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1.
Rheumatology (Oxford) ; 58(7): 1196-1205, 2019 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-30690571

RESUMO

OBJECTIVE: To develop a composite DAS for JDM and provide preliminary evidence of its validity. METHODS: The Juvenile DermatoMyositis Activity Index (JDMAI) is composed of four items: physician's global assessment of overall disease activity; parent's/child's global assessment of child's wellbeing; measurement of muscle strength; and assessment of skin disease activity. The score of the JDMAI is the arithmetic sum of the scores of each individual component. Six versions of the JDMAI were tested, which differed in the tools used to assess the third and fourth items. Validation procedures were conducted using three large multinational patient samples including a total of 627 patients. RESULTS: The JDMAI was found to possess face and content validity, good construct validity, satisfactory internal consistency (Cronbach's alpha = 0.58-0.89), fair responsiveness to clinically important change (standardized response mean = 0.82-3.12 among patients improved) and strong capacity to discriminate patients judged as being in the state of inactive disease or low, moderate or high disease activity by the physician (P < 0.001) or whose parents were satisfied or not satisfied with the course of their child's illness (P < 0.001). Overall, the six versions of the JDMAI showed similar metrological performances in validation analyses. CONCLUSION: The JDMAI was found to possess good measurement properties in a large population of patients with a wide range of disease activity, and is, therefore, suitable for use in both clinical and research settings. The final version of the JDMAI will be selected after its prospective validation.

2.
Clin Rheumatol ; 2018 Sep 28.
Artigo em Inglês | MEDLINE | ID: mdl-30267356

RESUMO

Juvenile idiopathic arthritis (JIA) is the most prevalent chronic rheumatic disease in children and young people (CYP) and a major cause of pain and disability. The vast majority of the world's children and their families live in less resourced countries (LRCs) and face significant socioeconomic and healthcare challenges. Current recommendations for standards of care and treatment for children with JIA do not consider children living in less resourced countries. In order to develop appropriate recommendations for the care of CYP with JIA in less resourced countries a meeting of experienced pediatric rheumatologists from less resourced countries was convened with additional input from a steering group of international pediatric rheumatologists with experience in developing recommendations and standards of care for JIA. Following a needs assessment survey of healthcare workers caring for CYP with JIA in LRC, a literature review was carried out and management recommendations formulated using Delphi technique and a final consensus conference. Responses from the needs assessment were received from 121/483 (25%) practitioners from 25/49 (51%) less resourced countries. From these responses, the initial 84 recommendations were refined and expanded through a series of 3 online Delphi rounds. A final list of 90 recommendations was proposed for evaluation. Evidence for each statement was reviewed, graded, and presented to the consensus group. The degree of consensus, level of agreement, and level of evidence for these recommendations are reported. Recommendations arrived at by consensus for CYP with JIA in less resourced countries cover 5 themes: (1) diagnosis, (2) referral and monitoring, (3) education and training, (4) advocacy and networks, and (5) research. Thirty-five statements were drafted. All but one statement achieved 100% consensus. The body of published evidence was small and the quality of evidence available for critical appraisal was low. Our recommendations offer novel insights and present consensus-based strategies for the management of JIA in less resourced countries. The emphasis on communicable and endemic diseases influencing the diagnosis and treatment of JIA serves as a valuable addition to existing JIA guidelines. With increasing globalization, these recommendations as a whole provide educational and clinical utility for clinicians worldwide. The low evidence base for our recommendations reflects a shortage of research specific to less resourced countries and serves as an impetus for further inquiry towards optimizing care for children with JIA around the world.

3.
Rheumatol Int ; 38(Suppl 1): 51-58, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29637334

RESUMO

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Argentinian Spanish language. The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, test-retest reliability, and construct validity (convergent and discriminant validity). A total of 373 JIA patients (23.1% systemic, 30.8% oligoarticular, 28.1% RF negative polyarthritis, 18% other categories) and 100 healthy children were enrolled in five centres. The JAMAR components discriminated well healthy subjects from JIA patients. Notably, there was no significant difference between healthy subjects and their affected peers in the school-related item. All JAMAR components revealed good psychometric performances. In conclusion, the Argentinian Spanish version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.


Assuntos
Artrite Juvenil/diagnóstico , Avaliação da Deficiência , Medidas de Resultados Relatados pelo Paciente , Reumatologia/métodos , Adolescente , Idade de Início , Argentina , Artrite Juvenil/fisiopatologia , Artrite Juvenil/psicologia , Artrite Juvenil/terapia , Estudos de Casos e Controles , Criança , Pré-Escolar , Características Culturais , Feminino , Nível de Saúde , Humanos , Masculino , Pais/psicologia , Pacientes/psicologia , Valor Preditivo dos Testes , Prognóstico , Psicometria , Qualidade de Vida , Reprodutibilidade dos Testes , Tradução
4.
Arthritis Care Res (Hoboken) ; 70(9): 1312-1319, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29245175

