Enfermedad de Kawasaki. Presentación atípica en un paciente pediátrico

La enfermedad de Kawasaki es un síndrome mucocutáneo caracterizado por vasculitis, que afecta medianos vasos; su principal manifestación es un síndrome febril agudo de, al menos, cinco días de duración y en muchas ocasiones de etiología desconocida. Se aprecia, fundamentalmente, en niños menores de cinco años de edad. Se considera que es frecuente, pero existen subregistros. Se caracteriza por tener dos formas de presentación: típica o atípica. Se presenta el caso clínico de un niño que fue hospitalizado debido a síndrome febril agudo asociado a malestar general e irritabilidad. Al examen físico se observaron edemas discretos en manos y pies así como erupción cutánea, no hepatomegalia ni alteraciones oculares. Teniendo en cuenta la epidemiología, lo atípico del cuadro clínico y los resultados de estudios hemoquímicos, se concluyó el diagnóstico de enfermedad de Kawasaki atípica. Se impuso tratamiento específico y se logró una evolución satisfactoria y la no aparición de complicaciones inmediatas. Esta entidad nosológica requiere de una adecuada valoración clínica-epidemiológica de los pacientes, así como de un minucioso examen físico y un diagnóstico precoz para lograr la evolución favorable de los pacientes y la no presencia de secuelas cardiacas, que pondrían en peligro la vida del paciente y/o su calidad de vida futura.

Kawasaki disease (KD) is a mucocutaneous syndrome characterized by vasculitis that affects medium vessels; its main manifestation is an acute febrile syndrome lasting at least five days and often of unknown etiology. It appears mainly in children under five years of age, it is considered to be frequent, but there are underreportings. It is characterized by having two presentation forms: typical or atypical. The clinical case of a child who was hospitalized due to acute febrile syndrome associated with malaise and irritability is presented. The physical examination revealed discrete edema in the hands and feet as well as a rash, no hepatomegaly or ocular alterations. Taking into account the epidemiology, the atypical clinical picture and the results of hemochemical studies, the diagnosis of atypical Kawasaki disease was concluded. Specific treatment was imposed and a satisfactory evolution was achieved with no immediate complications. This nosological entity requires an adequate clinical-epidemiological evaluation of the patients, as well as a meticulous physical examination and an early diagnosis to achieve a favorable patients' evolution and the absence of cardiac sequelae, which would endanger the patients' life and/or their future quality of life.

Analysis of coronary angiographic findings in 117 children with Kawasaki disease and coronary artery lesion / 中华实用儿科临床杂志

Objective:To analyze the coronary angiographic (CAG) characteristics of coronary artery lesion (CAL) in children with Kawasaki disease (KD), and to clarify the necessity of CAG in the diagnosis and treatment of KD combined with CAL in children.Methods:It was a retrospective study to analyze the clinical data, electrocardiogram, echocardiography, time and findings of CAG in children with KD and CAL who underwent CAG in Shanghai Children′s Medical Center of Shanghai Jiao Tong University School of Medicine from January 2013 to August 2022.The distribution, type, severity, and prognosis of CAL were analyzed.Results:A total of 117 children with KD and CAL were included in the analysis.The onset age of KD was from 2 months to 12.8 years old, and the age of performing CAG was from 8 months to 18.1 years old.A total of 234 coronary artery lesions were detected in 117 cases.Among them, CAL in the right coronary artery (RCA), left anterior descending branch (LAD), left main coronary artery and left circumflex artery were detected in 96 branches(41.1%), 78 branches(33.3%), 44 branches(18.8%), and 16 branches(6.8%), respectively.Unilateral coronary artery involvement was detected in 43 cases (36.8%), of which LAD was the dominant; while bilateral involvement was detected in 74 cases (63.2%), among which, LAD and RCA were the most involved arteries.Stratified by the degree of coronary involvement, large coronary aneurysms and severe coronary stenosis were most frequently occurred in the RCA and LAD.In contrast, 10 cases (13.6%), 20 cases (24.3%), 55 cases (45.8%) and 37 cases (67.3%) of intraluminal lesions were found in small, medium and large coronary aneurysms, and stenosis or occlusion, respectively.The incidence of intraluminal lesions tended to be higher in the site of severe lesions.CAG showed stenosis or occlusion in a total of 55 cases, and collateral circulation at varying degrees was found in cases of severe stenosis or occlusion.Conclusions:CAL in children with KD are complex and varied.Although clinical symptoms, routine electrocardiogram and cardiac ultrasound may indicate severe CAL.Their applications are limited by the diagnosis of the type (especially stenosis), degree, and extent of CAL, as well as the detection of extracoronary lesions.CAG is of great significance to identify vascular lesions and guide clinical management of KD combined with CAL in children.

