[Kawasaki disease versus Multisystem Inflammatory Syndrome COVID-19 with Kawasaki disease phenotype. Single-center experience]. / Enfermedad de Kawasaki versus Síndrome Inflamatorio Multisistémico COVID 19 con fenotipo enfermedad de Kawasaki. Experiencia de un centro.

In pediatrics, a process called Pediatric Inflammatory Multisystem Syndrome (PIMS) associated with recent infection by SARS-CoV-2 virus has been observed. One of its variants presents similarities with Kawasaki disease (KD). OBJECTIVE: to compare the clinical presentation, laboratory testing, and evolution of KD with PIMS Kawasaki phenotype (PIMS-KD) in patients hospitalized before the pandemic, compared with the pandemic period. PATIENTS AND METHOD: Cross-sectional study in two groups of patients at the Hospital Exequiel González Cortés: typical KD (group 1) and PIMS-KD (group 2). Data on demographic, clinical, and biochemical details were collected, as well as echocardiogram, treatment, and evolution records. IgG and IgM serology for SARS-CoV-2 was performed in both groups. RESULTS: In the KD group and the PIMS-KD group, 20 and 33 patients were analyzed, respectively. There were differences in age, days of fever, count of leukocytes, lymphocytes, and platelets, erythrocyte sedimentation rate (ESR), and hospital stay. In 25% of the KD group, there were alterations in the echocardiogram and, in the PIMS-K group, all patients received corticosteroids and 25 patients received intravenous immunoglobulin (IVIG). In both groups, a favorable clinical evolution was observed, characterized by the absence of complications and mortality. CONCLUSIONS: Based on the data obtained in our study, the importance of the epidemiological link is emphasized as an essential factor in differentiating between both pathologies, highlighting the need to consider factors such as age, duration of fever, count of leukocytes, lymphocytes, and platelets, and degree of cardiac involvement, for a differential evaluation between patients with PIMS-KD versus KD.

Parapharyngeal and retropharyngeal infections in children: Kawasaki disease needs vigilance.

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

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

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

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.

Kawasaki Disease Associated with SARS-CoV2 in a Pair of Triplets.

The etiology of Kawasaki disease (KD) and its precise genetic basics remain unknown. Genetic variants affecting immunity have been found in some patients. The occurrence of KD in siblings is rare, but KD pedigrees with multiple affected members have been described in Japan and North America. Cases in twins have been documented. We report 2 pairs of trizygotic triplets who developed KD associated with SARS-CoV2 infection from 2 different families. Our cases show that KD is multifactorial in origin, and both infectious etiology (particularly SARS-CoV2 as in our cases) and genetic factors are relevant in the disease.

The use of neutrophil-lymphocyte ratio for the prediction of refractory disease and coronary artery lesions in patients with Kawasaki disease.

BACKGROUND: Kawasaki disease is a vasculitis that can lead to cardiac complications, including coronary artery disease and cardiogenic shock. Various scoring systems have been developed to determine those that will be refractory to routine intravenous immunoglobulin therapy or develop coronary artery disease. The objective of this study was to determine if the neutrophil-lymphocyte ratio could predict refractory disease and coronary artery lesions in patients with Kawasaki disease. METHODS: A systematic review of the literature was performed to identify manuscripts describing comparisons of neutrophil-lymphocyte ratio between those who had refractory disease and those who did not, and between those who developed coronary artery lesions and those who did not. Mean difference was compared between groups. Areas under the curve were utilised to determine the pooled area under the curve. RESULTS: 12 studies with 5593 patients were included in the final analyses of neutrophil-lymphocyte ratio for the prediction of refractory disease. Neutrophil-lymphocyte ratio before therapy was higher in refractory disease with a mean difference of 2.55 (p < 0.01) and pooled area under the curve of 0.724. Neutrophil-lymphocyte ratio after therapy was higher in refractory disease with a mean difference of 1.42 (p < 0.01) and pooled area under the curve for of 0.803. Five studies with 1690 patients were included in the final analyses of neutrophil-lymphocyte ratio for the prediction of coronary artery lesions. Neutrophil-lymphocyte ratio before therapy was higher in coronary artery lesions with a mean difference of 0.65 (p < 0.01). CONCLUSION: The use of neutrophil-lymphocyte ratio may help physicians in the identification of patients at risk of refractory disease and coronary artery lesions in patients with Kawasaki disease.

Prediction of coronary artery lesions based on C-reactive protein levels in children with Kawasaki Disease: a retrospective cohort study.

