Clinical analysis of 5 cases of systemic juvenile idiopathic arthritis with coronary artery dilatation / 中华儿科杂志

Objective: To investigate the clinical characteristics of systemic juvenile idiopathic arthritis combined with coronary artery dilatation. Methods: A retrospective analysis was performed on the clinical data, including clinical manifestations, blood routine, inflammatory factors, echocardiography, vascular ultrasound and CT angiography, treatment and outcomes, etc, of 5 cases with systemic juvenile idiopathic arthritis combined with coronary artery dilation admitted to Department of Rheumatology in the affiliated Children's Hospital of Capital Institute of Pediatrics from May 2019 to June 2021. Results: There were 2 males and 3 females among 5 cases. The onset age ranged from 7 months to 4 years 7 months.The diagnostic time ranged from 1.5 months to 3.0 months.Four cases were diagnosed as atypical Kawasaki disease. Three cases showed unilateral coronary artery dilation.Two cases showed bilateral coronary artery dilation.Four cases developed multiple organ injuries.Three cases developed macrophage activation syndrome.Three cases developed lung injury.Two cases developed pericardial effusion.One case developed pulmonary hypertension.As for treatment, 3 cases treated with methylprednisolone pulse therapy and methotrexate combined with cyclosporine, improved after the final application of biological agents, and have stopped prednisone. The other 2 cases were treated with adequate oral prednisone and gradually reduced, and methotrexate was added at the same time, 1 case relapsed in the process of reduction. No other vascular involvement was found in 5 cases. Coronary artery dilation recovered completely after 1 to 3 months of treatment. Conclusions: Systemic juvenile idiopathic arthritis combined with coronary artery dilatation has the clinical characteristics of small onset age, long diagnostic time, prone to multiple organ injuries. Corticosteroids and conventional immunosuppressive agents are not sensitive, and biological agents should be used as soon as possible.The prognosis of coronary artery dilation is good after timely treatment.

Retrospective analysis of infliximab in the treatment of Kawasaki disease / 中华儿科杂志

Objective: To investigate the efficacy and safety of infliximab (IFX) therapy for children with Kawasaki disease. Methods: Sixty-eight children with Kawasaki disease who received IFX therapy in Children's Hospital of Fudan University from January 2014 to April 2021 were enrolled. The indications for IFX administration, changes in laboratory parameters before and after IFX administration, response rate, drug adverse events and complications and outcomes of coronary artery aneurysms (CAA) were retrospectively analyzed. Comparisons between groups were performed with unpaired Student t test or Mann-Whitney U test or chi-square test. Results: Among 68 children with Kawasaki disease, 52 (76%) were males and 16 (24%) were females. The age of onset was 2.1 (0.5, 3.8) years. IFX was administered to: (1) 35 children (51%) with persistent fever who did not respond to intravenous immunoglobulin (IVIG) or steroids, 28 of the 35 children (80%) developed CAA before IFX therapy; (2) 32 children (47%) with continuous progression of CAA; (3) 1 child with persistent arthritis. In all cases, IFX was administered as an additional treatment (the time from the onset of illness to IFX therapy was 21 (15, 30) days) which consisted of second line therapy in 20 (29%), third line therapy in 20 (29%), and fourth (or more) line therapy in 28 (41%). C-reactive protein (8 (4, 15) vs. 16 (8, 43) mg/L, Z=-3.38, P=0.001), serum amyloid protein A (17 (10, 42) vs. 88 (11, 327) mg/L, Z=-2.36, P=0.018) and the percentage of neutrophils (0.39±0.20 vs. 0.49±0.21, t=2.63, P=0.010) decreased significantly after IFX administration. Fourteen children (21%) did not respond to IFX and received additional therapies mainly including steroids and cyclophosphamide. There was no significant difference in gender, age at IFX administration, time from the onset of illness to IFX administration, the maximum coronary Z value before IFX administration, and the incidence of systemic aneurysms between IFX-sensitive group and IFX-resistant group (all P>0.05). Infections occurred in 11 cases (16%) after IFX administration, including respiratory tract, digestive tract, urinary tract, skin and oral infections. One case had Calmette-Guérin bacillus-related adverse reactions 2 months after IFX administration. All of these adverse events were cured successfully. One child died of CAA rupture, 6 children were lost to follow up, the remaining 61 children were followed up for 6 (4, 15) months. No CAA occurred in 7 children before and after IFX treatment, while CAA occurred in 54 children before IFX treatment. CAA regressed in 23 (43%) children at the last follow-up, and the diameter of coronary artery recovered to normal in 10 children. Conclusion: IFX is an effective and safe therapeutic choice for children with Kawasaki disease who are refractory to IVIG or steroids therapy or with continuous progression of CAA.

