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.

Canalización del crecimiento entre los 2 y los 5 años en niños aparentemente sanos con talla baja a los 2 años / Canalization of growth between 2 and 5 years of age in apparently healthy children with short stature at age 2 years

Introducción. El tamaño del recién nacido se asocia a condiciones intrauterinas. El potencial genético se expresa más tarde; la canalización del crecimiento se describe clásicamente hasta los 24 meses. Objetivo. Describir la canalización del crecimiento entre los 2 y los 5 años en niños aparentemente sanos con talla baja a los 2 años. Población y métodos. Estudio de cohorte retrospectiva. Se incluyeron niños seguidos en un hospital universitario de comunidad entre 2003 y 2019, con puntaje Z de talla menor a -2 DE para edad y sexo a los 2 años. Se excluyeron los nacidos prematuros, con bajo peso y con enfermedades crónicas. Se evaluó la trayectoria de crecimiento. Se definió canalización como la adquisición de talla normal para la población general. Resultados. Se incluyeron 64 niños, de los cuales 37 (58 %) presentaron canalización del crecimiento a los 5 años (20 a los 3 años, 8 a los 4 años, y 9 a los 5 años). La velocidad de crecimiento a los 3 y a los 5 años fue significativamente mayor en los que canalizaron en comparación con los que no lo hicieron; hubo una tendencia similar a los 4 años. De los 27 niños con talla baja a los 5 años, 25 tuvieron al menos un registro de velocidad de crecimiento anual menor al percentil 25. Conclusiones. La mayoría de los niños aparentemente sanos con baja talla a los 2 años alcanzan una talla normal a los 5 años. La velocidad de crecimiento anual permite detectar a los niños con riesgo de no canalizar.

Introduction. Newborn size is associated with intrauterine conditions. Genetic potential is expressed later; the canalization of growth is typically described up to 24 months of age. Objective. To describe the canalization of growth between 2 and 5 years of age in apparently healthy children with short stature at age 2 years. Population and methods. Retrospective, cohort study. Children seen at a community teaching hospital between 2003 and 2019, who had a Z-score for height below -2 SDs for age and sex at age 2 years were included. Infants born preterm, with a low birth weight, and chronic conditions were excluded. Growth patterns were assessed. Canalization was defined as reaching a normal stature for the general population. Results. Sixty-four children were included; 37 (58%) showed canalization of growth at 5 years old (20 at 3 years, 8 at 4 years, and 9 at 5 years). The growth rate at 3 and 5 years of age was significantly higher among those who showed canalization compared to those who did not; a similar trend was observed at 4 years of age. Among 27 children with short stature at 5 years of age, 25 had at least 1 annual growth velocity below the 25th centile. Conclusions. Most apparently healthy children with short stature at 2 years old reached a normal stature at 5 years old. The annual growth velocity allows to detect children at risk of not showing canalization.

Recent research on the nuclear factor-kappa B signaling pathway in cardiac injury in children with Kawasaki disease / 中国当代儿科杂志

Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome, is a systemic acute vasculitis belonging to autoimmune disease. Up to now, the specific pathogenesis of this disease remains unclear, and it may involve various factors such as immune response, inflammatory response, and vascular endothelial injury caused by the activation of the nuclear factor-kappa B (NF-κB) signaling pathway. In particular, children with KD and cardiac injury tend to have a poor prognosis, and researchers hope to explore the specific pathogenesis of cardiac injury in KD to provide new options for clinical diagnosis and treatment and reduce the incidence rate of this disorder. This article reviews the recent research on the role of the NF-κB signaling pathway in cardiac injury in children with KD, so as to provide a basis for future studies.

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.

Síndrome Inflamatória Multissistêmica Pediátrica (SIM-P) em crianças e adolescente com até 19 anos de idade, temporalmente associada à Covid-19 / Pediatric Multisystem Inflammatory Syndrome (P-SIM) in children and adolescents up to 19 years of age, temporally associated with Covid-19

A Síndrome Inflamatória Multissistêmica Pediátrica (SIM-P) é uma condição que afeta e prejudica diversas partes do corpo, incluindo coração, pulmões, rins, cérebro, pele, olhos ou órgãos gastrointestinais. É uma síndrome rara que ainda necessita de estudo. No entanto, é sabido que muitas crianças que apresentaram SIM-P tiveram o vírus que causa a COVID-19 ou entraram em contato com alguém com COVID-19. A SIM-P pode ser grave e fatal, e grande parte dos casos pode necessitar de internação em unidade de terapia intensiva

