Características del síndrome inflamatorio multisistémico asociado a COVID-19 en niños atendidos en un hospital peruano, 2020-2022 / Characteristics of COVID-19-associated multisystemic inflammatory syndrome in children treated in a Peruvian hospital, 2020-2022

RESUMEN El propósito del estudio fue describir las características del síndrome inflamatorio multisistémico asociado a COVID-19 (SIM-C) en los primeros tres años de pandemia en niños de un hospital pediátrico del Perú. Se realizó un estudio observacional, descriptivo con datos de 73 pacientes y se describieron las características clínicas, laboratoriales, tratamiento y complicaciones según la ola de la pandemia y si tuvieron shock. La mediana de edad fue 6 años, las manifestaciones gastrointestinales y mucocutáneas fueron frecuentes en las tres olas. El fenotipo similar a enfermedad de Kawasaki se presentó en 34 (46,6%) pacientes y 21 (28,8%) pacientes desarrollaron shock. El tratamiento más usado fue la inmunoglobulina (95,9%), ácido acetil salicílico (94,5%) y corticoide (86,3%). Cinco (7%) pacientes tuvieron aneurisma coronario y 17 (23,3%) ingresaron a la unidad de cuidados intensivos (UCI). Los pacientes con shock tuvieron mayor alteración laboratorial y necesidad de ventilación mecánica. En conclusión, el SIM-C ha disminuido en los primeros tres años de pandemia posiblemente por la vacunación de COVID-19 en niños.

ABSTRACT This study aimed to describe the characteristics of multisystemic inflammatory syndrome associated with COVID-19 (MIS-C) in the first three years of the pandemic in children in a pediatric hospital in Peru. We conducted an observational, descriptive study with data from 73 patients and described the clinical and laboratory characteristics, treatment and complications according to the wave of the pandemic and whether they had shock. The median age was 6 years, gastrointestinal and mucocutaneous manifestations were frequent in the three waves. Kawasaki disease-like phenotype was present in 34 (46.6%) patients and 21 (28.8%) patients developed shock. The most commonly used treatment was immunoglobulin (95.9%), followed by acetylsalicylic acid (94.5%) and corticosteroid (86.3%). Five (7%) patients had coronary aneurysm and 17 (23.3%) were admitted to the intensive care unit (ICU). Patients with shock had greater laboratorial alteration and need for mechanical ventilation. In conclusion, MIS-C has decreased in the first three years of the pandemic, possibly due to COVID-19 vaccination in children.

A prediction model for coronary artery abnormalities in children with Kawasaki disease older than 5 years

Abstract Objective: Reliably prediction models for coronary artery abnormalities (CAA) in children aged > 5 years with Kawasaki disease (KD) are still lacking. This study aimed to develop a nomogram model for predicting CAA at 4 to 8 weeks of illness in children with KD older than 5 years. Methods: A total of 644 eligible children were randomly assigned to a training cohort (n = 450) and a validation cohort (n = 194). The least absolute shrinkage and selection operator (LASSO) analysis was used for optimal predictors selection, and multivariate logistic regression was used to develop a nomogram model based on the selected predictors. Area under the receiver operating characteristic curve (AUC), calibration curves, Hosmer-Lemeshow test, Brier score, and decision curve analysis (DCA) were used to assess model performance. Results: Neutrophil to lymphocyte ratio, intravenous immunoglobulin resistance, and maximum baseline z-score ≥ 2.5 were identified by LASSO as significant predictors. The model incorporating these variables showed good discrimination and calibration capacities in both training and validation cohorts. The AUC of the training cohort and validation cohort were 0.854 and 0.850, respectively. The DCA confirmed the clinical usefulness of the nomogram model. Conclusions: A novel nomogram model was established to accurately assess the risk of CAA at 4-8 weeks of onset among KD children older than 5 years, which may aid clinical decisionmaking.

