Investigating the comparative effect of vitamin D level with the type of complications in Henoch Schönlein purpura and Kawasaki disease.

INTRODUCTION AND OBJECTIVES: Henoch Schönlein purpura (HSP) and Kawasaki disease (KD) are two main inflammatory diseases among childhood vasculitis. Considering the anti-inflammatory effects of 25-hydroxyvitamin D3, we decided to investigate the association of serum 25-hydroxy vitamin D3 level with the type and severity of these conditions. MATERIALS AND METHODS: The present study was performed as a historical cohort of 254 affected children with KD and HSP vasculitis. The required data were extracted, using a researcher-made questionnaire from patients' electronic file, and then they were analyzed after collecting information of the patients. RESULTS: In HSP group, 54% of participants were boys. Similarly, in KD group, boys were more affected than girls. The comparative 25-hydroxyvitamin vitamin D3 level in HSP patients with and without renal involvement (P=0.02), hematuria (P=0.14), and in two groups with and without heart disease, and also with and without coronary artery dilatation in KD patients (P<0.001) were significant. DISCUSSION AND CONCLUSIONS: The findings showed that insufficient level of vitamin D3 were significantly associated with the exacerbation of complications of both diseases, and therefore it seems that vitamin D deficiency can be an effective predictive factor of severity in HSP and KD patients.

The role of nailfold capillaroscopy in pediatric patients with Kawasaki disease.

BACKGROUND: Nailfold capillaroscopy has been used as a non-invasive diagnostic method for microvasculature evaluation in various rheumatological disorders. The present study aimed to determine the utility of nailfold capillaroscopy in the diagnosis of Kawasaki Disease (KD). METHOD: In this case-control study nailfold capillaroscopy was performed in 31 patients with KD and 30 healthy controls. All nailfold images were evaluated for capillary distribution and capillary morphology such as enlargement, tortuosity, and dilatation of the capillaries. RESULT: Abnormal capillaroscopic diameter was identified in 21 patients from the KD group and 4 patients in the control group. The most common abnormality in capillary diameter was irregular dilatation in 11 (35.4%) KD patients and in 4 people (13.3%) in the control group. Distortions of the normal capillary architecture was commonly seen in the KD group (n=8). A positive correlation was observed between coronary involvement and abnormal capillaroscopic results (r=.65, P<.03). The sensitivity and specificity of capillaroscopy for the diagnosis of KD were 84.0% (95%CI: 63.9-95.5%) and 72.2% (95%CI: 54.8-85.8%), respectively. The PPV and NPV of capillaroscopy for KD were 67.7% (95%CI: 48.6-83.3) and 86.7% (95% CI: 69.3-96.2), respectively. CONCLUSION: Capillary alterations are more common in KD patients compared to control group. Thus, nailfold capillaroscopy can be useful in detecting these alterations. Capillaroscopy is a sensitive test for detecting capillary alternations in KD patients. It could be used as a feasible diagnostic modality for evaluating microvascular damage in KD.

Immunoglobulin-resistant Kawasaki disease.

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.

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.

COVID-19 associated Kawasaki-like multisystem inflammatory syndrome in an adult.

Recently, few reports have described a serious condition linked to SARS-CoV-2 that mimics Kawasaki disease called multisystem inflammatory syndrome, especially in children (MIS-C) and young adults. In this work, we report on a severe form of MIS in a young female adult previously infected by SARS-CoV-2. She was treated by plasmapheresis with albumin and steroids, however outcome was fatal. We discuss the pathogenesis of this rare and life threatening entity and suggest some therapeutic regimen. This syndrome should not be misdiagnosed with an infectious or a drug induced cutaneous rash in the current context of COVID-19 pandemic.

COVID-19 associated Kawasaki-like multisystem inflammatory syndrome in an adult. / Síndrome inflamatorio multisistémico de tipo Kawasaki asociado a COVID-19 en un adulto.

Recently, few reports have described a serious condition linked to SARS-CoV-2 that mimics Kawasaki disease called Multisystem inflammatory syndrome, especially in children (MIS-C) and young adults. In this work, we report on a severe form of MIS in a young female adult previously infected by SARS-CoV-2. She was treated by plasmapheresis with albumin and steroids, however outcome was fatal. We discuss the pathogenesis of this rare and life threatening entity and suggest some therapeutic regimen. This syndrome should not be misdiagnosed with an infectious or a drug induced cutaneous rash in the current context of COVID-19 pandemic.