Síndrome multisistémico post COVID-19 y dificultad en su diagnóstico etiológico: a propósito de un caso clínico / Multisystem post COVID-19 syndrome and its difficult etiological diagnosis: a clinical case / Síndrome multissistêmica pós COVID-19 e a dificuldade no seu diagnóstico etiologico: relato de caso clínico

El SARS-CoV-2 afecta a un reducido número de pacientes pediátricos, que en su mayoría son asintomáticos o presentan compromiso respiratorio leve y evolución favorable. Sin embargo, en niños previamente sanos puede aparecer el síndrome inflamatorio multisistémico (SIM-C) o similar a Kawasaki (Kawasaki-like) asociado a la enfermedad por COVID-19, que evolucionan al shock y requiere internación en la unidad de cuidados intensivo. Presentamos el caso de un adolescente con antecedentes ambientales de SARS-CoV-2 37 días antes de su ingreso, que no se testeó en ese momento. Recibe la primera dosis de vacuna Pfizer 15 días antes de la presentación de un cuadro de sintomatología digestiva con dolor abdominal y fiebre, con test de antígenos COVID-19 y PCR viral negativos. Luego de una laparoscopia exploratoria, al sexto día de fiebre y dolor instaló conjuntivitis bilateral no supurada y exantema en tórax, odinofagia, astenia y lengua saburral con fenotipo Kawasaki. La serológica IgG anti spike fue positiva y la IgM negativa, con parámetros inflamatorios elevados, por lo que se planteó un síndrome multisistémico post COVID-19. Recibió tratamiento con inmunoglobulina intravenosa, ácido acetilsalicílico y metilprednisolona. Dada la necesidad de descartar la posibilidad de un evento atribuible a vacunación o inmunización (ESAVI), se realizó la búsqueda serológica para SARS-CoV-2, en un estudio cualitativo, buscando anticuerpos que no se generan con la vacuna Pfizer, que fueron positivos. De esta forma confirmamos la etiología post COVID-19 y descartamos la etiología por ESAVI, realizándose además el estudio cuantitativo de los anticuerpos anti spike con disminución de estos al mes del debut.

Summary: SARS-CoV-2 affects a low number of pediatric patients, most asymptomatic or with mild respiratory compromise and favorable evolution. However, in previously healthy children, Multisystem Inflammatory Syndrome (SIM-C) or Kawasaki-like Syndrome(Kawasaki-like) may appear linked to the COVID-19 disease, progressing to shock and requiring admission to the intensive care unit. We present the case of an adolescent with an environmental history of SARS-CoV-2 37 days prior to her admission, who was not tested at that time. She had received the first dose of Pfizer vaccine 15 days before the presentation of digestive symptoms with abdominal pain and fever. She had a negative Covid-19 antigen test and viral PCR. After an exploratory laparoscopy, on the sixth day of fever and pain, she developed bilateral non-suppurative conjunctivitis and a rash on the chest, odynophagia, asthenia, and coated tongue with the Kawasaki phenotype. The anti-spike IgG serology was positive and the IgM negative, with elevated inflammatory parameters, so a post-COVID-19 multisystem syndrome was suggested. She received treatment with intravenous immunoglobulin, ASA and methylprednisolone. In order to rule out the possibility of an event allegedly caused by vaccination or immunization (ESAVI), the serological search for SARS-CoV-2 was carried out through a qualitative study, looking for antibodies that are not generated with the Pfizer vaccine. The result was positive. Therefore, we confirmed the post-Covid etiology and ruled out the ESAVI etiology, and also performed the quantitative study of the anti-spike antibodies, which showed a decrease one month after the debut.

