Differences in the Clinical Characteristics of Kawasaki Disease Between Older and Younger Children (2015-2019): A Single-Center, Retrospective Study.

OBJECTIVE: The objective of this study was to investigate the differences in the clinical characteristics of Kawasaki disease between older and younger children. STUDY DESIGN: This retrospective study examined 405 children with Kawasaki disease admitted to Showa University Northern Yokohama Hospital between 2015 and 2019. RESULTS: Eligible patients were classified into the older (≥3.0 years of age, n = 169) and younger (<3.0 years of age, n = 236) groups. Skin rash was found in significantly fewer cases (112 [66.3%] vs 229 [97.0%], P < .001 in the younger group). Cervical lymphadenopathy was more common in older children (153 [90.5%] vs 165 [69.9%], P < .001) and in incomplete Kawasaki disease (3 or 4 findings) (34 [20.1%] vs 25 [10.6%], P = .0078). The diagnosis was more delayed in older children (median: 5.0 days vs 4.0 days, P = .003) than the younger group. Additionally, fever nonresponsive to a single intravenous immunoglobulin was more common, and the duration of fever was significantly longer in the older group (48 [28.4%] vs 47 [19.9%], P = .0479). CONCLUSIONS: Kawasaki disease should be suspected in children aged >3.0 years with cervical lymphadenopathy and fever, despite the absence of skin rash. Additionally, incomplete Kawasaki disease, fever unresolved by a single intravenous immunoglobulin infusion, and the tendency to delay treatment initiation are more common in children aged >3.0 years.

Recent Insights into COVID-19 in Children and Clinical Recommendations.

Pediatric coronavirus disease 2019 (COVID-19) and multisystem inflammatory syndrome in children (MIS-C) have been recognized in multiple countries globally. In this review, we provide recent insights into SARS-CoV-2 infection in children from epidemiological, clinical, and laboratory perspectives, including reports on the disease course and therapy. We highlight key features of SARS-CoV-2 infection in children, the relationship between MIS-C and Kawasaki disease, and summarize treatment guidelines for COVID-19 in children from institutional protocols from Colombia, case reports, recommendations based on expert consensus, and official statements from organizations such as the World Health Organization (WHO), United States Center for Disease Control (CDC), Colombian Association of Infectious Diseases, and the Colombian Society of Pediatrics. Finally, we discuss gaps in research with suggestions for future research on the pathogenesis underlying pediatric COVID-19.

Consenso sobre el tratamiento del síndrome inflamatorio multisistémico asociado a COVID-19 / Consensus on treatment of multisystemic inflammatory syndrome associated with COVID-19

La pandemia ocasionada por el nuevo coronavirus (SARS-CoV-2), declarada por la Organización Mundial de la Salud OMS) en marzo de 2020, afecta a un reducido número de pacientes pediátricos, quienes presentan, en su mayoría, compromiso respiratorio leve y evolución favorable. Sin embargo, en niños previamente sanos, comenzó a observarse un aumento de casos definidos como síndrome inflamatorio multisistémico (SIM-C) o similar a Kawasaki (Kawasaki-like) asociado a la enfermedad por el nuevo coronavirus (COVID-19) (KL-C) que evolucionan al shock y requieren internación en la unidad de cuidados intensivos.Los cuadros de SIM-C y los KL-C se caracterizan por fiebre, signos de inflamación, síntomas gastrointestinales y disfunción cardiovascular; las formas graves de presentación tienen mayor incidencia de hipotensión y/o shock. En el laboratorio se observan marcadores de inflamación, hipercoagulabilidad y daño miocárdico. El tratamiento farmacológico de primera línea consiste en la administración de inmunoglobulina por vía intravenosa más ácido acetilsalicílico por vía oral.Se recomienda un abordaje multidisciplinario para un diagnóstico certero y un tratamiento temprano y eficaz para disminuir la morbimortalidad.

The pandemic caused by the SARS-CoV-2 virus declared by the WHO in March 11th 2020, affects a small number of pediatric patients, who mostly present mild respiratory compromise and favorable evolution.However began to be observed in previously healthy children, an increase in cases defined as "Multisystemic Inflammatory Syndrome" (MIS-C) or "Kawasaki-like" post-COVID 19 (KL-C) that evolve to shock and require hospitalization in the Pediatric Intensive Care Unit.MIS-C and KL-C are characterized by fever; signs of inflammation, gastrointestinal symptoms, and cardiovascular dysfunction, associated with sever forms of presentation with higher incidence of hypotension and/or shock. In the laboratory, markers of inflammation, hypercoagulability and myocardial damage are observed. First-line drug treatment consists of intravenous immunoglobulin plus oral acetylsalicylic acid.A multidisciplinary approach is recommended for an accurate diagnosis and an early and effective treatment, in order to reduce morbidity and mortality.