OBJECTIVE: Since coronary artery lesions (CALs) are the most severe complication of Kawasaki disease (KD), clinically speaking, early prediction of CALs is crucial. The authors aimed to investigate the predictive value of C-reactive protein (CRP) in predicting CALs in KD patients. METHODS: KD patients were divided into the CALs group and the non-CALs group. The clinical and laboratory parameters were collected and compared. Multivariate logistic regression analysis was used to determine the independent risk factors of CALs. The receiver operating characteristic curve was applied to determine the optimal cut-off value. RESULTS: 851 KD patients who met the inclusion criteria were studied, including 206 in the CALs group and 645 in the non-CALs group. Children in the CALs group had significantly higher CRP levels than the non-CALs group (p < 0.05). Multivariable logistic regression analysis showed that incomplete KD, male, lower hemoglobin, and higher CRP were independent risk factors for predicting CAL (all p < 0.05). The optimal cut-off value of initial serum CRP for predicting CALs was 105.5 mg/L, with a sensitivity of 47.57% and a specificity of 69.61%. In addition, KD patients with high CRP (≥105.5 mg/L) had a higher occurrence of CALs than those with low CRP (<105.5 mg/L) (33% vs 19%, p < 0.001). CONCLUSION: The incidence of CALs was significantly higher in patients with high CRP. CRP is an independent risk factor for CALs formation and may be useful for predicting CALs in KD patients.

Clinical Presentation and Outcomes of Kawasaki Disease in Children from Latin America: A Multicenter Observational Study from the REKAMLATINA Network.

OBJECTIVES: To describe the clinical presentation, management, and outcomes of Kawasaki disease (KD) in Latin America and to evaluate early prognostic indicators of coronary artery aneurysm (CAA). STUDY DESIGN: An observational KD registry-based study was conducted in 64 participating pediatric centers across 19 Latin American countries retrospectively between January 1, 2009, and December 31, 2013, and prospectively from June 1, 2014, to May 31, 2017. Demographic and initial clinical and laboratory data were collected. Logistic regression incorporating clinical factors and maximum coronary artery z-score at initial presentation (between 10 days before and 5 days after intravenous immunoglobulin [IVIG]) was used to develop a prognostic model for CAA during follow-up (>5 days after IVIG). RESULTS: Of 1853 patients with KD, delayed admission (>10 days after fever onset) occurred in 16%, 25% had incomplete KD, and 11% were resistant to IVIG. Among 671 subjects with reported coronary artery z-score during follow-up (median: 79 days; IQR: 36, 186), 21% had CAA, including 4% with giant aneurysms. A simple prognostic model utilizing only a maximum coronary artery z-score ≥2.5 at initial presentation was optimal to predict CAA during follow-up (area under the curve: 0.84; 95% CI: 0.80, 0.88). CONCLUSION: From our Latin American population, coronary artery z-score ≥2.5 at initial presentation was the most important prognostic factor preceding CAA during follow-up. These results highlight the importance of early echocardiography during the initial presentation of KD.

Splenomegaly in Kawasaki Disease: A Pitfall in Diagnosis.

Among the 365 children diagnosed as having Kawasaki disease (KD), only 5 children (1.4%) presented with splenomegaly: 2 complicated by macrophage activation syndrome and 3 ultimately received a diagnosis of alternative systemic illness. Splenomegaly is atypical in KD and a potential marker of an underling complication, namely macrophage activation syndrome, or diagnosis other than 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.

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

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

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

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

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.

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.

Multisystem inflammatory syndrome in neonates associated with SARS-CoV-2 infection, a different entity?

BACKGROUND: Multisystemic inflammatory syndrome in children (MIS-C) is a novel disease that is associated with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV2). MIS-C usually affects children older than 5 years of age and adolescents, with a median of 8-years and an interquartile range of 3 to 11 years. A multisystemic inflammatory disease has been described in neonates and named MIS-N (multisystemic inflammatory syndrome in Neonates). We report three cases of Mexican newborns with MIS-N presenting with multiorgan compromise and a positive anti-SARS-CoV-2 IgG who developed Kawasaki disease (KD)-like cardiac features and discuss the current dilemma regarding diagnosis and treatment in these patients.

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.

Enfermedad tipo Kawasaki relacionado a COVID-19. Manejo perioperatorio de un caso pediátrico / Kawasaki-like disease related to COVID-19. Perioperative management of a pediatric case

The recently identified COVID-19-related Kawasaki-like disease has been considerad one of the phenotypes of the cardiovascular manifesta- tions of the Pediatric Multisystem Inflammatory Syndrome associated with SARS-CoV-2 (PIMS-TS), which stands out for few respiratory symptoms and múltiple manifestations cardiovascular, the most important being dilation and eventual coronary aneurysms associated or not with cardio- genic shock. The literature is scarce, so perioperative management is challenging for the anesthesiology team. We present the case of an infant with severe cardiovascular manifestations as a result of this disease that required anesthetic interventions to perform a plethysmography and amputation of his lower extremity. The article describes the most relevant considerations in the perioperative management of patients with this pathology.