[Previous or coincident infections with suspected Kawasaki disease. Should we change our approach?] / Infecciones previas o coincidentes con la sospecha de enfermedad de Kawasaki ¿debemos cambiar nuestra actitud?

INTRODUCTION: Kawasaki disease (KD) is a multisystem vasculitis associated with coronary artery abnormalities. Infections could be a trigger of the inflammation. The main aim of this study was to describe the presence of infections in children with KD, and to analyse the clinical characteristics and the presence of coronary abnormalities in these cases. PATIENTS AND METHODS: A retrospective study was performed within the Kawasaki Diseases Network (KAWA-RACE (2011-2016). An analysis was performed that included patients with positive microbiological findings (PMF) during the acute phase, as well as those with a previous recent infection (PRI) during the 4 weeks preceding KD diagnosis. RESULTS: The study included total of 621 children with KD, with PMF being found in 101 (16.3%) patients, and a PRI in 107 (17.2%). Significantly less echocardiographic abnormalities were found in the in the group with a PRI, when compared to those without a PRI (23 vs. 35%, P=.01) and also a lower proportion of overall coronary artery lesions (16 vs. 25%, P=.054). No significant differences were found in the proportion of aneurysms in either of these groups (PRI or PMF) when compared to those without infection. CONCLUSIONS: In the present study, no differences were found in the incidence of coronary aneurysms in either of the groups, with or without PRI or PMF. Therefore, if KD is suspected, appropriate treatment should be started despite having a confirmed infection.

Usefulness of age-adjusted N-terminal prohormone of brain natriuretic peptide level as a diagnostic marker for incomplete Kawasaki disease in children. / Validez del NT-proBNP ajustado por la edad como biomarcador diagnóstico de la enfermedad de Kawasaki incompleta en la población pediátrica.

OBJECTIVES: The main objective was to assess the diagnostic usefulness of age-adjusted level of N-terminal prohormone of brain natriuretic peptide (NT-proBNP) as a diagnostic marker in children suspected of having incomplete Kawasaki disease (IKD). The secondary aim was to compare the diagnostic yield of NT-proBNP level with the yield of other markers currently recommended by the American Heart Association (AHA). MATERIAL AND METHODS: Descriptive cross-sectional study of a case series of patients under the age of 14 years admitted with clinical suspicion of IKD between 2013 and 2017. We analyzed NT-proBNP level adjusted for age. Demographic, clinical, echocardiographic, analytic, and microbiologic findings were gathered from computerized charts. Two independent evaluators made the diagnosis of IKD according to AHA criteria. RESULTS: We included 70 cases, and 19 (27%) were diagnosed with IKD. Patients with IKD had higher NT-proBNP levels than patients with other febrile diseases, and the proportion of cases of elevated age-adjusted NT-proBNP level was also higher in the IKD group (84% vs 4%; P<.001). The diagnostic yield of age-adjusted NT-proBNP for IKD was good (area under the receiver operating characteristic curve, 0.90; 95% CI, 0.80-0.99) and significantly higher than the yields for C-reactive protein, erythrocyte sedimentation rate, albumin, and sterile pyuria (P<.001, all comparisons). CONCLUSION: NT-proBNP level may prove to be a valid diagnostic marker for IKD, possibly offering a higher diagnostic yield than the analyses currently recommended for children suspected of having IKD.

OBJETIVO: Evaluar la validez diagnóstica del NT-proBNP ajustado por la edad para enfermedad de Kawasaki incompleta (EKI) en pacientes pediátricos con sospecha clínica de EKI y comparar su capacidad diagnóstica con los parámetros analíticos diagnósticos recomendados actualmente por la American Heart Association (AHA). METODO: Se incluyó a todos los pacientes menores de 14 años ingresados por sospecha de EKI desde 2013 hasta 2017. Se registró el valor de NT-proBNP ajustado por la edad, datos demográficos, clínicos, analíticos, ecocardiográficos y microbiológicos. El diagnóstico de EKI lo realizaron dos evaluadores según los criterios de la AHA. RESULTADOS: Se incluyeron 70 pacientes: 19 (27%) fueron clasificados como EKI, y presentaron mayores concentraciones de NT-proBNP (p < 0,001), y un mayor porcentaje de casos de NT-proBNP elevado para la edad (84% vs 4%; p < 0,001) en comparación con otras enfermedades febriles. El NT-proBNP ajustado por la edad tuvo una buena capacidad diagnóstica para la EKI (área bajo la curva = 0,90; IC95% 0,80-0,99), superior a la proteína C reactiva (p < 0,001), velocidad globular de sedimentación (p < 0,001), albúmina (p < 0,001) y piuria estéril (p < 0,001). CONCLUSIONES: El NT-proBNP podría ser un test diagnóstico válido para la EKI y potencialmente superior a los parámetros analíticos actualmente recomendados en los pacientes pediátricos con sospecha de EKI.