Guide for cardiological referral for patients with paediatric multisystemic inflammatory syndrome and Kawasaki disease associated with SARS-CoV-2 / Guía para referencia cardiológica de pacientes con síndrome inflamatorio multisistémico pediátrico y enfermedad por Kawasaki asociados al SARS-CoV-2

We present an institutional guide for a referral to the specialized care center and initial management of pediatric patients infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) with severe manifestations of pediatric inflammatory multisystemic syndrome or symptoms similar to Kawasaki syndrome, and who must have a multidisciplinary approach to ensure adequate treatment and safety for the team of Health.

Presentamos una guía para la referencia al centro de atención especializada y el manejo inicial de pacientes pediátricos infectados por el coronavirus 2 del síndrome respiratorio agudo grave (SARS-CoV-2) con manifestaciones graves del síndrome multisistémico inflamatorio pediátrico o síntomas semejantes al síndrome de Kawasaki y que deben tener un abordaje multidisciplinario para garantizar un adecuado tratamiento y la mayor seguridad para el equipo de salud.

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.

Update on SARS-CoV-2 infection in children.

Despite the worldwide spread of SARS-CoV-2 infection (COVID-19), knowledge of the different clinical presentations, ways of transmission, severity and prognosis in children and adolescents is limited. An increasing number of reports describe some of these characteristics in this age range. A non-systematic review was undertaken using MEDLINE (PubMed), LILACS (VHL), Scopus, Web of Science, Cochrane and CAPES Portal databases from 1 January until 30 September 2020 [103] with the search terms SARS-CoV-2, COVID-19, child, children, youth, adolescent and newborn to identify the more recent clinical aspects of SARS-CoV-2 infection in children. In general, SARS-CoV-2 infection in children tends to be asymptomatic or to have mild or moderate signs, and most young ones are infected by family members. Recent reports offer new insights into the disease. Current evidence on SARS-CoV-2 infection in children and adolescents is presented, especially concerning the clinical presentation, imaging and uncommon severe forms of the disease, particularly the COVID-19-associated multisystem inflammatory syndrome. The impact of COVID-19 infection in the perinatal period is described in detail. Knowledge of the various clinical presentations of SARS-CoV-2 in children and adolescents allows the paediatrician to diagnose earlier, monitor warnings signs, implement treatment and, especially, establish preventive measures.Abbreviations : ACE-2, angiotensin-converting enzyme 2; ARDS, acute respiratory distress syndrome; ARF, acute rheumatic fever; CAA, coronary artery aneurysms; CK-MB, creatine kinase-MB; COVID-19, coronavirus disease-2019; HLA, specific human leucocyte antigen; IPC, infection prevention and control; IVIG, intravenous immunoglobulin; KD, Kawasaki disease; MIS-C, COVID-19-associated multisystem inflammatory syndrome; RNA, ribonucleic acid; RT-PCR, reserve transcription-polymerase chain reaction; SARS-CoV-2, severe acute respiratory syndrome coronavirus-2; TCT, thoracic computed tomography; TSS, toxic shock syndrome; WHO, World Health Organization.

Multiple Emergency Department Visits for a Diagnosis of Kawasaki Disease: An Examination of Risk Factors and Outcomes.

OBJECTIVES: To determine predictors of >1 emergency department (ED) visit for a Kawasaki disease diagnosis in a quaternary care pediatric hospital and compare outcomes between patients with 1 vs >1 visit for Kawasaki disease diagnosis. STUDY DESIGN: Medical records of patients evaluated for Kawasaki disease between January 2006 and August 2018 at Boston Children's Hospital were abstracted for demographic and clinical data. Predictors of >1 visit were explored using logistic regression and classification and regression tree analysis. RESULTS: Of 530 patients diagnosed with Kawasaki disease, 117 (22%) required multiple ED visits for Kawasaki disease diagnosis. Multivariable regression and classification and regression tree analysis identified ≤2 Kawasaki disease criteria (OR 33.9; 95% CI 18.1-63.6), <3 days of fever at the first visit (OR 3.47; 95% CI 1.77-6.84), and non-White race (OR 2.15; 95% CI 1.18-3.95) as predictors of >1 visit. There were no significant differences in duration of hospitalization, day of illness at initial Kawasaki disease treatment, intravenous immunoglobulin resistance, need for adjunctive therapies, or coronary artery outcomes between patients diagnosed with Kawasaki disease at initial visit vs subsequent visits. CONCLUSIONS: Incomplete Kawasaki disease criteria, fewer days of fever, and non-White race were significant predictors of multiple ED visits for Kawasaki disease diagnosis in this single institution study. Our findings underscore the importance of maintaining a high index of suspicion for Kawasaki disease in patients with <4 Kawasaki disease criteria. Further research is needed to determine causes for increased healthcare use in non-White patients to receive a Kawasaki disease diagnosis.

