Relapsing polychandritis in the childhood: case report and literature review

Relapsing polychondritis is a rare disease characterized by recurrent episodes of inflammation of the articular, auricular, nasal and laryngotracheal cartilages of variable intensity. Differential diagnosis includes connective tissue diseases, vasculitis and periodic febrile syndromes of childhood. We report a case of 11 year-old boy with knee arthritis, acute conjunctivitis, ear, nose and upper airway cartilage involvement whose disease was controlled with the use of corticosteroids and immunosuppressive drugs. The clinical and laboratory findings were discussed and compared with case reports from literature.A policondrite recidivante é uma doença rara caracterizada por episódios recorrentes de inflamação das cartilagens articular, auricular, nasal e laringotraqueal de intensidade variável. O diagnóstico diferencial inclui as doenças difusas do tecido conjuntivo, as vasculites e as síndromes febris periódicas na infância. Relatamos o caso de um adolescente de 11 anos com artrite de joelhos, conjuntivite aguda, acometimento de orelha, nariz e vias aéreas superiores cuja doença foi controlada com o uso de corticosteróides e imunossupressores. Os achados clínicos e laboratoriais são discutidos e comparados com os dados da literatura.UNIFESP-EPM Departamento de PediatriaUNIFESP, EPM, Depto. de PediatriaSciEL

Rehabilitation in juvenile idiopathic arthritis

INTRODUCTION AND AIMS: juvenile idiopathic arthritis (JIA) may cause permanent physical disabilities in children and adolescents. This study aimed to describe the several kinds of rehabilitation procedures, ranging from evaluation to prescription of exercises, as well as the elaboration of a practical rehabilitation guide for JIA patients. SOURCES OF DATA: the research was based on data from Medline and Lilacs. The opinion of experts working on the Pediatric Rheumatology service from Lar Escola São Francisco and Universidade Federal de São Paulo (UNIFESP) was considered on the debate of several topics. SUMMARY: JIA patients may present pain and limitation of joint movement thereby leading to decrease in physical capacity, affecting both aerobic and anaerobic activities. In addition to the joint compromise, cardiac and autonomic dysfunctions collaborate on this process, impairing sport and everyday activities. The American College of Rheumatology recommends 30-minute activity with moderate intensity, two to three times weekly. Hydrotherapy is associated to treatment adherence, besides helping in decreasing pain perception and adding to cope with daily activities. Other rehabilitation modalities, such as massage, education, joint protection, energy conservation, and splints are also considered in the present review. CONCLUSION: there are few studies in the literature focusing on rehabilitation in children with JIA. Particularly, there is a lack of studies concerning aspects of adequate prescription of exercises, weight-bearing, number of series and repetitions, as well as the best choice regarding ground or water activity. We believe that additional information is needed in order to improve the physical care to these patients.INTRODUÇÃO E OBJETIVOS: a artrite idiopática juvenil (AIJ) pode acarretar em seu curso clínico incapacidades físicas permanentes em crianças e adolescentes. Este estudo teve como objetivos a des-crição das diversas modalidades de reabilitação, desde a avaliação até a prescrição de exercícios, bem como a elaboração de um guia prático de reabilitação para pacientes com AIJ. FONTE DE DADOS: a pesquisa foi realizada nas bases de dados do Medline e do Lilacs. Na discussão dos diversos tópicos, foi considerada a experiência dos especialistas em reumatologia pediátrica e reabilitação do Lar Escola São Francisco e da Universidade Federal de São Paulo (UNIFESP). RESUMO: os pacientes com AIJ podem apresentar dor e limitação da amplitude de movimento articular e conseqüente diminuição da capacidade física, com comprometimento das capacidades aeróbia e anaeróbia. Não só o comprometimento articular, mas as disfunções cardíacas e autonômicas colaboram nesse processo, tendo como conseqüência uma baixa capacidade de executar atividades esportivas e atividades de vida diárias (AVDs). O American College of Rheumatology recomenda 30 minutos de atividade com intensidade moderada de duas a três vezes por semana. A hidroterapia está relacionada a uma maior aderência ao tratamento, além de auxiliar na diminuição da percepção dolorosa e dificuldade apresentada na realização das AVDs. As outras modalidades de reabilitação, tais como massagem, educação, proteção articular, conservação de ener-gia e órteses, também são discutidas nesta revisão. CONCLUSÃO: há poucos estudos na literatura sobre reabilitação em crianças com AIJ, especialmente no que se refere a temas como prescrição adequada de exercícios, cargas, número de séries e repetições, bem como qual a melhor opção a ser utilizada - solo ou piscina. Acreditamos que mais estudos científicos são necessários para que possamos prescrever adequadamente os diversos tipos de exercícios.UNIFESP-EPM Departamento de MedicinaUNIFESP-EPM Departamento de Neurologia e NeurocirurgiaUNIFESP-EPM Departamento de PediatriaUNIFESP Departamento de Pediatria Setor de Reumatologia PediátricaUNIFESP, EPM, Depto. de MedicinaUNIFESP, EPM Depto. de Neurologia e NeurocirurgiaUNIFESP, EPM Depto. de PediatriaUNIFESP, Depto. de Pediatria Setor de Reumatologia PediátricaSciEL