RESUMO

OBJECTIVE: To develop and test a hybrid measure of muscle strength for juvenile dermatomyositis (JDM), which is based on the combination of the Manual Muscle Testing in 8 muscles (MMT-8) and the Childhood Myositis Assessment Scale (CMAS) but is more comprehensive than the former and more feasible than the latter. METHODS: The hybrid MMT-8/CMAS (hMC) is composed of all 8 items of the MMT-8 and 3 items of the CMAS: time of head lift, assessment of abdominal muscles, and floor rise. The score ranges 0-100, with 100 indicating normal muscle strength. Validation procedures were conducted using 3 large multinational patient samples, including a total of 810 JDM patients. RESULTS: The hMC revealed face and content validity, good construct validity, excellent test-retest reliability (intraclass correlation coefficient = 0.99), and internal consistency (Cronbach's α = 0.94), strong responsiveness to clinical change over time (standardized response mean = 0.8 among patients judged as improved by the caring physician), and satisfactory capacity to discriminate patients judged as being in the states of inactive disease or low, moderate, or high disease activity by the physician (P < 0.001) or patients whose parents were satisfied or not satisfied with the illness course (P < 0.001). CONCLUSION: The hMC was found to possess good measurement properties in a large population of patients with a wide range of disease activity and severity. The new tool, which is primarily intended for use in routine clinical care, should be further tested in other populations of patients evaluated prospectively.

5.
Pediatr Rheumatol Online J ; 14(1): 17, 2016 Mar 12.
Artigo em Inglês | MEDLINE | ID: mdl-26968522

RESUMO

The juvenile spondyloarthropathies (JSpA) are a group of related rheumatic diseases characterized by involvement of peripheral large joints, axial joints, and entheses (enthesitis) that begin in the early years of life (prior to 16(th) birthday).The nomenclature and concept of spondyloarthropathies has changed during the last few decades. Although there is not any specific classification of JSpA, diseases under the spondyloarthropathy nomenclature umbrella in the younger patients include: the seronegative enthesitis and arthropathy (SEA) syndrome, juvenile ankylosing spondylitis, reactive arthritis, and inflammatory bowel disease-associated arthritis. Moreover, the ILAR criteria for Juvenile Idiopathic Arthritis includes two categories closely related to spondyloarthritis: Enthesitis-related arthritis and psoriatic arthritis.We review the pathophysiology and the use of biological agents in JSpA. JSpA are idiopathic inflammatory diseases driven by an altered balance in the proinflammatory cytokines. There is ample evidence on the role of tumor necrosis factor (TNF) and interleukin-17 in the physiopathology of these entities. Several non-biologic and biologic agents have been used with conflicting results in the treatment of these complex diseases. The efficacy and safety of anti-TNF agents, such as etanercept, infliximab and adalimumab, have been analysed in controlled and uncontrolled trials, usually showing satisfactory outcomes. Other biologic agents, such as abatacept, tocilizumab and rituximab, have been insufficiently studied and their role in the therapy of SpA is uncertain. Interleukin-17-blocking agents are promising alternatives for the treatment of JSpA patients in the near future. Recommendations for the treatment of patients with JSpA have recently been proposed and are discussed in the present review.


Assuntos
Fatores Biológicos/uso terapêutico , Imunidade Inata , Espondiloartropatias/tratamento farmacológico , Adolescente , Humanos , Espondiloartropatias/imunologia
6.
Arch Argent Pediatr ; 110(3): 251-5, 2012 May-Jun.
Artigo em Espanhol | MEDLINE | ID: mdl-22760753

RESUMO

Takayasu's arteritis is a chronic inflammatory disease that primarily affects the large vessels, such as the aorta and its branches. It represents the third most frequent vasculitis during pediatric age. Our objective was to describe clinical and complementary exams features as well as treatment modalities of a case series of pediatric patients. We present 11 patients (10 girls) with median age at onset of 8 years (range: 2-15). The median diagnosis delay was 16 months (range: 2-96). Clinical presentations were lower limb claudication, arterial hypertension, CNS involvement, presence of murmurs, systemic symptoms, lymphadenopathy, chest pain, abdominal pain and arthritis. Laboratory tests showed: elevated ESR, anemia and trombocytosis. Vascular imaging studies exhibited stenosis, dilatation, occlussion and aneurysms. The outcome of the disease was persistent active condition (1 patient), relapse (4 patients), remission (3 patients), motor sequelae (1 patient) and death (2 patients). All patients were treated with steroids and immunosuppressants. Takayasu 's arteritis is a condition that can potentially be life-threatening. The diagnosis should be suspected in a variety of clinical manifestations during childhood.