CLINICAL-EPIDEMIOLOGICAL RELATION BETWEEN SARS-COV-2 AND KAWASAKI DISEASE: AN INTEGRATIVE LITERATURE.

OBJECTIVE: To analyze the current scientific literature to document, in an integrative review, the main findings that correlate Kawasaki disease (KD) to COVID-19. DATA SOURCES: The search was carried out in June 2020 in the following databases: Biblioteca Virtual em Saúde (BVS), periódico da CAPES and U.S National Library of Medicine (PubMed). The combination of descriptors used was [(COVID-19 OR SARS-CoV-2) AND (Kawasaki disease)], and the inclusion criteria stipulated were studies published from January 2019 to June 2020, without restriction of language or location, and available online in full. News, editorials, comments, and letters, as well as duplicates and articles that did not answer the guiding question were excluded. DATA SYNTHESIS: A total of 97 articles were identified, of which seven comprised this review. The association of KD to the new coronavirus appears to trigger a severe clinical condition of vasculitis. Different from the usual, in this inflammatory syndrome, patients are older, and prevalence is higher in children from African or Caribbean ancestry; clinical and laboratory manifestations are also atypical, with a predominance of abdominal complaints and exaggerated elevation of inflammatory markers. In addition, there was a greater report of rare complications and greater resistance to the recommended treatment for KD. CONCLUSIONS: Pediatric COVID-19 and its potential association to severe KD, still unfamiliar to health professionals, reinforces the importance of testing patients with vasculitis for the new coronavirus and the need to wage high surveillance and preparation of the health system during the current pandemic.

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.

Kawasaki Disease Shock Syndrome Versus Septic Shock: Early Differentiating Features Despite Overlapping Clinical Profiles.

OBJECTIVES: To compare the clinical features and resuscitative measures of children with Kawasaki disease shock syndrome vs septic shock. STUDY DESIGN: In this retrospective case-control study, children with Kawasaki disease shock syndrome admitted to the intensive care unit from 2007 to 2017 were identified and compared with age-matched controls with septic shock. We studied 9 children with Kawasaki disease shock syndrome and 18 children with septic shock. Clinical characteristics were abstracted and between-group differences were compared. RESULTS: Compared with septic shock controls, children with Kawasaki disease shock syndrome were less likely to have an underlying comorbid illness (1/9 [11%] vs 11/18 [61%]; P = .02) and were more likely to have at least 1 of the 5 classic diagnostic signs of Kawasaki disease at presentation (9/9 [100%] vs 0/18 [0%]; P < .001), a longer duration of illness before admission (9 days [IQR, 7-14 days] vs 3 days [IQR, 1-5 days]; P = .004), and a lower platelet count at presentation (140 [IQR 73, 167]) vs 258 [IQR, 137-334]; P = .02). Among patients who underwent echocardiography, abnormalities such as ventricular dysfunction, valvulitis, and coronary artery dilation were more common in the Kawasaki disease shock syndrome cohort (5/9 [56%] vs 0/7 [0%]; P = .03). There were no differences in volume of fluid resuscitation, vasoactive-inotropic scores, duration of inotropic therapy, or biochemical markers of illness severity (other than platelet count) between the matched groups. CONCLUSIONS: A longer duration of illness before admission, lack of any significant underlying medical comorbidities, a lower platelet count, echocardiographic abnormalities, and the presence of classic diagnostic signs of Kawasaki disease at presentation may be useful early features to differentiate Kawasaki disease shock syndrome from septic shock.

