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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.
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BACKGROUND: Paediatric inflammatory multisystem syndrome (PIMS) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been described since mid-April 2020 with the first reports coming from Europe. Our objective was to describe the characteristics of patients among the Brazilian population. METHODS: A multicenter retrospective study was conducted with the participation of five pediatric rheumatology centers in Brazil during the period from March to November 2020. Children and adolescents with PIMS temporally associated with SARS-CoV-2 (TS) who met the definition criteria for the disease according to the Royal College of Paediatrics and Child Health were included. Demographic, clinical, laboratory, therapeutic characteristics and molecular and serological diagnosis of SARS-CoV-2 infection were described. RESULTS: Fifty-seven children and adolescents with PIMS-TS were evaluated, 54% female, with a median age of 8 (3-11) years. Most (86%) were previously healthy, with asthma being the main comorbidity, present in 10% of the patients. Fever was the main manifestation, present in all patients, followed by mucocutaneous and gastrointestinal features, present in 89% and 81% of the patients, respectively. Myocarditis occurred in 21% of the patients and in 68% of them required intensive care. The Kawasaki disease phenotype occurred in most patients (77%). All patients had elevated inflammatory markers, with elevated CRP being the most found (98%). Anemia and lymphopenia were present in 79% and 72%, respectively. Laboratory evidence of SARS-CoV-2 was found in 77% of the patients, with 39% positive RT-PCR and 84% positive serology for SARS-CoV-2. An immunomodulatory treatment was performed in 91% of the patients, with 67% receiving intravenous immunoglobulin (IVIG) associated with glucocorticoid, 21% receiving IVIG, and 3.5% receiving glucocorticoid. The median length of hospitalization was 10 days. CONCLUSIONS: This study showed a high morbidity of PIMS-TS in Brazilian children, with a prolonged length of hospitalization and a high rate of admission to pediatric intensive care unit. Multicenter prospective studies are needed to assess the morbidity of the disease in the medium and long term.
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COVID-19 , Adolescente , Brasil/epidemiologia , COVID-19/complicações , Criança , Feminino , Humanos , Masculino , Estudos Retrospectivos , SARS-CoV-2 , Síndrome de Resposta Inflamatória SistêmicaRESUMO
Abstract Background: Paediatric inflammatory multisystem syndrome (PIMS) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been described since mid-April 2020 with the first reports coming from Europe. Our objective was to describe the characteristics of patients among the Brazilian population. Methods: A multicenter retrospective study was conducted with the participation of five pediatric rheumatology centers in Brazil during the period from March to November 2020. Children and adolescents with PIMS temporally associated with SARS-CoV-2 (TS) who met the definition criteria for the disease according to the Royal College of Paediatrics and Child Health were included. Demographic, clinical, laboratory, therapeutic characteristics and molecular and serological diagnosis of SARS-CoV-2 infection were described. Results: Fifty-seven children and adolescents with PIMS-TS were evaluated, 54% female, with a median age of 8 (3-11) years. Most (86%) were previously healthy, with asthma being the main comorbidity, present in 10% of the patients. Fever was the main manifestation, present in all patients, followed by mucocutaneous and gastrointestinal features, present in 89% and 81% of the patients, respectively. Myocarditis occurred in 21% of the patients and in 68% of them required intensive care. The Kawasaki disease phenotype occurred in most patients (77%). All patients had elevated inflammatory markers, with elevated CRP being the most found (98%). Anemia and lymphopenia were present in 79% and 72%, respectively. Laboratory evidence of SARS-CoV-2 was found in 77% of the patients, with 39% positive RT-PCR and 84% positive serology for SARS-CoV-2. An immunomodulatory treatment was performed in 91% of the patients, with 67% receiving intravenous immunoglobulin (IVIG) associated with glucocorticoid, 21% receiving IVIG, and 3.5% receiving glucocorticoid. The median length of hospitalization was 10 days. Conclusions: This study showed a high morbidity of PIMS-TS in Brazilian children, with a prolonged length of hospitalization and a high rate of admission to pediatric intensive care unit. Multicenter prospective studies are needed to assess the morbidity of the disease in the medium and long term.