Acute kidney injury and cholestasis associated with Kawasaki disease in a 9-year-old: Case report. / Daño renal agudo y colestasis asociados a la enfermedad de Kawasaki en una paciente de 9 años; reporte de caso.

Kawasaki disease (KD) is a systemic vasculitis frequent in children younger than 5 years of age. It involves coronary arteries and other medium-sized vessels. There also exists evidence of inflammatory and proliferative changes affecting the biliary tract and lymphocyte infiltration of the renal interstitial. We describe the case of a 9-year-old girl who developed high-grade fever, bilateral non-purulent conjunctivitis, «strawberry¼ tongue, desquamation of the fingers and toes, cholestatic syndrome, edema and elevated serum creatinine. KD is a diagnostic challenge for the pediatrician. In every patient with high-grade fever, cholestasis and acute kidney injury, KD should be included in the differential diagnosis, even though more research is necessary to evaluate this atypical association.

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.

[Cardiac manifestations in the acute phase of Kawasaki disease in a third level children's hospital in Mexico City]. / Manifestaciones cardiacas en la etapa aguda de la enfermedad de Kawasaki en un hospital pediátrico de tercer nivel en la Ciudad de México.

OBJECTIVES: To describe the cardiac manifestations in the acute phase of patients with Kawasaki disease treated in a third level Children's hospital in Mexico City, Mexico. METHODS: A cross-sectional study was conducted in patients with a diagnosis of Kawasaki disease treated in this hospital from August 1995 to December 2016. Information included patient demographics, clinical features, treatment used, electrocardiographic findings, extra-coronary echocardiographic findings, and the development of coronary artery aneurysms in the acute phase of the disease. RESULTS: The study included 508 cases of Kawasaki disease, with a mean age at diagnosis of 37.64±35.56 months (range from 2 to 200 months). Almost two-thirds (65.4%) of the patients were male, with a male/female ratio of 1.88:1. Complete Kawasaki disease was diagnosed in 79.2% of cases. Almost all cases (92.4%) received intravenous immunoglobulin. Twenty-eight patients (5.5%) developed arrhythmias, ST changes developed in 29 patients (5.6%), and 5 patients presented with ischaemic changes. In the initial echocardiographic evaluation, 51 patients (9.9%) were diagnosed with myocarditis, 72 patients (14.0%) with pericarditis and 77 cases (15.0%) developed pericardial effusion. Coronary artery anomalies were detected in 169 cases (32.9%). 32 cases were diagnosed as giant coronary aneurysms. Four patients died from cardiac complications in the acute phase of the disease. CONCLUSIONS: There has been an increase in the diagnosis of Kawasaki disease in Mexico. They presented with more cardiac complications than reported in literature. An increased knowledge of Kawasaki disease is required in Mexico in order to establish the cardiac outcomes of this group of patients.

[Risk factors related to cardiovascular complications in patients diagnosed with Kawasaki disease in northwestern Mexico]. / Factores de riesgo asociados a complicaciones cardiovasculares en pacientes con enfermedad de Kawasaki en el noroeste de México.

Background: Kawasaki disease (KD) is an acute systemic vasculitis of small and medium vessels, which occurs primarily in children; it manifests itself as a febrile syndrome coupled with vasculitis data and can cause coronary artery abnormalities in 25% of untreated patients. The objective of this study was to describe the clinical behavior and to identify risk factors for cardiovascular complications in pediatric patients with KD, in a second level hospital in Northwestern Mexico. Methods: Under a case series design, we studied pediatric patients with diagnosis of KD. We measured clinical variables, laboratory values and the presence of cardiac complications; the probability of risk was determined with odds ratio (OR) and the association with chi squared test. Results: 12 patients were included, and the female gender predominated; the mean age of presentation of KD was 2 years. The clinical presentation was complete in 100 % of the cases and patients also presented atypical manifestations. 50% of the patients studied had cardiovascular complications, the most common of which was coronary disease (33%). The variables male gender, age under 2 years and anemia reported OR of 5.5 and 10 to present cardiovascular complications. Conclusions: Cardiovascular complications of KD are frequent (more than 30%). Male gender, age under 2 years and anemia increase the probability of risk for the presence of cardiovascular complications.

