RESUMO
OBJECTIVES: To define disease activity measures, muscle strength and functional assessments in new-onset juvenile dermatomyositis (JDM) patients, at disease onset and follow up. METHODS: A registry was set up in 18 hospitals, enrolling patients over 3-years (2015-2018). Clinical assessments were performed at baseline, and at 6, 12, 18 and 24 months after diagnosis. Disease Activity Score (DAS20), skin and musculoskeletal DAS sub-scales; Manual Muscle Test (MMT8); Childhood Myositis Assessment Scale (CMAS); Childhood Health Assessment Questionnaire disability index (CHAQ_DI 0-3) and 10 cm Visual Analog Scale (VAS) for overall wellbeing scores were compared by Poisson Model and Wald post-test for repeated measures. RESULTS: Ninety-six cases, being 61 (64%) females, median age 10 years had JDM diagnosis and 12 (13%) onset calcinosis. Mean ±SD scores at diagnosis and 6 months intervals for DAS20 (0-20) were 7.8±5, 6.3 ±4.8, 5±4, 4.9 ±5 and 0.5 ±2.3; with significant difference from baseline (p<0.01). Skin DAS subscales were 2.8±3.3, 1.8±2.9, 1,1±2.2, 0.6±1.8, 0.4±1.5. MMT (0-80) 62.6±20.4, 70.2±13.5, 73.3±11, 75.7±7.9 and 74.8±7.8, with significant difference from baseline up to 6 months (p=0.016); CMAS (0-53) 29.5±11.4, 33.1±8.3, 34.2±5.8, 34±6 and 33.3±5.4. CHAQ-DI (0-3) 1±0.9, 0.6±0.7, 0.8±0.8, 1±0.8 and 1±0.3; parents VAS 4.1±2.5, 2±2.1; 1.3±2.8, 4.1±3.1, 1.7±2.2. There was no significant difference for CMAS, CHAQ-DI and parents VAS from baseline up to 24-month assessment. CONCLUSIONS: DAS20 scores improved gradually during follow up, MMT8 improved significantly during the first 6 months and CMAS, CHAQ-DI and parents VAS scores had no significant improvement with persistent functional impairment over 2-years.
RESUMO
BACKGROUND: Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology in which the main complication is coronary arteritis. Sensorineural hearing loss (SNHL) has also been described as a complication of this disorder. The objective of this study was to evaluate the prevalence of SNHL in patients with KD and to determine the possible causative abnormality, correlating the findings with the prevalence of cardiac complications, with the use of gammaglobulin, and with the results of laboratory tests. METHODS: A clinical cohort of 40 patients with KD seen between 2005 and 2007 was evaluated by acoustic immittance measurement and brainstem-evoked response audiometry that were performed within the first 30 days of disease and 6 months after the first evaluation. Patients with hearing disability after this period underwent further testing through otoacoustic emissions analysis. All patients were treated with intravenous administration of immunoglobulin. RESULTS: Ten (25%) of the 40 patients developed coronary aneurysm, and 22 (55%) disclosed hearing loss within the first 30 days, although this problem was noticed by the parents in only 3 children. In 12 (30%), the hearing loss persisted after 6 months. Ten (83.3%) of these 12 patients also showed thrombocytosis (platelets, >500,000), 8 (66%) had anemia (hemoglobin, <10 mg/dL), and all had high erythrocyte sedimentation rate (>50 mm/hr) persisting more than 30 days. CONCLUSION: SNHL was more prevalent than coronary complications. A significant association was found between persistent SNHL, although often not initially noted, with a prolonged presence of thrombocytosis, anemia, and high erythrocyte sedimentation rate and the delayed use of intravenous administration of immunoglobulin after the first 10 days of illness.
Assuntos
Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/epidemiologia , Síndrome de Linfonodos Mucocutâneos/complicações , Audiometria , Criança , Pré-Escolar , Estudos de Coortes , Esquema de Medicação , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/terapia , Prevalência , Fatores de TempoRESUMO
This is a prospective study of a cohort of 70 children with Kawasaki disease (KD) admitted from April 2002 to April 2007 to a tertiary hospital in Brasilia, Brazil. Of the 70 children, only 32 (45.7%) were referred during the acute phase of the disease and only 15 (21.4%) were referred with the correct diagnosis. Coronary aneurysms were detected in 13 (18.5%). A high suspicion index is essential in order to detect new cases as KD is probably underdiagnosed in Brazil and other developing countries, and an early diagnosis will dramatically decrease its life-threatening complications.
Assuntos
Síndrome de Linfonodos Mucocutâneos/epidemiologia , Distribuição por Idade , Brasil/epidemiologia , Criança , Pré-Escolar , Aneurisma Coronário/diagnóstico , Aneurisma Coronário/epidemiologia , Aneurisma Coronário/etiologia , Diagnóstico Diferencial , Ecocardiografia Doppler , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Incidência , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Síndrome de Linfonodos Mucocutâneos/terapia , Estudos Prospectivos , Resultado do TratamentoRESUMO
Objetivos: Chamar a atençäo dos pediatras para as características clínicas, critérios diagnósticos e a possibilidade de recorrência da fase aguda da Doença de Kawasaki e suas possíveis complicaçöes. Métodos: Descriçäo de um caso registrado no Hospital Universitário de Brasília, em prontuário n§ 40.0682 e revisäo bibliográfica. Resultado: Relato de caso de um menino que, após o desaparecimento dos sinais e sintomas de fase aguda, recidivou todo o quadro clínico da doença, vindo a ter, como complicaçäo, aneurisma de coronária esquerda. Conclusäo: O diagnóstico da Doença de Kawasaki é clínico, devendo o pediatra estar sempre atento para os critérios do diagnóstico, por se tratar de doença que cursa com complicaçöes cardíacas na fase aguda e com muito mais freqüência na sua recorrência. Essas complicaçöes cardíacas levam ao óbito 2 por cento dos pacientes