developed in the resistant group (39/111; 14.9% vs. 117/785; 35.1%, P < 0.01). The decision tree for predicting IVIG resistance was classified based on total bilirubin (0.7 mg/mL, 1.46 mg/dL) and NT-proBNP (1561 pg/mL), consisting of two layers and four nodes, with 86.2% training accuracy and 90.5% evaluation accuracy. The Receiver Operating Characteristic (ROC) evaluated the predictive ability of the decision tree, and the area under the curve (AUC) (0.834; 95% confidence interval, 0.675-0.973; P < 0.05) showed relatively higher accuracy. The group with CADs had significantly higher total bilirubin and NT-proBNP levels than the control group (0.64 ± 0.82 mg/dL vs 1.04 ± 1.14 mg/dL and 1192 ± 2049 pg/mL vs 2268 ± 4136 pg/mL, respectively, P < 0.01). The decision trees for predicting CADs were classified into two nodes based on NT-proBNP (789 pg/mL) alone, with 83.5% training accuracy and 90.3% evaluation accuracy. CONCLUSION: A new algorithm decision tree model presents for predicting IVIG resistance and CADs in KD, confirming the usefulness of NT-proBNP as a predictor of KD.

Cost-effectiveness analysis of infliximab for the treatment of Kawasaki disease refractory to the initial treatment: A retrospective cohort study.

BACKGROUND: Infliximab (IFX) treatment is approved in Japan for health insurance coverage in patients with Kawasaki disease (KD). This study aimed to compare the cost-effectiveness of IFX and other therapeutic strategies for KD refractory to initial treatment, including intravenous immunoglobulin (IVIG), steroids, immunosuppressants, and plasma exchange therapy. METHODS: This multicenter, retrospective cohort study utilized data from the public medical insurance system of Japan. The target population included those who received treatment for KD between April 2012 and March 2019. Eligibility criteria were as follows: 1) initial onset of KD, 2) age < 15 years at onset, and 3) administration of 3rd line treatment if the 1st line treatment was IVIG alone or 2nd line treatment if the 1st line treatment was a combination of IVIG and steroids, in accordance with Japanese guidelines (2012). Those with KD-related cardiovascular complications before admission and those with congenital cardiac disease were excluded. The primary outcome was cost-effectiveness, which was calculated based on the number of admission events per annum divided by medical expenses per annum (times/10,000 US dollars). The Wilcoxon test was applied to analyze the difference in cost-effectiveness between patients who had received IFX and those who had not. RESULTS: Among 1267 patients with KD, 25 received IFX treatment, while 206 received another treatment after the disease was designated refractory to initial treatment. The frequency of steroid use during initial IVIG treatment (a predictor of severity) was higher in the non-IFX group than in the IFX group (70.4% vs. 32.0%, p < 0.001) but became comparable after propensity-score matching. Our analysis indicated that IFX was more cost-effective than other treatments [1.04 (0.86, 1.34) vs. 1.38 (1.03, 1.79) (times/10,000 US dollars), p = 0.006]. CONCLUSIONS: IFX treatment may be more cost-effective than non-IFX treatment for patients with KD that is refractory to initial treatment.

Trends and Resource Utilization in Kawasaki Disease Hospitalizations in the United States, 2008-2017.

OBJECTIVES: To explore trends in hospitalization rate, resource use, and outcomes of Kawasaki Disease (KD) in children in the United States from 2008 to 2017. METHODS: This was a retrospective, serial cross-sectional analysis of pediatric hospitalizations with International Classification of Disease diagnostic codes for KD in the National Inpatient Sample. Hospitalization rates per 100 000 populations were calculated and stratified by age group, gender, race, and US census region. Prevalence of coronary artery aneurysms (CAA) were expressed as proportions of KD hospitalizations. Resource use was defined in terms of length of stay and hospital cost. Cochran-Armitage and Jonckheere-Terpstra trend tests were used for categorical and continuous variables, respectively. P <.05 was considered significant. RESULTS: A total of 43 028 pediatric hospitalizations identified with KD, yielding an overall hospitalization rate of 5.5 per 100 000 children. The overall KD hospitalization rate remained stable over the study period (P = .18). Although KD hospitalization rates differed by age group, gender, race, and census region, a significant increase was observed among Native Americans (P = .048). Rates of CAA among KD hospitalization increased from 2.4% to 6.8% (P = .04). Length of stay remained stable at 2 to 3 days, but inflation-adjusted hospital cost increased from $6819 in 2008 to $10 061 in 2017 (Ptrend < 0.001). CONCLUSIONS: Hospitalization-associated costs and rates of CAA diagnostic codes among KD hospitalizations increased, despite a stable KD hospitalization rate between 2008 and 2017. These findings warrant further investigation and confirmation with databases with granular clinical information.