Association between duration of fever before treatment and intravenous immunoglobulin resistance in Kawasaki disease / 中国当代儿科杂志

OBJECTIVES@#To examine the association between duration of fever before intravenous immunoglobulin (IVIG) treatment and IVIG resistance in children with Kawasaki disease (KD).@*METHODS@#A retrospective analysis was performed on the medical data of 317 children with KD who were admitted from January 2018 to December 2020. According to the duration of fever before IVIG treatment, they were divided into two groups: short fever duration group (≤4 days) with 92 children and long fever duration group (>4 days) with 225 children. According to the presence or absence of IVIG resistance, each group was further divided into a drug-resistance group and a non-drug-resistance group. Baseline data and laboratory results were compared between groups. A multivariate logistic regression analysis was used to identify the influencing factors for IVIG resistance.@*RESULTS@#In the short fever duration group, 19 children (20.7%) had IVIG resistance and 5 children (5.4%) had coronary artery aneurysm, and in the long fever duration group, 22 children (9.8%) had IVIG resistance and 19 children (8.4%) had coronary artery aneurysm, suggesting that the short fever duration group had a significantly higher rate of IVIG resistance than the long fever duration group (P<0.05), while there was no significant difference in the incidence rate of coronary artery aneurysm between the two groups (P>0.05). In the short fever duration group, compared with the children without drug resistance, the children with drug resistance had a significantly lower level of blood sodium and significantly higher levels of procalcitonin, C-reactive protein, and N-terminal B-type natriuretic peptide before treatment (P<0.05). In the long fever duration group, the children with drug resistance had significantly lower levels of blood sodium and creatine kinase before treatment than those without drug resistance (P<0.05). The multivariate logistic regression analysis showed that a reduction in blood sodium level was associated with IVIG resistance in the long fever duration group (P<0.05).@*CONCLUSIONS@#IVIG resistance in children with KD varies with the duration of fever before treatment. A reduction in blood sodium is associated with IVIG resistance in KD children with a duration of fever of >4 days before treatment.

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

Kawasaki disease (KD) is one of the common acquired heart diseases in under-5-year-old children and is an acute self-limiting vasculitis. After nearly 60 years of research, intravenous immunoglobulin combined with oral aspirin has become the first-line treatment for preventing coronary artery aneurysm in the acute stage of KD. However, glucocorticoid (GC), infliximab, and other immunosuppressants are options for the treatment of KD patients with a high risk of coronary artery aneurysm, no response to intravenous immunoglobulin and a confirmed diagnosis of coronary artery aneurysm. At present, there are still controversies over the use of GC in the treatment of KD. With reference to the latest research findings of KD treatment in China and overseas, this consensus invited domestic pediatric experts to fully discuss and put forward recommendations on the indications, dosage, and usage of GC in the first-line and second-line treatment of KD.