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Background and objectives: The present study is the first known in Latin America to enroll a substantial number of Kawasaki disease (KD) patients with an extended follow-up. This study aimed to: (1) to expose the difficulties and delays in the diagnosis of KD in a developing country, (2) to describe and correlate the clinical features of this disorder with the children's age at the time of disease onset, (3) to correlate the frequent lack of early diagnosis with a delayed application of appropriate treatment, and (4) to describe the outcome and eventual recurrences of KD in our region. Materials and Methods: Three hundred and one participants (183 males and 118 females) included in the study were diagnosed and, subsequently, clinically followed for ten years (January 2007 to December 2016) at the Pediatric Rheumatology Walk-in Clinic of the Children's Hospital of Brasilia. Results: Episodes ranged from four months to two years. This rate of recurrence was well-above that disclosed by previous reports. Delay in diagnosis, in all age groups, caused an undesirable delay between the disease onset, the final diagnosis, and the administration of intravenous immunoglobulin (IVIG). KD recurred in 25 (8.3%) of the children during the first three years of follow-up. In seven patients, KD recurred twice, with an interval between episodes ranging from four months to two years. Conclusions: This rate of recurrence was well-above that disclosed by previous reports. In Latin America, aside from a handful of physicians and researchers, KD is being ignored. There is a pressing need to educate primary health care physicians and bring awareness to the fact that KD is not an exotic condition that affects only the Asian populations but a disorder that already exists among us and that frequently results in severe consequences.
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Fatores Etários , Síndrome de Linfonodos Mucocutâneos/complicações , Brasil/epidemiologia , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Países em Desenvolvimento , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Síndrome de Linfonodos Mucocutâneos/fisiopatologiaRESUMO
Digital photoelasticity is an important segment of optical metrology for stress analysis by digital photographic images. Advances in digital image processing, data acquisition, standard recognition and data storage allow the utilization of computer-aided techniques in the automation and improvement of the digital photoelastic technique. The objective of this study is to develop new techniques using 3D rapid prototyping with transparent resins in digital photoelasticity. Some innovations are proposed (e.g., a tapered roller bearing built with 3D rapid prototyping with transparent resin and the final assembly with the specimens prototyped separately). A metrology study is carried out with the new techniques developed.
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BACKGROUND: Kawasaki disease (KD) is a self-limited acute systemic vasculitis of unknown aetiology that predominantly affects infants and young children eventually associated with immunological abnormalities. Coeliac disease (CD) is an inflammatory autoimmune disease characterised by a permanent gluten intolerance, which affects genetically susceptible individuals of any age group, and can cause intestinal and systemic symptoms. Association of CD with KD has been previously described in a single study that disclosed a surprisingly high prevalence of CD in children with a history of KD. OBJECTIVE: To confirm the existence of a higher prevalence of CD among individuals with a history of KD, which would turn the screening for CD in patients with history of KD highly advisable. SETTING: Children with history of KD, diagnosed and followed at the Rheumatology Clinic of the Children's Hospital of Brasilia (Brasilia, Brazil). PARTICIPANTS: This study included 110 children with history of KD and a control group composed of 110 presumably healthy children. INTERVENTIONS: Participants underwent anti-transglutaminase and anti-endomysial antibodies tests and genetic typing for the presence of CD predisposing alleles (HLA-DQ2 and DQ8). Jejunal biopsy was performed when necessary, according the European Society of Paediatric Gastroenterology, Hepatology and Nutrition guidelines. RESULTS: Diagnosis of CD was confirmed in one (0.91%) patient with KD by positive serological tests, presence of predisposing alleles and CD typical lesions on duodenal biopsy. All serological tests were negative among the controls. The prevalence of CD predisposing alleles among patients with KD was 29.09%, similar to the prevalence found among controls, 33.64%. CONCLUSION: The detected CD prevalence (0.91%) does not confirm the existence of an association between KD and CD since this prevalence is similar to that found in the general population (≃1%).
