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1.
Lancet Child Adolesc Health ; 3(4): 255-263, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30819662

RESUMO

BACKGROUND: To our knowledge, the characteristics and burden of childhood arthritis have never been studied on a worldwide basis. We aimed to investigate, with a cross-sectional study, the prevalence of disease categories, treatment methods, and disease status in patients from across different geographical areas and from countries with diverse wealth status. METHODS: In this multinational, cross-sectional, observational cohort study, we asked international paediatric rheumatologists from specialised centres to enrol children with a diagnosis of juvenile idiopathic arthritis, according to International League of Associations for Rheumatology criteria, who were seen consecutively for a period of 6 months. Each patient underwent retrospective and cross-sectional assessments, including measures of disease activity and damage and questionnaires on the wellbeing and quality of life of the children. We qualitatively compared the collected data across eight geographical areas, and we explored an association between disease activity and damage and a country's gross domestic product (GDP) with a multiple logistic regression analysis. FINDINGS: Between April 4, 2011, and Nov 21, 2016, 9081 patients were enrolled at 130 centres in 49 countries, grouped into eight geographical areas. Systemic arthritis (125 [33·0%] of 379 patients) and enthesitis-related arthritis (113 [29·8%] of 379) were more common in southeast Asia, whereas oligoarthritis was more prevalent in southern Europe (1360 [56·7%] of 2400) and rheumatoid factor-negative polyarthritis was more frequent in North America (165 [31·5%] of 523) than in the other areas. Prevalence of uveitis was highest in northern Europe (161 [19·1%] of 845 patients) and southern Europe (450 [18·8%] of 2400) and lowest in Latin America (54 [6·4%] of 849), Africa and Middle East (71 [5·9%] of 1209), and southeast Asia (19 [5·0%] of 379). Median age at disease onset was lower in southern Europe (3·5 years, IQR 1·9-7·3) than in other regions. Biological, disease-modifying antirheumatic drugs were prescribed more frequently in northern Europe and North America than in other geographical settings. Patients living in countries with lower GDP had greater disease activity and damage than those living in wealthier countries. Damage was associated with referral delay. INTERPRETATION: Our study documents a variability in prevalence of disease phenotypes and disparities in therapeutic choices and outcomes across geographical areas and wealth status of countries. The greater disease burden in lower-resource settings highlights the need for public health efforts aimed at improving equity in access to effective treatments and care for juvenile idiopathic arthritis. FUNDING: IRCCS Istituto Giannina Gaslini.

2.
J Pediatr ; 209: 236-239.e2, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30679051

RESUMO

We explored, through a national survey, pediatrician beliefs and misconceptions that could interfere with early referral of patients with juvenile idiopathic arthritis. A total of 831 pediatricians participated. Approximately one-half of the respondents underestimated the incidence of the disease and thought that pain was the leading symptom of oligoarticular forms.

3.
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.

4.
Artigo em Inglês | MEDLINE | ID: mdl-30273710

RESUMO

BACKGROUND: Postzygotic de novo mutations lead to the phenomenon of gene mosaicism. The 3 main types are called somatic, gonadal, and gonosomal mosaicism, which differ in terms of the body distribution of postzygotic mutations. Mosaicism has been reported occasionally in patients with primary immunodeficiency diseases (PIDs) since the early 1990s, but its real involvement has not been systematically addressed. OBJECTIVE: We sought to investigate the incidence of gene mosaicism in patients with PIDs. METHODS: The amplicon-based deep sequencing method was used in the 3 parts of the study that establish (1) the allele frequency of germline variants (n = 100), (2) the incidence of parental gonosomal mosaicism in families with PIDs with de novo mutations (n = 92), and (3) the incidence of mosaicism in families with PIDs with moderate-to-high suspicion of gene mosaicism (n = 36). Additional investigations evaluated body distribution of postzygotic mutations, their stability over time, and their characteristics. RESULTS: The range of allele frequency (44.1% to 55.6%) was established for germline variants. Those with minor allele frequencies of less than 44.1% were assumed to be postzygotic. Mosaicism was detected in 30 (23.4%) of 128 families with PIDs, with a variable minor allele frequency (0.8% to 40.5%). Parental gonosomal mosaicism was detected in 6 (6.5%) of 92 families with de novo mutations, and a high incidence of mosaicism (63.9%) was detected among families with moderate-to-high suspicion of gene mosaicism. In most analyzed cases mosaicism was found to be both uniformly distributed and stable over time. CONCLUSION: This study represents the largest performed to date to investigate mosaicism in patients with PIDs, revealing that it affects approximately 25% of enrolled families. Our results might have serious consequences regarding treatment and genetic counseling and reinforce the use of next-generation sequencing-based methods in the routine analyses of PIDs.