Clinical-epidemiological relation between sars-cov-2 and kawasaki disease: an integrative literature / Relação clínico-epidemiológica entre sars-cov-2 e doença de kawasaki: uma revisão integrativa da literatura

ABSTRACT Objective: To analyze the current scientific literature to document, in an integrative review, the main findings that correlate Kawasaki disease (KD) to COVID-19. Data sources: The search was carried out in June 2020 in the following databases: Biblioteca Virtual em Saúde (BVS), periódico da CAPES and U.S National Library of Medicine (PubMed). The combination of descriptors used was [(COVID-19 OR SARS-CoV-2) AND (Kawasaki disease)], and the inclusion criteria stipulated were studies published from January 2019 to June 2020, without restriction of language or location, and available online in full. News, editorials, comments, and letters, as well as duplicates and articles that did not answer the guiding question were excluded. Data synthesis: A total of 97 articles were identified, of which seven comprised this review. The association of KD to the new coronavirus appears to trigger a severe clinical condition of vasculitis. Different from the usual, in this inflammatory syndrome, patients are older, and prevalence is higher in children from African or Caribbean ancestry; clinical and laboratory manifestations are also atypical, with a predominance of abdominal complaints and exaggerated elevation of inflammatory markers. In addition, there was a greater report of rare complications and greater resistance to the recommended treatment for KD. Conclusions: Pediatric COVID-19 and its potential association to severe KD, still unfamiliar to health professionals, reinforces the importance of testing patients with vasculitis for the new coronavirus and the need to wage high surveillance and preparation of the health system during the current pandemic.

RESUMO Objetivo: Analisar a literatura científica atual a fim de documentar, por meio de revisão integrativa, os principais achados que associam a doença de Kawasaki (DK) à doença do coronavírus (COVID-19). Fonte de dados: A busca ocorreu em junho de 2020, nas bases de dados: Biblioteca Virtual em Saúde (BVS), periódico da Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES) e U.S. National Library of Medicine (PubMed). Os descritores utilizados foram [(COVID-19 OR SARS-CoV-2) AND (Kawasaki Disease)], e os critérios de inclusão estipulados compreenderam estudos publicados de janeiro de 2019 a junho de 2020, sem restrição de idioma ou localização e disponíveis online integralmente. Foram excluídos notícias, editoriais, comentários e cartas de apresentação, assim como duplicatas e artigos que não respondiam à pergunta norteadora. Síntese dos dados: Identificaram-se 97 artigos, dos quais sete compuseram esta revisão. A associação da DK com o novo coronavírus parece desencadear um quadro de vasculite grave. Diferentemente do usual, nessa síndrome inflamatória, os pacientes são mais velhos e a descendência prevalente é africana ou caribenha; as manifestações clínicas e laboratoriais também são atípicas, com predomínio de queixas abdominais e elevação exagerada de marcadores inflamatórios. Além disso, houve maior relato de complicações raras e maior resistência ao tratamento preconizado para DK. Conclusões: A COVID-19 pediátrica e sua potencial associação com uma DK grave, ainda pouco conhecida pelos profissionais da saúde, reforçam a importância da testagem de pacientes com vasculite para o novo coronavírus e a necessidade de empreender alta vigilância e preparação do sistema de saúde durante a atual pandemia.

Síndrome inflamatorio en pacientes pediátricos en el contexto de pandemia por COVID-19 / Inflammatory syndrome in pediatric patients in the context of the COVID-19 pandemic

Introducción: En el contexto de la pandemia por COVID-19, el aumento en las informaciones sobre niños gravemente afectados por una nueva forma de enfermedad similar al síndrome de Kawasaki, encendió las alarmas a nivel mundial. Objetivo: Analizar la evidencia disponible y describir la presentación, síntomas y tratamientos de la enfermedad denominada nueva Kawasaki asociada a COVID-19 en la población pediátrica. Métodos: Se efectuó una revisión sistemática de estudios publicados en buscadores científicos: PubMed, Cochrane, Science Direct, Medline; entre diciembre 2019 y septiembre 2020, que incluyeran a la población pediátrica entre 0-18 años Análisis e integración de la información: Luego de la aplicación de los criterios de inclusión y exclusión quedaron para el análisis 15 artículos, con una muestra total de 932 pacientes, que presentaron la enfermedad asociada a COVID-19. Se encuentra un porcentaje elevado de signos de gravedad como complicaciones cardiacas. En los estudios que registraron estos signos la prevalencia varía entre 60-100 por ciento de los pacientes y entre 50-100 por ciento en el shock asociado a aumento de parámetros inflamatorios. En general, el pronóstico fue favorable en la mayoría de los pacientes. Solo se presentaron 9 fallecimientos (0,97 por ciento) respecto a la muestra total. Conclusiones: El tratamiento oportuno se asocia a buen pronóstico en estos pacientes, con baja tasa de mortalidad. Por lo anteriormente expuesto es fundamental que tanto la comunidad médica como la población general estén debidamente informadas frente a esta afección y su presentación clínica(AU)