Study on the evolution of TCM syndromes of 171 cases of Kawasaki disease under the intervention of gamma globulin therapy based on factor analysis / 国际中医中药杂志

Objective:To study the evolution of Traditional Chinese Medicine (TCM) syndromes of 171 cases of Kawasaki disease (KD) under the intervention of gamma globulin therapy based on factor analysis.Methods:A cross-sectional study. 171 cases of KD children hospitalized in the Department of Cardiology of Guangzhou Women's and Children's Medical Center from July 2019 to December 2020 were collected. All patients were treated with intravenous gamma globulin (2 g/kg) for 1 week. According to the results of the treatment with C-ball, 171 children with C-ball sensitive KD were selected to collect the four diagnostic data, and the representative syndromes of defensive level, qi level, yin level, and nutritive level were observed. Factor analysis was used to analyze the evolution of syndrome in 171 children with KD c-ball sensitivity.Results:The result of factor analysis showed that the KMO statistics of 171 children with c-ball sensitivity before treatment was 0.792, and Bartley test was significant ( P<0.01). 16 common factors were extracted, and 23 syndromes were screened, mainly including defensive level disorder, qi level disorder,nutritive level disorder,yin level disorder, heat stagnation and blood stasis syndrome. One week after treatment, the statistic of KMO test was 0.787, and Bartley test was significant ( P<0.01). 9 common factors were extracted, and 10 syndromes were screened, mainly including qi deficiency syndrome, yin deficiency syndrome and blood stasis syndrome. Conclusion:Before treatment, the TCM syndromes in KD C-cell sensitive children are mainly nutritive level disorder, defensive level disorder, qi level disorder yin level disorder, and heat stagnation and blood stasis syndrome; after treatment, the main TCM syndromes are mainly qi deficiency syndrome, yin deficiency syndrome and blood stasis syndrome.

Surgical Correction of Ascending Aortic Aneurysm Without Coronary Dilatation After Kawasaki Disease in a 3-Year-Old Child

Abstract Kawasaki disease (KD) is an inflammatory condition that may affect genetically predisposed individuals in pediatric ages after infectious/environmental triggering. An infrequent finding associated with KD is ascending aortic aneurysm during or after the acute phase of the disease. In this Multimedia presentation, we describe a three-year-old girl submitted to surgical treatment.

Successful Surgical Treatment of Coronary Aneurysm, Ascending Aortic Aneurysm, and Bicuspid Aorta for a Kawasaki Disease Patient

Abstract Kawasaki disease was first reported in 1967, and it was classified as an autoimmune vasculitis of the small and medium arteries. It is a self-limiting condition that occurs mostly in childhood, but it may involve complications — such as coronary artery aneurysms, myocardial ischemia, and arrhythmias — with significant morbidity and mortality that occur later in life. In this article, we present the association of an ascending aortic aneurysm with bicuspid aortic disease in addition to coronary aneurysm in a 55-year-old patient diagnosed with Kawasaki disease.

Comparative analysis of clinical laboratory indicators between Kawasaki disease and systemic juvenile idiopathic arthritis / 中国基层医药