O SARS-CoV-2 afeta um número reduzido de pacientes pediátricos, em sua maioria, assintomáticos ou apresentando comprometimento do nível respiratório e evolução favorável. Porém, em crianças previamente sãs, pode aparecer a Síndrome Inflamatória Multissistêmica (SIM-C) ou similar a Kawasaki (Kawasaki-like) associada à doença por COVID-19, que pode evoluir a choque e requer a internação na UTI. Apresentamos o caso de um adolescente com antecedentes ambientais de SARS-CoV-2 37 dias antes do seu ingresso, que não se testou nesse momento. Recebendo a dose da primeira vacina Pfizer 15 dias antes da apresentação de sintomatologia digestiva com dor abdominal e febre. Teve um teste de antígenos COVID-19 viral negativo. Depois de una laparoscopia exploratória, ao sexto dia de febre e dor, teve conjuntivite bilateral não supurada e exantema en tórax, odinofagia, astenia e língua saburral com fenótipo Kawasaki. A sorologia IgG anti-spike foi positiva e a gM negativa, com parâmetros inflamatórios superiores, indicando uma síndrome multissistêmica pós Covid 19. O tratamento recebido foi imunoglobulina intravenosa, AAS e Metilprednisolona. Com o fim de descartar a possibilidade de um evento supostamente atribuível à vacinação ou vacinação positiva (ESAVI), realizou-se uma busca sorológica para SARS COV2, através de um estúdio qualitativo, procurando anticorpos não gerados pela vacina Pfizer, os quais foram confirmados. Nesta forma, confirmamos a etiologia pós-Covid e descartamos a etiologia por ESAVI. Aliás, realizamos um estudo quantitativo dos anticorpos anti-spike e comprovamos a sua diminuição a um mês de ter acontecido o debut.

Proteomics of serum exosomes in children in the acute stage of Kawasaki disease: a prospective study / 中国当代儿科杂志

OBJECTIVES@#To study the biological processes and functions of serum exosomes in children in the acute stage of Kawasaki disease (KD), so as to provide new biomarkers for the early diagnosis of KD.@*METHODS@#In this prospective study, 13 children with KD who were treated in Children's Hospital of Soochow University from June 2019 to August 2020 were enrolled as the KD group, and 13 children who were hospitalized due to bacterial infection during the same period were enrolled as the control group. Whole blood was collected on the next morning after admission, serum samples were obtained by centrifugation, and exosomes were extracted through ultracentrifugation. Serum exosomes were analyzed by label-free quantitative proteomics, and differentially expressed proteins (DEPs) were screened out for functional enrichment analysis. A protein-protein interaction (PPI) network was plotted, and unique proteins were validated by targeted proteomics.@*RESULTS@#A total of 131 DEPs were screened out for the two groups, among which 27 proteins were detected in both groups. There were 48 unique DEPs in the KD group, among which 23 were upregulated and 25 were downregulated, and these proteins acted on "complement and coagulation cascades" and "the MAPK signaling pathway". Validation by targeted proteomics showed that FGG, SERPING1, C1R, C1QA, IGHG4, and C1QC proteins were quantifiable in the KD group. A total of 29 proteins were only expressed in the control group, among which 12 were upregulated and 17 were downregulated. Four proteins were quantifiable based on targeted proteomics, i.e., VWF, ECM1, F13A1, and TTR. A PPI network was plotted for each group. In the KD group, FGG and C1QC had close interaction with other proteins, while in the control group, VWF had close interaction with other proteins.@*CONCLUSIONS@#The serum exosomes FGG and C1QC in children in the acute stage of KD are expected to become the biomarkers for the early diagnosis of KD. For children with unexplained fever, detection of FGG, C1QC1, and VWF may help with etiological screening.

Kawasaki-Like Disease, a New Phenotype in Sars-CoV-2?

Abstract The coronavirus disease (COVID-19), that assumed pandemic proportions in March 2020, mainly affects the respiratory tract, causing severe interstitial pneumonia in adults. Worldwide data indicate that COVID-19 tends to be more benign in children, which is evidenced by a high incidence of asymptomatic or mild upper airways' infection cases in this population. However, recent studies have been associating Kawasaki-like symptoms as a nonclassical presentation of coronavirus disease in pediatrics. It is suggested that the intense cytokine cascade, promoted by the SARS-CoV-2 infection, can trigger a multisystem inflammatory response as an atypical Kawasaki form in genetically predisposed individuals. In this context, patients may develop more severe clinical features with a greater predisposition to myocardial involvement, Macrophage Activation Syndrome, and Kawasaki Disease Shock Syndrome. Despite critical conditions, patients usually respond to conventional treatment of Kawasaki Disease with intravenous immunoglobulin. This article intends to provide an approach to the association between Kawasaki-Like Syndrome and 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.