Introduction: In the context of the COVID-19 pandemic, the increase of information on children severely affected by a new form of disease similar to Kawasaki syndrome set off alarms worldwide. Objective: Analyze the available evidence and describe the presentation, symptoms and treatments of the disease called new Kawasaki associated with COVID-19 in the pediatric population. Methods: A systematic review of studies published in scientific search engines like PubMed, Cochrane, ScienceDirect, Medline was carried out, between December 2019 and September 2020, which included the pediatric population between 0-18 years. Analysis and integration of information: After the application of the inclusion and exclusion criteria, 15 articles remained for analysis, with a total sample of 932 patients, who presented the disease associated with COVID-19. A high percentage of signs of severity such as cardiac complications are found. In the studies that recorded these signs, the prevalence varies between 60-100 pencent of patients and between 50-100 percent in the shock associated with increased inflammatory parameters. In general terms, the prognosis was favorable in most of the patients. There were only 9 deaths (0.97 percent) of the total sample. Conclusions: Timely treatment is associated with a good prognosis in these patients, with a low mortality rate. Therefore, it is essential that both the medical community and the general population are properly informed about this condition and its clinical presentation(AU)

Recomendación para la sospecha, manejo y seguimiento del compromiso cardiovascular en pacientes con Síndrome Inflamatorio Multisistémico en Pediatría asociado a COVID-19 (PIMS-TC). Declaración de Posición de la Sociedad Chilena de Pediatría (SOCHIPE) y Sociedad Chilena de Cardiología y Cirugía Cardiovascular (SOCHICAR) / Recommendation for the recognition, management and follow up of cardiovascular compromise in patients with Pediatric Multisystemic Inflammatory Syndrome associated with COVID-19 (PIMS-CT). Position statement of Chilean Scientific Societies

Desde la aparición de la pandemia por SARS-CoV-2, la población pediátrica ha sido menos afectada por la enfermedad tanto en frecuencia como en severidad. Sin embargo, desde abril de este año se han reportado casos de presentación y gravedad variables, caracterizados por fenómenos inflamato rios que afectan múltiples órganos, condición denominada Síndrome Inflamatorio Multisistémico Pediátrico (PIMS). La literatura describe frecuente compromiso cardíaco, hasta en un 80%. Este se caracteriza por injuria miocárdica con significativa elevación de biomarcadores: Troponinas séricas I/T, BNP o NT-ProBNP, unido a diversos grados de disfunción ventricular, pericarditis, valvulitis y arritmias. Además, se ha evidenciado la presencia de compromiso coronario el cual puede ocurrir hasta en un 23% de los casos, en un rango que va desde dilataciones hasta aneurismas. El seguimien to cardiológico hospitalizado y ambulatorio se ha sistematizado en base a los fenotipos clínicos de presentación: injuria miocárdica (miocarditis, valvulitis, pericarditis), shock (habitualmente de tipo "vasopléjico"), manifestaciones tipo Enfermedad de Kawasaki y aquellos casos PIMS que no cumplen con la clínica de los tres precedentes. Este último grupo es el que representa el mayor desafío en el cor to, mediano y seguimiento a largo plazo. Por esta razón se requiere un equipo multidisciplinario para su manejo. Considerando la alta frecuencia del compromiso cardíaco en el PIMS y la importancia de lograr un consenso en su manejo y seguimiento, se presentan estas recomendaciones según el estado actual del conocimiento de esta patología recientemente descrita.

Since the onset of the SARS-CoV-2 pandemic, the pediatric population has been less affected by the disease both in frequency and severity. However, since April cases of variable presentation and severity characterized by inflammatory phenomena that affect multiple organs have been reported, a condition called Multisystem Inflammatory Syndrome in Children (MIS-C). The literature has reported frequent cardiac involvement, up to 80%. This is characterized by myocardial injury with a significant increase of biomarkers such as serum troponins I and T, BNP, or NT-ProBNP coupled with varying degrees of ventricular dysfunction, pericarditis, valvulitis, and arrhythmias. Coronary compromise has also been described, which can occur in up to 23% of cases, and ranges from dila tations to aneurysms. Inpatient and outpatient cardiology follow-up has been systematized based on the clinical phenotypes such as myocardial injury (myocarditis, valvulitis, pericarditis), shock (usua lly vasoplegic), Kawasaki disease-type manifestations, and those MIS-C that do not comply with the clinic of the previous three. This last group represents the main challenge in the short-, medium- and long-term follow-up, therefore, it is necessary a multidisciplinary team for managing these patients. Considering the high frequency of cardiac compromise in MIS-C, and the importance of reaching a consensus regarding its management and follow-up, we present these recommendations according to the current state of knowledge regarding this recently described pathology.