Study on the influencing factors of coronary artery lesions in patients with Kawasaki disease / 重庆医学

Objective To analyze the influencing factors of coronary artery lesions in patients with Ka-wasaki disease(KD)in Xinjiang.Methods A total of 1 500 KD patients who were treated in the First Affilia-ted Hospital of Xinjiang Medical University from December 2020 to December 2022 were selected as the re-search objects.According to whether coronary artery disease occurred,they were divided into two groups.886 patients with coronary artery lesions were included in the experimental group,and 614 patients without coro-nary artery lesions were included in the control group.Univariate and multivariate logistic regression analysis was used to analyze the risk factors of coronary artery lesions.Results The results of logistic regression anal-ysis showed that the duration of fever(＞10 d),high Gensini score,high level of N-terminal pro-brain natri-uretic peptide(NT-proBNP),C-reactive protein(CRP),total cholesterol(TC),low-density lipoprotein cho-lesterol(LDL-C)and triglycerides(TG)may be risk factors for coronary artery lesions(P＜0.05).Conclusion In clinical diagnosis and treatment,targeted intervention should be given as early as possible to prevent the occurrence of coronary artery lesions.

Study on the expression levels and clinical significance of serum GSDMD and ACE2 in children with Kawasaki disease / 国际检验医学杂志

Objective To explore the expression levels and clinical significance of serum Gasdermin D(GS-DMD)and angiotensin converting enzyme 2(ACE2)in children with Kawasaki disease(KD).Methods A to-tal of 90 children with KD treated in the hospital from January 2020 to January 2022 were collected as KD group.According to whether the children with KD had coronary artery lesions(CAL),the KD group was di-vided into CAL group(32 cases)and non-CAL group(58 cases),and 50 children with fever due to acute re-spiratory infection admitted to the hospital during the same period were selected as fever control group.An-other 50 children with oblique inguinal hernia who underwent elective surgery in the same period were selected as the control group.Serum GSDMD and ACE2 levels were detected by enzyme-linked immunosorbent assay.The correlation between serum GSDMD,ACE2 and clinical indicators was analyzed by Pearson correlation.Multivariate Logisitic regression was used to analyze the influencing factors of CAL occurrence in KD pa-tients.The diagnostic value of serum GSDMD and ACE2 for CAL in children with KD was analyzed by receiv-er operating characteristic(ROC)curve.Results The serum GSDMD and ACE2 levels in KD group were higher than those in fever control group and control group,and the differences were statistically significant(all P＜0.05).Compared with the non-CAL group,the fever duration,gamma globulin treatment time,erythro-cyte sedimentation rate,platelet count,C reactive protein,GSDMD and ACE2 levels of KD children in the CAL group were significantly higher,while the blood sodium and albumin were significantly lower,with statistical significance(all P＜0.05).The results of Pearson correlation analysis showed that serum GSDMD and ACE2 levels in KD group were positively correlated with fever duration,gamma globulin treatment time,erythrocyte sedimentation rate,platelet count and C reactive protein(all P＜0.05),and negatively correlated with blood sodium and albumin(all P＜0.05).Multivariate Logistic regression analysis showed that the increase of ser-um GSDMD and ACE2 was an independent risk factor for the development of CAL in KD children.ROC curve analysis results showed that the area under the curve(AUC)and 95％CI of the combined detection of serum GSDMD and ACE2 for CAL in KD children were 0.918(0.868-0.949).The AUC and 95％CI of serum GS-DMD and ACE2 were significantly higher than that of serum GSDMD and ACE2[0.838(0.789-0.887)and 0.865(0.811-0.912),respectively],and the differences were statistically significant(Z=5.116,4.217,all P＜0.05).Conclusion The combined detection of serum GSDMD and ACE2 has high diagnostic value for CAL in children with KD.

Clinical analysis of aortographic manifestations in 30 child patients with Kawasaki disease / 介入放射学杂志

Objective To summarize the aortographic features of child patients with Kawasaki disease(KD)complicated by coronary artery lesion(CAL)who were treated at the Wuhan Children's Hospital of China.Methods The clinical data of 30 child patients with early recovery stage KD complicated by CAL,who received aortic angiography examination at Wuhan Children's Hospital of China between June 2016 and June 2023,were retrospectively analyzed.Preoperative echocardiography was adopted to evaluate the severity of CAL,and aortic angiography under laryngeal mask general anesthesia was performed.Results Successful aortic angiography examination was accomplished in all the 30 child patients with KD complicated by CAL.After angiography,the CAL risk classification results showed that grade I was seen in 5,grade Ⅱ in 7,grade Ⅲ in 12,grade Ⅲ in 4,and grade V in 2 child patients.Of all child patients,old coronary thrombosis was found in one(grade Ⅲb),and coronary artery stenosis with collateral circulation formation in one(grade Ⅴ).The CAL was located at the proximal segment(n=15),middle segment(n=3)and distal segment(n=2)of the right coronary artery,at the left main coronary artery(n=12),and at the proximal segment of left coronary artery anterior descending branch(n=9).Electrocardiography showed sinus rhythm(n=17),sinus arrhythmia(n=4),right bundle branch block(n=4),atrial premature beat(n=1),ventricular premature beat(n=2),T-wave change(n=1),and Q-wave change(n=1,coronary lesion grade V,echocardiography suggested left heart enlargement).No cardiac enlargement,cardiac functional insufficiency,or valvular regurgitation signs were observed in the remaining child patients.Conclusion It is clinically safe and feasible to perform aortic angiography examination in child patients with early recovery stage KD complicated by CAL.In this series of child patients with KD,CAL mainly occurs at the proximal segment of coronary artery,especially at the proximal segment of right coronary artery as well as at the left main artery and left anterior descending branch.