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Alelos , Autoanticorpos/sangue , Doença Celíaca/complicações , Duodeno/patologia , Síndrome de Linfonodos Mucocutâneos/complicações , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/epidemiologia , Biópsia , Brasil/epidemiologia , Estudos de Casos e Controles , Doença Celíaca/diagnóstico , Doença Celíaca/epidemiologia , Criança , Pré-Escolar , Estudos Transversais , Feminino , Antígenos HLA-DQ/genética , Humanos , Masculino , Chaperonas Moleculares/genética , Síndrome de Linfonodos Mucocutâneos/sangue , Síndrome de Linfonodos Mucocutâneos/genética , Síndrome de Linfonodos Mucocutâneos/imunologia , Prevalência , Valores de ReferênciaRESUMO
OBJECTIVE: To establish guidelines based on scientific evidence for the management of familial Mediterranean fever. DESCRIPTION OF THE EVIDENCE COLLECTION METHOD: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. RESULTS: 10,341 articles were retrieved and evaluated by title and abstract; from these, 46 articles were selected to support the recommendations. RECOMMENDATIONS: 1. The diagnosis of FMF is based on clinical manifestations, characterized by recurrent febrile episodes associated with abdominal pain, chest or arthritis of large joints. 2. FMF is a genetic disease presenting an autosomal recessive trait, caused by mutation in the MEFV gene. 3. Laboratory tests are not specific, demonstrating high serum levels of inflammatory proteins in the acute phase of the disease, but also often showing high levels even between attacks. SAA serum levels may be especially useful in monitoring the effectiveness of treatment. 4. The therapy of choice is colchicine; this drug has proven its effectiveness in preventing acute inflammatory episodes and progression toward amyloidosis in adults. 5. Based on the available information, the use of biological drugs appears to be an alternative for patients with FMF who do not respond or are intolerant to therapy with colchicine.
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Amiloidose Familiar/prevenção & controle , Colchicina/uso terapêutico , Febre Familiar do Mediterrâneo/diagnóstico , Febre Familiar do Mediterrâneo/terapia , Guias de Prática Clínica como Assunto , Pirina/genética , Amiloidose Familiar/genética , Medicina Baseada em Evidências , Febre Familiar do Mediterrâneo/genética , Humanos , Fenótipo , SíndromeRESUMO
OBJECTIVE: To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes. DESCRIPTION OF THE EVIDENCE COLLECTION METHOD: The Guideline was prepared from 4 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. RESULTS: 1215 articles were retrieved and evaluated by title and abstract; from these, 42 articles were selected to support the recommendations. RECOMMENDATIONS: 1. The diagnosis of CAPS is based on clinical history and clinical manifestations, and later confirmed by genetic study. CAPS may manifest itself in three phenotypes: FCAS (mild form), MWS (intermediate form) and CINCA (severe form). Neurological, ophthalmic, otorhinolaryngological and radiological assessments may be highly valuable in distinguishing between syndromes; 2. The genetic diagnosis with NLRP3 gene analysis must be conducted in suspected cases of CAPS, i.e., individuals presenting before 20 years of age, recurrent episodes of inflammation expressed by a mild fever and urticaria; 3. Laboratory abnormalities include leukocytosis and elevated serum levels of inflammatory proteins; and 4. Targeted therapies directed against interleukin-1 lead to rapid remission of symptoms in most patients. However, there are important limitations on the long-term safety. None of the three anti-IL-1ß inhibitors prevents progression of bone lesions.
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Síndromes Periódicas Associadas à Criopirina/diagnóstico , Síndromes Periódicas Associadas à Criopirina/terapia , Proteína 3 que Contém Domínio de Pirina da Família NLR/genética , Guias de Prática Clínica como Assunto , Idade de Início , Síndromes Periódicas Associadas à Criopirina/genética , Medicina Baseada em Evidências , Febre , Humanos , Inflamação/genética , Inflamação/imunologia , Interleucina-1beta , Mutação , Prognóstico , Índice de Gravidade de Doença , UrticáriaRESUMO
OBJECTIVE: To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. DESCRIPTION OF THE EVIDENCE COLLECTION METHOD: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. RESULTS: 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. RECOMMENDATIONS: 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development. 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes. 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients. 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA. 5. Despite promising results obtained in studies with IL-1ß inhibitors, such studies are limited to a few case reports.