Severe clinical spectrum with high mortality in pediatric patients with COVID-19 and multisystem inflammatory syndrome.

OBJECTIVES: To assess the outcomes of pediatric patients with laboratory-confirmed coronavirus disease (COVID-19) with or without multisystem inflammatory syndrome in children (MIS-C). METHODS: This cross-sectional study included 471 samples collected from 371 patients (age<18 years) suspected of having severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The study group comprised 66/371 (18%) laboratory-confirmed pediatric COVID-19 patients: 61 (92.5%) patients tested positive on real-time reverse transcription-polymerase chain reaction tests for SARS-CoV-2, and 5 (7.5%) patients tested positive on serological tests. MIS-C was diagnosed according to the criteria of the Center for Disease Control. RESULTS: MIS-C was diagnosed in 6/66 (9%) patients. The frequencies of diarrhea, vomiting, and/or abdominal pain (67% vs. 22%, p=0.034); pediatric SARS (67% vs. 13%, p=0.008); hypoxemia (83% vs. 23%, p=0.006); and arterial hypotension (50% vs. 3%, p=0.004) were significantly higher in patients with MIS-C than in those without MIS-C. The frequencies of C-reactive protein levels >50 mg/L (83% vs. 25%, p=0.008) and D-dimer levels >1000 ng/mL (100% vs. 40%, p=0.007) and the median D-dimer, troponin T, and ferritin levels (p<0.05) were significantly higher in patients with MIS-C. The frequencies of pediatric intensive care unit admission (100% vs. 60%, p=0.003), mechanical ventilation (83% vs. 7%, p<0.001), vasoactive agent use (83% vs. 3%, p<0.001), shock (83% vs. 5%, p<0.001), cardiac abnormalities (100% vs. 2%, p<0.001), and death (67% vs. 3%, p<0.001) were also significantly higher in patients with MIS-C. Similarly, the frequencies of oxygen therapy (100% vs. 33%, p=0.003), intravenous immunoglobulin therapy (67% vs. 2%, p<0.001), aspirin therapy (50% vs. 0%, p<0.001), and current acute renal replacement therapy (50% vs. 2%, p=0.002) were also significantly higher in patients with MIS-C. Logistic regression analysis showed that the presence of MIS-C was significantly associated with gastrointestinal manifestations [odds ratio (OR)=10.98; 95%CI (95% confidence interval)=1.20-100.86; p=0.034] and hypoxemia [OR=16.85; 95%CI=1.34-211.80; p=0.029]. Further univariate analysis showed a positive association between MIS-C and death [OR=58.00; 95%CI=6.39-526.79; p<0.0001]. CONCLUSIONS: Pediatric patients with laboratory-confirmed COVID-19 with MIS-C had a severe clinical spectrum with a high mortality rate. Our study emphasizes the importance of investigating MIS-C in pediatric patients with COVID-19 presenting with gastrointestinal involvement and hypoxemia.

Epidemiology, Treatments, and Cardiac Complications in Patients with Kawasaki Disease: The Nationwide Survey in Japan, 2017-2018.

OBJECTIVE: To report the epidemiologic characteristics, treatments, and cardiac complications of Kawasaki disease, using data from the nationwide survey in Japan. STUDY DESIGN: The nationwide Kawasaki disease survey in Japan has been conducted biennially since 1970. The most recent survey was completed in 2019, obtaining information for patients who developed Kawasaki disease during 2017-2018. Survey respondents were hospitals specializing in pediatrics and those with ≥100 beds and a pediatric department throughout Japan, where patients with Kawasaki disease were eventually hospitalized. RESULTS: The survey identified 32 528 patients with Kawasaki disease, which consisted of 15 164 (46.6%) in 2017 and 17 364 (53.4%) in 2018. The highest annual incidence rate was recorded in 2018 (359 per 100 000 children aged 0-4 years). After 1982, patients with ≤4 principal Kawasaki disease signs gradually increased, resulting in 6847 (21.1%) patients diagnosed during 2017-2018. Among the 30 784 patients receiving initial intravenous immunoglobulin administration, 6061 (19.7%) did not respond. Within 30 days of Kawasaki disease onset, 9.0% of patients were diagnosed with cardiac complications, and 2.6% of patients developed cardiac sequelae after the acute illness. CONCLUSIONS: The annual number of patients developing Kawasaki disease in Japan increased from 1970 through 2018, whereas the proportion of patients with Kawasaki disease with cardiac complications decreased in the most recent 2 decades. Early diagnosis of Kawasaki disease as well as advances in initial treatments could explain these findings.

