RESUMO
We described the characteristics of 11 children with pediatric multisystem inflammatory syndrome-temporally associated with SARS-CoV-2. The main clinical indications for hospital admission were vasogenic toxic shock (n = 2), Kawasaki disease (n = 4), and Kawasaki disease shock syndrome (n = 5). The echocardiography findings were abnormal in 63% of cases. All patients had 2 or more organ dysfunctions, and the mortality rate was 18%.
Assuntos
Infecções por Coronavirus/fisiopatologia , Pneumonia Viral/fisiopatologia , Síndrome de Resposta Inflamatória Sistêmica/virologia , Adolescente , Betacoronavirus/isolamento & purificação , Brasil/epidemiologia , COVID-19 , Criança , Pré-Escolar , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/mortalidade , Infecções por Coronavirus/virologia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Mortalidade , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/mortalidade , Síndrome de Linfonodos Mucocutâneos/fisiopatologia , Pandemias , Pneumonia Viral/diagnóstico , Pneumonia Viral/mortalidade , Pneumonia Viral/virologia , Estudos Prospectivos , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/mortalidade , Síndrome de Resposta Inflamatória Sistêmica/fisiopatologiaRESUMO
BACKGROUND: Kawasaki disease shock syndrome (KDSS) is an uncommon presentation of Kawasaki disease (KD). KDSS has been associated with more severe markers of inflammation, coronary abnormalities and i.v. immunoglobulin (IVIG) resistance. METHODS: A retrospective, descriptive study of children with KDSS in two hospitals was performed. Relevant articles about KD and shock were collected, and demographic data, clinical presentation, laboratory variables, echocardiogram findings, treatment and special features were analyzed when available. Twelve patients diagnosed with KDSS were retrospectively reviewed from two centers in Mexico, along with 91 additional cases from the literature. RESULTS: Seventy-two patients presented with complete KD (69.9%), and 30.1% (31/103) had unusual KD manifestations. The most frequent diagnosis at the time of admission was toxic shock syndrome (TSS; n = 20). Sixteen of the 20 had coronary artery abnormalities. Overall, abnormalities in the coronary arteries were documented in 65% of the patients. The mortality rate was 6.8%. CONCLUSION: The presence of coronary aneurysms was significantly and positively correlated with male gender, IVIG resistance, inotrope treatment, cardiac failure, abdominal pain and neurological symptoms. IVIG-resistant patients had higher neutrophil : lymphocyte ratio. Abdominal symptoms, hypoalbuminemia and elevated C-reactive protein were present in almost all of the patients. Multisystem involvement with atypical presentation in KDSS is frequent. An important differential diagnosis is TSS. Mechanical ventilation, gastrointestinal and neurological symptoms were associated with IVIG resistance and the presence of coronary aneurysms. The first line of treatment includes IVIG and pulse corticosteroids; in severe cases, infliximab, anakinra, cyclosporine or plasmapheresis are alternative treatment options.
Assuntos
Síndrome de Linfonodos Mucocutâneos/complicações , Choque/etiologia , Adolescente , Biomarcadores/sangue , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/mortalidade , Síndrome de Linfonodos Mucocutâneos/terapia , Estudos Retrospectivos , Choque/diagnóstico , Choque/epidemiologia , Taxa de SobrevidaRESUMO
OBJECTIVES: To clarify the occurrence of cardiac events based on the maximal diameter of the maximal coronary artery aneurysm (CAA) in Kawasaki disease (KD). STUDY DESIGN: Two hundred fourteen patients (160 male and 54 female) who had had at least 1 CAA in the selective coronary angiogram less than 100 days after the onset of KD were studied. We measured the maximal CAA diameters in the major branches of the initial coronary angiograms. Death, myocardial infarction and coronary artery revascularization were included as cardiac events in this study. We divided the patients into three groups based on the maximal CAA diameter (large ≥8.0 mm; medium ≥6.0 mm and <8.0 mm; small <6.0 mm). Further, we also analyzed the cardiac events based on laterality of maximal CAA (bilateral, unilateral) and body surface area (BSA). RESULTS: Cardiac events occurred in 44 patients (21%). For BSA < 0.50 m2, the 30-year cardiac event-free survival in the large and medium groups was 66% (n = 38, 95% CI, 49-80) and 62% (n = 27, 95% CI, 38-81), respectively. For BSA ≥ 0.50 m2, that in large group was 54% (n = 58, 95% CI, 40-67). There were no cardiac events in the medium group for BSA ≥0.50 m2 (n = 36) and the small group (n = 56). In the large analyzed group, the 30-year cardiac event-free survival in the bilateral and unilateral groups was 40% (n = 48, 95% CI, 27-55) and 78% (n = 48, 95% CI, 63-89), respectively (P < .0001). CONCLUSIONS: The group with the highest risk of cardiac events was the patient group with the maximal CAA diameter ≥6.0 mm with BSA < 0.50 m2 and the maximal CAA diameter ≥8.0 mm with BSA ≥ 0.50 m2. At 30 years after the onset of KD, cardiac event-free survival was about 60%. Given the high rate of cardiac events in this patient population, life-long cardiovascular surveillance is advised.