Objective:To explore the differences of clinical laboratory indicators between Kawasaki disease (KD) and systemic juvenile idiopathic arthritis (SJIA), providing objective evidence for diagnosis and differential diagnosis of these diseases.Methods:A total of 41 children patients with KD (KD group) and 33 children patients with SJIA (SJIA group) who received treatment in Huainan Maternal and Child Health Hospital between September 2017 and January 2022 were retrospectively analyzed. An additional 50 healthy children who concurrently received physical examination in the same hospital were included in the control group. Platelet count (PLT), white blood cell count (WBC), and erythrocyte sedimentation rate (ESR) as well as C-reactive protein (CRP), serum procalcitonin (PCT), interleukin-6 (IL-6), interleukin-10 (IL-10), and serum ferritin (SF) levels were compared among groups before treatment.Results:One-way analysis of variance and pairwise q test were performed to compare laboratory indicators among KD, SJIA and control groups. CRP, ESR, SF and IL-6 levels in the KD group were significantly lower than those in the SJIA group [CRP: (57.80 ± 25.23) mg/L vs. (77.72 ± 45.64) mg/L; ESR: (67.02 ± 28.80) mm/h vs. (83.84 ± 47.64) mm/h; SF: (320.21 ± 182.53) μg/L vs. (945.58 ± 604.65) μg/L; IL-6: (50.35 ± 20.54) ng/L vs. (89.35 ± 45.54) ng/L, q = 4.34, 3.42, 11.51, 8.85, all P < 0.05]. IL-10 level in the KD group was significantly higher than that in the SJIA group [(18.52 ± 16.71) ng/L vs. (10.01 ± 3.24) ng/L, q = -5.25, P < 0.05]. WBC, CRP, ESR, PCT, PLT, IL-6, IL-10 and SF in the KD and SJIA groups were significantly higher than those in the control group (all P < 0.05). Conclusion:Detection of CRP, ESR, SF, IL-6, IL-10 in blood can provide objective evidence for the early diagnosis and differential diagnosis of KD and SJIA, thereby reducing the misjudgment of clinical diagnosis.

Changes of humoral and cellular immune indexes in peripheral blood of children with Kawasaki disease at different stages / 中国医师进修杂志

Objective:To investigate the dynamic change characteristics of peripheral blood interferon-γ (INF-γ), interleukin (IL)-4 levels and T helper cell (Th)1/Th2 balance in acute, subacute and restoration stages of children with Kawasaki disease (KD).Methods:Forty-one children with KD received treatment in Women′s and Children′s Hospital Affiliated of School of Medicine University of Electronic Science and Technology of China, Chengdu Women′s and Children′s Central Hospital from May 2017 to January 2020 were enrolled as the observation group, and 41 healthy children examinee from the same period were enrolled as the control group. Children with KD of the observation group were performed with tuberculin pure protein derivative (PPD) test when acute and restoration stage of KD respectively. Peripheral venous blood were drawn from KD children of the observation group in acute, subacute and restoration stage and the control group respectively, serum immune globulin IgG, IgA, IgM and IgE, serum IFN-γ and IL-4 levels were detected by enzyme linked immunosorbent assay (ELISA).Results:Positive rates of PPD test in the restoration stage was higher than that in the acute stage: 65.85%(27/41) vs.17.07%(7/41), there was statistical difference ( χ2 = 20.10, P<0.05). The levels of serum IgG, IgA, IgM and IgE in the acute stage , subacute stage and restoration stagewere gradually decreased ( P<0.05). The levels of serum IgG, IgA, IgM and IgE in the restoration stage and the control group had no significant differences ( P>0.05). The levels of serum IFN-γ and IFN-γ/IL-4 in the acute stage , subacute stage and restoration stage were gradually increased ( P<0.05), the level of IL-4 was gradually decreased ( P<0.05), but the levels of serum IFN-γ, IL-4 and IFN-γ/IL-4 in the restoration stage and the control group had no significant differences ( P>0.05). Conclusions:Among the children with KD in acute stage, serum level of IFN-γ is decreased while serum IL-4 level is increased, and Th1/Th2 balance shifts to Th2. Along with the stabilization of disease, the levels of serum IFN-γ and IL-4 are normalized, and Th1/Th2 balance presents a recovering trend and they almost recover to normal after entering the restoration stage.

Oblivion: autopsy findings of a 31-year-old man with sudden cardiac arrest, a case report of a sequalae of Kawasaki disease

ABSTRACT A 31-year-old man presented to the hospital after suffering a sudden cardiac arrest. Despite optimal therapy, the patient passed away. His medical history included febrile rash at age 2. At autopsy, there was aneurysmal dilation and severe coronary artery stenosis by atherosclerotic plaques and myocardial fibrosis. These findings were presumed to be due to complications of Kawasaki disease, given the remote history of severe febrile rash as a toddler and the presence of chronic coronary artery injury, recanalization, and thrombosis with ischemic heart disease leading to sudden cardiac collapse and death.