Actualización en enfermedad de kawasaki: diagnóstico, estudio y tratamiento / Kawasaki disease update: diagnosis, study and treatment

La enfermedad de Kawasaki (EK) es una vasculitis aguda de vaso mediano que afecta principalmente a niños menores de 5 años, que de no ser tratada, se asocia al desarrollo de aneurismas de las arterias coronarias en aproximadamente el 25% de los casos. Típicamente la EK se presenta con fiebre, cambios mucocutáneos y linfadenopatía. Sin embargo, EK es una enfermedad excepcional en la que las formas incompletas de la enfermedad son muy comunes y a menudo asociación sintomatologías atípicas. Éstas pueden crear un desafío diagnóstico para los tratantes y retrasar el inicio de la terapia. No existe un gold standard para el diagnóstico de EK pero hay estudios de laboratorio y hallazgos ecocardiográficos que permiten apoyar el diagnóstico en casos incompletos. El estudio con ecocardiograma debe realizarse lo más pronto posible cuando se sospecha el diagnóstico, pero no debe retrasar el inicio de tratamiento. El objetivo de la terapia en EK es disminuir el riesgo de desarrollar aneurismas de las arterias coronarias y de esta forma, la morbimortalidad asociada a dicha condición. El propósito de esta revisión es conocer las características clínicas y las posibles formas de presentación de esta patología, además del tratamiento actual.

Kawasaki disease (KD) is an acute vasculitis of the medium vessel that mainly affects children under 5 years old, which if it's not treated, is associated with the development of coronary artery aneurysms in approximately 25% of all cases. Typically, KD presents with fever, mucocutaneous changes and lymphadenopathy. However, EK is an exceptional disease in which incomplete forms of the disease are very common and atypical presentations often occur. These presentations may create a diagnostic puzzle for pediatricians and may delay the start of therapy. There is no specific study for the diagnosis of KD but there are laboratory studies and findings in ECG that support the diagnosis in atypical cases. The echocardiogram study should be performed as soon as possible when the diagnosis is suspected, but should not delay the treatment. The goal of treatment in KD is to reduce the risk of developing aneurysms of the coronary arteries and thereby decrease the morbidity and mortality associated with this condition.

[Recommendation for the recognition, management and follow up of cardiovascular compromise in patients with Pediatric Multisystemic Inflammatory Syndrome associated with COVID-19 (PIMS-CT). Position statement of Chilean Scientific Societies]. / Recomendación para la sospecha, manejo y seguimiento del compromiso cardiovascular en pacientes con Síndrome Inflamatorio Multisistémico en Pediatría asociado a COVID-19 (PIMS-TC). Declaración de Posición de la Sociedad Chilena de Pediatría (SOCHIPE) y Sociedad Chilena de Cardiología y Cirugía Cardiovascular (SOCHICAR).

Since the onset of the SARS-CoV-2 pandemic, the pediatric population has been less affected by the disease both in frequency and severity. However, since April cases of variable presentation and severity characterized by inflammatory phenomena that affect multiple organs have been reported, a condition called Multisystem Inflammatory Syndrome in Children (MIS-C). The literature has reported frequent cardiac involvement, up to 80%. This is characterized by myocardial injury with a significant increase of biomarkers such as serum troponins I and T, BNP, or NT-ProBNP coupled with varying degrees of ventricular dysfunction, pericarditis, valvulitis, and arrhythmias. Coronary compromise has also been described, which can occur in up to 23% of cases, and ranges from dila tations to aneurysms. Inpatient and outpatient cardiology follow-up has been systematized based on the clinical phenotypes such as myocardial injury (myocarditis, valvulitis, pericarditis), shock (usua lly vasoplegic), Kawasaki disease-type manifestations, and those MIS-C that do not comply with the clinic of the previous three. This last group represents the main challenge in the short-, medium- and long-term follow-up, therefore, it is necessary a multidisciplinary team for managing these patients. Considering the high frequency of cardiac compromise in MIS-C, and the importance of reaching a consensus regarding its management and follow-up, we present these recommendations according to the current state of knowledge regarding this recently described pathology.

