Role and mechanisms of CHI3L1 in coronary artery lesions in a mouse model of Kawasaki disease-like vasculitis / 中国当代儿科杂志

OBJECTIVES@#To explore the role and potential mechanisms of chitinase-3-like protein 1 (CHI3L1) in coronary artery lesions in a mouse model of Kawasaki disease (KD)-like vasculitis.@*METHODS@#Four-week-old male SPF-grade C57BL/6 mice were randomly divided into a control group and a model group, with 10 mice in each group. The model group mice were intraperitoneally injected with 0.5 mL of lactobacillus casei cell wall extract (LCWE) to establish a mouse model of KD-like vasculitis, while the control group mice were injected with an equal volume of normal saline. The general conditions of the mice were observed on the 3rd, 7th, and 14th day after injection. Changes in coronary artery tissue pathology were observed using hematoxylin-eosin staining. The level of CHI3L1 in mouse serum was measured by enzyme-linked immunosorbent assay. Immunofluorescence staining was used to detect the expression and localization of CHI3L1, von Willebrand factor (vWF), and α-smooth muscle actin (α-SMA) in coronary artery tissue. Western blot analysis was used to detect the expression of CHI3L1, vWF, vascular endothelial cadherin (VE cadherin), Caspase-3, B cell lymphoma-2 (Bcl-2), Bcl-2 associated X protein (Bax), nuclear factor κB (NF-κB), and phosphorylated NF-κB (p-NF-κB) in coronary artery tissue.@*RESULTS@#The serum level of CHI3L1 in the model group was significantly higher than that in the control group (P<0.05). Compared to the control group, the expression of CHI3L1 in the coronary artery tissue was higher, while the expression of vWF was lower in the model group. The relative expression levels of CHI3L1, Bax, Caspase-3, NF-κB, and p-NF-κB were significantly higher in the model group than in the control group (P<0.05). The relative expression levels of vWF, VE cadherin, and Bcl-2 were lower in the model group than in the control group (P<0.05).@*CONCLUSIONS@#In the LCWE-induced mouse model of KD-like vasculitis, the expression levels of CHI3L1 in serum and coronary arteries increase, and it may play a role in coronary artery lesions through endothelial cell apoptosis mediated by inflammatory reactions.

Platelet-to-lymphocyte ratio as a biomarker for predicting coronary artery lesions in Chinese children with Kawasaki disease: a Meta analysis / 中国当代儿科杂志

OBJECTIVES@#To systematically evaluate the value of the platelet-to-lymphocyte ratio (PLR) in predicting coronary artery lesions (CAL) in Chinese children with Kawasaki Disease (KD).@*METHODS@#A comprehensive search was conducted in databases including PubMed, Embase, Cochrane Library, Web of Science, China National Knowledge Infrastructure, Wanfang Data, China Biomedical Literature Database, and China Science and Technology Journal Database from inception to December 2022. The quality of the included literature was assessed using the Newcastle-Ottawa Scale, and a Meta analysis was performed using Stata 15.1.@*RESULTS@#A total of ten published reports, involving 3 664 Chinese children with KD, were included in this Meta analysis, of whom 1 328 developed CAL. The Meta analysis revealed a sensitivity of 0.78 (95%CI: 0.71-0.83), specificity of 0.71 (95%CI: 0.61-0.80), overall diagnostic odds ratio of 8.69 (95%CI: 5.02-15.06), and an area under the curve of the summary receiver operating characteristic of 0.82 (95%CI: 0.78-0.85) for PLR in predicting CAL in the children with KD. The sensitivity, specificity, and area under the curve of summary receiver operating characteristic were lower for PLR alone compared to PLR in combination with other indicators. Sensitivity analysis demonstrated the stability of the Meta analysis results with no significant changes upon excluding individual studies. However, a significant publication bias was observed (P<0.001).@*CONCLUSIONS@#PLR demonstrates certain predictive value for CAL in Chinese children with KD.