The value of the ratio of heparin binding protein to albumin in evaluating the state of shock syndrome in Kawasaki disease / 中华急诊医学杂志

Objective:To investigate the value of the ratio of heparin binding protein (HBP) to albumin (ALB) in diagnosis and predict the severity of Kawasaki disease shock syndrome (KDSS).Methods:This study was a retrospective study. Pediatric patients with Kawasaki disease (KD) admitted to the Children's Intensive Care Unit and department of Pediatric Emergency Center of Hunan Children's Hospital from January 2019 to May 2022 were enrolled. The HBP/ALB ratio was calculated according to HBP and serum ALB. The children were divided into three groups (low, medium, and high ratio groups) according to the median and upper and lower quartiles of the HBP/ALB ratio. The differences of each index among the three groups were compared. The receiver operating characteristic curves were drawn to evaluate the clinical value of the HBP/ALB ratio in diagnosis of KDSS and the severity of the disease.Results:A total of 111 cases were included in this study, including 28 cases in the low ratio group, 56 cases in the medium ratio group, and 27 cases in the high ratio group. There were 24 cases with coronary artery damage, 87 cases without coronary artery damage, 27 cases with abnormal ECG findings, and 17 children with KDSS (including 5 cases in the medium ratio group, and 12 cases in the high ratio group). The incidence of KDSS, coronary involvement, and abnormal electrocardiogram proportions in the high ratio group were significantly higher than those in the other two groups. Compared with low and medium ratio groups, the levels of cardiac troponin I, N-terminal pro-brain natriuretic peptide, lactate, stroke output variation, trends in thoracic fluid content, white blood cell count, C-reactive protein, procalcitonin, and D-dimer levels were higher in the high ratio group, while ALB and blood sodium levels were lower in the high ratio group (all P<0.05). There was no significant difference in above indicators between the low and medium ratio groups (all P>0.05). The HBP/ALB ratio had a higher area under the curve, sensitivity, and specificity (0.942, 0.882, and 0.883, respectively) in predicting KDSS compared to HBP alone (0.776, 0.842, and 0.670, respectively). Conclusion:The HBP/ALB ratio could reflect the severity of children with KD and has certain clinical value for prognostic evaluation.

Application of Non-Contrast-Enhanced Whole-Heart Coronary Magnetic Resonance Angiography in Coronary Artery Lesions of Kawasaki Disease in Children / 中国医学影像学杂志

Purpose To demonstrate the advantages of non-contrast-enhanced whole-heart coronary magnetic resonance angiography(NCE-CMRA)in evaluating coronary arteries by comparing ultrasonography(US),and to explore the clinical value of NCE-CMRA in the diagnosis of coronary artery lesions of Kawasaki disease(KD)in children.Materials and Methods NCE-CMRA and US imaging data of 41 children with KD from June 2017 to June 2021 who were diagnosed clinically in Lanzhou University Second Hospital were analyzed retrospectively.The display ability of US and NCE-CMRA in coronary arteries were compared.At the same time,the imaging characteristics of NCE-CMRA were analyzed,and the imaging characteristics such as the range and degree of coronary artery lesions displayed by NCE-CMRA were summarized.Results The overall segment display rate of NCE-CMRA in 41 children with KD was 75.6%;the overall segment display rate of US was 46.3%,with statistical difference between the two techniques(χ2=59.04,P<0.001).Regarding the display of the middle and distal segments of coronary arteries,NCE-CMRA had a clear imaging advantage over US(χ2=57.98 and 161.47,P<0.001).In all cases,25 patients(200 segments)had coronary artery lesions,and 94 segments of coronary artery showed different degrees of dilatation,including 8 segments(8.6%)of giant coronary artery aneurysm,35 segments(37.2%)of medium coronary artery aneurysm,and 51 segments(54.2%)of small coronary artery aneurysm or coronary artery dilation.Conclusion NCE-CMRA technology can objectively and accurately display coronary artery in children,and it can specifically evaluate the degree of damage caused by KD.It has important clinical significance in the diagnosis and evaluation of coronary artery lesions in children with KD.