Diffuse idiopathic musculoskeletal pain in childhood and adolescence

OBJECTIVE: Persistent or intermittent diffuse idiopathic musculoskeletal pain is defined by the presence of pain over three months with no apparent etiology. The diagnosis of this condition in children and adolescents is increasing. The objective of this study was to report the cases of two patients with disabling pain that received multidiscipli-nary treatment and to review the current literature about this subject. CASE DESCRIPTION: The first patient is a nine-year old girl with history of headache, musculoskeletal pain and abdominal pain for two years. During this period she had four episodes of absence of gait with no apparent organic cause. The second patient is a 14-year girl with severe daily low back pain for 14 months, followed by headache and fatigue. Both patients presented a normal physical examina-tion, except for allodynia (case 1) and fibromyalgia tender points (case 2). All tests resulted negative regarding the search for organic reasons for pain complaints. Significant clinical improvement was observed with a multidisciplinary approach offered by pediatricians, psychologists, physical therapists, and nutritionists. Both patients resumed their daily regular activities. COMMENTS: Team integrated performance in a short-term period can provide the attenuation of pain symptoms since emotional, physical and nutritional factors are related to the pain expressed by the patients.OBJETIVO: A dor musculoesquelética difusa idiopática persistente ou intermitente é definida pela presença de dor com duração de três meses ou mais, sem etiologia aparente, e tem acometido cada vez mais crianças e adolescentes. Este estudo objetivou relatar dois pacientes com dor incapacitante submetidos à abordagem e ao tratamento multiprofissional, além de discutir o tratamento conduzido, por meio de revisão da literatura atual. DESCRIÇÃO DO CASO: O primeiro caso é de uma menina com nove anos de idade, com histórico de cefaleia, dor mus-culoesquelética e dor abdominal há dois anos, período no qual apresentou quatro episódios de ausência de marcha, sem causas orgânicas aparentes. O segundo caso refere-se a uma adolescente de 14 anos com lombalgia diária há 14 meses de forte intensidade, com irradiação para membros inferiores, acompanhada de cefaleia, fadiga e adinamia. Ambas apresentavam exame físico normal, exceto pela presença de alodínia (caso 1) e pontos de fibromialgia (caso 2); além de exames subsidiários normais, sem justificativas orgânicas para as queixas dolorosas. Houve melhora clínica significativa após diagnóstico e tratamento diferencial por meio de abordagem multiprofissional (médico, psicólogo, fisioterapeuta e nutricionista), com retomada das atividades regulares pelas pacientes. COMENTÁRIOS: A atuação integrada da equipe em um curto período de tempo proporcionou às pacientes a retomada de suas atividades normais, visto que fatores emocionais, físicos e nutricionais relacionam-se à dor.Universidade Federal de São Paulo (UNIFESP)Universidade Federal de São Paulo, EPM, São Paulo, BrazilSciEL