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Febre/terapia , Linfadenite/terapia , Faringite/terapia , Guias de Prática Clínica como Assunto , Estomatite Aftosa/terapia , Adenoidectomia , Febre/diagnóstico , Febre/cirurgia , Humanos , Linfadenite/diagnóstico , Linfadenite/cirurgia , Faringite/diagnóstico , Faringite/cirurgia , Estomatite Aftosa/diagnóstico , Estomatite Aftosa/cirurgia , Síndrome , TonsilectomiaRESUMO
Resumo Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo da febre familiar do Mediterrâneo (FFM). Descrição do método de coleta de evidência: A diretriz foi elaborada a partir de 5 questões clínicas que foram estruturadas por meio do PICO (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados: Foram recuperados, e avaliados pelo título e resumo, 10.341 trabalhos e selecionados 46 artigos para sustentar as recomendações. Recomendações: 1. O diagnóstico da FFM é baseado nas manifestações clínicas, caracterizadas por episódios febris recorrentes associados a dor abdominal, torácica ou artrite de grandes articulações; 2. A FFM é uma doença genética que apresenta traço autossômico recessivo ocasionada por mutação no gene MEFV; 3. Exames laboratoriais são inespecíficos e demonstram níveis séricos elevados de proteínas inflamatórias na fase aguda da doença, mas também, com frequência, níveis elevados mesmo entre os ataques. Níveis séricos de SAA podem ser especialmente úteis no monitoramento da eficácia do tratamento; 4. A colchicina é a terapia de escolha e demonstrou eficácia na prevenção dos episódios inflamatórios agudos e progressão para amiloidose em adultos; 5. Com base na informação disponível, o uso de medicamentos biológicos parece ser opção para pacientes com FFM que não respondem ou que são intolerantes à terapia com colchicina.
Abstract Objective: To establish guidelines based on scientific evidence for the management of familial Mediterranean fever. Description of the evidence collection method: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. Results: 10,341 articles were retrieved and evaluated by title and abstract; from these, 46 articles were selected to support the recommendations. Recommendations: 1. The diagnosis of FMF is based on clinical manifestations, characterized by recurrent febrile episodes associated with abdominal pain, chest or arthritis of large joints; 2. FMF is a genetic disease presenting an autosomal recessive trait, caused by mutation in the MEFV gene; 3. Laboratory tests are not specific, demonstrating high serum levels of inflammatory proteins in the acute phase of the disease, but also often showing high levels even between attacks. SAA serum levels may be especially useful in monitoring the effectiveness of treatment; 4. The therapy of choice is colchicine; this drug has proven effectiveness in preventing acute inflammatory episodes and progression towards amyloidosis in adults; 5. Based on the available information, the use of biological drugs appears to be an alternative for patients with FMF who do not respond or are intolerant to therapy with colchicine.
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Humanos , Febre Familiar do Mediterrâneo/diagnóstico , Febre Familiar do Mediterrâneo/terapia , Colchicina/uso terapêutico , Guias de Prática Clínica como Assunto , Amiloidose Familiar/prevenção & controle , Pirina/genética , Febre Familiar do Mediterrâneo/genética , Fenótipo , Síndrome , Medicina Baseada em Evidências , Amiloidose Familiar/genéticaRESUMO
Resumo Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo das síndromes periódicas associadas à criopirina (criopirinopatias – Caps). Descrição do método de coleta de evidência: A diretriz foi elaborada a partir de quatro questões clínicas que foram estruturadas por meio do PICO (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultado: Foram recuperados, e avaliados pelo título e resumo, 1.215 artigos e selecionados 42 trabalhos para sustentar as recomendações. Recomendações: 1. O diagnóstico de Caps é baseado na anamnese e nas manifestações clínicas e posteriormente confirmado por estudo genético. Pode se manifestar sob três fenótipos: FCAS (forma leve), MWS (forma intermediária) e Cinca (forma grave). Avaliações neurológica, oftalmológica, otorrinolaringológica e radiológica podem ser de grande valia na distinção entre as síndromes; 2. O diagnóstico genético com análise do gene NLRP3 deve ser conduzido nos casos suspeitos de Caps, isto é, indivíduos que apresentam, antes dos 20 anos, episódios recorrentes de inflamação expressa por urticária e febre moderada; 3. As alterações laboratoriais incluem leucocitose e elevação nos níveis séricos de proteínas inflamatórias; 4. Terapias alvo dirigidas contra a interleucina 1 levam a rápida remissão dos sintomas na maioria dos pacientes. Contudo, existem limitações importantes em relação à segurança em longo prazo. Nenhuma das três medicações anti-IL1β evita progressão das lesões ósseas.