Pediatric expert consensus on the application of aspirin in Kawasaki disease / 中国当代儿科杂志

Kawasaki disease (KD) is one of the common acquired heart diseases in children aged <5 years and is an acute systemic vasculitis. After nearly 60 years of research, intravenous immunoglobulin combined with oral aspirin has become the first-line treatment for the prevention of coronary artery lesion in acute KD; however, there are still controversies over the role and optimal dose of aspirin. The consensus was formulated based on the latest research findings of KD treatment in China and overseas and comprehensive discussion of pediatric experts in China and put forward recommendations on the dose, usage, and course of aspirin treatment in the first-line treatment of KD.

Deformación sistólica longitudinal del ventrículo izquierdo en pacientes pediátricos con antecedente de enfermedad de Kawasaki / Left ventricular longitudinal systolic strain in children with history of Kawasaki disease

Resumen Objetivo La enfermedad de Kawasaki (EK) es una vasculitis sistémica cuya complicación más grave es la formación de lesiones coronarias, las cuales pueden llevar a infarto del miocardio y muerte súbita. El estudio ecocardiográfico es parte del seguimiento obligado de los pacientes con EK. La deformación sistólica longitudinal (DSL) medida mediante speckle tracking es una herramienta precisa para evaluar la función de la fibra miocárdica (longitudinal) del ventrículo izquierdo. No se ha establecido la utilidad en la práctica clínica de la DSL en niños con antecedente de EK. El objetivo de este estudio fue analizar si la presencia de lesiones coronarias condiciona alteraciones en la DSL segmentaria y su correspondencia con el territorio coronario en donde se encuentra la lesión. Método Serie de casos. Se realizó un estudio ecocardiográfico completo y la evaluación de la DSL a niños con antecedente de EK al menos 6 meses después de la fase aguda. Resultados Se estudiaron 9 pacientes. La mediana de edad fue de 6 años (mínimo 2 y máximo 17). El 56% era de sexo masculino. El 77% presentó aneurismas coronarios. La DSL resultó alterada en el 56% de la muestra estudiada. Dentro de los pacientes que presentaron una DSL anormal, todos mostraron aneurismas coronarios y lesiones estenóticas u oclusivas demostradas mediante cateterismo de arterias coronarias, además de alteraciones de la perfusión miocárdica en estudio de Medicina Nuclear. Conclusiones En la muestra estudiada, los pacientes en quienes se encontró una DSL anormal, resultaron tener lesiones coronarias que condicionaban isquemia o infarto.

Abstract Objective Kawasaki disease (KD) is a systemic vasculitis that affects young children. Coronary artery aneurisms, ectasia and stenosis are its main complications and may lead to ischemic heart disease or sudden death. Echocardiography evaluation it's mandatory in all patients with history of KD. Left ventricular longitudinal systolic strain (LVLSS) measured by speckle tracking it's an accurate tool to evaluate global and segmental left ventricle mechanics. Clinical utility of LVLSS in children with KD hasn't been established. The goal of this study was to analyse if the presence of coronary lesions alters segmental LVLSS and if there is a relationship with the affected coronary territory. Method Case series. A complete transthoracic echocardiography with LVLSS was performed in children with history of KD with at least 6 months after the acute phase. Results Nine patients where studied, with a median age of 6 years (minimum 2 and maximum 17). A percentage of 56 were male, and 77% had coronary aneurisms. An abnormal LVLSS was found in 56% of the population studied. All of the patients that had an abnormal LVLSS had coronary aneurisms with stenosis or complete occlusion confirmed by invasive coronary angiography and abnormal Nuclear Medicine perfusion scans. Conclusions On the population studied, all patients with an abnormal LVLSS had obstructive coronary lesions and ischemic heart disease.

Drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) / Reação a drogas com eosinofilia e sintomas sistêmicos (síndrome DRESS)

SUMMARY Objective: To review the hypersensitivity reaction to drugs known as drug reaction with eosinophilia and systemic symptoms (DRESS syndrome), based on a case report. We also intend to discuss the difficulty and importance of disease recognition, since none of the changes is pathognomonic of this disease and failure to identify it may have disastrous consequences for the patient. Method: To describe this case report, in addition to the information collected for clinical assessment, a literature review was performed in the PubMed and Bireme databases in order to retrieve the latest information published in literature on DRESS syndrome. Results: The case of a 20-year old patient is reported. After anamnesis, physical examination and laboratory tests a diagnosis of DRESS syndrome was performed, characterized by rash, hematologic alterations, lymphadenopathy and lesions in target organ. This is a rare syndrome, whose frequency varies according to the drug used and the immune status of the patient, being more often associated with the use of anticonvulsants. Conclusion: The approach and discussion of the topic are of paramount importance, in view of the potential lethality of this treatable syndrome. Recognizing the occurrence of DRESS syndrome and starting treatment as soon as possible is crucial to reduce the risk of mortality and improve prognosis.

RESUMO Objetivo: fazer uma revisão da reação de hipersensibilidade a drogas denominada reação a drogas com eosinofilia e sintomas sistêmicos (síndrome DRESS), com base em um relato de caso clínico. Pretende-se ainda discutir a dificuldade e importância de seu reconhecimento, uma vez que nenhuma alteração dessa doença é patognomônica e sua não identificação pode trazer consequências desastrosas para o paciente. Método: para descrever este relato, além das informações coletadas no caso clínico, uma revisão da literatura nas bases de dados PubMed e Bireme foi realizada com o intuito de rever as informações mais recentes publicadas na literatura acerca da síndrome DRESS. Resultados: relatou-se o caso de uma paciente de 20 anos de idade que, após anamnese, exame físico e exames laboratoriais, foi diagnosticada com síndrome DRESS, caracterizada por erupção cutânea, alterações hematológicas, linfonodomegalia e lesões em órgão-alvo. É uma síndrome rara, cuja frequência varia conforme a droga utilizada e o estado imunológico do indivíduo, sendo mais associada ao uso de anticonvulsivantes. Conclusão: a abordagem e discussão do tema são de extrema importância, tendo em vista o potencial de letalidade dessa síndrome, que possui tratamento. Reconhecer precocemente a DRESS e instituir terapêutica é fundamental para reduzir o risco de mortalidade e melhorar o prognóstico.

Riesgo de afectación coronaria en la enfermedad de Kawasaki / Risk of coronary artery involvement in Kawasaki disease

Introducción: La enfermedad de Kawasaki es una vasculitis sistémica con riesgo de afectación coronaria. Nuestro objetivo es identificar los factores de riesgo asociados a la afectación coronaria en pacientes con enfermedad de Kawasaki completa e incompleta. Material y métodos: Estudio descriptivo retrospectivo de los pacientes diagnosticados con enfermedad de Kawasaki en un hospital terciario entre 2008 y 2014. Se utilizaron los criterios diagnósticos de la Asociación Americana de Cardiología para definir la enfermedad de Kawasaki en su forma completa e incompleta. Resultados: Treinta y un niños fueron diagnosticados con enfermedad de Kawasaki; 24 cumplían criterios para la forma completa y 7, para la incompleta. Cinco presentaron afectación coronaria. Uno de ellos presentaba enfermedad de Kawasaki incompleta (1/7= 14,3%), y los 4 restantes, enfermedad de Kawasaki completa (4/24= 16,7%). No se encontraron diferencias significativas en el riesgo de afectación coronaria entre ambos grupos (p= 1,0). Los pacientes con afectación coronaria tenían una proteína C reactiva mayor (mediana: 16,2 mg/dl vs. 8,4 mg/dl; p= 0,047) y una menor albuminemia (mediana: 3,2 mg/dl vs. 3,99 mg/dl; p= 0,002). Conclusiones: El riesgo de afectación coronaria de la enfermedad de Kawasaki incompleta es similar al de la enfermedad de Kawasaki completa, por lo que, en pacientes con la forma incompleta de la enfermedad, no se debería demorar el tratamiento con inmunoglobulina. En nuestra población, los valores de proteína C reactiva y de albúmina se relacionan con un mayor riesgo de afectación coronaria.