Infecção por Influenza B e doença de Kawasaki em adolescente durante a pandemia da COVID-19: relato de caso / Influenza B infection and Kawasaki disease in an adolescent during the COVID-19 pandemic: a case report

RESUMO Apresentação de um caso de infecção por Influenza B e doença de Kawasaki em adolescente ocorrido durante a pandemia da COVID-19. Adolescente asmática evoluiu com febre e síndrome gripal por 7 dias e deu entrada com quadro de insuficiência respiratória aguda, necessitando de intubação orotraqueal. Evoluiu também com instabilidade hemodinâmica respondedora ao uso de droga vasoativa. Foram introduzidas antibioticoterapia e medidas de suporte. Apresentou melhora hemodinâmica e respiratória progressiva, porém mantinha febre e alteração de provas inflamatórias. Durante internação, evoluiu com conjuntivite não purulenta bilateral, descamação de mão e pés, língua em framboesa e linfonodomegalia cervical, recebendo diagnóstico de doença de Kawasaki. Recebeu gamaglobulina e, por conta de quadro clínico refratário, foi administrado também corticoide, evoluindo afebril 24 horas após. Não apresentou alterações coronarianas. O único agente isolado foi Influenza B, mesmo realizando painel viral e investigação para COVID-19 com reação em cadeia da polimerase e sorologia. Durante internação, apresentou tromboembolismo pulmonar, e, em investigação de coagulopatias, foi diagnosticada com mutação em heterozigose de fator V de Leiden. Há uma potencial associação entre doença de Kawasaki e infecção por Influenza B ou outros vírus, como o coronavírus e, por isso, esses diagnósticos devem ser investigados nos pacientes pediátricos, incluindo adolescentes, com quadros febris prolongados.

ABSTRACT We report a case of Influenza B infection and Kawasaki disease in an adolescent, diagnosed during the COVID-19 pandemic. An asthmatic female adolescent presented with fever and flu-like symptoms for 7 days and was admitted with acute respiratory failure requiring mechanical ventilation. She progressed with hemodynamic instability responsive to vasoactive drugs. Antibiotic therapy and support measures were introduced, showing progressive hemodynamics and respiratory improvement, however with persistent fever and increased inflammatory markers. During the hospitalization, she developed bilateral non-purulent conjunctivitis, hand and feet desquamation, strawberry tongue, and cervical adenopathy, and was diagnosed with Kawasaki disease. She was prescribed intravenous immunoglobulin and, due to the refractory clinical conditions, corticosteroid therapy was added; 24 hours later, the patient was afebrile. No coronary changes were found. A full viral panel including COVID-19 C-reactive protein and serology could only isolate the Influenza B virus. During the hospitalization, she was diagnosed with pulmonary thromboembolism; coagulopathies were investigated, and she was diagnosed with heterozygous factor V Leiden mutation. There is a potential association between Kawasaki disease and infection with Influenza B or with other viruses such as coronavirus. Therefore, this association should be considered in pediatric patients, adolescents included, with prolonged febrile conditions.

Age-related differences in clinical characteristics of Kawasaki disease

This study aimed to examine and summarize clinical characteristics of Kawasaki disease (KD) at different ages to further strengthen clinicians understanding of children with KD, improving the level of diagnosis, and reducing coronary artery complications of KD. A total of 398 patients with KD who were diagnosed between January 2016 and December 2017 were reviewed retrospectively. These participants were allocated into three groups according to age: group A (<1 year, n=62), group B (≥1 and <5 years, n=286), and group C (≥5 years, n=50). Clinical manifestations, laboratory results, and echocardiographic findings were compared among the groups. Most (71.86%) patients with KD were aged 1-5 years. The prevalence of cervical lymphadenopathy was lowest in group A. The duration of fever before admission was longest in group A. The rate of cervical lymphadenopathy and laboratory data were different among the groups. Group A had higher frequencies of gastrointestinal involvement, neurological symptoms, and redness at the Bacillus Calmette-Guerin inoculation site than the other groups. Infants aged <1 year with KD often have a longer duration of fever before admission, a lower prevalence of cervical lymphadenopathy, and a higher prevalence of gastrointestinal and neurological symptoms.