The value of neutrophil lymphocyte ratio and systemic immune inflammatory index for diagnosis of coronary artery lesions in patients with Kawasaki disease / 中华全科医师杂志

Objective:To investigate the value of neutrophil lymphocyte ratio (NLR) and systemic immune inflammatory index (SII) for the diagnosis of coronary artery lesions in patients with acute phase of Kawasaki disease (KD).Methods:It was a cross-sectional study. Children with acute Kawasaki disease admitted to Linyi Central Hospital from January 2018 to December 2021 were enrolled and divided into the Kawasaki disease complicated with coronary artery disease group (KD-CAL group) and the Kawasaki disease without coronary artery disease group (KD-NCAL group). The demographic data, blood routine and C-reactive protein (CRP) test results before intravenous injection of γ globulin were collected, and NLR and SII were calculated. Multivariate logistic regression model was used to analyze the risk factors of coronary artery disease in children with Kawasaki disease. Receiver operating characteristic (ROC) curve was used to analyze the diagnostic value of NLR and SII for coronary artery lesions in children with Kawasaki disease.Results:A total of 109 children with acute phase of KD aged 1 month to 9 years were enrolled, including 66 boys (60.6%). There were no significant differences in age and gender composition between the KD-CAL group ( n=17) and the KD-NCAL group ( n=92) (all P>0.05). Compared with the KD-NCAL group, the NLR, SII and CRP levels in the KD-CAL group were higher (all P<0.05). Multivariate logistic regression analysis showed that NLR and SII were independent risk factors of coronary artery disease in children with Kawasaki disease (NLR, OR=1.265, 95% CI:1.066-1.502, P=0.007; SII, OR=1.001, 95% CI:1.000-1.002, P=0.015). ROC curve results showed that thearea under the curve ( AUC) of NLR in diagnosis of coronary artery lesions was 0.812 (95% CI:0.703-0.921, P<0.05), taking 5.4 as cutoff value, the sensitivity was 73.9% and specificity was 76.5%; the AUC of SII in diagnosis of coronary artery lesions was 0.830 (95% CI:0.741-0.919, P<0.05), taking 1 623 as the cut-off value, the sensitivity was 66.3% and specificity was 88.2%; the AUC of the combination of NLR and SII was 0.875(95% CI:0.790-0.959, P<0.05), the sensitivity and specificity were 85.9% and 76.5%, respectively. Conclusion:NLR and SII have certain diagnostic value for coronary artery disease in children with acute Kawasaki disease, and the combination of NLR and SII has better value than single index.

Multisystem inflammatory syndrome in children related with COVID-19 infection / 中国免疫学杂志

Multisystem inflammatory syndrome in children is a disease related with severe acute respiratory syndrome corona-virus 2 infection,which can involve multiple system damage.Most cases included features of shock,cardiac dysfunction,gastrointestinal symptoms,significantly elevated markers of inflammation and cardiac damage,and positive test results for SARS-CoV-2 by serology.Although its clinical features overlap with Kawasaki disease,it is more likely to occur in older children and adolescents,and most of them need to be treated with intensive care and a variety of immunomodulators.At present,the pathogenesis and long-term prognosis of the disease need to be further studied.

Parapharyngeal and retropharyngeal infections in children: Kawasaki disease needs vigilance