5.
Rheumatol Int ; 38(Suppl 1): 91-98, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29637328

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 Castilian 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 526 JIA patients (8.6% systemic, 49.4% oligoarticular, 18.2% RF negative polyarthritis, 23.8% other categories) and 78 healthy children, were enrolled in six centres. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the Castilian 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 practise and clinical research.


Assuntos
Artrite Juvenil/diagnóstico , Avaliação da Deficiência , Medidas de Resultados Relatados pelo Paciente , Reumatologia/métodos , Adolescente , Idade de Início , 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
6.
Rheumatol Int ; 38(Suppl 1): 107-113, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29637372

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 Colombian Spanish language. The reading comprehension of the questionnaire was tested in ten 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, and construct validity (convergent and discriminant validity). A total of 22 JIA patients (9.1% systemic, 27.3% RF-negative polyarthritis, 36.4% enthesitis-related arthritis, 27.2% other categories) were enrolled in the paediatric centre of Bogota. All JAMAR components revealed good psychometric performances. In conclusion, the Colombian 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 , 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
7.
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.

8.
Front Immunol ; 9: 3148, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30761159

RESUMO

Background: NF-κB1 is a master regulator of both acquired and innate responses. NFKB1 loss-of-function mutations elicit a wide clinical phenotype with asymptomatic individuals at one end of the spectrum and patients with common variable immunodeficiency, combined immunodeficiency or autoinflammation at the other. Impairment of acquired and innate immunity and disseminated Mycobacterium genavense infection expands the clinical and immunological phenotype of NF-κB1 deficiency. Objective: Functional and molecular characterization of a patient with a novel phenotype of NF-κB1 deficiency. Methods: Circulating T, B, dendritic cell subsets and innate or unconventional T-cells were quantified. The cytokine production in stimulated whole blood samples was assessed and molecular characterization by next generation sequencing and gene expression assays were also performed. Results: We report a patient presenting with features of combined immunodeficiency (CID) and disseminated Mycobacterium genavense infection. Sequencing of genomic DNA identified a novel synonymous mutation (c.705G > A) in NFKB1 gene which resulted in exon 8 skipping and haploinsufficiency of the NF-κB1 subunit p50. The susceptibility to atypical mycobacterial infection has not been previously reported and may be the result of a dendritic cell deficiency. A selective deficiency of circulating follicular helper T (cTFH) cells responsible for mediating the differentiation of naive B cells into memory and plasma cells was also present in the patient. It could affect the maturation of innate or unconventional T cells where NF-κB1 could also be involved. Conclusion: These findings showed that the role of NF-κB1 in humans could be critical for the development of acquired and innate immunity and further highlights the role of human T cells in anti-mycobacterial immunity.


Assuntos
Imunidade Adaptativa , Imunidade Inata , Síndromes de Imunodeficiência/complicações , Síndromes de Imunodeficiência/genética , Infecções por Mycobacterium/diagnóstico , Infecções por Mycobacterium/etiologia , Mycobacterium/imunologia , Subunidade p50 de NF-kappa B/deficiência , Biópsia , Medula Óssea/metabolismo , Criança , Citocinas/metabolismo , Humanos , Imunofenotipagem , Masculino , Mutação , Linhagem , Fenótipo , Pele/patologia
9.
Int Ophthalmol ; 37(3): 719-725, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27432171