Association of left ventricular systolic dysfunction with coronary artery dilation in Kawasaki disease patients: Assessment with cardiovascular magnetic resonance.

PURPOSE: To quantify global and regional left ventricular (LV) strain parameters in patients with Kawasaki disease (KD) using cardiovascular magnetic resonance (CMR) tissue tracking and assess the association of coronary artery dilation (CA dilation) with LV systolic dysfunction. METHODS: Thirty-one KD patients with CA dilation, 22 patients without CA dilation and 27 age- and sex-matched normal controls underwent 3.0 T CMR examination. Z score of >2 was defined as CA dilation. Global LV strain parameters and regional LV strain parameters in 16 American Heart Association segmentation, including radial, circumferential and longitudinal peak strain (PS) and LV function were measured and compared among groups. RESULTS: No significant difference in LV ejection fraction has been observed among controls, KD patients with CA dilation and without CA dilation (all p > 0.05). However, global longitudinal PS (GLPS) was lower in groups with CA dilation than those without CA dilation (-12.6 ± 4.1% vs -14.9 ± 2.6%, p < 0.05). For regional strain parameters, the segments with CA dilation (n = 301) were lower than those in both normal controls (n = 416) and segments without CA dilation (n = 547) in regional radial, circumferential and longitudinal PS (all p < 0.05). The severity of CA dilation was positively correlated to GLPS and regional longitudinal PS (r = 0.388 and r = 0.222; both p < 0.05) in KD patients. After adjusting for clinical characteristics, the multivariate analysis demonstrated that Z score was independently associated with GLPS in KD patients (ß = 0.469, p = 0.000, model R2 = 0.355). CONCLUSIONS: CMR tissue tracking could sensitively identify subclinical LV dysfunction in KD patients with CA dilation. LV systolic dysfunction occurs particularly in the myocardium dominated by the dilated coronary artery. CA dilation is an independent predictor of LV systolic dysfunction.

Temporal clustering of Kawasaki disease cases around the world.

In a single-site study (San Diego, CA, USA), we previously showed that Kawasaki Disease (KD) cases cluster temporally in bursts of approximately 7 days. These clusters occurred more often than would be expected at random even after accounting for long-term trends and seasonality. This finding raised the question of whether other locations around the world experience similar temporal clusters of KD that might offer clues to disease etiology. Here we combine data from San Diego and nine additional sites around the world with hospitals that care for large numbers of KD patients, as well as two multi-hospital catchment regions. We found that across these sites, KD cases clustered at short time scales and there were anomalously long quiet periods with no cases. Both of these phenomena occurred more often than would be expected given local trends and seasonality. Additionally, we found unusually frequent temporal overlaps of KD clusters and quiet periods between pairs of sites. These findings suggest that regional and planetary range environmental influences create periods of higher or lower exposure to KD triggers that may offer clues to the etiology of KD.

Assessment of Pediatric Admissions for Kawasaki Disease or Infectious Disease During the COVID-19 State of Emergency in Japan.