Surgical Treatment for a Super-Giant Right Coronary Artery Aneurysm Induced by Kawasaki Disease

Abstract A 32-year-old man diagnosed with Kawasaki disease at the age of three years presented with coronary artery aneurysm (CAA). The aneurysm increased in size, and the patient was referred to our hospital for surgery. Preoperative computed tomography scan showed a super-giant right CAA and giant left CAAs; surgery was performed. The super-giant right CAA was resected, and the ostium of the right coronary artery was closed; then, coronary artery bypass grafting was performed. The left CAAs were not treated surgically because the risk of rupture was low. Here, we describe the successful surgical treatment of a right super-giant CAA.

Aneurisma coronario secundario a enfermedad de Kawasaki en paciente adulto: caso Clínico / Coronary aneurysm secondary to Kawasaki disease in an adult: report of one case

Kawasaki disease (KD) can be a primary cause of an acute coronary syndrome in young adults, with or without a history of KD during childhood. We report a 31year old female, admitted for acute chest pain. She had negative T waves on DIII and AVF leads of the electrocardiogram. Troponins were positive. A cardiac magnetic resonance was compatible with an acute inferior myocardial infarction. In the coronary angiography she had a calcified complete occlusion of the proximal right coronary artery. The left coronary artery had no visible lesions. A computed angiogram showed an extensive calcified and thrombotic aneurysm of the right coronary artery, compatible with Kawasaki disease (KD). The patient was discharged seven days after admission in good conditions.

Enfermedad de Kawasaki: ¿una entidad exclusiva de niños? / Kawasaki disease, is it exclusive of children?

Resumen La enfermedad de Kawasaki es un síndrome febril agudo y autolimitado, de presunta etiología autoinmune, que característicamente afecta a niños menores de 5 años. Esta enfermedad consiste en una vasculitis aguda de pequeños y medianos vasos que favorece la formación de aneurismas en las arterias coronarias. El diagnóstico en la fase aguda es esencialmente clínico, mientras que en la fase crónica suele hacerse al documentar las secuelas vasculares. La importancia de la identificación apropiada de esta enfermedad radica en que muchos de los infartos fatales y no fatales en personas menores de 40 años tienen hallazgos compatibles con este diagnóstico. Se presenta el caso de un adulto joven con dolor torácico y angiotomografía coronaria con hallazgos compatibles con secuelas de enfermedad de Kawasaki del adulto.

Abstract Kawasaki disease is an acute and self-limited febrile syndrome with a presumed autoimmune etiology that characteristically affects children under 5 years of age. Kawasaki disease is an acute vasculitis of small and medium vessels that facilitates the formation of coronary aneurysms. The diagnosis of the disease during the acute phase is essentially clinical, while the diagnosis of Kawasaki disease during the chronic phase is performed when the vascular complications are detected. The importance of proper identification of Kawasaki disease lies in the fact that many of the fatal and non-fatal infarcts in young adults (<40 years) have findings compatible with this diagnosis. This article describes the case of a young patient with chest pain and coronary angiotomography showing sequelae of Kawasaki disease in the adult.

Aneurisma coronario de localización inusual asociado a infarto agudo de miocardio / A coronary aneurysm in an unusual location associated with acute myocardial infarction

Resumen Se presenta un caso clínico de difícil abordaje terapéutico. Paciente masculino joven con obesidad tipo II, quien ingresa por síndrome coronario agudo con elevación del segmento ST, se realiza coronariografía que pone en evidencia un aneurisma ubicado en el tronco coronario con imagen sugestiva de trombo en su interior que se trató con inhibidor de glicoproteína IIb/IIIa. En la evolución presenta falla cardiaca aguda manejada con inotrópicos y vasopresores. En segunda coronariografía se observó desaparición completa del componente trombótico. Como prevención secundaria se deja tratamiento antiagregante plaquetario y antitrombótico. El manejo de estos pacientes a la fecha es un reto ante la posibilidad de presentar un nuevo evento trombótico por la alteración en el flujo laminar intracoronario.