Severe clinical spectrum with high mortality in pediatric patients with COVID-19 and multisystem inflammatory syndrome

OBJECTIVES: To assess the outcomes of pediatric patients with laboratory-confirmed coronavirus disease (COVID-19) with or without multisystem inflammatory syndrome in children (MIS-C). METHODS: This cross-sectional study included 471 samples collected from 371 patients (age<18 years) suspected of having severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The study group comprised 66/371 (18%) laboratory-confirmed pediatric COVID-19 patients: 61 (92.5%) patients tested positive on real-time reverse transcription-polymerase chain reaction tests for SARS-CoV-2, and 5 (7.5%) patients tested positive on serological tests. MIS-C was diagnosed according to the criteria of the Center for Disease Control. RESULTS: MIS-C was diagnosed in 6/66 (9%) patients. The frequencies of diarrhea, vomiting, and/or abdominal pain (67% vs. 22%, p=0.034); pediatric SARS (67% vs. 13%, p=0.008); hypoxemia (83% vs. 23%, p=0.006); and arterial hypotension (50% vs. 3%, p=0.004) were significantly higher in patients with MIS-C than in those without MIS-C. The frequencies of C-reactive protein levels >50 mg/L (83% vs. 25%, p=0.008) and D-dimer levels >1000 ng/mL (100% vs. 40%, p=0.007) and the median D-dimer, troponin T, and ferritin levels (p<0.05) were significantly higher in patients with MIS-C. The frequencies of pediatric intensive care unit admission (100% vs. 60%, p=0.003), mechanical ventilation (83% vs. 7%, p<0.001), vasoactive agent use (83% vs. 3%, p<0.001), shock (83% vs. 5%, p<0.001), cardiac abnormalities (100% vs. 2%, p<0.001), and death (67% vs. 3%, p<0.001) were also significantly higher in patients with MIS-C. Similarly, the frequencies of oxygen therapy (100% vs. 33%, p=0.003), intravenous immunoglobulin therapy (67% vs. 2%, p<0.001), aspirin therapy (50% vs. 0%, p<0.001), and current acute renal replacement therapy (50% vs. 2%, p=0.002) were also significantly higher in patients with MIS-C. Logistic regression analysis showed that the presence of MIS-C was significantly associated with gastrointestinal manifestations [odds ratio (OR)=10.98; 95%CI (95% confidence interval)=1.20-100.86; p=0.034] and hypoxemia [OR=16.85; 95%CI=1.34-211.80; p=0.029]. Further univariate analysis showed a positive association between MIS-C and death [OR=58.00; 95%CI=6.39-526.79; p<0.0001]. CONCLUSIONS: Pediatric patients with laboratory-confirmed COVID-19 with MIS-C had a severe clinical spectrum with a high mortality rate. Our study emphasizes the importance of investigating MIS-C in pediatric patients with COVID-19 presenting with gastrointestinal involvement and hypoxemia.

[Clinical- epidemiological characteristics and predictors of coronary complications in children of Argentina with Kawasaki disease]. / Características clínico-epidemiológicas y predictores de complicaciones coronarias en niños de Argentina con enfermedad de Kawasaki.

BACKGROUND: Kawasaki disease (EK) is an acute systemic vasculitis with a risk of developing coronary aneurysms. AIM: To describe the clinical and epidemiological characteristics of children with EK in Argentina and to analyse the risk factors for the development of coronary's complications (CC). METHODS: Multicenter, retrospective, cross-sectional, observational and analytical study. It included patients younger than 18 years of age diagnosed with EK in hospitals in Argentina, between January the 1st, 2010 and December the 31th, 2013. RESULTS: N = 193 subjects. Age: medium: 29 months. Total incidence 5 cases / 10,000 hospital discharges. CC was observed in 15.5% of patients. Increased risk factors for CC: Elevated number of days with fever at the time of treatment placement (p = 0.0033); Increased of: heart frequency (p = 0.0021), erythrosedimentation (ESR) (p = 0.005), C-reactive protein (CRP) (p < 0.0001), leukocytes (p = 0.0006), neutrophils (p = 0.0021); Decreased of hematocrit (p = 0.0007) and hemoglobin (p < 0.0001).Association with CC: non-coronary cardiological alterations (OR = 10,818); PCR greater than 68 mg /L (OR = 11,596); leukocytes greater than 20,000 / mm3 (OR = 4.316); and ESR greater than 64 mm / 1 hour (OR = 4.267). CONCLUSION: The most frequent form of presentation was complete EK, the risk of CC was higher in males, younger than 5 years old, the risk factors (clinical and laboratory) were similar to those described in the literature.