Introduction: Kawasaki disease refers to systemic vasculitis with risk of coronary artery disease. Our objective is to identify risk factors associated with coronary artery disease in patients with complete and incomplete Kawasaki disease. Material and methods: Descriptive, retrospective study conducted in patients diagnosed with Kawasaki disease in a tertiary-care hospital between 2008 and 2014. The American Heart Association diagnostic criteria were used to define complete and incomplete Kawasaki disease. Results: Thirty-one children were diagnosed with Kawasaki disease; 24 met the criteria for the complete form, and 7, for the incomplete form of this condition. Five had coronary artery disease. One of them had incomplete Kawasaki disease (1/7= 14.3%), and the remaining four had the complete form (4/24= 16.7%). No significant differences were found between both groups (p= 1.0). Patients with coronary artery involvement had a higher C-reactive protein level (median: 16.2 mg/dL versus 8.4 mg/dL, p= 0.047) and lower albuminemia (median: 3.2 mg/dL versus 3.99 mg/dL, p= 0.002). Conclusions: The risk of coronary artery involvement in incomplete Kawasaki disease is similar to that in complete Kawasaki disease; therefore, in patients with the incomplete form, immunoglobulin therapy should not be delayed. In our population, C-reactive protein and albumin levels were related to a higher risk of coronary artery involvement.

Síndrome de Kawasaki. Persistencia de la fiebre luego del tratamiento / Kawasaki syndrome. Fever persistence after treatment

El síndrome de Kawasaki es un trastorno multisistémico, de etiología desconocida, que afecta a niños menores de 5 años. Presentamos el caso de un niño de 2 años con síndrome de Kawasaki, que recibió tratamiento con inmunoglobulina en infusión única y ácido acetilsalicílico, con respuesta rápida y favorable del cuadro cutáneo pero persistencia de la fiebre y reaparición del exantema a las 72 horas, por lo que se debió repetir la administración de inmunoglobulina. El ecocardiograma inicial revelaba dilatación de la arteria coronaria izquierda de 1,6 vez el diámetro del vaso adyacente.

Coronary Artery Dilatation in Incomplete Kawasaki Disease.

We conducted this study to compare the incidence of coronary artery dilatation in children with Incomplete and Classical Kawasaki disease, diagnosed as per AHA criteria. Subjects were included on a retrospective review of records (2002-2007); those with a discharge diagnosis of Kawasaki disease were enrolled. A total of 29 patients were identified (3.1 per 1000 pediatric admissions), out of which 22 were boys (median age: 4.8 years; range: 4 months-11 years). Seventeen (58.6%) had Classical KD and twelve (41.4%) children had Incomplete KD. All children received IVIG and underwent echocardiography. Coronary involvement was more in Incomplete KD (11/12 = 91.6 %) as compared to Classical KD (7/17= 41.1%). The sensitivity, specificity and predictive value of AHA criteria to detect coronary artery dilatation was 39%, 9%, and 41%, respectively. We conclude that children presenting with Incomplete Kawasaki disease are at a higher risk of coronary artery abnormalities.

Síndrome de kawasaki: presentación de un caso poco común / Kawasaki syndrome: presentation of an uncommon case

La enfermedad de Kawasaki es un proceso vasculítico multisistémico cuya etiología es poco conocida. La presentación clínica es florida y su evolución está condicionada al inicio temprano de la terapia específica con gammaglobulinas, de allí la importancia de un diagnóstico precoz. Habitualmente estos casos cursan con dilatación del lecho arterial coronario siendo excepcional la aparición de alteraciones a nivel de arterias de mayor calibre, menos común aneurismas a nivel cerebral, que confiere un peor pronóstico para estos pacientes. Presentamos el caso de lactante de 3 meses de edad con aneurisma de ambas arterias coronarias, arteria subclavia izquierda, cerebral media derecha y ambas Iliacas, y obstrucción de las mismas.