Importance: The development of Kawasaki disease (KD) has been suggested to be associated with droplet- or contact-transmitted infection; however, its triggers and transmission modes remain to be determined. Under an epidemic of SARS-CoV-2, the COVID-19 state of emergency in Japan served as a nationwide social experiment to investigate the impact of quarantine or isolation on the incidence of KD. Objective: To assess the role of droplet or contact transmission in the etiopathogenesis of KD. Design, Setting, and Participants: This multicenter, longitudinal, cross-sectional study was conducted from 2015 to 2020 at Fukuoka Children's Hospital and 5 adjacent general hospitals. The number of admissions for KD and infectious diseases were analyzed. Participants were pediatric patients admitted to the participating hospitals for KD or infectious diseases. Exposures: Quarantine and isolation owing to the COVID-19 state of emergency. Main Outcomes and Measures: The primary end points were the ratios of patients with KD to patients with respiratory tract or gastrointestinal infections admitted from April to May in 2015 to 2019 and 2020. A Poisson regression model was used to analyze them. Results: The study participants included 1649 patients with KD (median [interquartile range] age, 25 [13-43] months; 901 boys [54.6%]) and 15 586 patients with infectious disease (data on age and sex were not available for these patients). The number of admissions for KD showed no significant change between April and May in 2015 to 2019 vs the same months in 2020 (mean [SD], 24.8 [5.6] vs 18.0 [4.0] admissions per month; 27.4% decrease; adjusted incidence rate ratio [aIRR], 0.73; 95% CI, 0.48-1.10; P = .12). However, the number of admissions for droplet-transmitted or contact-transmitted respiratory tract infections (mean [SD], 157.6 [14.4] vs 39.0 [15.0] admissions per month; 75.3% decrease; aIRR, 0.25; 95% CI, 0.17-0.35; P < .001) and gastrointestinal infections (mean [SD], 43.8 [12.9] vs 6.0 [2.0] admissions per month; 86.3% decrease; aIRR, 0.14; 95% CI, 0.04-0.43; P < .001) showed significant decreases between April and May in 2015 to 2019 vs the same months in 2020 (total, 12 254 infections). Thus, the ratio of KD to droplet- or contact-transmitted respiratory tract and gastrointestinal infections incidence in April and May 2020 was significantly increased (ratio, 0.40 vs 0.12; χ21 = 22.76; P < .001). Conclusions and Relevance: In this study, the significantly increased incidence of KD compared with respiratory tract and gastrointestinal infections during the COVID-19 state of emergency suggests that contact or droplet transmission is not a major route for KD development and that KD may be associated with airborne infections in most cases.

Assessing Health Care Utilization and Compliance in Kawasaki Disease.

OBJECTIVE: To characterize health care utilization and costs associated with care after diagnosis of Kawasaki disease including adherence to guidelines for echocardiograms. STUDY DESIGN: We analyzed children hospitalized for Kawasaki disease using 2015-2017 national Truven MarketScan commercial claims data. The mean 90-day prehospitalization utilization and costs were quantified and compared with the 90 days posthospitalization via Wilcoxon 2-sample test. Adherence to echocardiogram guidelines was examined using multivariable logistic regression to identify factors associated with adherence. RESULTS: The mean total payments 90 days prior to hospitalization ($2090; n = 360) were significantly lower than those after discharge ($3778), though out of pocket costs were higher ($400 vs $270) (P < .0001). There was an increase in office visits, medical procedures, and echocardiograms after discharge. A majority of health care utilization before hospitalization occurred in the 7 days immediately prior to the date of admission; 51% obtained an echocardiogram within the first 2 weeks, and 14% were completely adherent with recommendations. Children with greater utilization prior to admission were more likely to adhere to American Heart Association guidelines for follow-up echocardiograms (OR 1.03, 95% CI 1.01-1.06). CONCLUSIONS: Outpatient health care expenditure nearly doubles after Kawasaki disease hospital discharge when compared with prehospitalization, suggesting the financial ramifications of this diagnosis persist beyond costs incurred during hospitalization. A significant portion of patients do not receive guideline recommended follow-up echocardiograms. This issue should be explored in more detail given the morbidity and mortality associated with this diagnosis.

RADIATION DOSE FOR PATIENTS WITH KAWASAKI DISEASE UNDERGOING FLUOROSCOPICALLY GUIDED CARDIAC CATHETERIZATION.

The goal of the present study was to estimate the radiation dose for a group of 45 Kawasaki disease (KD) patients undergoing fluoroscopically guided cardiac catheterization. The sample of procedures corresponds to a single hospital and was collected in 10 years. Anthropometric characteristics and the quantities of air kerma-area product (PKA) among others were recorded for each procedure. Monte Carlo PCXMC 2.0 software was used to estimate organ and effective doses. The PKA value of 7.2 Gy cm2 was proposed as the local Diagnostic Reference Level for KD. For organ absorbed doses, median values for thyroid, heart, lungs, esophagus, skin, active bone and breast were 1.2; 2.2; 4.6; 2.7; 1.1; 1.2 and 2.7 mGy, respectively. For effective dose, the mean value was 2.7 ± 2.5 mSv. This paper presents the first patient dose values for the KD using catheterization techniques, in Latin America and the Caribbean Region.

Assessment of Endothelial Dysfunction in Acute and Convalescent Phases of Kawasaki Disease Using Automated Edge Detection Software: A Preliminary Study From North India.