Abstract We present a clinical case with difficult treatment. A young male patient with type II obesity was admitted due to acute coronary syndrome with ST elevation. Coronariography showed an aneurysm on the coronary trunk suggestive of having a thrombus inside. It was treated with a glycoprotein IIb/IIIa antagonist. The patient progressed to acute heart failure managed with inotropes and vasopressors. A second coronariography showed complete disappearance of the thrombotic component. He was prescribed antiplatelet and antithrombotic treatment as secondary prevention. To date, the management of these patients has been a challenge, given the possibility of experiencing a new thrombotic event due to altered intracoronary laminar flow.

A Associação da Relação Proteína C-Reativa/Albumina em Pacientes com Ectasia da Artéria Coronária Isolada / Association of C-Reactive Protein to Albumin Ratio in Patients with Isolated Coronary Artery Ectasia

Resumo Fundamento A ectasia da artéria coronária (EAC) é definida como a dilatação difusa ou localizada do lúmen da artéria coronária com diâmetro de 1,5 a 2,0 vezes o diâmetro da artéria coronária normal adjacente. A relação proteína C-reativa/albumina (CAR, sigla em inglês) é um marcador inflamatório útil que tem sido documentado em doença arterial coronariana. Objetivo Analisar a associação entre a EAC e a CAR. Métodos Um protocolo caso-controle foi utilizado neste estudo. Foram incluídos 102 pacientesconsecutivos com EAC isolada sem estenose (56 homens e 46 mulheres; idade média de 60,4 ± 8,8 anos). O grupo controle era constituido pelo mesmo número de pacientes pareados por sexo e idade com artérias coronárias normais (55 homens e 47 mulheres; idade média de 61,2 ± 9,1 anos). Características clínicas, achados laboratoriais e histórico de uso de medicamentos foram registrados. Foram realizados teste t de Student, teste U de Mann-Whitney, teste do qui-quadrado, análise de regressão linear e logística. Foi considerado estatisticamente significativo p bilateral < 0,05. Resultados A CAR estava aumentada nos pacientes com EAC em comparação com os controles (32 e 16; p < 0,001). Além disso, foi verificado que a CAR era um preditor independente da EAC (razão de chances = 2,202; intervalo de confiança 95%, 1,184 - 5,365; p < 0,001). Conclusão No presente estudo, determinamos que os níveis da CAR estavam significativamente mais altos no grupo EAC que no grupo controle e a CAR estava significativamente correlacionada com a EAC. (Arq Bras Cardiol. 2020; [online].ahead print, PP.0-0)

Abstract Background Coronary artery ectasia (CAE) is defined as diffuse or localized dilatation of coronary artery lumen with a diameter of 1.5 to 2.0 times the adjacent normal coronary artery. The C-reactive protein to albumin ratio (CAR) is a useful inflammatory marker, which has been documented in coronary artery disease. Objective To analyze the association of CAE and CAR. Methods A case-control protocol was used in this study. We included 102 consecutive patients with isolated CAE without stenosis (56 men and 46 women; mean age 60.4 ± 8.8 years). The control subjects consisted of an equal number of sex and age matched patients with normal coronary arteries (55 men and 47 women; mean age 61.2 ± 9.1 years). Clinical features, laboratory findings, and medication use history were recorded. Student's t test, Mann-Whitney U test, chi-square test, and linear and logistic regression analysis were performed. A 2-sided p < 0.05 was statistically considered significant. Results The CAR was increased in patients with CAE compared to the controls (32 and 16; p < 0.001). In addition, the CAR was found to be an independent predictor of CAE (OR = 2.202; 95% CI 1.184 - 5.365; p < 0.001). Conclusion In the present study, we determined that CAR levels were significantly higher in the CAE group than in the control group, and the CAR was significantly correlated with CAE. (Arq Bras Cardiol. 2020; [online].ahead print, PP.0-0)

Interpretation of the JCS/JSCS 2020 guideline on diagnosis and management of cardiovascular sequelae in Kawasaki disease / 中国当代儿科杂志