Abstract Objective: To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes. Description of the evidence collection method: The Guideline was prepared from 4 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. Results: 1215 articles were retrieved and evaluated by title and abstract; from these, 42 articles were selected to support the recommendations. Recommendations: 1. The diagnosis of CAPS is based on clinical history and clinical manifestations, and later confirmed by genetic study. CAPS may manifest itself in three phenotypes: FCAS (mild form), MWS (intermediate form) and CINCA (severe form). Neurological, ophthalmic, otorhinolaryngological and radiological assessments may be highly valuable in distinguishing between syndromes; 2. The genetic diagnosis with NLRP3 gene analysis must be conducted in suspected cases of CAPS, i.e., individuals presenting before 20 years of age, recurrent episodes of inflammation expressed by a mild fever and urticaria; 3. Laboratory abnormalities include leukocytosis and elevated serum levels of inflammatory proteins; and 4. Targeted therapies directed against interleukin-1 lead to rapid remission of symptoms in most patients. However, there are important limitations on the long-term safety. None of the three anti-IL-1β inhibitors prevents progression of bone lesions.
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Humanos , Guias de Prática Clínica como Assunto , Síndromes Periódicas Associadas à Criopirina/diagnóstico , Síndromes Periódicas Associadas à Criopirina/terapia , Proteína 3 que Contém Domínio de Pirina da Família NLR/genética , Prognóstico , Urticária , Índice de Gravidade de Doença , Idade de Início , Medicina Baseada em Evidências , Interleucina-1beta , Síndromes Periódicas Associadas à Criopirina/genética , Febre , Inflamação/genética , Inflamação/imunologia , MutaçãoRESUMO
Resumo Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo da síndrome de febre periódica, estomatite aftosa, faringite e adenite (PFAPA). Descrição do método de coleta de evidência: A Diretriz foi elaborada a partir de cinco questões clínicas que foram estruturadas por meio do Pico (Paciente, Intervenção ou Indicador, Comparação e Outcome), com busca nas principais bases primárias de informação científica. Após definir os estudos potenciais para sustento das recomendações, esses foram graduados pela força da evidência e pelo grau de recomendação. Resultados: Foram recuperados e avaliados pelo título e resumo 806 trabalhos e selecionados 32 artigos, para sustentar as recomendações. Recomendações: 1. O diagnóstico da PFAPA é clínico e de exclusão, deve a suspeita ser considerada em crianças que apresentam episódios febris de origem indeterminada recorrentes e periódicos ou amidalites de repetição, intercalados com períodos assintomáticos, sobretudo em crianças em bom estado geral e com desenvolvimento pondero-estatural mantido; 2. Os achados laboratoriais são inespecíficos. Não existem alterações patognomônicas nos exames complementares; 3. A evidência que sustenta a indicação do tratamento cirúrgico (tonsilectomia com ou sem adenoidectomia) é baseada em dois ensaios clínicos randomizados não cegos que incluíram pequeno número de pacientes; 4. O uso de prednisona no início do quadro febril em pacientes com PFAPA mostrou ser eficaz. Melhores evidências ainda são necessárias para apoiar seu uso na PFAPA; 5. Apesar de os resultados obtidos de estudos com inibidores de IL-1ß serem promissores, esses são limitados a poucos relatos de casos.
Abstract Objective: To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. Description of the evidence collection method: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. Results: 806 articles were retrieved and evaluated by title and abstract; from these, 32 articles were selected to support the recommendations. Recommendations: 1. PFAPA is a diagnosis of exclusion established on clinical grounds, and one must suspect of this problem in children with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis interspersed with asymptomatic periods, especially in children in good general condition and with preservation of weight and height development. 2. Laboratory findings are nonspecific. Additional tests do not reveal pathognomonic changes. 3. The evidence supporting an indication for surgical treatment (tonsillectomy with or without adenoidectomy), is based on two non-blinded randomized clinical trials with small numbers of patients. 4. The use of prednisone at the onset of fever in patients with PFAPA proved to be an effective strategy. There is still need for more qualified evidence to support its use in patients with PFAPA. 5. Despite promising results obtained in studies with IL-1β inhibitors, such studies are limited to a few case reports.