Assuntos
Arterite de Takayasu/diagnóstico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos
7.
Clin Exp Rheumatol ; 29(1): 117-24, 2011 Jan-Feb.
Artigo em Inglês | MEDLINE | ID: mdl-21345298

RESUMO

OBJECTIVES: To compare the demographic features, presenting manifestations, diagnostic investigations, disease course, and drug therapies of children with juvenile dermatomyositis (JDM) followed in Europe and Latin America. METHODS: Patients were inception cohorts seen between 1980 and 2004 in 27 paediatric rheumatology centres. The following information was collected through the review of patient charts: sex; age at disease onset; date of disease onset and diagnosis; onset type; presenting clinical features; diagnostic investigations; course type; and medications received during disease course. RESULTS: Four hundred and ninety patients (65.5% females, mean onset age 7.0 years, mean disease duration 7.7 years) were included. Disease presentation was acute or insidious in 57.1% and 42.9% of the patients, respectively. The course type was monophasic in 41.3% of patients and chronic polycyclic or continuous in 58.6% of patients. The more common presenting manifestations were muscle weakness (84.9%), Gottron's papules (72.9%), heliotrope rash (62%), and malar rash (56.7%). Overall, the demographic and clinical features of the 2 continental cohorts were comparable. European patients received more frequently high-dose intravenous methylprednisolone, cyclosporine, cyclophosphamide, and azathioprine, while methotrexate and antimalarials medications were used more commonly by Latin American physicians. CONCLUSIONS: The demographic and clinical characteristics of JDM are similar in European and Latin American patients. We found, however, several differences in the use of medications between European and Latin American paediatric rheumatologists.


Assuntos
Preparações Farmacêuticas/classificação , Adolescente , Idade de Início , Criança , Pré-Escolar , Demografia , Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Dermatomiosite/etnologia , Europa (Continente)/etnologia , Feminino , Nível de Saúde , Humanos , Lactente , Cooperação Internacional , América Latina/etnologia , Masculino , Índice de Gravidade de Doença
8.
Arthritis Care Res (Hoboken) ; 62(1): 63-72, 2010 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-20191492

RESUMO

OBJECTIVE: To investigate the long-term outcome and prognostic factors of juvenile dermatomyositis (DM) through a multinational, multicenter study. METHODS: Patients consisted of inception cohorts seen between 1980 and 2004 in 27 centers in Europe and Latin America. Predictor variables were sex, continent, ethnicity, onset year, onset age, onset type, onset manifestations, course type, disease duration, and active disease duration. Outcomes were muscle strength/endurance, continued disease activity, cumulative damage, muscle damage, cutaneous damage, calcinosis, lipodystrophy, physical function, and health-related quality of life (HRQOL). RESULTS: A total of 490 patients with a mean disease duration of 7.7 years were included. At the cross-sectional visit, 41.2-52.8% of patients, depending on the instrument used, had reduced muscle strength/endurance, but less than 10% had severe impairment. Persistently active disease was recorded in 41.2-60.5% of the patients, depending on the activity measure used. Sixty-nine percent of the patients had cumulative damage. The frequency of calcinosis and lipodystrophy was 23.6% and 9.7%, respectively. A total of 40.7% of the patients had decreased functional ability, but only 6.5% had major impairment. Only a small fraction had decreased HRQOL. A chronic course, either polycyclic or continuous, consistently predicted a poorer outcome. Mortality rate was 3.1%. CONCLUSION: This study confirms the marked improvement in functional outcome of juvenile DM when compared with earlier literature. However, many patients had continued disease activity and cumulative damage at followup. A chronic course was the strongest predictor of poor prognosis. These findings highlight the need for treatment strategies that enable a better control of disease activity over time and the reduction of nonreversible damage.


Assuntos
Dermatomiosite/diagnóstico , Dermatomiosite/terapia , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Dermatomiosite/mortalidade , Dermatomiosite/fisiopatologia , Feminino , Humanos , Lactente , Internacionalidade , Masculino , Prognóstico , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
9.
Arch. argent. pediatr ; 103(2): 162-164, abr.2005. ilus
Artigo em Espanhol | LILACS | ID: lil-469527

RESUMO

La osteoartropatía hipertrófica hepática es una rara entidad que se asocia con enfermedades hepáticas graves, en especial relacionadas con colestasis crónica.Se define por la presencia de hipocratismo digital, reacción perióstica y artritis. Describimos el caso de un paciente con osteoartropatía hipertróficahepática que se desarrolló después de un cuadro de enfermedad hepática con colestasis persistente,quien presentó poliartritis que imposibilitaba ladeambulación. Los signos clínicos y radiológicos desaparecieron después del trasplante hepático.Palabras clave: osteoartropatía hipertrófica, colestasis,niños.


Assuntos
Criança , Artrite , Colestase , Insuficiência Hepática/terapia , Periostite , Transplante de Fígado
10.
Arch. argent. pediatr ; 103(2): 162-164, abr.2005. ilus
Artigo em Espanhol | BINACIS | ID: bin-120686

RESUMO

La osteoartropatía hipertrófica hepática es una rara entidad que se asocia con enfermedades hepáticas graves, en especial relacionadas con colestasis crónica.Se define por la presencia de hipocratismo digital, reacción perióstica y artritis. Describimos el caso de un paciente con osteoartropatía hipertróficahepática que se desarrolló después de un cuadro de enfermedad hepática con colestasis persistente,quien presentó poliartritis que imposibilitaba ladeambulación. Los signos clínicos y radiológicos desaparecieron después del trasplante hepático.Palabras clave: osteoartropatía hipertrófica, colestasis,niños.(AU)


Assuntos
Criança , Colestase , Artrite , Insuficiência Hepática/terapia , Periostite , Transplante de Fígado
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