Assuntos
Aneurisma Coronário/complicações , Vasos Coronários/patologia , Síndrome de Linfonodos Mucocutâneos/complicações , Adolescente , Criança , Pré-Escolar , Aneurisma Coronário/mortalidade , Angiografia Coronária , Feminino , Humanos , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/mortalidade , Taxa de SobrevidaRESUMO
A doença de Kawasaki é uma enfermidade que afeta principalmente crianças menores de 5 anos, ainda de etiologia desconhecida. Diagnóstico precoce e tratamento adequado reduzem o risco de complicaçöes. Os autores fazem aqui uma breve atualizaçäo da literatura e relatam sua experiência de 15 anos de seguimento. Säo analisados 153 casos, com estudo ecocardiográfico em 143, cateterismo cardíaco e estudo com radioisótopos nos que apresentam aneurismas gigantes das artérias coronárias. Detectou-se coronarite em 37 (28,9 'por cento'), dos quais 7 apresentavam diâmetro arterial igual ou maior que 8 mm. Cinco evoluíram com formaçäo de trombos. Näo houve óbitos nessa estatística. Os autores, baseados em sua experiência propöem tratamento com gamaglobulina, aspirina e diridamol, dependendo da fase da doença, e seguimento dos pacientes a longo prazo.
Assuntos
Humanos , Pré-Escolar , Coração/fisiopatologia , Doença das Coronárias , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/mortalidade , Síndrome de Linfonodos Mucocutâneos/terapiaRESUMO
OBJECTIVE: To determine whether the mortality rate of patients with a history of Kawasaki disease is higher than that of the general population. DESIGN: In a cohort study, 6585 patients with Kawasaki disease were observed from the first medical encounter because of the disease through the end of 1992, or until death. Standardized mortality ratios (SMRs) with 95% confidence intervals (CI) were calculated with vital statistics data of Japan for the control. RESULTS: Of 6585 patients who met the eligibility criteria, 6550 (99.5%) were followed through either the end of the study or the date of death. Nineteen patients (14 male subjects) died during the study period; an overall SMR of 1.56 (955 CI, 0.94 to 2.43) was calculated for the entire study period. The SMR was 1.78 (95% CI, 0.97 to 2.99) for male subjects and 1.16 (95% CI, 0.38 to 2.71) for female subjects. During the acute phase of the disease (the first 2 months after onset), the SMR was higher, particularly in male subjects (SMR, 10.12; 95% CI, 3.72 to 22.07). After the acute phase, however, both boys and girls had low SMRs. Nine of the 19 deaths were caused by Kawasaki disease; there were 2 deaths as a result of congenital anomalies of the circulatory system and 2 subjects died of malignant neoplasms of lymphatic or hematopoietic tissues. CONCLUSIONS: Although the mortality rate among those with a history of Kawasaki disease was elevated in Japan, many of the deaths that caused the elevation occurred during the acute phase of the disease. The mortality rate was not increased after the acute phase of the disease.
Assuntos
Síndrome de Linfonodos Mucocutâneos/mortalidade , Distribuição por Idade , Causas de Morte , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Japão/epidemiologia , Masculino , Análise por Pareamento , Distribuição por SexoRESUMO
We report an unselected series of eight patients younger than 6 months of age with Kawasaki disease evaluated between January 1982 and May 1984. The incidence of coronary artery aneurysms (six patients) and the mortality (two patients) were unusually high in this small series. Because of the confusing clinical presentation in three patients, diagnosis was delayed until pathologic or echocardiographic evidence of coronary vasculitis or aneurysm was discovered. The currently accepted clinical criteria for Kawasaki disease may not always identify patients with the pathologic findings of the syndrome who are younger than 6 months of age. The diagnosis of Kawasaki disease and echocardiographic evaluation of the coronary arteries should be considered in young infants with prolonged fever of unknown origin.