Introducción: La enfermedad de Kawasaki (EK) es una vasculitis sistémica aguda de pequeños y medianos vasos, que se presenta principalmente en niños. Se manifiesta como síndrome febril aunado a datos de vasculitis, y puede causar anormalidades en las arterias coronarias en el 25% de los pacientes no tratados. El objetivo de este estudio fue describir el comportamiento clínico y la identificación de factores de riesgo para complicaciones cardiovasculares en pacientes pediátricos con EK atendidos en un hospital de segundo nivel del noroeste de México. Métodos: Bajo un diseño de serie de casos, se estudiaron pacientes pediátricos con diagnóstico de EK. Se midieron variables clínicas, de laboratorio y presencia de complicaciones cardiacas, y se estimó la probabilidad de riesgo con razón de momios (RM) y asociación con prueba de ji al cuadrado. Resultados: Se incluyeron 12 pacientes y predominó el sexo femenino; la edad media de presentación de la EK fue de 2 años. La presentación clínica fue completa en el 100% de los casos y hubo además manifestaciones atípicas. El 50% de los pacientes estudiados presentaron complicaciones cardiovasculares; la más común fue afección coronaria (33%). Las variables sexo masculino, edad menor de 2 años y anemia tuvieron RM de 5.5 y 10 de presentar complicaciones cardiovasculares. Conclusiones: Las complicaciones cardiovasculares de la EK son frecuentes (más del 30% de los pacientes). Las variables sexo masculino, edad menor de 2 años y anemia incrementaron la probabilidad de riesgo para la presencia de complicaciones cardiovasculares.

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.

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.

[National consensus on the cardiological treatment and follow-up of Kawasaki disease]. / Consenso nacional sobre diagnóstico, tratamiento y seguimiento cardiológico de la enfermedad de Kawasaki.

Kawasaki disease is a self-limiting acute vasculitis that affects small and medium-sized vessels, and is the most common cause of acquired heart disease in children in our environment. Up to 25% of untreated patients develop coronary aneurysms. It is suspected that an infectious agent may be the trigger of the disease, but the causative agent is still unknown. Based on the previous evidence, recommendations are proposed for the diagnosis, treatment of acute disease, and the long-term management of these patients, in order to unify criteria. The diagnosis must be quick, based on easy-to-use algorithms and with the support of complementary tests. This document includes the indication of available imaging techniques, as well as the planning of cardiological examinations based on the initial involvement. Intravenous immunoglobulin is the basis of the initial treatment. The role of corticosteroids is still controversial, but there are studies that support its use as adjuvant treatment. A multidisciplinary working group has developed a scheme with different treatment guidelines depending on the risk factors at diagnosis, the patient's clinical situation, and response to previous treatment, including indications for thromboprophylaxis in patients with coronary involvement. The stratification of risk for long-term treatment is essential, as well as the recommendations on the procedures based on the initial cardiological involvement and its progression. Patients with coronary aneurysms require continuous and uninterrupted cardiological monitoring for life.

Síndrome inflamatorio multisistémico post-COVID en niños: reporte de casos en Montevideo, Uruguay, 2020-2021 / Multsystemic inflammatory syndrome in post-Covid children: clinical case studies in Montevideo, Uruguay, 2020-2021 / Sindrome inflamatório multisistêmico pós-COVID em crianças: relato de casos em Montevdéu, Uruguai, 2020-2021

El síndrome inflamatorio multisistémico asociado a COVID-19 es una presentación postinfecciosa, que puede ser particularmente grave, pudiendo producir una severa disfunción multiorgánica. Se presenta una serie de 6 casos clínicos, asistidos en Servicio Médico Integral, Montevideo-Uruguay, con el fin de exponer las características clínicas y paraclínicas de este nuevo síndrome, y la evolución clínica de los mismos. Se realiza un estudio observacional descriptivo. La edad media fue de 6 años, con predominio en sexo femenino. Estos pacientes tuvieron una presentación clínica leve a moderada, con buena evolución Las manifestaciones clínicas principales fueron: fiebre, compromiso gastrointestinal, mucocutáneo y afectación ocular; dos de estos pacientes presentaron compromiso cardiovascular. Todos tuvieron alteraciones en la paraclínica, destacándose reactantes de fase aguda elevados. Todos los pacientes recibieron inmunoglobulina intravenosa y corticoides, tres de ellos recibieron heparina de bajo peso molecular por valor de D-dímeros 5 veces por encima del valor de referencia. No descartamos el impacto del diagnóstico y tratamiento precoz en la buena evolución de estos niños.

