Assuntos
Doenças Hereditárias Autoinflamatórias/diagnóstico , Criança , Pré-Escolar , Congressos como Assunto , Diagnóstico Diferencial , Febre/etiologia , Doenças Hereditárias Autoinflamatórias/etiologia , Doenças Hereditárias Autoinflamatórias/terapia , Humanos , Linfadenite/etiologia , Faringite/etiologia , Estomatite Aftosa/etiologiaRESUMO
OBJECTIVE: To identify any adverse effects of colchicine in a pediatric patients with familial Mediterranean fever (FMF). STUDY DESIGN: Clinical presentation, Mediterranean fever gene genotype, disease duration, colchicine dose, laboratory tests, and reported adverse effects in children with FMF were analyzed. RESULTS: Of the 153 patients with FMF, 22 (14.4%) developed diarrhea during a follow-up of 4 years; the colchicine dose was reduced to control this symptom in only 4 patients. In 18 (11.8%) patients, a mild transitory increase of transaminases (45-158 IU/L) was found during a follow-up of 1 year. Blood cell counts and kidney function tests were normal in all patients. No correlation was found between the adverse effects and patient's age, disease onset, treatment duration, or any of the clinical characteristics of the disease. CONCLUSION: Colchicine is a safe drug in the treatment of children with FMF, even in infancy. The only significant adverse effects are diarrhea (in a small number of patients), which can be controlled by a decrease in the colchicine dose and transitory elevation of transaminases.
Assuntos
Colchicina/efeitos adversos , Febre Familiar do Mediterrâneo/tratamento farmacológico , Moduladores de Tubulina/efeitos adversos , Adolescente , Fatores Etários , Criança , Pré-Escolar , Colchicina/uso terapêutico , Proteínas do Citoesqueleto/genética , Análise Mutacional de DNA , Diarreia/induzido quimicamente , Relação Dose-Resposta a Droga , Esquema de Medicação , Febre Familiar do Mediterrâneo/diagnóstico , Febre Familiar do Mediterrâneo/genética , Feminino , Seguimentos , Marcadores Genéticos , Humanos , Lactente , Masculino , Estudos Prospectivos , Pirina , Sistema de Registros , Fatores de Tempo , Resultado do Tratamento , Moduladores de Tubulina/uso terapêuticoRESUMO
OBJECTIVE: To assess linear growth in patients with persistent oligoarticular juvenile idiopathic arthritis (JIA) treated by intra-articular corticosteroid injections (IACSI). STUDY DESIGN: Data were obtained from a retrospective review of the charts of 95 patients with persistent oligoarticular JIA (69 females). The mean age at first visit was 4.9 ± 3.4 years, with follow-up of 6 ± 3.7 years. The height SDS for chronologic age (z-score) was correlated with the clinical course of the disease and compared among patients treated by IACSI alone (group I) or by a combination of disease-modifying antirheumatic drugs (DMARDs) (group II). RESULTS: Growth retardation was found in 35.8% of patients (Δ z-score <-0.3), including 11.6% with severe growth retardation (Δ z-score <-1.0). Growth retardation was found in a smaller proportion of patients in group I (any growth retardation, 30.6%; severe growth retardation, 6.5%) than in patients in group II (any growth retardation, 44.4%; severe growth retardation, 21.2%; P < .05). Elevated erythrocyte sedimentation rate values (≥ 40 mm/1sth) indicated a significantly higher risk for growth retardation. All other clinical variables had no association with growth retardation. CONCLUSION: A significant proportion of patients with persistent oligoarticular JIA have growth retardation and a minority have severe growth retardation. Only elevated erythrocyte sedimentation rate values were proven to be a good predictor of risk for growth retardation.
Assuntos
Artrite Juvenil/complicações , Transtornos do Crescimento/etiologia , Adolescente , Idade de Início , Antirreumáticos/uso terapêutico , Artrite Juvenil/tratamento farmacológico , Sedimentação Sanguínea , Estatura , Criança , Pré-Escolar , Feminino , Seguimentos , Glucocorticoides/uso terapêutico , Humanos , Lactente , Injeções Intra-Articulares , Masculino , Estudos Retrospectivos , Triancinolona Acetonida/uso terapêuticoRESUMO
OBJECTIVE: To characterize the clinical and genetic features of familial Mediterranean fever (FMF). STUDY DESIGN: Clinical presentation and MEditerranean FeVer mutation type of all patients with FMF, who first manifested the disease at < or =2 years of age were analyzed and compared with patients who first presented with FMF between 2 and 16 years. RESULTS: Of 814 patients with FMF, in 254 patients (31.2%) the first FMF attack was at < or =2 years of age, with a mean age at onset of 1.1 +/- 0.8 years. They were compared with 242 patients who presented with their first manifestation of FMF at 2 to 16 years. The clinical manifestations of FMF were comparable in the 2 patient groups, but the delay of diagnosis was longer in patients with early presentation (3.2 +/- 3.2 years vs.1.9 +/- 2.7 years in the group with onset at 2-16 years, P < .001). A subgroup of patients (60/254), who were diagnosed at < or =2 years had the highest rate of attacks of fever alone as their sole manifestation (40.0% vs 8.4%, P < .05), and less peritonitis (45% vs 86.1%, P < .05) and pleuritis (3.4% vs 32.9%, P < .05). Most of these patients were homozygous for the M694V mutation and were of North African (Sephardic Jewish) extraction. CONCLUSION: In early life, FMF often begins with an atypical presentation, characterized by attacks of fever alone, and its diagnosis and initiation of treatment is therefore significantly delayed.
Assuntos
Febre Familiar do Mediterrâneo/diagnóstico , Adolescente , Idade de Início , Criança , Pré-Escolar , Proteínas do Citoesqueleto/genética , Análise Mutacional de DNA , Febre Familiar do Mediterrâneo/epidemiologia , Febre Familiar do Mediterrâneo/genética , Feminino , Humanos , Lactente , Masculino , Mutação , Fenótipo , PirinaRESUMO
OBJECTIVE: To perform a retrospective study comparing clinical and laboratory aspects of patients with acute rheumatic fever (ARF) and patients with post-streptococcal reactive arthritis (PSRA), to discern whether these are 2 separate entities or varying clinical manifestations of the same disease. STUDY DESIGN: We located the records of 68 patients with ARF and 159 patients with PSRA, whose diseases were diagnosed with standardized criteria and treated by 8 pediatric rheumatologists in 7 medical centers, using the Israeli internet-based pediatric rheumatology registry. The medical records of these patients were reviewed for demographic, clinical, and laboratory variables, and the data were compared and analyzed with univariate, multivariate, and discriminatory analysis. RESULTS: Four variables were found to differ significantly between ARF and PSRA and serve also as predictors: sedimentation rate, C-reactive protein, duration of joint symptoms after starting anti-inflammatory treatment, and relapse of joint symptoms after cessation of treatment. A discriminative equation was derived that enabled us to correctly classify >80% of the patients. CONCLUSION: On the basis of simple clinical and laboratory variables, we were able to differentiate ARF from PSRA and correctly classify >80% of the patients. It appears that ARF and PSRA are distinct entities.