Clinical analysis of 105 cases of Kawasaki disease complicated by hypoproteinemia / 中国基层医药

Objective:To analyze the clinical characteristics of children with Kawasaki disease complicated by hypoproteinemia.Methods:The clinical data of 105 children patients with Kawasaki disease who received treatment in Ji'an Central People's Hospital, China between January 2015 and December 2019 were retrospectively analyzed. These patients were divided into normal-level albumin (ALB) group (≥ 35 g/L, n = 54) and low-level ALB group (< 35 g/L, n = 51). White blood cell count, hemoglobin, C-reactive protein, erythrocyte sedimentation rate, alanine aminotransferase, aspartate aminotransferase, total bile acid, and coronary artery B-ultrasound examination results were compared between the two groups. Results:The incidence of hypoproteinemia was similar among all age groups. There was significant difference in coronary artery dilatation rate between the two groups (χ 2 = 5.49, P < 0.05). There were no significant differences in the percentage of patients with hand and foot scleredema and urinary protein-positive rate between the two groups (χ 2 = 0.40, 0.39, both P > 0.05). White blood cell count, erythrocyte sedimentation rate, C-reactive protein level in the low-level ALB group were 15.2 × 10 9/L, (82.1 ± 26.1) mm/h, 94.7 mg/L, respectively, which were significantly higher than those in the normal-level ALB group [11.5 × 10 9/L, (54.5 ± 26.2) mm/h, 43.65 mg/L, Z = -2.94, t = 5.40, Z = -6.01, all P < 0.01]. There were no significant differences in alanine aminotransferase, aspartate aminotransferase, and total bile acid levels between the two groups (all P > 0.05). Conclusion:The incidence of Kawasaki disease complicated by hypoproteinemia is unrelated to age and it is related to inflammatory reaction. Hypoproteinemia is a risk factor of coronary artery dilatation. This study is of great science.

Effects of Notch1 signaling on regulatory T cells and coronary artery lesions in childhood Kawasaki disease / 中华风湿病学杂志