Potential link between SARS-CoV-2 and Kawasaki disease: importance of dentists for the diagnosis

Abstract: Kawasaki disease (KD) is a vasculitis with predilection for coronary arteries. Due to a lack of reliable confirmatory laboratory tests, the diagnosis of KD is based on a characteristic pattern of clinical findings that appear in a typical temporal sequence. The diagnostic criteria have been periodically modified and the American Heart Association has proposed the most recent guidelines for its diagnosis. However, patients may have incomplete or atypical forms of KD and diagnosis can often be difficult. Because oropharyngeal manifestations are a common and important feature for diagnosing KD and recent studies have hypothesized a possible association between KD and the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), in this review we highlight the importance of dentists in the diagnosis of KD and its potential association with SARS-CoV-2.

Diagnostic value of the reaction at the bacillus calmette-guérin vaccination site in kawasaki disease / Utilidade diagnóstica da reação do sítio de vacinação bacillus calmette-guérin na doença de kawasaki

ABSTRACT Objective: To describe the case of an infant - diagnosed with incomplete Kawasaki disease - who developed BCG scar reactivation. Case description: A 6-month-old patient was admitted to hospital with fever associated with ocular hyperemia, cervical lymphadenopathy, and hyperemic lips, and remained hospitalized for 12 days. The physical examination revealed an inflammatory reaction at the site of the BCG scar, leading to the diagnosis of incomplete Kawasaki disease. The patient was treated with venous immunoglobulin, but presented recurrence of Kawasaki disease, with subsequent onset of coronary artery disease. Comments: BCG scar reactivation is an important finding in countries where the vaccine is routinely given and may be a useful marker for early diagnosis of Kawasaki disease, especially in its incomplete form.

RESUMO Objetivo: Descrever o caso de um lactente - com diagnóstico de Doença de Kawasaki incompleta - que desenvolveu reativação da cicatriz da vacina BCG. Descrição do caso: Um paciente de 6 meses de idade foi admitido no hospital com febre, associada à hiperemia ocular, linfadenomegalia cervical e fissuras labiais, permanecendo hospitalizado por 12 dias. Apresentava, no exame físico, reação inflamatória no local da cicatriz da vacina BCG, tendo sido feito o diagnóstico de Kawasaki incompleto. O paciente foi tratado com imunoglobulina venosa, mas apresentou recorrência da doença, com posterior surgimento de coronariopatia. Comentários: A reativação da BCG é um achado importante na doença de Kawasaki em países onde a vacina é aplicada de forma rotineira e pode ser um marcador útil para o diagnóstico precoce da doença de Kawasaki, principalmente em sua forma incompleta.

Síndrome Inflamatorio Sistémico Pediátrico Asociado a SARS-CoV-2 / Pediatric Systemic Inflammatory Syndrome Associated with SARS-CoV-2

La infección por SARS-CoV-2 es poco frecuente en niños, niñas y adolescentes, con manifestaciones clínicas leves o asintomáticos, pero desde abril del 2020, se han reportado niños gravemente enfermos en las zonas de mayor incidencia de infecciones por coronavirus, caracterizado por fiebre, síntomas gastrointestinales y marcadores de inflamación sistémica, compromiso cardiovascular importante (shock, disfunción miocárdica o miocarditis), con semejanzas a la Enfermedad de Kawasaki, tormenta de citoquinas y síndrome de activación macrofágica, denominado Síndrome Inflamatorio Multisistémico Pediátrico (PIMS/MIS-C). La patogénesis no se conoce exactamente, pero una respuesta inmune innata y adaptativa alterada asociada a autoinmunidad podría ser el mecanismo. Si bien no existe una guía terapéutica estandarizada, la mayoría de los pacientes reciben gamaglobulina intravenosa y corticoides sistémicos, y en algunos casos se requiere el uso inhibidores de interleuquinas. Se ha reportado una buena respuesta y mejoría en casi todos los niños, con una baja letalidad de 1,7-2%.

SARS-CoV-2 infection is rare in children and adolescents, with mild or asymptomatic clinical manifestations, but since April 2020, seriously ill children have been reported in areas with the highest incidence of coronavirus infections, characterized by fever, gastrointestinal symptoms and markers of systemic inflammation, significant cardiovascular compromise (shock, myocardial dysfunction or myocarditis), with similarities to Kawasaki disease, cytokine storm and macrophage activation syndrome, called Pediatric Multisystemic Inflammatory Syndrome (PIMS / MIS-C) ). The pathogenesis is not exactly known, but an altered innate and adaptive immune response associated with autoimmunity could be the mechanism. Although there is no standardized therapeutic guide, most patients receive intravenous gamma globulin and systemic corticosteroids, and in some cases the use of interleukin inhibitors is required. A good response and improvement has been reported in almost all children, with a low fatality rate of 1.7-2%.