Abstract Objective Kawasaki Disease (KD) may mimic Parapharyngeal (PPI) and Retropharyngeal Infections (RPI), leading to misdiagnosis as Deep Neck Infections (DNIs). The treatment plans for the two diseases are different, and delayed treatment can lead to serious complications. Therefore, prompt diagnosis and management are necessary. This study was performed to evaluate the clinical features of KD mimicking DNIs and explore the treatment options. Methods Children with cellulitis or abscess in parapharyngeal or retropharyngeal space in neck CT were included in this study. The medical records of enrolled children were retrospectively reviewed. Results In total, 56 children were diagnosed with PPI or/and RPI. Twenty-two (39.3%) participants were eventually diagnosed with KD, and 34 (60.7%) were diagnosed with DNIs. Compared with the DNIs group, the KD group had a higher body temperature (p = 0.007), and higher levels of AST (p = 0.040), ALT (p = 0.027), and ESR (p = 0.030). Deep cervical cellulitis (p = 0.005) were more common in the KD group. However, deep neck abscess often occurred in the DNIs group (p = 0.002), with parapharyngeal abscess being the most common type of abscess (p = 0.004). The KD mimicking DNIs cases did not respond to antibiotic treatment, but symptoms significantly improved after the use of Immunoglobulin (IVIG) and aspirin. Conclusion Children with KD may exhibit retropharyngeal or parapharyngeal inflammation in the early stages. KD should be considered a differential diagnosis for children with DNIs, high fever, and no response to antibiotic therapy. Surgery in KD mimicking deep neck abscess requires caution. Level of Evidence I.

Immunoglobulin-resistant Kawasaki disease / Enfermedad de Kawasaki refractaria a tratamiento con inmunoglobulina

Abstract Background: Kawasaki disease is a systemic vasculitis that affects small and medium-sized vessels, primarily the coronary arteries. First-line treatment includes intravenous immunoglobulin (IVIG) and acetylsalicylic acid; however, 20% do not respond adequately despite treatment. We describe a case treated with etanercept after initial IVIG failure, showing a good response. Case report: A 5-year-old female was diagnosed with classic Kawasaki disease. Echocardiography and angiotomography revealed giant and fusiform aneurysms in the coronary arteries. A first dose of IVIG therapy was administered without improvement; after the second dose, the fever persisted, so etanercept was administered, and the fever subsided. There were no new lesions in medium-caliber vessels and the previously identified coronary lesions did not progress. Conclusions: The use of etanercept in Kawasaki disease has demonstrated a clinically favorable response. Controlled clinical trials of this drug are needed to establish it as a formal therapy in cases of initial IVIG failure.

Resumen Introducción: La enfermedad de Kawasaki es una vasculitis sistémica que afecta los vasos de pequeño y mediano calibre con predominio de las arterias coronarias. El tratamiento de primera línea incluye inmunoglobulina intravenosa (IGIV) y ácido acetilsalicílico; a pesar del tratamiento, el 20% de los pacientes no responden adecuadamente. Se presenta un caso tratado con etanercept debido a la falla inicial a IGIV, con buena respuesta. Caso clínico: Se trata de una paciente de 5 años de edad, a quien se diagnosticó con enfermedad de Kawasaki clásica. En ecocardiografía y angiotomografía se evidenciaron aneurismas gigantes y fusiformes en las coronarias. Se administró una primera dosis con IGIV, sin mejoría; después de la segunda dosis, la paciente persistió con fiebre, por lo que se administró etanercept, tras lo cual esta cesó. No aparecieron nuevas lesiones en vasos de mediano calibre y las lesiones coronarias previas no progresaron. Conclusiones: Con el uso de etanercept se presentó una respuesta favorable clínicamente en la enfermedad de Kawasaki. Se requieren ensayos clínicos controlados con este fármaco para establecerlo como terapia formal en los casos de falla inicial a IGIV.

Multisystem Inflammatory Syndrome in Children (MIS-C) Associated With COVID-19 – Single-Center Experience

Objectives: To describe the clinical presentation, phenotype and outcome of multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 (COVID-19) from a tertiary care center in southern India. Methods: 257 children fulfilling the inclusion criteria of MIS-C were prospectively enrolled from June, 2020 to March, 2022. Results: Median (range) age at presentation was 6 year (35 day to 12 years). Presenting features were fever (98%), vomiting (75.8%), red eyes (63%), rashes (49%), pain abdomen (49%), shock (45.9%), lymphopenia (73%, thrombocytopenia (58.3%) and anemia (45%). 103 (39.7%) children required intensive care admission. Shock phenotype, Kawasaki-like phenotype and no specific phenotype were diagnosed in 45.9%, 44.4%, and 36.6% children, respectively. Left ventricular dysfunction (30.3%), acute kidney injury (13%), acute liver failure (17.4%), and hemophagolymphohistiocytosis (HLH) (13.6%) were the major system involvement in MIS-C. Mitral regurgitation (P=0.029), hyperechogenic coronaries (P=0.006), left ventricular dysfunction (P=0.001) and low ejection fraction (P=0.007) were significantly associated with shock. Overall mortality was 11.7%. Conclusions: Kawasaki-like and shock-like presentation were common in MIS-C. Coronary abnormalities were seen in 118 (45.9%) children. Children with acute kidney injury, HLH, need for mechanical ventilation, and echocardiogram evidence of mitral regurgitation in MIS-C have a poor outcome.