Objective:To explore the effect of Notch1 signaling on regulatory T cells and its roles in vascular damage in patients with Kawasaki disease (KD).Methods:A total of 42 children with KD were enrolled in the present study from March 2019 to June 2020, as 32 age-matched healthy children were recruited as control. The proportions of CD4 +CD25 hiFoxp 3+ regulatory T cells (Treg) and expressions of transcription factor forkhead box protein 3 (Foxp3), cytotoxic T lymphocyte associated antigen-4 (CTLA4), glucocorticoid-induced tumor necrosis factor receptor (GITR), and Notch1 protein were evaluated by flow cytometry. Chromatin immunoprecipitation was conducted to detect acetylation level of histone H4 (H4Ac) associated with the promoter of Foxp3 gene and its binding abilities of Notch1 intracellular domain 1 (NICD1), recombination signal binding protein for immunoglobulin kappa J region (RBP-J) and p300 in CD4 + T cells. Transcription levels of Foxp3, presenilin 1 (PSEN1), mastermind like transcriptional coactivator 1 (MAML1), and RBP-J in CD4 + T cells were determined by real-time polymerase chain reaction (PCR). Concentrations of interleukin (IL)-10 and transforming growth factor-β (TGF-β) in plasma and culture supernatant stimulated with Jagged1 were measured by enzyme linked immunosorbent assay. Independent-sample t-test, Pearson correlation analysis was used as the statistical method in this study. Results:① The frequencies of Treg in acute KD patients decreased significantly [(4.3±1.5)% vs (7.9±2.9)%; t=6.41, P<0.001], as protein levels of Foxp3, CTLA4 and GITR and concentrations of IL-10 and TGF-β in plasma reduced remarkably in acute KD patients ( t=6.87, P<0.001; t=4.26, P<0.001; t=7.88, P<0.001; t=8.42, P<0.001; t=13.01, P<0.001). All parameters afore-mentioned in patients combined with coronary artery lesions (CAL) were lower than those of patients without coronary artery lesions (NCAL) ( t=5.83, P<0.001; t=3.83, P<0.001; t=3.28, P=0.002; t=5.05, P<0.001; t=5.96, P<0.001; t=5.17, P<0.001), and increased after therapy ( t=7.13, P<0.001; t=6.10, P<0.001; t=4.31, P<0.001; t=6.55, P<0.001; t=7.40, P<0.001; t=7.84, P<0.001). ② H4Ac associated with promoter of Foxp3 gene and the binding abilities of NICD1 and p300 in acute KD patients were lower than those of the controls ( t=10.25, P<0.001; t=6.93, P<0.001; t=6.75, P<0.001), and increased remarkably after therapy ( t=7.72, P<0.001; t=4.16, P<0.001; t=5.76, P<0.001). Meanwhile, the three items in CAL group were found to be less than those of NCAL group ( t=6.08, P<0.001; t=2.66, P=0.011; t=6.02, P<0.001). Pearson correlation analysis showed a positive correlation between H4Ac associated with Foxp3 promoter and its mRNA level in acute KD patients ( r=0.47, P<0.001). No statistical significant difference about the binding ability of RBP-J with Foxp3 promoter were found among the groups ( t=0.57, P>0.05; t=0.61, P>0.05; t=1.20, P>0.05). ③ Protein level of Notch1 and the expressions of PSEN1, MAML1 and RBP-J mRNA in CD4 + T cells from acute KD patients were down-regulated remarkably ( t=5.28, P<0.001; t=6.31, P<0.001; t=11.78, P<0.001; t=8.06, P<0.001), and restored after therapy ( t=4.77, P<0.001; t=6.43, P<0.001; t=11.95, P<0.001; t=7.79, P<0.001). In parallel, the four indexes aforementioned of CAL group were lower than those of NCAL group ( t=3.16, P=0.003; t=4.13, P<0.001; t=5.42, P<0.001; t=4.05, P<0.001). Upon rhJagged1 stimulation for 48 hours, H4Ac level of Foxp3 promoter and its binding abilities with NICD1 and p300 in CD4 + T cells in KD patients and control group was significantly higher than those of untreated group [(KD: t=15.36, P<0.001; t=7.25, P<0.001; t=14.29, P<0.001), (Ctrl: t=7.87, P<0.001; t=5.71, P<0.001; t=8.74, P<0.001)], as the binding ability of RBP-J with Foxp3 promoter increased slightly without statistically significant difference (KD: t=1.11, P>0.05; Ctrl: t=1.37, P>0.05). Simultaneously, H4Ac level of Foxp3 promoter and its binding abilities with NICD1 and p300 in KD group were still lower than those of the control group after stimulation ( t=3.86, P<0.001; t=3.42, P=0.001; t=2.85, P=0.006). ④ After incubation of PBMC from heathy children with KD serum, the proportion of Treg cells, protein level of Foxp3 and expressions of Notch1 and RBP-J in CD4 + T cells in the group treated with IVIG increased significantly compared with the untreated group ( t=7.10, P<0.001; t=10.16, P<0.001; t=8.06, P<0.001; t=9.77, P<0.001), as well as H4Ac level of Foxp3 promoter and its binding abilities with NICD1 in the group treat with IVIG were also higher than the latter ( t=7.24, P<0.001; t=8.24, P<0.001). Conclusion:Insufficiency and impaired function of Treg caused by aberrant Notch1 signaling may be the important factor contributing to immune dysfunction and vascular damage in KD.

Síndrome Inflamatória Multissistêmica associada à COVID-19: uma complicação pediátrica da pandemia / COVID-19-associated Multisystem Inflammatory Syndrome: a pediatric complication during the pandemic

Desde o surgimento da pandemia de COVID-19, casos de febre e inflamação sistêmica tardias, similares à Doença de Kawasaki, têm surgido na população pediátrica, sendo denominados Síndrome Inflamatória Multissistêmica associada à COVID-19. Estes quadros podem ir de apenas febre prolongada, até grave envolvimento gastrointestinal e cardíaco, com choque refratário e falência de múltiplos órgãos. Aneurismas de carótida podem surgir na evolução, levando a complicações em longo prazo. O pronto reconhecimento desta entidade com tratamento precoce de suporte geral, uso de imunoglobulina humana endovenosa e outras drogas imunomoduladoras, pode evitar evolução para casos graves e até mesmo fatais, assim como proteger o paciente de complicações crônicas, principalmente cardíacas.