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Humanos , Estomatite Aftosa/terapia , Faringite/terapia , Guias de Prática Clínica como Assunto , Febre/terapia , Linfadenite/terapia , Estomatite Aftosa/cirurgia , Estomatite Aftosa/diagnóstico , Síndrome , Tonsilectomia , Adenoidectomia , Faringite/cirurgia , Faringite/diagnóstico , Febre/cirurgia , Febre/diagnóstico , Linfadenite/cirurgia , Linfadenite/diagnósticoRESUMO
This paper describes the methodology used to translate the MCMI-III into Brazilian Portuguese and presents results from a reliability study conducted in Brazil with the translated instrument. The sample was comprised of 222 Brazilian college students. Test-retest correlations between first and second testings (over a period of 7 to 14 days) ranged from .70 to .93 across 27 scales. Cronbach's alpha coefficients ranged from .54 to .87. The procedures described in this paper represent preliminary steps in the development of a Brazilian Portuguese translation of the MCMI-III. Further studies are needed to obtain reliability data with a clinical sample, evaluate the diagnostic validity of the scales, and derive appropriate base rates for the Brazilian population.
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Idioma , Inventário de Personalidade/normas , Inquéritos e Questionários/normas , Adulto , Brasil , Feminino , Humanos , Masculino , Inventário de Personalidade/estatística & dados numéricos , Reprodutibilidade dos Testes , Estudantes/psicologia , Traduções , Adulto JovemRESUMO
The present report describes the severe evolution of Kawasaki disease in a three-month-old infant. The ailment was initially atypical in its presentation, with the patient exhibiting only persistent fever in association with a progressive lethargy and maculopapular rash on the face, trunk and limbs erroneously diagnosed as roseola infantum. On the 10th day of the condition, mainly due to the unexplained persistence of fever, the infant was admitted to a local hospital. The typical features of KD appeared only on the 14th day of illness with the relapse of the maculopapular rash in association with non-purulent conjunctivitis; dry, reddish and fissured lips; tongue with reddish and hypertrophic papillae; erythema and edema of the palms and soles. During the following days, the ailment rapidly evolved to a catastrophic clinical picture characterized by generalized vasculitis, splenic infarction, pulmonary thrombosis, giant right and left coronary aneurysms, dilatation of common and internal iliac arteries and progressive ischemia of the distal third of the feet resulting in necrotic lesions of both halluces. Appropriate therapy was initiated, but repeated administration of intravenous immunoglobulin G (IVIG) followed by three days of administration of methylprednisolone did not abate the intense inflammatory activity. The remission of inflammation and regression of vascular lesions were only achieved during the following five weeks after the introduction of methotrexate associated with etanercept. The report of this case aims to draw attention to severe forms of KD that exhibit an unfavorable evolution and can be extremely refractory to the conventional therapy.
RESUMO
OBJECTIVE: To draw attention to complications that might arise in any Kawasaki disease (KD) stage, risk factors contributing to the onset of complications and possible transient or permanent disease sequelae. METHODS: Prospective study (clinical cohort) conducted between April 2002 and April 2009 of 115 patients with KD admitted to the Pediatric Rheumatology Clinic of the General Hospital of the Federal District, Brazil. All patients were sequentially assessed with clinical and laboratory examinations, Doppler echocardiography, imitanciometry, auditory evoked potentials, psychological evaluation, ophthalmologic examination and, in one patient with chorea, cerebral magnetic resonance angiography. In all patients, a questionnaire assessing the possible presence of cognitive, emotional, behavioral and social disorders was applied. RESULTS: Twenty-five patients (21.7%) had coronary aneurisms. Thirty eight patients (33%) had a sensorineural auditory loss during the acute and subacute phases of the disease and 13 patients (11.3%) maintained the auditory loss six months after the first assessment. Other complications observed were as follows: facial palsy in one patient (0.9%), ataxia in acute and subacute phases in 11 (9.5%); 15 patients had ophthalmologic complications (13.2%), with uveitis in 13, papilledema in one patient, and conjunctival hemorrhage in another patient. One patient experienced chorea (0.9%), with a magnetic resonance angiography showing changes consistent with cerebral ischemia. In one patient, a thoracic aorta aneurism was found (0.9%) and another patient had a necrotizing vasculitis progressing to peripheral gangrene and tongue tip loss (0.9%). Behavioral changes over convalescence were observed in 23 children. CONCLUSION: KD may progress with several complications even within months of the disease acute phase, eventually resulting in permanent sequelae. The earlier the diagnosis and therapeutic intervention with IV IgG administration are, the lower will be the occurrence of complications; the presence of thrombocytosis, anemia and elevated and extended inflammatory activity are risk factors for complication arising.