Resource utilization and cost of rheumatic fever

Objective. The socioeconomic effects of rheumatic fever (RF) in Brazil, including direct and indirect costs to patients and their families and to society, are largely unknown. We evaluated the utilization of resources and costs related to RF in a tertiary center caring for low income patients in the city of Sao Paulo, Brazil.Methods. One hundred patients with RE younger than 18 yrs, with followup of at least one year, were sequentially selected to provide complete information on a questionnaire. Additional data were collected from patients' charts. The utilization of resources was evaluated for each patient throughout the entire disease course. Costs were determined for patients and their families as well as for the society, using variables from 3 different systems: the national public health system, used by most lower income groups; the Brazilian Medical Association, which regulates charges and fees utilized by health plans and insurance companies; and costs charged by private practitioners, paid directly by patients.Results, The RF population studied belonged to a low socioeconomic level. The mean monthly family income was $625.20 US. The mean disease duration was 3.9 yrs (range 1-10). Patients had a total of 1657 medical consultations, 22 hospital admissions, and 4 admissions to intensive care unit. Work absenteeism among parents was calculated as 22.9$105,860 US ($271/patient/yr),$18,803 US ($48/patient/yr), and$124,663 US (US $319/patient/yr), respectively. When health care plan and private systems were taken as reference, the total costs were$423,550 US and $684,351 US, respectively.Conclusion. RF and rheumatic heart disease have an important socioeconomic impact in Brazil; costs of RF made up roughly 1.3$51,144,347.00 US.Univ Fed Sao Paulo, Escola Paulista Med, Dept Pediat, Div Rheumatol Allergy & Immunol,UNIFESP, Sao Paulo, BrazilUniv Fed Sao Paulo, Escola Paulista Med, Dept Med, Unit Clin Epidemiol,Div Rheumatol,UNIFESP, Sao Paulo, BrazilUniv Fed Sao Paulo, Escola Paulista Med, Dept Pediat, Div Rheumatol Allergy & Immunol,UNIFESP, Sao Paulo, BrazilUniv Fed Sao Paulo, Escola Paulista Med, Dept Med, Unit Clin Epidemiol,Div Rheumatol,UNIFESP, Sao Paulo, BrazilWeb of Scienc

Leprosy: An unusual cause of arthritis in children. A report of two cases

Universidade Federal de São Paulo,ESCOLA PAULISTA MED,DEPT MED,DIV RHEUMATOL,São Paulo,BRAZILUniversidade Federal de São Paulo,ESCOLA PAULISTA MED,DEPT MED,DIV RHEUMATOL,São Paulo,BRAZILWeb of Scienc