Therapeutic Window of Intravenous Immunoglobulin (IVIG) and its correlation with IVIG-resistant in Kawasaki Disease: a retrospective study

Abstract Objective: To investigate the optimal timing of initial intravenous immunoglobulin (IVIG) treatment in Kawasaki disease (KD) patients. Methods: KD patients were classified as the early group (day 1-4), conventional group (day 5-7), conventional group (day 8-10), and late group (after day 10). Differences among the groups were analyzed by ANOVA and Chi-square analysis. Predictors of IVIG resistance and the optimal cut-off value were determined by multiple logistic regression analyses and receiver operating characteristic (ROC) curve analysis. Results: There were no significant differences in IVIG resistance among the 4 groups (p = 0.335). The sensitivity analysis also confirmed no difference in the IVIG resistance between those who started the initial IVIG ≤ day 7 of illness and those who received IVIG >day 7 of illness (p = 0.761). In addition, patients who received IVIG administration more than 7 days from the onset had a higher proportion of coronary artery abnormalities (p = 0.034) and longer length of hospitalization (p = 0.033) than those who started IVIG administration less than 7 days. The optimal cut-off value of initial IVIG administration time for predicting IVIG resistance was >7 days, with a sensitivity of 75.25% and specificity of 82.41%. Conclusions: IVIG therapy within 7 days of illness is found to be more effective for reducing the risk of coronary artery abnormalities than those who received IVIG >day 7 of illness. IVIG treatment within the 7 days of illness seems to be the optimal therapeutic window of IVIG. However, further prospective studies with long-term follow-up are required.

Características, estacionalidad y tendencia de las hospitalizaciones por enfermedad de Kawasaki en niños de Perú, 2015-2019

Introducción: Existen limitados reportes epidemiológicos de la enfermedad de Kawasaki (EK) en Latinoamérica. Objetivo: Describir las características, estacionalidad y tendencia de las hospitalizaciones por EK en niños. Métodos: Se realizó un estudio descriptivo de análisis secundario con datos de hospitalizaciones por EK de instituciones de salud de Perú en el periodo 2015-2019. Resultados: 297 hospitalizaciones fueron registradas. El 59,9% fueron varones. El 82,2% fue menor de cinco años y el 16,2% menor de un año. Los departamentos con más hospitalizaciones fueron Lima, Lambayeque y Piura con 202, 21 y 17 casos. Las estaciones con mayor registro fueron invierno y primavera. La incidencia de EK se incrementó de 0,52 a 2,28 casos/100 000 niños menores de cinco años entre el 2015 al 2019. Conclusiones: Hubo un incremento de las hospitalizaciones por EK, este fue más frecuente en niños menores de cinco años, varones y se presentó en todas las estaciones.

Introduction: There are limited epidemiological reports of Kawasaki disease (KD) in Latin America. Objetive: To describe the characteristics, seasonality, and trend of hospitalizations for KD in children. Methods: Descriptive study of secondary analysis was performed with data on hospitalizations due to KD from healthcare institutions from Peru, 2015-2019. Results: Two hundred ninety-seven hospitalizations were registered. 59.9% were male. 82.2% were under five years old, and 16.2% were under one year old. The departments with more hospitalizations were Lima (202 cases), Lambayeque (21 cases), and Piura (17 cases). The seasons of the year with more hospitalizations were winter and spring. The incidence of KD increased from 0.52 to 2.28 cases per 100,000 children under five years old between 2015 and 2019. Conclusions: There was an increase in hospitalizations due to KD, this was more frequent in children under five years of age and males, and it occurred in all seasons.