Since the emergence of the COVID-19 pandemic, cases of late fever and systemic inflammation similar to Kawasaki Disease have appeared in the pediatric population, this entity was called Multisystem Inflammatory Syndrome associated with COVID-19. The presentation can range from just prolonged fever to severe gastrointestinal and cardiac involvement with refractory shock and multiple organ failure. Carotid aneurysms can arise in the course leading to long-term complications. The prompt recognition of this syndrome with early treatment with general support, use of Human Intravenous Immunoglobulin and other immunomodulatory drugs, can prevent progression to severe and even fatal cases, as well protect the patient from chronic complications, especially the cardiac ones.

Adenitis cervical imitando a un absceso cervical bacteriano en un lactante con Enfermedad de Kawasaki incompleta y aneurisma coronario: Reporte de un caso / Cervical adenitis mimicking a bacterial cervical abscess in an infant with incomplete Kawasaki disease and coronary aneurysm: A case repor

Antecedentes: La enfermedad de Kawasaki (EK) es una vasculitis aguda y autolimitada frecuente en menores de cinco años; sin embargo, su diagnóstico es un reto en menores de seis meses. Reporte de caso: Reportamos un lactante de tres meses con fiebre y tumoración cervical dolorosa de tres días de enfermedad. Fue diagnosticado como tumoración cervical abscedada y recibió tratamiento con antibióticos por 10 días. En hospitalización presentó lesiones eritematosas maculares en todo el cuerpo, persistencia de fiebre y elevación de marcadores inflamatorios. Se realizó una ecocardiografía encontrándose dilatación de las arterias coronarias siendo diagnosticado de EK incompleto. Recibió inmunoglobulina humana endovenosa y ácido acetil salicílico. Actualmente está en controles por cardiología con ácido acetil salicílico por persistencia de dilatación de las arterias coronarias. Conclusiones: La adenitis cervical puede imitar a otras infecciones cutáneas como el absceso cervical ocasionado retraso en el diagnóstico de EK produciendo complicaciones como el aneurisma coronario.

Background: Kawasaki disease (KD) is an acute and self-limited vasculitis common in children under five years. The diagnosis is a challenge in children under six months of age. Report case: We report a three-month-old infant with fever and a painful neck tumor of three days of disease. He was diagnosed with an abscessed cervical mass and received antibiotic treatment for ten days. During hospitalization, macular erythematous lesions developed throughout the body, persistence of fever, and elevated inflammatory markers. Echocardiography was performed, finding dilatation of the coronary arteries, being diagnosed with incomplete KD. He received intravenous human immunoglobulin and acetylsalicylic acid. He is currently undergoing cardiology controls with acetylsalicylic acid due to persistent dilatation of the coronary arteries. Conclusions: Cervical adenitis can mimic other skin infections such as cervical abscesses. It can cause a delay in the diagnosis of KD, producing complications such as a coronary aneurysm.

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

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

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.

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

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

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

Repercussão da COVID-19 em crianças e adolescentes: nova inflamação multissistêmica ou desencadeamento da doença de Kawasaki? / COVID-19 repercussion in children and adolescents: a new multisystem inflammation or a Kawasaki disease trigger?

A doença causada pelo novo coronavírus (COVID-19) teve início em dezembro de 2019 na China e rapidamente adquiriu grande importância epidemiológica, com um crescente número de casos e mortes no Brasil e no mundo. Entre os diversos estudos sobre a doença causada pelo SARS-CoV-2, foram relatados casos de crianças infectadas que apresentaram inflamação multissistêmica, mimetizando a doença de Kawasaki, o que instigou a possibilidade de uma associação entre esta e a COVID-19. O presente artigo de revisão visa destacar esta nova possibilidade diagnóstica, discutindo principalmente os sintomas, exames laboratoriais e a epidemiologia que podem diferenciar a síndrome inflamatória multissistêmica em crianças (MIS-C) como decorrente da COVID- 19 ou desencadeante da doença de Kawasaki. O banco de dados eletrônico PubMed foi utilizado para a pesquisa de artigos originais e de revisão de 2020 e 2021, selecionados de acordo com critérios de atualidade e menção a descritores científicos como infecções por coronavírus, síndrome de linfonodos mucocutâneos e inflamação. A revisão dos sintomas da MIS-C e achados de exames laboratoriais relacionados à COVID-19 foi feita por meio de artigos com estudos realizados com mais de 10 pacientes. Entre os principais resultados obtidos tem-se a diferença entre a faixa etária e etnia acometidas, presença de sintomas gastrointestinais, mecanismos de lesão cardíaca envolvidos e alterações de exames laboratoriais na doença de Kawasaki e na MIS-C. Assim, a conclusão é que a MIS-C está relacionada à infecção prévia pelo SARS-CoV-2, compartilhando dos sintomas da doença de Kawasaki, sem atuar como um desencadeante desta doença.