Uveitis in juvenile idiopathic arthritis

Objective: to evaluate the frequency of chronic anterior uveitis in patients with juvenile idiopathic arthritis and its association with the presence of antinuclear antibodies. Patients and methods: we retrospectively studied 72 patients with juvenile idiopathic arthritis. All of them were submitted to slit-lamp examination of the anterior chamber at diagnosis. Both antinuclear antibodies and rheumatoid factor were determined. Patients with positive results for antinuclear antibodies were evaluated every three months and those with negative results were assessed every six months.Results: forty patients were male (55.5%) and 36 were Caucasoid (50%). The mean age at the onset of juvenile idiopathic arthritis was 6.4 years (range = 1 to 14 years) and the mean age at the beginning of the study was 10.4 years (1 to 19 years). According to the type of disease at onset, 32 were pauciarticular (44.4%) (17 boys and 15 girls), 30 were polyarticular (41.6%) (17 boys and 13 girls) and 10 were systemic (14%) (6 boys and 4 girls). We observed chronic anterior uveitis in five patients (6.5%) (mean age = 11.4 years). Among them, four (80%) had pauciarticular juvenile idiopathic arthritis at disease onset (three girls with type I juvenile idiopathic arthritis and positive antinuclear antibodies and one boy with type I juvenile idiopathic arthritis and negative antinuclear antibodies) and one girl with polyarticular juvenile idiopathic arthritis (negative antinuclear antibodies and rheumatoid factor). In this group, the mean age at the onset of juvenile idiopathic arthritis was 5.1 years and the mean age of uveitis onset was 9 years. Antinuclear antibodies were positive in 3/5 patients (60%) with uveitis. Antinuclear antibodies were positive in 12% of the patients without uveitis (n = 67). Among the patients with uveitis, three had only one flare and the other two had four flares with cataract. The frequency of antinuclear antibodies was statistically higher in the patients with uveitis (P< 0.05).Conclusion: although the incidence of uveitis in our study was lower than that reported in the literature, the frequency of uveitis was higher in females, in those with pauciarticular juvenile idiopathic arthritis and in patients with positive antinuclear antibodies.Objetivo: avaliar a freqüência de uveíte anterior crônica em pacientes com artrite idiopática juvenil (AIJ) e sua associação com a presença do fator antinúcleo (FAN). Casuística e métodos: foram avaliadas, retrospectivamente, 72 crianças com diagnóstico confirmado de AIJ, que tiveram avaliação oftalmológica através da biomicroscopia para exame da câmara anterior da úvea, determinação do FAN (imunofluorescência indireta) e fator reumatóide (FR) (prova do látex) no período inicial da doença. Para os pacientes com FAN positivo, esta avaliação foi realizada a cada três meses, e, caso contrário, a cada seis meses.Resultados: dos 72 pacientes com AIJ, 40 (55,5%) crianças eram do sexo masculino e 36 (50%) caucasóides. A idade média de início da AIJ foi 6,4 anos (1 a 14 anos), e a idade média na época do estudo foi de 10,4 anos (1 a 19 anos). De acordo com o tipo de início da AIJ, em 32 (44,4%) crianças era pauciarticular (17 meninos e 15 meninas), em 30 (41,6%) era poliarticular (17 meninos e 13 meninas), e em 10 (14%) era sistêmico (6 meninos e 4 meninas). A presença de uveíte anterior crônica foi detectada em cinco (6,5%) crianças com idade média de 11,4 anos. Destas, quatro (80%) eram do tipo de início pauciarticular (três meninas do tipo I, com FAN positivo, e um menino do tipo I, FAN negativo), e uma menina com doença poliarticular (FAN e FR negativos). Neste grupo, a idade de início da AIJ foi em média 5,1 anos (3 a 12 anos), e a idade de início da uveíte foi em média de 9 anos (4 a 16 anos). O FAN foi positivo em 3/5 pacientes (60%) com uveíte. Dentre os pacientes com AIJ e sem uveíte (67 crianças), oito (12%) apresentaram FAN positivo. Quanto à evolução dos pacientes com comprometimento ocular, três crianças tiveram apenas um episódio de uveíte, e duas crianças apresentaram quatro recorrências da uveíte com catarata sem atividade articular associada. A presença do FAN foi estatisticamente mais freqüente na população de pacientes com AIJ associada com uveíte (60%) do que naqueles sem uveíte (12%) (p<0,05).Conclusão: concluímos que, apesar do pequeno número de pacientes com AIJ e uveíte anterior crônica encontrados (6,5%), a freqüência foi maior no sexo feminino e na doença de início pauciarticular, com presença do FAN na maioria dos casos

Health related quality of life of children with rheumatic heart diseases: reliability of the Brazilian version of the pediatric quality of life inventory (TM) cardiac module scale