RESUMO

To report the experience of our center with the use of adalimumab (ADA) for the treatment of severe refractory noninfectious paediatric uveitis. The study is a retrospective case series of all paediatric patients with refractory uveitis who were treated with ADA at the Paediatric Uveitis Unit of our center from 2008 to 2015. We present 12 patients (6 Juvenile idiopathic arthritis-associated uveitis, 4 idiopathic panuveitis, 1 early-onset sarcoidosis-associated panuveitis, and 1 intermediate uveitis), with uveitis in 19/24 eyes. Once ADA therapy was started, all the patients presented improved activity according to Standardization of Uveitis Nomenclature (SUN) criteria. Nine out of the 12 patients had structural damage before ADA could be started: cataract (n = 4), glaucoma (n = 2), cystic macular edema (n = 1), exudative retinal detachment (n = 1), and optic disk edema (n = 5). Visual acuity improved or maintained stable in 17/19 affected eyes, and only 2 eyes decreased its visual acuity because of structural damage, which was already present before ADA therapy. In our experience, ADA presents a good safety profile and is efficacious in the treatment of paediatric patients with different forms of refractory noninfectious uveitis.


Assuntos
Adalimumab/administração & dosagem , Artrite Juvenil/complicações , Uveíte/tratamento farmacológico , Acuidade Visual , Adolescente , Anti-Inflamatórios/administração & dosagem , Criança , Relação Dose-Resposta a Droga , Feminino , Seguimentos , Humanos , Masculino , Soluções Oftálmicas/administração & dosagem , Estudos Retrospectivos , Tomografia de Coerência Óptica , Resultado do Tratamento , Uveíte/diagnóstico , Uveíte/etiologia
10.
Pediatrics ; 137(4)2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26966131

RESUMO

Juvenile dermatomyositis (JDM) is the most common form of juvenile idiopathic inflammatory myopathy. We report a child with steroid-dependent JDM refractory to hydroxychloroquine and subcutaneous methotrexate who experienced systemic reactions to intravenous immunoglobulin and was successfully treated with subcutaneous immunoglobulin. This form of therapy has been shown to be safe, has a very low rate of adverse effects, does not require hospital admission, reduces the number of missed school days, and decreases the costs associated with treatment.


Assuntos
Dermatomiosite/tratamento farmacológico , Imunoglobulinas/administração & dosagem , Pré-Escolar , Feminino , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Injeções Subcutâneas , Prednisona/uso terapêutico
11.
Rheumatol Int ; 36(1): 83-9, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26259985

RESUMO

The objectives of this study were (1) to determine the percentage of emergency department (ED) visits due to musculoskeletal pain (MSP) by children 3-14 years of age during a period of 1 year; (2) to determine the most frequent presenting complaints; and (3) to characterize their etiology. A cross-sectional study was performed on children aged 3-14(11/12) years attended at the ED of a tertiary hospital due to MSP. The demographic and clinical characteristics of the patients were reviewed 5 days each month for 12 consecutive months. Study days were selected by computer-generated simple random sampling. Out of 4,531 visits to the ED, 826 were due to MSP (18.2 %; 95 % CI 17.1-19.4 %). When compared with children with no skeletal complaints, children with MSP had a similar sex distribution but were older (mean ± SD 7 ± 3.5 years vs 9.9 ± 3.1 years; p < 0.0001). The most common complaints were pain at the wrist (19 %), ankle (19 %) and finger (15 %). The most common etiology was trauma (88.4 %), including contusions (38 %), fractures (21 %) and sprains (18 %). Children with hip (6.7 ± 3 years; p < 0.0001) and elbow (7.8 ± 3.5 years; p < 0.0001) complaints were younger than children with pain in other locations, whereas children with wrist pain (10.5 ± 2.6 years; p < 0.002) and joint sprains (10.7 ± 2.7 years; p < 0.0001) were older. Fractures were more frequent in boys (64 vs 36 %, p = 0.008; OR 1.6; CI 1.1-2.2). Visits to the ED due to MSP increased with age. Pain at three locations represented 50 % of the presenting complaints. Trauma was the principal etiology, but fractures only represented one-fifth of the total.