Assuntos
Síndrome de Linfonodos Mucocutâneos/complicações , Aneurisma da Aorta Torácica/etiologia , Ataxia/etiologia , Criança , Pré-Escolar , Aneurisma Coronário/etiologia , Paralisia Facial/etiologia , Feminino , Perda Auditiva Neurossensorial/etiologia , Humanos , Lactente , Masculino , Transtornos Mentais/etiologia , Estudos Prospectivos , Fatores de RiscoRESUMO
O objetivo deste relato é alertar a comunidade médica sobre o escorbuto que, apesar de ser doença rara nos dias atuais, deve ser lembrado quando o paciente manifesta distúrbios hemorrágicos e história de erro alimentar. Reporta-se o caso de uma criança que foi hospitalizada por dor progressiva nos membros inferiores, edema e manifestaçõeshemorrágicas. O diagnóstico de escorbuto foi baseado nos achados clínicos, no relato histórico alimentar de ingestão somente de bolo e leite e na remissão dos sinais e sintomas após a administração de vitamina C.
The purpose of this report is to alert the medical community about scurvy. Although it is nowadays a rare disease, it must be taken into consideration when the patient presents bleeding disorders and bad eating habits. A case is presented of a child hospitalized with progressive pain in his lower limbs, edema and hemorrhages. The diagnosis of scurvy was based on clinical findings, on the alimentary history of only cake and milk intake and, on the remission of symptoms and signs after the administration of vitamin C.
RESUMO
OBJETIVO: Chamar a atenção para as complicações, que podem surgir em qualquer fase da Doença de Kawasaki (DK), para os fatores de risco que contribuem para o aparecimento dessas complicações e para as possíveis sequelas da doença, sejam elas transitórias ou permanentes. MÉTODOS: Estudo prospectivo (coorte clínica) realizado entre abril de 2002 e abril de 2009 de 115 pacientes com DK internados no serviço de Reumatologia Pediátrica do Hospital Geral do Distrito Federal. Todos os pacientes foram sequencialmente avaliados com exames clínicos e laboratoriais, ecocardiogramas com Doppler, imitanciometria, potenciais evocados auditivos, avaliação psicológica, exame oftalmológico e, em um paciente com coreia, angiorressonância magnética cerebral. Em todos os pacientes foram aplicados questionários avaliando a possível presença de dificuldades cognitivas, emocionais, comportamentais e sociais. RESULTADOS: Vinte e cinco pacientes (21,7 por cento) apresentaram aneurismas de coronárias. Trinta e oito pacientes (33 por cento) apresentaram perda auditiva neurossensorial durante a doença aguda e subaguda, e 13 pacientes (11,3 por cento) mantiveram a perda auditiva seis meses após a primeira avaliação. Outras complicações observadas foram: paralisia facial em um paciente (0,9 por cento), ataxia na fase aguda e subaguda em 11 pacientes (9,5 por cento), complicações oftalmológicas em 15 pacientes (13,2 por cento), constatando-se uveíte em 13, edema de papila em um paciente e hemorragia conjuntival em outro. Um paciente apresentou coreia (0,9 por cento) sendo que a angioressonância magnética evidenciou alterações compatíveis com isquemia cerebral. Em um paciente constatou-se a presença de aneurisma de aorta torácica (0,9 por cento), e outro apresentou vasculite necrosante que evoluiu com gangrena periférica e perda da ponta da língua (0,9 por cento). Alterações de comportamento durante a convalescença (20 por cento) foi observada em 23 crianças. CONCLUSÃO: A DK pode evoluir com complicações diversas, mesmo meses após a fase aguda da doença, eventualmente resultando em sequelas permanentes. Quanto mais precoce forem o diagnóstico e a intervenção terapêutica com a administração de IgG IV, menor será a ocorrência de complicações. Presença de trombocitose, anemia e de atividade inflamatória elevada e por tempo prolongado são fatores de risco para o aparecimento de complicações.