Estudio multicéntrico retrospectivo de los aspectos epidemiológicos, clínicos y terapéuticos de la enfermedad de Kawasaki en niños de Panamá

Resumen Antecedentes: La enfermedad de Kawasaki (EK) representa la principal causa de cardiopatía pediátrica adquirida en muchos países; sin embargo, hay pocos estudios publicados en Latinoamérica. Objetivo: Describir la epidemiología, los aspectos clínicos y el tratamiento de EK en niños en Panamá como parte del estudio de vigilancia de REKAMLATINA-2 en Latinoamérica. Pacientes y Métodos: Estudio retrospectivo, descriptivo, de pacientes internados con diagnóstico de EK, atendidos en tres hospitales pediátricos de Panamá del 1-enero-2009 al 31-diciembre-2013. Resultados: Se analizaron 111 pacientes, 61(54,9%) eran hombres. Todos fueron hospitalizados, siendo la media de hospitalización de 5,8 (4-7) días. La mediana de edad al ingreso fue de 28,9 (12-38) meses. Un 63,9% recibió antimicrobianos recientemente por otros posibles diagnósticos. Recibieron inmunoglobulina intravenosa (IGIV) 105 (94,6%) pacientes; de éstos, 10 (9,5%) fueron resistentes. Un 11,7% tuvo alteraciones cardiovasculares en el ECO inicial, de las cuales las lesiones coronarias se detectaron en 3 (2,9%) pacientes. Conclusiones: Los datos sugieren que EK en Panamá tiene una incidencia cercana a 2,05 x 100,000 bajo 15 años de edad y una frecuencia 2,6 veces mayor bajo 3 años de edad. Se observó un alto porcentaje de uso de antimicrobianos ambulatoriamente antes de la confirmación diagnóstica, lo cual sugiere reconocimiento tardío de EK en Panamá.

Abstract Background: Kawasaki disease (KD) is the leading cause of pediatric acquired heart disease in many countries, however, there are few published studies from Latin America (LA). Aim: To describe the epidemiology, clinical aspects, and treatment of KD in children from Panama as part of the REKAMLATINA-2 in LA. Methods: Retrospective descriptive review of hospitalized patients diagnosed with KD, attended at three main pediatric hospitals of Panama from January-1-2009 to December-31-2013. Results: 111 patients were analyzed, 61(54.9 %) were male. All children were hospitalized, and had a mean length of hospitalization of 5.8 (4-7) days. Median age at admission was 28.9 (12-38) months. Prior to KD final diagnosis, 63.9% patients received antibiotics for other presumed diagnoses. 105 (94.6%) patients received IGIV, 10 (9.5%) were resistant. On initial echocardiogram, 11.7% of cardiovascular complications were reported, of which coronary artery lesions (CALs) were detected in 3 (2.9 %) patients. Conclusion: The data suggest that KD in Panama has an incidence of about 2.05 x 100,000 in children under 15 years of age, and with a frequency 2.6 times higher in children under 3 years. A high rate of antibiotic misuse on outpatient prior to diagnostic confirmation was observed, suggesting KD unawareness and late recognition in Panamá.

Síndrome Inflamatória Multissistêmica Pediátrica (SIM-P) em crianças e adolescente com até 19 anos de idade, temporalmente associada à Covid-19 / Pediatric Multisystem Inflammatory Syndrome (P-SIM) in 19-year-old children and adolescents, temporarily associated with Covid-19

Em 26 de abril de 2020 no auge da pandemia da Covid-19 o Sistema Nacional de Saúde Inglês (NHS) publicou um "Alerta" sobre uma nova apresentação clínica em crianças, possivelmente associada com a infecção pelo SARS-CoV-2 (vírus causador da Covid-19), com características semelhantes à síndrome do choque tóxico, doença de Kawasaki completa e incompleta e a síndrome de ativação macrofágica. Porém, as crianças apresentam sintomas incomuns como dor abdominal, sintomas gastrointestinais, com marcadores inflamatórios elevados, lesão cardíaca e ocorrem em crianças mais velhas, escolares e adolescentes. Esse quadro clínico foi denominado de Síndrome Inflamatória Multissistêmica Pediátrica (SIM-P) e pode ocorrer dias ou semanas após a infecção pelo SARS-CoV-2.