Assuntos
Fraturas Ósseas/epidemiologia , Dor Musculoesquelética/epidemiologia , Entorses e Distensões/epidemiologia , Adolescente , Fatores Etários , Criança , Pré-Escolar , Estudos Transversais , Serviço Hospitalar de Emergência , Feminino , Fraturas Ósseas/complicações , Humanos , Masculino , Dor Musculoesquelética/etiologia , Prevalência , Estudos Retrospectivos , Distribuição por Sexo , Fatores Sexuais , Entorses e Distensões/complicações
12.
Rheumatol Int ; 35(10): 1615-24, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25917856

RESUMO

To develop recommendations on the transition from pediatric care to adult care in patients with chronic inflammatory rheumatic diseases with childhood onset based. Recommendations were generated following nominal group methodology and Delphi technique. A panel of 16 experts was established. A systematic literature review (on transitional care) and a narrative review were performed and presented to the panel in the first panel meeting to be discussed. A first draft of recommendations was generated and circulated. Focal groups with adolescents, young adults and parents were organized. In a second meeting, the focus group results along with the input from invited psychologist were used to establish definitive recommendations. Then, a Delphi process (two rounds) was carried out. A group of 72 pediatric and adult rheumatologists took part. Recommendations were voted from 1 (total disagreement) to 10 (total agreement). We defined agreement if at least 70 % voted ≥7. The level of evidence and grade or recommendation was assessed using the Oxford center for evidence-based medicine levels of evidence. Transition care was defined as a purposeful, planned process that addresses the medical, psychosocial and educational/vocational needs of adolescents and young adults with chronic inflammatory rheumatic diseases with childhood onset as they move from child-centered to adult-oriented healthcare systems. The consensus covers: transition needs, barriers and facilitators, transitional issues (objectives, participants, content, phases, timing, plans, documentation and responsibilities), physicians' and other health professionals' knowledge and skill requirements, models/programs, and strategies and guideline for implementation. Preliminary recommendations and agreement grade are shown in the Table (first Delphi round). These recommendations are intended to provide health professionals, patients, families and other stakeholders with a consensus on the transition process from pediatric to adult care.


Assuntos
Pediatria , Doenças Reumáticas/terapia , Reumatologia , Transição para Assistência do Adulto , Adolescente , Adulto , Consenso , Humanos , Espanha , Adulto Jovem
13.
J Rheumatol ; 42(6): 994-1001, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25877504

RESUMO

OBJECTIVE: To seek insights into the heterogeneity of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA) through the analysis of a large patient sample collected in a multinational survey. METHODS: International pediatric rheumatologists and hemato-oncologists entered their patient data, collected retrospectively, in a Web-based database. The demographic, clinical, laboratory, histopathologic, therapeutic, and outcome data were analyzed in relation to (1) geographic location of caring hospital, (2) subspecialty of attending physician, (3) demonstration of hemophagocytosis, and (4) severity of clinical course. RESULTS: A total of 362 patients were included by 95 investigators from 33 countries. Demographic, clinical, laboratory, and histopathologic features were comparable among patients seen in diverse geographic areas or by different pediatric specialists. Patients seen in North America were given biologics more frequently. Patients entered by pediatric hemato-oncologists were treated more commonly with biologics and etoposide, whereas patients seen by pediatric rheumatologists more frequently received cyclosporine. Patients with demonstration of hemophagocytosis had shorter duration of sJIA at MAS onset, higher prevalence of hepatosplenomegaly, lower levels of platelets and fibrinogen, and were more frequently administered cyclosporine, intravenous immunoglobulin (IVIG), and etoposide. Patients with severe course were older, had longer duration of sJIA at MAS onset, had more full-blown clinical picture, and were more commonly given cyclosporine, IVIG, and etoposide. CONCLUSION: The clinical spectrum of MAS is comparable across patients seen in different geographic settings or by diverse pediatric subspecialists. There was a disparity in the therapeutic choices among physicians that underscores the need to establish uniform therapeutic protocols.


Assuntos
Artrite Juvenil/epidemiologia , Artrite Juvenil/terapia , Síndrome de Ativação Macrofágica/epidemiologia , Síndrome de Ativação Macrofágica/terapia , Adolescente , Distribuição por Idade , Artrite Juvenil/diagnóstico , Criança , Pré-Escolar , Estudos de Coortes , Comorbidade , Bases de Dados Factuais , Feminino , Humanos , Internacionalidade , Síndrome de Ativação Macrofágica/diagnóstico , Masculino , Análise Multivariada , Prevalência , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de Doença , Distribuição por Sexo , Análise de Sobrevida
15.
Rheumatol Int ; 35(5): 777-85, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-25656443