Kawasaki disease, a multisystem vasculitis of unknown etiology can present in various ways. It is crucial to make an early diagnosis, and consequently give gamma globulin in order to abort its insidious evolution and not infrequently fatal outcome. Aneurysms and obstruction of the coronary arteries are the most characteristic presentations. Other large vessels may be involved, with the cerebral circulation being affected least often but having the worst prognosis. We present a clinical case of a 3 month old breast fed infant with aneurysms and obstructions in both coronary arteries, the left subclavian, right middle cerebral, and both iliac arteries.

Kawasaki disease in Jordanian children

Nineteen children with signs and symptoms suggesting Kawasaki Disease [KD] were admitted to the pediatric ward at Jordan University Hospital in the period from February 1997 to September 2001. To evaluate our experience in diagnosing and managing children with KD, the clinical notes, investigations including ESR, platelets count, ECG, echocardiogram and drugs used for those children were studied. Their ages ranged from 40 days to 12 yrs, and 70% were aged up to 2 years. Male to female ratio was 8 to 1. Seventeen patients fulfilled the diagnostic criteria of KD and two had atypical KD. All received 2 gm/kg IV infusion of gamma globulin over 12 hrs, and 80-100 mg /kg/ day aspirin. One patient, received prednisone, 52% had cardiac involvement, 26% had coronary artery aneurysms and four had pericardial effusion. All except three had complete recovery, two continued to have coronary artery aneurysms and one died [5%]. Early management is important for good prognosis in KD. Further national multi-center studies are recommended to evaluate the incidence and the outcome of KD in Jordan

Enfermedad de Kawasaki: primer caso diagnósticado en el Hospital Central de San Cristobal Estado Táchira / Kawasaki disease: case first diagnostic in the Hospital Central of San Cristobal Tachira State

La enfermedad de Kawasaki o Síndrome Mucocutaneo de Ganglios Linfáticos, es un síndrome febril agudo de etiología desconocida que se presenta en lactantes y niños pequeños y que se caracteriza por fiebre, alteraciones cutaneo-mucosas, tumefacción de ganglios linfáticos cervicales y en casos graves compromisos cardíaco que puede llevar a la muerte del paciente si no se detecta y se trata a tiempo. Se reporta el primer caso diagnosticado en el HCSC, Estado Táchira. Lactantes mayor femenino de 16 meses de edad, presenta de 1 mes de evolución lesiones eritematosas generalizadas y posteriormente descamativas, acompañadas de hipertermica no cuantificada y conjuntivitis por lo cual acude; se detecta soplo cardíaco grado II/IV. Ecocardiograma reporta Miocarditis Coronaria Derecha Dilatada. Se instala tratamiento con Gammaglobulina y ASA. Paciente egresa en buenas condiciones generales con tratamiento y control cardiológico ambulatorio

Enfermedad de Kawasaki: Pautas Terapéuticas. Revisión Bibliográfica / Kawasaki disease: therapeutic guidelines bibliographic review

Se revisa la bibliografía internacional sobre la base de la terapeútica óptima para aquellos niños afectados por la enfermedad de Kawasaki

Diagnóstico Diferencial. Enfermedad de Kawasaki: Enfoque Práctico / Differential Diagnosis: Kawasaki disease. Practical approach

Se expone de una manera práctica para el clínico pediatra aquellos aspectos que permiten un diagnóstico diferencial de la enfermedad de Kawasaki con otras entidades comunes en Pediatría

Kawasaki syndrome.

Kawasaki and associates first described a mucocutaneous lymph node syndrome from Japan. Since then this febrile illness has been reported from several countries. The patient described in this report had all the clinical manifestations of this entity excepting coronary aneurysms.