Coronavirus disease 2019 (COVID-19) arose in December 2019 in China and quickly assumed great epidemiological importance, with an increasing number of cases and deaths in Brazil and worldwide. Among the several studies on this disease, cases of children infected with SARS-CoV-2 who had multisystem inflammation, mimicking Kawasaki disease, were reported, which raised the possibility of an association between Kawasaki disease and COVID-19. This review article aims to highlight this new diagnostic possibility and mainly discusses the symptoms, laboratory tests, and epidemiology that may differentiate multisystem inflammatory syndrome in children (MIS-C) as a result of COVID-19 or as a Kawasaki disease trigger. Original and review articles from 2020 and 2021 that were current and mentioned keywords such as coronavirus infections, mucocutaneous lymph node syndrome, and inflammation were selected from the electronic database PubMed. The review of MIS-C symptoms and laboratory findings related to COVID-19 was based on articles of studies that included more than 10 patients. The main results obtained were differences between the affected age group and ethnicity, presence of gastrointestinal tract symptoms, different mechanisms of cardiac injury involved, and changes in laboratory tests in Kawasaki disease and MIS-C. In conclusion, MIS-C is related to previous SARS-CoV-2 infection and leads to the same symptoms of Kawasaki disease, without acting as a trigger for this disease.

Doença de Kawasaki: Preditores de Resistência à Imunoglobulina Intravenosa e Complicações Cardíacas / Kawasaki Disease: Predictors of Resistance to Intravenous Immunoglobulin and Cardiac Complications

Resumo Fundamento: A doença de Kawasaki (DK) é a principal causa de cardiopatia adquirida em idade pediátrica nos países desenvolvidos. Objetivos: Identificar fatores preditores de resistência à imunoglobulina intravenosa (IGIV), calcular a eficácia dos modelos preditores japoneses e caracterizar as complicações cardíacas. Métodos: Análise retrospectiva dos casos de DK entre janeiro de 2006 e julho de 2018 em um hospital pediátrico português. Foram construídas curvas ROC para encontrar fatores preditores de resistência e utilizada regressão logística multivariada para elaborar o modelo preditor. O nível de significância utilizado foi de 5%. Resultados: Foram incluídos 48 pacientes com mediana de idade de 36 meses. Verificou-se resistência à IGIV em 21%. Ocorreram alterações ecocardiográficas em 46%, com envolvimento coronário em 25%. Como variáveis preditoras de resistência, a proteína C-reativa (PC-R) apresentou uma AUC ROC = 0,789, ponto de corte = 15,1 mg/dL, sensibilidade (S) = 77,8% e especificidade (E) = 78,9%. A velocidade de sedimentação (VS) apresentou uma AUC ROC = 0,781, ponto de corte = 90,5 mm/h, S = 66,7% e E = 85,7%. O modelo com as duas variáveis apresentou valor p = 0,042 e AUC ROC = 0,790. O modelo Kobayashi apresentou S = 63,6% e E = 77,3%; Egami, S = 66,7% e E = 73,1%; e Sano, S = 28,6% e E = 94,1%. Conclusão: A PC-R e a VS são variáveis independentes que mostraram tendência preditora de resistência à IGIV com pontos de corte ótimos de 15,1 mg/dL e 90,5 mm/h, respectivamente. Cerca de metade dos pacientes teve algum tipo de envolvimento cardíaco. Os modelos japoneses não têm utilidade nessa população. (Arq Bras Cardiol. 2021; 116(3):485-491)