Características clínicas y evolución de los pacientes con enfermedad de Kawasaki en un hospital general / Clinical characteristics and course of patients with Kawasaki disease at a general hospital

La enfermedad de Kawasaki (EK) es la principal causa de cardiopatía adquirida en menores de cinco años. Nuestro objetivo fue conocer las características clínicas, el compromiso coronario y la evolución de pacientes atendidos en nuestra institución. Se revisó una serie de casos desde 2001 hasta 2018. Se incluyeron 63 pacientes, 58 % varones; la mediana de edad fue 2,6 años. La mediana de días de fiebre al diagnóstico fue 5,5 días. El 33 % presentó la forma incompleta y se detectó compromiso coronario en el 20 %. El 60 % de los pacientes con afectación coronaria presentaron EK incompleta versus el 28 % de presentación incompleta en los pacientes sin compromiso coronario (p 0,06). No se observaron diferencias en datos de laboratorio entre los grupos según el compromiso coronario. En conclusión, 33 % presentó EK incompleta y el 20 %, afectación coronaria. Hubo una tendencia de mayor riesgo para daño coronario en la forma incompleta.

Kawasaki disease (KD) is considered the leading cause of acquired heart disease in children younger than 5 years. Our objective was to know the clinical characteristics, coronary involvement, and course of patients seen at our facility. A case series from 2001 to 2018 was reviewed. Sixty-three patients were included; their median age was 2.6 years; 58% were males. The median duration of fever at the time of diagnosis was 5.5 days. The incomplete form was observed in 33% and coronary involvement, in 20%. Among patients with coronary involvement, 60% had incomplete KD versus 28% among those without coronary involvement (p: 0.06). No differences were observed between groups in laboratory data based on coronary involvement. To conclude, 33% had incomplete KD and 20%, coronary involvement. There was a trend to a higher risk for coronary artery damage in the incomplete form of KD.

Clinical, biochemical and echocardiographic characteristics in children with multisystem inflammatory syndrome associated with coronavirus disease: a tertiary care experience

Background: We describe the clinical, biochemical and echocardiographic characteristics in children diagnosed with multisystem inflammatory syndrome (MISC) temporally associated with severe acute respiratory syndrome coronavirus 2 (SARS-Co-V-2), admitted to a tertiary care hospital in Goa, India.Methods: This was a retrospective observational study conducted at Goa Medical College, Goa, India. Parameters including demographics, clinical features, laboratory markers, medications and outcomes were obtained from patient hospital records; analyzed for patients treated for MISC (as per WHO case definition) for a period of 11 months from 01 July 2020 to 31 July 2021.Results: 30 patients (18 males) with median age of 7 years (IQR: 2 months – 12 years) were included. The proportion of children with gastrointestinal symptoms was 20 (64.5%), shock was a presenting feature in 15 (48%) and coronary ectasia and/or low ejection fraction was seen in 12 children (38.7%).Conclusions: A high index of suspicion for the diagnosis of MIS-C in children presenting with fever and Kawasaki disease like symptoms or signs of shock is recommended; since early aggressive therapy with Intravenous Immunoglobulins and methylprednisolone can have favourable outcomes.

Clinical application of plasma exchange combined with continuous veno-venous hemofiltration dialysis in children with refractory Kawasaki disease shock syndrome / 中国当代儿科杂志

OBJECTIVES@#To study the role of plasma exchange combined with continuous blood purification in the treatment of refractory Kawasaki disease shock syndrome (KDSS).@*METHODS@#A total of 35 children with KDSS who were hospitalized in the Department of Pediatric Intensive Care Unit, Hunan Children's Hospital, from January 2019 to August 2022 were included as subjects. According to whether plasma exchange combined with continuous veno-venous hemofiltration dialysis was performed, they were divided into a purification group with 12 patients and a conventional group with 23 patients. The two groups were compared in terms of clinical data, laboratory markers, and prognosis.@*RESULTS@#Compared with the conventional group, the purification group had significantly shorter time to recovery from shock and length of hospital stay in the pediatric intensive care unit, as well as a significantly lower number of organs involved during the course of the disease (P<0.05). After treatment, the purification group had significant reductions in the levels of interleukin-6, tumor necrosis factor-α, heparin-binding protein, and brain natriuretic peptide (P<0.05), while the conventional group had significant increases in these indices after treatment (P<0.05). After treatment, the children in the purification group tended to have reductions in stroke volume variation, thoracic fluid content, and systemic vascular resistance and an increase in cardiac output over the time of treatment.@*CONCLUSIONS@#Plasma exchange combined with continuous veno-venous hemofiltration dialysis for the treatment of KDSS can alleviate inflammation, maintain fluid balance inside and outside blood vessels, and shorten the course of disease, the duration of shock and the length of hospital stay in the pediatric intensive care unit.