The Multisystem Inflammatory Syndrome associated with COVID-19 is a postinfectious presentation, which can be particularly serious, and can produce severe multiorgan dysfunction. A report of 6 clinical cases, assisted in the Comprehensive Medical Service, Montevideo-Uruguay, is presented in order to expose the clinical and paraclinical characteristics of this new syndrome, and their clinical evolution. A descriptive observational study was carried out. The mean age was 6 years, with a predominance of females. These patients had a mild to moderate clinical presentation, with good evolution. The main clinical manifestations were: fever, gastrointestinal and mucocutaneous involvement, and ocular involvement; two of these patients had cardiovascular compromise. All had alterations in the paraclinical, standing out elevated acute phase reactants. All patients received intravenous immunoglobulin and corticosteroids, three of them received low molecular weight heparin with a value of D-dimers 5 times higher than the reference value. We do not rule out the impact of early diagnosis and treatment on the positive evolution of these children.

A Síndrome Inflamatória Multissistêmica associada à COVID-19 é uma apresentação pósinfecciosa, que pode ser particularmente grave e produzir disfunção grave de múltiplos órgãos. Apresentamos um relato de 6 casos clínicos, atendidos no Serviço Médico Integral, Montevidéu-Uruguai, com o objetivo de expor as características clínicas e para clínicas desta nova síndrome, e sua evolução clínica. Tratase de um estudo observacional descritivo. A média de idade foi de 6 anos, com predomínio do sexo feminino. Esses pacientes tinham apresentação clínica leve a moderada, com boa evolução. As principais manifestações clínicas foram: febre, envolvimento gastrointestinal, muco cutâneo e ocular; dois desses pacientes apresentaram comprometimento cardiovascular. Todos apresentavam alterações para clínicas, destacandose elevados reagentes na fase aguda. Todos os pacientes receberam imunoglobulina e corticosteroides endovenosos, três deles receberam heparina de baixo peso molecular com valor do dímero D 5 vezes acima do valor de referência. Não descartamos o impacto do diagnóstico e tratamento precoces na evolução positiva dessas crianças.

Caracterización de la enfermedad de Kawasaki en niños entre 3 meses y 15 años en 2 instituciones de salud en Bogotá, Colombia / Characterization of Kawasaki disease in children between 3 months and 15 years in 2 health institutions in Bogotá, Colombia

Resumen Objetivo: Disminuir la brecha del conocimiento de la Enfermedad de Kawasaki (EK) y dar herramientas al personal médico por medio de la descripción de la demografía, presentación clínica, los hallazgos de laboratorio, la frecuencia de lesiones coronarias y desenlaces en 2 instituciones de salud en Bogotá, Colombia. Metodología: Estudio observacional retrospectivo mediante la revisión de historias clínicas de los códigos CIE-10 de paciente pediátricos donde se evaluaron características demográficas, presentación clínica, datos paraclínicos (incluidos hallazgos ecocardiográficos), tratamiento recibido y respuesta a este, en pacientes admitidos entre junio de 2015 y junio de 2020. Resultados: Se incluyeron 36 pacientes entre 3 meses y 15 años. La edad media de los pacientes fue de 2.9 años, siendo la EK más frecuente en niños en una rela ción 2:1. El 61.1% presentó EK completa o clásica, el 30.5% EK incompleta y el 8.3% EK atípica. Todos los pacientes recibieron inmunoglobulina intravenosa antes del día 10 del curso de la enfermedad, con remisión de la fiebre antes de 12 horas luego de la administración. La incidencia de compromiso coronario fue de 30.6%. Conclusiones: La Enfermedad de Kawasaki tiene un curso clínico característico que afecta especialmente a niños menores de 5 años. Es una entidad clínica que, al ser reconocida con mayor frecuencia por pediatras, permite instaurar diagnóstico y tratamiento tempranos evitando complicaciones y secuelas a mediano y largo plazo.