La enfermedad tipo Kawasaki relacionada a COVID-19 recientemente identificada ha sido considerada como uno de los fenotipos de las manifestaciones cardiovasculares del Síndrome Inflamatorio Multisistémico Pediátrico asociado a SARS-CoV-2 (PIMS-TS), que destaca por escasos síntomas respiratorios y múltiples manifestaciones cardiovasculares, siendo la más importante, la dilatación y eventuales aneurismas coronarios asociados o no a shock cardiogénico. La bibliografía es escasa, por lo que, el manejo perioperatorio es desafiante para el equipo anestesiológico. Presentamos el caso de un lactante con manifestaciones cardiovasculares severas producto de esta enfermedad que requirió intervenciones anestésicas para la realización de una pletismografía y la amputación de su extremidad inferior. El artículo describe las consideraciones más relevantes en el manejo perioperatorio de los pacientes con esta patología.

Kawasaki Disease Associated with Covid-19 in a Pediatric Patient.

Kawasaki disease (KD) is a multisystemic vasculitis of small and medium-size vessels that mainly affects children under five years of age. The etiology of KD is still uncertain; however, evidence suggests that infectious agents and genetic susceptibility act as a trigger for its development, which could explain the increase in cases of children with COVID-19 who developed a classic or incomplete form of KD. The aim of this report is to discuss a case of a five-year-old boy diagnosed with incomplete KD associated with COVID-19.

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

Protocolo de atención y manejo de la enfermedad de Kawasaki / Protocol for care and management of Kawasaki disease

OBJETIVO: El objetivo de este estudio es la elaboración de un protocolo para el diagnóstico y el abordaje de la enfermedad de Kawasaki, basado en la evidencia científica disponible. METODOLOGÍA: Realización de una revisión sistemática para localizar, sintetizar y analizar la evidencia científica disponible. Las bases de datos interrogadas fueron: MEDLINE (mediante OVID), EMBASE, Web of Science (WOS), Epistemonikos database, Cochrane Library y páginas web específicas de guías de práctica clínica, con los términos "'mucocutaneous lymph node síndrome", "Kawasaki disease" y "Kawasaki syndrome". Así mismo, se realizó un cuestionario telemático a pacientes y familiares. Con la información localizada y las principales cuestiones reflejadas por los pacientes, se elaboraron una serie de recomendaciones. 168 recomendaciones se sometieron a votación mediante metodología Delphi por un panel de expertos multidisciplinar, implicados en la atención a los pacientes de esta patología. RESULTADOS: Se obtuvieron un total de 165 recomendaciones para el diagnóstico, tratamiento y seguimiento, con consenso del panel de expertos. Dichas recomendaciones quedaron recogidas en un protocolo asistencial, y gráficamente en 2 algoritmos para facilitar al personal sanitario la comprensión y aplicación de las recomendaciones seleccionadas. CONCLUSIONES: La enfermedad de Kawasaki sigue siendo una enfermedad poco conocida, pero controlable con un diagnóstico, tratamiento temprano y seguimiento adecuado. Para lo cual se hace imprescindible la unificación de criterios en el manejo de estos pacientes. Los protocolos clínicos son una valiosa herramienta que aporta información a los profesionales y ayuda en la toma de decisiones.

OBJETIVE: The aim of this study is to develop a protocol for the diagnosis and management of Kawasaki disease, based on the available scientific evidence. METHODOLOGY: A systematic review was carried out to locate, synthesise and analyse the available scientific evidence. The databases searched were: MEDLINE (via OVID), EMBASE, Web of Science (WOS), Epistemonikos database, Cochrane Library and specific web pages of clinical practice guidelines, with the terms "'mucocutaneous lymph node syndrome", "Kawasaki disease" and "Kawasaki syndrome". A telematic questionnaire was also administered to patients and relatives. A series of recommendations were drawn up based on the information collected and the main issues raised by patients. 168 recommendations were voted on using Delphi methodology by a panel of multidisciplinary experts involved in the care of patients with this pathology. RESULTS: A total of 165 recommendations for diagnosis, treatment and follow-up were obtained, with the consensus of the panel of experts. These recommendations were set out in a care protocol, and graphically in 2 algorithms to make it easier for healthcare personnel to understand and apply the selected recommendations. CONCLUSIONS: Kawasaki disease remains a poorly understood disease, but controllable with diagnosis, early treatment and appropriate followup. For this reason, it is essential to unify criteria in the management of these patients. Clinical protocols are a valuable tool that provides information to professionals and helps in decision-making.