Clinical features of Kawasaki disease complicated by macrophage activation syndrome: an analysis of 27 cases / 中国当代儿科杂志

OBJECTIVES@#To investigate the clinical manifestations and laboratory examination results of children with Kawasaki disease complicated by macrophage activation syndrome (KD-MAS), and to provide a basis for identifying early warning indicators for the early diagnosis and treatment of KD-MAS.@*METHODS@#A retrospective study was performed on 27 children with KD-MAS (KD-MAS group) and 110 children with KD (KD group) who were admitted to Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology, from January 2014 to January 2022. Clinical and laboratory data were compared between the two groups. The receiver operating characteristic (ROC) curve was used to investigate the value of laboratory markers with statistical significance in the diagnosis of KD-MAS.@*RESULTS@#Compared with the KD group, the KD-MAS group had significantly higher incidence rates of hepatomegaly, splenomegaly, incomplete KD, no response to intravenous immunoglobulin, coronary artery damage, multiple organ damage, and KD recurrence, as well as a significantly longer length of hospital stay (P<0.05). Compared with the KD group, the KD-MAS group had significantly lower levels of white blood cell count, absolute neutrophil count, hemoglobin, platelet count (PLT), erythrocyte sedimentation rate, serum albumin, serum sodium, prealbumin, and fibrinogen (FIB), a significantly lower incidence rate of non-exudative conjunctiva, and significantly higher levels of C-reactive protein, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase (LDH), and serum ferritin (SF) (P<0.05). The ROC curve analysis showed that SF, PLT, FIB, and LDH had high value in the diagnosis of KD-MAS, with areas under the curve (AUC) of 0.989, 0.966, 0.932, and 0.897, respectively (P<0.001), and optimal cut-off values of 349.95 μg/L, 159×109/L, 3.85 g/L, and 403.50 U/L, respectively. The combination of SF, PLT, FIB, and LDH had a larger AUC than PLT, FIB, and LDH alone in the diagnosis of KD-MAS (P<0.05), but there was no significant difference in the AUC between the combination of SF, PLT, FIB, and LDH and SF alone (P>0.05).@*CONCLUSIONS@#KD-MAS should be considered when children with KD have hepatosplenomegaly, no response to intravenous immunoglobulin, coronary artery damage, and KD recurrence during treatment. SF, PLT, FIB, and LDH are of high value in the diagnosis of KD-MAS, especially SF is of great significance in the diagnosis of KD-MAS.

Role and mechanism of platelet-derived growth factor BB in thrombocytosis in Kawasaki disease / 中国当代儿科杂志

OBJECTIVES@#To study the role and mechanism of platelet-derived growth factor BB (PDGF-BB) on platelet production in Kawasaki disease (KD) mice and human megakaryocytic Dami cells through in vitro and invivo experiments.@*METHODS@#ELISA was used to measure the expression of PDGF in the serum of 40 children with KD and 40 healthy children. C57BL/6 mice were used to establish a model of KD and were then randomly divided into a normal group, a KD group, and an imatinib group (30 mice in each group). Routine blood test was performed for each group, and the expression of PDGF-BB, megakaryocyte colony forming unit (CFU-MK), and the megakaryocyte marker CD41 were measured. CCK-8, flow cytometry, quantitative real-time PCR, and Western blot were used to analyze the role and mechanism of PDGF-BB in platelet production in Dami cells.@*RESULTS@#PDGF-BB was highly expressed in the serum of KD children (P<0.001). The KD group had a higher expression level of PDGF-BB in serum (P<0.05) and significant increases in the expression of CFU-MK and CD41 (P<0.001), and the imatinib group had significant reductions in the expression of CFU-MK and CD41 (P<0.001). In vitro experiments showed that PDGF-BB promoted Dami cell proliferation, platelet production, mRNA expression of PDGFR-β, and protein expression of p-Akt (P<0.05). Compared with the PDGF-BB group, the combination group (PDGF-BB 25 ng/mL + imatinib 20 μmol/L) had significantly lower levels of platelet production, mRNA expression of PDGFR-β, and protein expression of p-Akt (P<0.05).@*CONCLUSIONS@#PDGF-BB may promote megakaryocyte proliferation, differentiation, and platelet production by binding to PDGFR-β and activating the PI3K/Akt pathway, and the PDGFR-β inhibitor imatinib can reduce platelet production, which provides a new strategy for the treatment of thrombocytosis in KD.