OBJECTIVE: The aim of this study was to assess endothelial dysfunction in acute and convalescent phases of Kawasaki disease (KD) using automated edge detection software. METHODS: This was a case-control study to assess the flow-mediated dilatation (FMD) of brachial artery (BA) in patients with KD during acute phase and at least 3 months after diagnosis. A 10-MHz multifrequency linear array probe attached to a high-resolution ultrasound machine (PHILIPS Medical System-IU22) was used to acquire the images. Automated edge detection software was used to assess BA diameter. RESULTS: A total of 16 children with KD and 16 healthy children were enrolled in the study. Mean ± SD maximum BA diameter was found to be significantly low during the acute stage of KD (2.56 ± 0.36 mm) as compared with the convalescence phase (2.93 mm ± 0.31) and in healthy controls (2.95 mm ± 0.56). The mean ± SD percentage change in the FMD was found to be significantly low in the acute phase of KD (12.32 ± 6.2) as compared with the convalescence phase of KD (17.99 ± 8.13) and healthy controls (26.88 ± 12.76). The mean ± SD percentage change in the FMD was also found to be significantly low in the convalescence phase of KD as compared with healthy controls. CONCLUSIONS: The FMD of the BA is significantly reduced in patients during the acute and convalescence phase of KD as compared with normal healthy children. The endothelial dysfunction was present even in patients who had no obvious coronary artery abnormalities during the acute stage.

Kawasaki Disease and Clinical Outcome Disparities Among Black Children.

OBJECTIVE: To determine whether Black children with Kawasaki disease exhibit disparities in prevalence, sequelae, and response to intravenous gamma globulin (IVIG) treatment. STUDY DESIGN: International Classification of Diseases codes were used to identify children with Kawasaki disease admitted to a tertiary center in the southeastern US. Subjects diagnosed and treated according to American Heart Association criteria were included. Demographic, laboratory, clinical, and echocardiographic data from the electronic medical record (2000-2015) were compared between Blacks and Whites. RESULTS: Data from 369 subjects (52% Whites and 48% Blacks) were included in our analysis. No significant differences related to timely admission, IVIG treatment, or coronary artery (CA) abnormalities during hospitalization were observed. Blacks showed lower IVIG response rates than Whites for patients administered IVIG within 10 days of fever onset (86.6% vs 95.6%; P = .007). Blacks received more ancillary drugs (9.6% vs 2.6%; P = .003), and endured longer hospitalizations (mean, 5 ± 3.9 days vs 3.4 ± 2.2 days; P = .001). Blacks presented with higher C-reactive protein level and erythrocyte sedimentation rate and lower hemoglobin, albumin, and sodium levels. Blacks had a higher proportion of persistent CA abnormalities than Whites at second follow-up echocardiogram (14.5% vs 6.3%; P = .03), and at third follow-up echocardiogram (21.2% vs 6.9%; P = .01). CONCLUSIONS: Compared with White children, Black children with Kawasaki disease had higher IVIG refractory prevalence, more severe inflammation, more ancillary treatments, and longer hospitalizations. Despite no racial differences in time to diagnosis or initial treatment, there was greater CA abnormality persistence among Black children at follow-up.

Temporal Clusters of Kawasaki Disease Cases Share Distinct Phenotypes That Suggest Response to Diverse Triggers.

OBJECTIVE: To test the hypothesis that cases of Kawasaki disease within a temporal cluster have a similar pattern of host response that is distinct from cases of Kawasaki disease in different observed clusters and randomly constructed clusters. STUDY DESIGN: We designed a case-control study to analyze 47 clusters derived from 1332 patients with Kawasaki disease over a 17-year period (2002-2019) from a single clinical site and compared the cluster characteristics with those of 2 control groups of synthetic Kawasaki disease clusters. We defined a "true" Kawasaki disease cluster as at least 5 patients within a 7-day moving window. The observed and synthetic Kawasaki disease clusters were compared with respect to demographic and clinical characteristics and median values for standard laboratory data using univariate analysis and a multivariate, rotated empirical orthogonal function analysis. RESULTS: In a univariate analysis, the median values for age, coronary artery z-score, white blood cell count, erythrocyte sedimentation rate, C-reactive protein, and age-adjusted hemoglobin for several of the true Kawasaki disease clusters exceeded the 95th percentile for the 2 synthetic clusters. REOF analyses revealed distinct patterns of demographic and clinical measures within clusters. CONCLUSIONS: Cases of Kawasaki disease within a cluster were more similar with respect to demographic and clinical features and levels of inflammation than would be expected by chance. These observations suggest that different triggers and/or different intensities of exposures result in clusters of cases of Kawasaki disease that share a similar response pattern. Analyzing cases within clusters or cases who share demographic and clinical features may lead to new insights into the etiology of Kawasaki disease.