Abstract Objective: To reduce the knowledge gap about Kawasaki Disease (KD) and to provide tools to medical personnel through the description of demographics, clinical presentation, laboratory findings, frequency of coronary lesions and outcomes in 2 health institutions in Bogota Colombia. Methodology: Retrospective observational study by reviewing the clinical records of the ICD-10 codes of pediatric patients where demographic characteristics, clinical presentation, paraclinical data (including echocardiographic findings), treatment received and response to it were evaluated, in patients admitted between June 2015 and June 2020. Results: The mean age of the patients was 2.9 years, being KD more frequent in boys a 2:1 ratio. 61.1% had complete or classic KD, 30.5% had incomplete KD, and 8.3% atypical KD. All patients received intravenous immunoglobulin before day 10 of the course of the disease, with remission of fever within 12 hours after administration. The incidence of coronary compromise was 30.6%. Conclusions: KD has a characteristic clinical course that especially affects children under 5 years. A more frequent recognition of this clinical entity by pediatricians, allows for an early diagnosis and treatment avoiding complications and sequelae in the medium and long term.

Has joint involvement lessened in Kawasaki disease? / ¿La enfermedad de Kawasaki ha perdido su afectación articular?

OBJECTIVE: Kawasaki disease (KD) is an acute systemic vasculitis affecting medium-sized arteries, particularly the coronary arteries. Classic diagnosis is based in prolonged fever and different clinical features, including acute arthritis. Our objective is to determine the prevalence of arthritis at the moment of the diagnosis, the response to intravenous immunoglobulin infusion and the relation with cardiac findings. MATERIAL AND METHODS: Retrospective study through review of medical records of 42 patients with KD from 1988 to 2013. Demographic, clinical, laboratory variables and treatment were reviewed. RESULTS: Male sex was predominant (57%). Fever (100%), exanthema (92,9%), conjunctivitis (78,6%), oropharingeal changes (76,2%), cervical lymphadenopathy (71,4%), edema (52,4%) and peripheral desquamation (46,3%) were reported. Eight patients presented ecocardiography alterations (ectasia and aneurism). Acute articular involvement was reported in 7 (16%) patients, including oligoarticular (57%), monoarticular (29%) and polyarticular (14%) patterns. All patients had elevation of acute phase reactants with neutrophilia (57%) and hypoalbuminemia (71,5%), but showed a good therapeutic response to intravenous immunoglobulin, without sequelaes. Sixteen patients had incomplete KD nine males, with 100% of fever exanthema (75%), conjunctivitis (56%) and 50% of cervical lymphadenopathy. Whereas oropharingeal changes and edemas was described in 44% and 25% of them. Four patients with incomplete KD had coronary artery abnormalities. CONCLUSIONS: Acute arthritis was an uncommon finding (16%) and resulted in no sequelae. Maybe the treatment with intravenous immunoglobulin and aspirin prevents the development of articular abnormalities and then leading to a decrease in its follow-up requierement by reumathologist. The cardiovascular sequelae, mainly incomplete KD, remains determining its prognosis. The presence of articular involvement seems not to have influence over cardiac involvement.

[Giant coronary aneurysms in infants with Kawasaki disease]. / Aneurismas coronarios gigantes en lactantes con enfermedad de Kawasaki.

INTRODUCTION: Kawasaki disease (KD) is an acute vasculitis of unknown origin and predominant in males. The long-term effects of the disease depend on whether there are coronary lesions, particularly aneurysms. The prognosis of patients with giant aneurysms is very poor due to their natural progression to coronary thrombosis or severe obstructive lesions. OBJECTIVES: A series of 8 cases is presented where the epidemiology and diagnostic methods are described. The treatment of the acute and long-term cardiovascular sequelae is also reviewed. METHODS: A descriptive analysis was conducted on patients admitted to the Paediatric Cardiology Unit of La Fe University Hospital (Valencia) with KD and a coronary lesion. RESULTS: More than one artery was involved in all patients. Although early diagnosis was established in only two cases, none of the patients had severe impairment of ventricular function during the acute phase. Treatment included intravenous gammaglobulin and acetylsalicylic acid at anti-inflammatory doses during the acute phase. A combination of dual antiplatelet therapy and corticosteroids was given in cases of coronary thrombosis. The silent aneurysms continue to persist. CONCLUSIONS: KD is the most common cause of acquired heart disease in children. The delay in diagnosis is associated with a greater likelihood of coronary lesions that could increase the risk of cardiovascular events in adulthood. Thus, this subgroup requires close clinical monitoring for a better control of cardiovascular risk factors over time.