Background: This study aimed to translate the 'Pediatric Quality of Life Inventory (TM) (PedsQL (TM) 3.0) Cardiac Module' into Portuguese, adapt it to Brazilian culture, and assess its psychometric properties (validity and reproducibility), and to calculate health-related quality of life scores on the PedsQL 4.0 and PedsQL (TM) 3.0 Cardiac Module Scales for a group of patients 5 to 18 years old with rheumatic heart disease.Methods: the methods suggested by the authors of the original version of the questionnaire included 1) translation by an expert panel; 2) translation back into English and revision by the authors of the original version; 3) pilot study with seven children and parents in each of three age ranges (5 to 7, 8 to 12, and 13 to 18 years old); and 4) assessment of the measurement properties. in this stage, the PedsQL (TM) 3.0 Cardiac Module and the PedsQL 4.0 Generic Scale were applied to a sample comprising 109 children and adolescents with rheumatic heart disease and their parents or caregivers. the version for parents or caregivers was administered separately on the same day.Results: the values of Cronbach's alpha for all scales assessed in the questionnaire (heart problems and treatment [symptoms], problems with perceived physical appearance, treatment anxiety, cognitive problems, and communication problems) varied from 0.6 to 0.8, indicating good internal consistency. Correlation was found between the scores for the Cardiac Module and the Generic Scale (0.36-0.86), demonstrating convergent validity (Spearman's correlation coefficient, p < 0.01). the symptoms, problems with perceived physical appearance, and cognitive and communication problem domains were able to distinguish between groups of patients with mild and moderate/severe heart disease (Student's t-test, p < 0.05). the intraclass correlation of the interobserver reproducibility was adequate (0.76 to 0.94 among the patients [children/adolescents] and 0.76 to 0.84 among their caregivers). the correlation between the patients' scores and their parents' scores varied from 0.50 to 0.86 (Pearson's correlation coefficient, p < 0.01).Conclusions: the Brazilian version of the PedsQL (TM) 3.0 Cardiac Module was shown to be reliable. the application of this questionnaire in practice will be very useful for all professionals charged with the care of children and adolescents with heart diseases

Reliability of the Pediatric Quality of Life Inventory - Healthcare Satisfaction Generic Module 3.0 version for the assessment of the quality of care of children with chronic diseases

OBJECTIVE: To translate to Brazilian Portuguese and to evaluate the reliability of the Healthcare Satisfaction (HS) Generic Module version 3.0 of the PedsQL inventory for the Brazilian culture. METHOD: The methods recommended by the authors of the original version of the questionnaire were used: 1) translation by bilingual experts; 2) back translation into English and evaluation by the authors of the original version; 3) a pilot study including 10 parents (cognitive interview); and 4) evaluation of the measurement properties of the questionnaire. The questionnaire was administered to 60 parents of children and adolescents with chronic diseases in key units of the Department of Pediatrics. RESULTS: Cronbach's alpha ranged from 0.57 to 0.78 for all domains (information, family inclusion, communication, technical skills, emotional needs and overall satisfaction), which confirmed good internal consistency. Test-retest reproducibility was evaluated using 20% of the sample (Spearman correlation coefficient = 0.909). The satisfaction score was good in all areas (> 4.3 points) except in the emotional needs domain. CONCLUSION: The Brazilian version of the HS-PedsQL is a valid and reliable tool to measure the overall satisfaction of parents and caregivers with the treatment of their children.OBJETIVO: Traduzir e avaliar a confiabilidade do módulo Satisfação com o Serviço de Saúde do questionário Pediatric Quality of Life Inventory versão 3.0 (SSS-PedsQL) para o idioma português e para a cultura brasileira. MÉTODO: Utilizamos a metodologia proposta pelos autores da versão original do questionário: 1) tradução por especialistas bilíngues; 2) tradução reversa para o inglês com avaliação pelos autores da versão original; 3) estudo piloto envolvendo 10 pais (entrevista cognitiva); e 4) avaliação das propriedades de medida do questionário, sendo administrado a um grupo de 60 pais de crianças e adolescentes com doenças crônicas em acompanhamento nos departamentos de pediatria de um hospital-escola. RESULTADOS: Obtivemos alfa de Cronbach entre 0,57 e 0,78 para os domínios do questionário (informação, inclusão familiar, comunicação, habilidades técnicas, necessidades emocionais e satisfação geral), demonstrando uma boa consistência interna. Foi avaliada a reprodutibilidade interobservador em 20% da amostra, com coeficiente de correlação de Spearman de 0,909. Observamos um ótimo escore de satisfação com as equipes multiprofissionais para todos os domínios (> 4,3 pontos), exceto para os domínios necessidades emocionais, com 3,5 pontos, e comunicação, com 3,9. CONCLUSÃO: A versão brasileira do SSS-PedsQL é válida e confiável e é útil na mensuração da satisfação global dos pais e/ou cuidadores com o tratamento dos seus filhos