OBJECTIVE: To draw attention to complications that might arise in any Kawasaki disease (KD) stage, risk factors contributing to the onset of complications and possible transient or permanent disease sequelae. METHODS: Prospective study (clinical cohort) conducted between April 2002 and April 2009 of 115 patients with KD admitted to the Pediatric Rheumatology Clinic of the General Hospital of the Federal District, Brazil. All patients were sequentially assessed with clinical and laboratory examinations, Doppler echocardiography, imitanciometry, auditory evoked potentials, psychological evaluation, ophthalmologic examination and, in one patient with chorea, cerebral magnetic resonance angiography. In all patients, a questionnaire assessing the possible presence of cognitive, emotional, behavioral and social disorders was applied. RESULTS: Twenty-five patients (21.7 percent) had coronary aneurisms. Thirty eight patients (33 percent) had a sensorineural auditory loss during the acute and subacute phases of the disease and 13 patients (11.3 percent) maintained the auditory loss six months after the first assessment. Other complications observed were as follows: facial palsy in one patient (0.9 percent), ataxia in acute and subacute phases in 11 (9.5 percent); 15 patients had ophthalmologic complications (13.2 percent), with uveitis in 13, papilledema in one patient, and conjunctival hemorrhage in another patient. One patient experienced chorea (0.9 percent), with a magnetic resonance angiography showing changes consistent with cerebral ischemia. In one patient, a thoracic aorta aneurism was found (0.9 percent) and another patient had a necrotizing vasculitis progressing to peripheral gangrene and tongue tip loss (0.9 percent). Behavioral changes over convalescence were observed in 23 children. CONCLUSION: KD may progress with several complications even within months of the disease acute phase, eventually resulting in permanent sequelae. The earlier the diagnosis and therapeutic intervention with IV IgG administration are, the lower will be the occurrence of complications; the presence of thrombocytosis, anemia and elevated and extended inflammatory activity are risk factors for complication arising.
Assuntos
Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Aneurisma da Aorta Torácica/etiologia , Ataxia/etiologia , Aneurisma Coronário/etiologia , Paralisia Facial/etiologia , Perda Auditiva Neurossensorial/etiologia , Transtornos Mentais/etiologia , Estudos Prospectivos , Fatores de RiscoRESUMO
Suid herpesvirus 1 (SuHV-1) is the causative agent of Aujeszky's disease. The infectious agent has only one serotype, but it was classified by restriction enzyme analysis of the whole genome into four genotypes, named I to IV. The aim of this study was to standardize a rapid method for genotyping SuHV-1 without virus isolation, using a multiplex-PCR followed by enzymatic restriction analysis. The complete genome of the virus was analyzed in silico to determine the restriction sites for the enzyme BamHI. Primers were designed to flank sites with emphasis on certain points of differentiation of genotypes. The standard PCRs were able to detect the SuHV-1 and also to differentiate genotypes from brain tissue of infected pigs. The BamHI-PCR is a rapid, practical, and sensitive way to genotype SuHV-1.
RESUMO
A doença de Camurati-Engelmann é uma displasia diafisária progressiva, autossômica dominante, caracterizada por progressiva expansão cortical e esclerose, que afeta, na maioria das vezes, as diáfises dos ossos longos, associada à hiperostose craniana. As manifestações clínicas são dor óssea nos membros inferiores, fraqueza muscular e atrofia muscular. Os autores relatam um caso de doença de Camurati-Engelmann em paciente masculino, de um ano de idade com diagnóstico avaliado pela radiografia simples, tomografia computadorizada, cintilografiaóssea e exames laboratoriais durante oito anos de evolução da doença. Ressalta-se a importância do diagnóstico diferencial com outras doenças que cursam com hiperostose e o emprego de corticosteroide para controle do quadro álgico.
Camurati-Engelmann disease is a rare progressive diaphyseal dysplasia, autosomal dominant, characterized by progressive cortical expansion and sclerosis mainly affecting the diaphysis of the long bones associated with cranial hyperostosis. The clinical manifestations are lower limbs bone pain, muscle weakness and muscular atrophy. The authors report a case of Camurati-Engelmann disease in a 1-year-old male patient who was submitted to plain radiography, computed tomography, bone scintigraphy and laboratory exams for eight years. The authors emphasize theimportance of the differential diagnosis with other diseases that attend hyperostosis and the use of corticosteroids to control the pain.
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.