[Kawasaki disease is more prevalent in rural areas of Catalonia (Spain)]. / En Cataluña la enfermedad de Kawasaki es más prevalente en las zonas rurales.

INTRODUCTION: Kawasaki disease (KD) is an acute self-limited systemic vasculitis relatively common in childhood. The etiology of KD is still unknown, although clinical, laboratory and epidemiological features suggest an infectious origin or trigger. Differences on incidence between countries have been related to specific genetic factors, ethnicity, country of birth and some other sociocultural and environmental factors. We present a population-based study on incidence of KD in Catalonia (Spain), focusing on differences between patients in rural and non-rural areas of the region. METHODS: Observational population-based study including all Pediatric Units in Catalan hospitals, between 2004 and 2014. A 12-month (March 2013-March 2014) prospective collection of new cases of KD was carried out to determine the incidence of KD. The rest of the data was retrieved retrospectively. RESULTS: Data from 399 patients over the 10-year study period was analyzed. Among the total KD patients, 353 (88.5%) lived in non-rural areas and 46 (11.5%) in rural areas. It was found that there is a significant difference (P<.001) between the percentage of rural population observed in patients with KD (11.5%), and the expected 5% of the Catalan population. CONCLUSION: This is the first population-based study showing significant differences on KD incidence rates between rural and non-rural areas.

Enfermedad de Kawasaki, enfermedad similar a Kawasaki y MIS-C asociado a COVID-19 en niños: revisión sistemática / Kawasaki disease, Kawasaki-like disease, and MIS-C associated with COVID-19 in children: systematic review

Resumen Objetivo: Sintetizar las características epidemiológicas y clínicas de los niños de COVID-19 con EK, KLD y MIS-C. Métodos: Se realizó una búsqueda en 4 bases de datos y preprints hasta el 31 de Mayo del 2021. Se incluyeron reportes/series de caso que evaluaron las caracte rísticas clínicas del EK, KLD o MIS-C en pacientes pediátricos con COVID-19. Resultados: Se incluyeron 16 estudios (seis informes de casos y diez series de casos, 367 pacientes en total, 58 pacientes con EK, 87 con KLD y 290 pacientes con MIS-C); con edades entre los 6 meses y los 10 años, y el 62% eran mujeres. Se observó COVID-19 positivo en 75,2%. Respecto a EK, KLD y MIS-C, las características clínicas repor tadas fueron compatibles con los cuadros diagnósticos estandarizados en el contexto de COVID-19. La duración de la hospitalización fue de 5 a 14 días para EK y de 4,3 a 13 para MIS-C. Once pacientes con MIS-C (2,8%) necesitaron ECMO. Seis pacientes con MIS-C fueron reportados muertos. Ocho estudios reportaron pacientes en la UCI. Conclusiones: EK o KLD puede asociarse a COVID-19 en niños, y pueden complicarse con MIS-C. El tiempo de hospitalización es prolongado si se presenta EK o KLD asociado a COVID-19 en niños.

Abstract Objective: To synthesize the epidemiological and clinical characteristics of COVID-19 children with MIS-C, KLD and EK. Methods: Databases and preprints were searched until May 31, 2021. Reports/case series that evaluated the clinical features of EK, KLD, or MIS-C in pediatric patients with COVID-19 were included. Results: Sixteen studies were included (six case reports and ten case series, 367 patients total, 58 patients with EK, 87 with KLD, and 290 patients with MIS-C); with ages ranging from 6 months to 10 years, and 62% were female. Positive COVID-19 was observed in 75.2%. Regarding EK, KLD and MIS-C, the reported clinical characteristics were compatible with the standardized diagnostic pictures in the context of COVID-19. The duration of hospitalization was 5 to 14 days for EK and 4.3 to 13 for MIS-C. Eleven patients with MIS-C (2.8%) needed ECMO. Eleven patients with MIS-C (2.8%) needed ECMO. Six patients with MIS-C were reported dead. Eight studies reported patients in the ICU. Conclusions: Children with COVID-19 develop EK or KLD, and can be complicated by MIS-C. Prevention, diagnosis, and treatment measures are needed.