Perfil da doença de Kawasaki em crianças encaminhadas para dois serviços de reumatologia pediátrica do Rio de Janeiro, Brasil

Abstract

Objectives:To describe a population of children diagnosed with Kawasaki's disease (KD) in pediatric rheumatology centers of Rio de Janeiro, Brazil, defining the magnitude of the delay period in diagnosing KD and initiating treatment due to confusion with common childhood febrile illnesses and the impact of this delay on the frequency of coronary sequels. Methods: Data analysis from hospital records summarized in a dedicated form, including name, gender, age, date of first recorded clinical signs, date of admission to the specialty service, information about symptoms, clinical evolution, intravenous immunoglobulin (IVIG) use and coronary sequels. Results: Of 125 patients, 63% were males. 40% were under 2 years at diagnosis. Average lapse between earliest signs and KD diagnosis was 12 days (mean fever duration, 14 d). Only 22.4% had a diagnosis of KD before entering the specialty service. For the remainder, initial hypotheses included: bacterial (60%) and viral infections (12%), rheumatological diseases (4%) and adverse vaccination reactions (1.6%). Hence, prevalent febrile illnesses of childhood were major confounding factors. For records (85.6%) mentioning treatment, 46.7% reported IVIG treatment, beginning after day 10 in 23 cases (21.5%). 20 patients (16%) presented coronary sequels, 9 of which were diagnosed late, including 3 given IVIG after day 10, and 6 given no IVIG. We found no significant association between the frequency of coronary sequels and: a) sex; b) age; c) clinical criteria; d) initiation of IVIG treatment (before or after day 10). Conclusions: Common febrile illnesses of childhood often confound the diagnosis of KD.

Clinical Manifestations of Kawasaki Disease at Different Age Spectrum: A Ten-Year Study

“…Different from other studies, we conducted a study in which the children were followed during the entire period. Our data corroborated previous studies [4,7,16] showing the highest prevalence of KD among males (1.55 male:1 female) for all of the age groups (Table 1). Behavioral changes were observed with great frequency, not only in the acute phase but also in the chronic phase of the disease, which corroborates with findings published in other studies [13,17].…”

“…KD presents a challenge to physicians working in Latin American countries, since it is an uncommon rheumatologic affliction that requires highly specific intervention to prevent serious or fatal sequelae but has a nonspecific clinical presentation in a critical period during which proper treatment should be instituted [16]. The age group in which the disease is most prevalent, between one and four years of age, coincides with the age at which childhood febrile exanthematic diseases are more common, which often hampers and delays the definitive diagnosis.…”

“…Coronary aneurysms appear in 15% to 25% of cases [7], often silent, and may be recognized only years later in the event of sudden death or myocardial infarction [4,7]. There are many infectious diseases more prevalent than KD that present as acute febrile illnesses of childhood, confounding the diagnosis of KD [11,16].…”

O papel do gene e da molécula HLA-G na expressão clínica das doenças reumatológicas

“…Currently, studies of genomic scan have been gaining attention in the investigation and discovery of alleles and regions related to genetic susceptibility to autoimmune diseases. In a study of genomic scan that analyzed 2360 SNPs within the MHC region in 92 patients with Kawasaki disease (KD), a systemic vasculitis of childhood of unknown cause, 51 HLA-G was identifi ed as the only candidate locus for a signifi cant association with that vasculitis. 52 The 14-bp polymorphism was investigated in other situations and has been associated with several diseases -the insertion allele has been observed with higher frequency in patients with sarcoidosis 53 and Behçet's disease (BD), 54 while the deletion allele has been reported as a risk factor for idiopathic dilated cardiomyopathy 55 and pemphigus vulgaris (PV).…”

Infarto agudo do miocárdio silencioso associado a aneurisma de artéria coronária decorrente de doença de Kawasaki: relato de caso / Silent acute myocardial infarction associated with coronary artery aneurysm due to Kawasaki disease: case report

“…A presença de cinco desses critérios define o diagnóstico da doença. 7,8 O acometimento cardíaco é um aspecto marcante da DK em decorrência de sua frequência e gravidade, com possibilidade de ocorrer miocardite, pericardite, endocardite, comprometimentos valvulares e prejuízo à estrutura das artérias coronárias. Os danos coronarianos podem se caracterizar por estenoses ou formação de aneurismas, aparecendo em cerca de 18 a 25 dias após a instalação da doença.…”