Kawasaki disease is the main cause of acquired heart disease in children. The cardiovascular sequelae of Kawasaki disease, such as coronary artery lesion and giant coronary aneurysm, have a great impact on children's physical and mental health. The Japanese Circulatory Society and the Japanese Society of Cardiac Surgery jointly released the JCS/JSCS 2020 guideline on diagnosis and management of cardiovascular sequelae in Kawasaki disease in July, 2020, which systematically introduces the advances in the diagnosis and management of cardiovascular sequelae of Kawasaki disease. The article gives an interpretation in the severity evaluation of Kawasaki disease and diagnosis, treatment and long-term management of cardiovascular sequelae in the guideline.

Compromiso cardiovascular en pacientes con síndrome inflamatorio pediátrico multisistémico, asociado a infección por SARS-CoV-2 / Cardiovascular involvement in pediatric patients with SARS-CoV-2 associated to multisystemic inflammatory syndrome

INTRODUCCION: El Síndrome Inflamatorio Pediátrico Multisistémico (PIMS) ha emergido como una nueva enfermedad en niños, secundaria a infección por SARSCoV-2. Se caracteriza por presentar compromiso multiorgánico con parámetros inflamatorios elevados y manifestaciones clínicas graves, siendo el corazón el órgano más severamente comprometido. OBJETIVO: Describir las características clínicas y de laboratorio de 23 pacientes con diagnóstico de PIMS con compromiso cardiovascular hospitalizados en un centro único. MÉTODO: Se efectuó un estudio retrospectivo analizando los hallazgos clínicos y de laboratorio junto a las manifestaciones cardiovasculares que presentaron estos pacientes. RESULTADOS: 23/29 pacientes con PIMS (78%) presentaron manifestaciones digestivas y mucocutáneas. Las manifestaciones cardiovasculares fueron: Síndrome Kawasaki y "Kawasaki like" sin compromiso coronario en 15/23 (65%) y con compromiso coronario en 3 (13%). Shock en 9 pacientes (39%), injuria miocárdica- miocarditis en 8 (35%) y derrame pericárdico en 13 (56%). Trastornos del ritmo cardíaco se observaron en 6 pacientes (26%). La terapia más utilizada fue inmunoglobulina y corticoides. 18 /23 requirieron manejo en unidades de intermedio y/o intensivo. Un 70% de los pacientes se recuperó del compromiso cardiovascular antes del alta. CONCLUSIÓN: El compromiso cardiovascular en PIMS es la complicación más frecuente de esta enfermedad, que se acompaña de manifestaciones inmunológicas y hematológicas graves lo que hace necesario un tratamiento multidisciplinario para un mejor manejo de estos pacientes.

INTRODUCTION: Pediatric Multisystemic Inflammatory Syndrome (PIMS) has emerged as a new disease in children, secondary to SARSCoV-2 infection. It is characterized by multi-organ involvement with elevated inflammatory parameters and severe clinical manifestations, the heart being the organ most severely involved. OBJETIVE: to describe the clinical and laboratory characteristics of 23 patients diagnosed with PIMS with cardiovascular involvement hospitalized in a single center. METHOD: We conducted a retrospective study in which we analyzed the clinical and laboratory findings along with the cardiovascular manifestations presented by these patients. Results: 23/29 patients with PIMS and cardiovascular involvement were selected, 78% had digestive and mucocutaneous manifestations. Cardiovascular manifestations consisted of KawasakiKawasaki like syndrome without coronary involvement in 15/23 (65%) and coronary involvement in 3 (13%). Nine patients developed shock (39%), 8 (35%) myocardial injury in and 13 (56%) pericardial effusion.. Heart rhythm disorders were observed in 6 patients (26%). The main therapy was immunoglobulin and corticosteroids. 18 /23 required management in intermediate and/or intensive care unit. 70% of patients recovered from cardiovascular involvement before discharge. CONCLUSION: Cardiovascular involvement in PIMS is the most frequent complication of this disease, but it is associated with severe immunological and hematological manifestations, which makes necessary a multidisciplinary treatment for a better management

