OBJECTIVE: 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.
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.
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.

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.
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.
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.
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.
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.
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.

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).
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.

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.

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.

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).
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.
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).
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.
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).
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.
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).
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.

BACKGROUND: 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.
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.

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.
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.
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.
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.
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.
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.
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.
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.

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.

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.
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.
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.
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.