Abstract Background: Kawasaki disease (KD) is the leading cause of acquired cardiac disease in children, in developed countries. Objectives: To identify predictive factors for resistance to intravenous immunoglobulin (IVIG), calculate the effectiveness of Japanese predictive models and characterize cardiac complications. Methods: Retrospective analysis of KD cases admitted in a Portuguese paediatric hospital between january 2006 and july 2018. ROC curves were used to determine predictive factors for resistance and the multivariate logistic regression analysis was used to develop the predictive model. A significance level of 5% was used. Results: 48 patients with a median age of 36 months were included. The IVIG resistance was 21%. Echocardiographic anomalies were noted in 46%, with coronary involvement in 25% of the sample population. As predictive variable of resistance, the C-reactive protein (CRP) presented an AUC ROC = 0.789, optimal cut-off value 15.1 mg/dL, sensitivity (Sn) 77.8% and specificity (Sp) 78.9%. The erythrocyte sedimentation rate (ESR) presented an AUC ROC = 0.781, optimal cut-off value 90.5 mm/h, Sn 66.7% and Sp 85.7%. The model with the two variables showed p = 0.042 and AUC ROC = 0.790. Predictive strength of Japanese models were: Kobayashi (Sn 63.6%, Sp 77.3%), Egami (Sn 66.7%, Sp 73.1%), Sano (Sn 28.6%, Sp 94.1%). Conclusion: CRP and ESR are independent variables that were related to IVIG resistance, with optimal cut-off points of 15.1 mg/dL and 90.5 mm/h, respectively. About half of the patients had some form of cardiac involvement. The Japanese models appeared to be inadequate in our population. (Arq Bras Cardiol. 2021; 116(3):485-491)

Perfil clínico y epidemiológico de la enfermedad de Kawasaki: Hospital Pediátrico Dr. Agustín Zubillaga / Clinical and epidemiological profile of Kawasaki disease: Hospital Pediátrico Dr. Agustín Zubillaga

Se realizó una investigación descriptiva transversal retrospectiva con el fin de determinar el perfil clínico y epidemiológico de la enfermedad de Kawasaki (EK) en 95 niños egresados del Hospital Pediátrico Dr. Agustín Zubillaga durante el período 2014-2017. El promedio de edad de los pacientes se ubicó en 3,9 ± 3,3 años, siendo 69,5% del sexo masculino. El tiempo de evolución de la enfermedad fue menor de 10 días en el 68,4% de los casos. Las manifestaciones clínicas predominantes fueron fiebre (100%), edema y/o descamación de palmas y plantas (85,3%), alteración de la mucosa oral (65,3%), inyección conjuntival (59%) y eritema polimorfo (55,8%). El tratamiento recibido consistió en aspirina (100%) e inmunoglobulina (96,8%). La respuesta al tratamiento fue satisfactoria en el 95,8% de los pacientes. Los hallazgos de laboratorio evidenciaron una PCR elevada (51,6%) y trombocitosis (97,9%) con hemoglobina normal en 62,1% de los casos. 41% de los pacientes presentaron ecocardiograma anormal y 97,9% presentaron ultrasonido abdominal normal. El diagnóstico de ingreso de la EK fue incompleto en 53,7% de los pacientes y el diagnóstico de egreso de la EK fue completo en 51,6%. Los resultados de este estudio brindan datos epidemiológicos recientes sobre la enfermedad de Kawasaki en nuestra institución prestadora de salud(AU)

A descriptive cross-sectional retrospective investigation was carried out to evaluate the clinical and epidemiological profile of Kawasaki disease (KD) in 95 children discharged from the Pediatric Hospital Dr. Agustín Zubillaga during the period 2014-2017. Mean age of children was 3.9 ± 3.3 years and 69.5% were male. Time of symptom onset was less than ten days in 68.4% patients. Predominant clinical manifestations were fever (100%), edema and/or desquamation of palms and soles (85.3%), alteration of the oral mucosa (65.3%), conjunctival injection (59%) and polymorphic erythema (55.8%). Treatment received included aspirin (100%) and immunoglobulin (96.8%). Response to treatment was satisfactory in 95.8% of cases. Laboratory findings showed elevated CRP (51.6%), thrombocytosis (97.9%) and normal hemoglobin in 62.1% patients. 41% had an abnormal echocardiogram and 97.9% showed a normal abdominal ultrasound. The diagnosis of CHD admission was incomplete in 53.7% of cases and the diagnosis of EK was complete in 51.6%. Results of this study show recent epidemiological data about Kawasaki disease in our healthcare institution(AU)