Enfermedad aneurismática coronaria / Coronary aneurysm disease

Resumen La enfermedad aneurismática coronaria se define como la dilatación de un segmento arterial coronario más 1,5 veces el diámetro de las arterias adyacentes de tamaño normal. Puede ser difusa, y afectar todo el trayecto de la arteria, o localizada y afectar sólo un segmento arterial específico. Puede encontrarse entre un 3 a un 8% de los estudios angiográficos y entre un 0,22% a un 1,4% de las necropsias clínicas. Se presenta 3 reportes de casos, donde la presentación clínica, los hallazgos angiográficos y el manejo farmacológico es diverso como corresponde a esta entidad.

Abstract Coronary aneurysm disease is defined as the dilation of a coronary artery segment more than 1.5 times the diameter of the adjacent arteries of normal size. It may be diffuse, and affect the whole trajectory of the artery, or localised and only affect a specific arterial segment. It can be found in between 3% and 8% of angiograph studies, with between 0.22% and 1.4% in clinical post-mortems. Three case reports are presented in which the clinical presentation, the angiographic findings, and the pharmacological management are shown to be as diverse as this condition itself.

Aneurisma gigante de la arteria coronaria derecha, reporte de un caso y revisión de la literatura / Giant aneurysm of the right coronary artery, report of a case and review of the literature

Resumen Los aneurismas de las arterias coronarias se definen como una dilatación localizada que excede el diámetro normal en 1.5 veces. Esta es una condición poco frecuente, su incidencia varía del 0.3 hasta el 5.3% de las angiografías coronarias. Los aneurismas que exceden cuatro veces el diámetro del vaso normal se consideran gigantes. Estos son aún más raros y se presentan en el 0.02 a 0.2% de todos los casos. Existe controversia en cuanto a su fisiopatología, sin embargo, hasta el 50% de los casos se relacionan con la aterosclerosis. Se diagnostican más frecuentemente entre la sexta y séptima décadas de vida. Las principales manifestaciones clínicas están relacionadas con la cardiopatía isquémica. Respecto a su tratamiento, no existe un consenso del manejo en los pacientes adultos, las opciones son: médico, quirúrgico o intervencionismo. Reportamos la presencia de un aneurisma gigante de la coronaria derecha y ectasia gigante del sistema izquierdo con trombosis activa en un hombre con antecedentes de un aneurisma en la aorta abdominal, tratado por vía endovascular, e infarto agudo al miocardio sin elevación del segmento ST no reperfundido. Requirió de estudio de angiotomografía coronaria, el cual permitió la identificación de las características anatómicas de esta enfermedad.

Abstract Coronary artery aneurysms are described as a localized dilatation that exceeds the normal diameter by 1.5 times. This is a rare condition; its incidence varies from 0.3% up to 5.3% of all coronary angiographies. Those aneurysms that exceed 4 times the diameter of a normal artery are considered giant aneurysms, which are even more uncommon, presenting between 0.02% and 0.2% of all cases. There is controversy regarding its pathophysiology, however, up to 50% of the cases are related to atherosclerosis. They are diagnosed more frequently between the sixth and seventh decade of life. The main clinical manifestations are related to ischemic heart disease. Regarding their treatment, there is no general consensus towards its management in adult patients. The options are medical, surgical or percutaneous treatment. We report the presence of a giant aneurysm of the right coronary artery and giant ectasia of the left coronary system with active thrombosis in a man with history of an abdominal aortic aneurysm, with endovascular treatment and a non-ST segment elevation myocardial infarction with no reperfusion strategy, who required a coronary computed tomography, identifying the anatomical characteristics of this disease.