RESUMO

Uveitis associated with juvenile idiopathic arthritis (JIA) typically involves the anterior chamber segment, follows an indolent chronic course, and presents a high rate of uveitic complications and a worse outcome as compared to other aetiologies of uveitis. Disease assessment, treatment, and outcome measures have not been standardized. Collaboration between pediatric rheumatologists and ophthalmologists is critical for effective management and prevention of morbidity, impaired vision, and irreparable visual loss. Although the Standardization of Uveitis Nomenclature Working Group recommendations have been a great advance to help clinicians to improve consistency in grading and reporting data, difficulties arise at the time of deciding the best treatment approach in the individual patient in routine daily practice. For this reason, recommendations for a systematized control and treatment strategies according to clinical characteristics and disease severity in children with JIA-related uveitis were developed by a panel of experts with special interest in uveitis associated with JIA. A clinical management algorithm organized in a stepwise regimen is here presented.


Assuntos
Corticosteroides/uso terapêutico , Algoritmos , Antirreumáticos/uso terapêutico , Artrite Juvenil/complicações , Midriáticos/uso terapêutico , Uveíte/tratamento farmacológico , Abatacepte/uso terapêutico , Adalimumab/uso terapêutico , Administração Oftálmica , Anticorpos Monoclonais Humanizados/uso terapêutico , Criança , Pré-Escolar , Comportamento Cooperativo , Gerenciamento Clínico , Humanos , Infliximab/uso terapêutico , Metotrexato/uso terapêutico , Oftalmologia , Guias de Prática Clínica como Assunto , Reumatologia , Índice de Gravidade de Doença , Uveíte/complicações , Acuidade Visual
16.
Pediatrics ; 134(5): e1441-6, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25287461

RESUMO

Recurrent pericarditis is a troublesome complication of idiopathic acute pericarditis and occurs more frequently in pediatric patients after cardiac surgery (postpericardiotomy syndrome). Conventional treatment with nonsteroidal antiinflammatory drugs, corticosteroids, and colchicine is not always effective or may cause serious adverse effects. There is no consensus, however, on how to proceed in those patients whose disease is refractory to conventional therapy. In such cases, human intravenous immunoglobulin, immunosuppressive drugs, and biological agents have been used. In this report we describe 2 patients with refractory recurrent pericarditis after cardiac surgery who were successfully treated with 3 and 5 monthly high-dose (2 g/kg) intravenous immunoglobulin until resolution of the effusion. Our experience supports the effectiveness and safety of this therapy.


Assuntos
Imunoglobulinas Intravenosas/administração & dosagem , Pericardite/diagnóstico , Pericardite/tratamento farmacológico , Pré-Escolar , Feminino , Humanos , Masculino , Recidiva
18.
Eur J Pediatr ; 172(10): 1411-3, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-23715656

RESUMO

UNLABELLED: Intra-articular corticosteroid injections (IACI) are one of the mainstays of treatment for children with juvenile idiopathic arthritis. The most important disadvantage of IACI is the pain associated with the procedure. Little is known about the children or parents' perception of this pain. This study was undertaken to determine whether patients and their parents prefer sedation to receive IACI or not and why. A survey form was presented to patients and/or their parents from January to March 2010 to evaluate their choice of anesthesiologist-controlled deep sedation (with sevoflurane) vs. no sedation-no local anesthesia and the reasons for it. All participants had experienced the two options. In addition, there were two visual analog scales (VAS) to evaluate pain associated with blood draws and IACI, respectively. A total of 45 patients and their parents filled out the survey form. There were 34 females; the median age was 10.6 years, and the median duration of the disease was 6.4 years. Median VAS score was 1.3 for pain associated with blood draws, and 6, for IACI. Most children preferred sedation for IACI (26 vs. 15), although four did not show preference for either method. Children who preferred sedation for IACI were younger (p = 0.03) and had a shorter course of disease (p = 0.04). CONCLUSIONS: While most children prefer to receive IACI under sedation, a majority of parents prefer to avoid its risks. Children who prefer IACI without sedation are significantly older and have a longer course of disease.


Assuntos
Artrite Juvenil/tratamento farmacológico , Sedação Consciente/métodos , Glucocorticoides/administração & dosagem , Dor/tratamento farmacológico , Preferência do Paciente/estatística & dados numéricos , Adolescente , Anestésicos Inalatórios/administração & dosagem , Criança , Coleta de Dados , Feminino , Glucocorticoides/uso terapêutico , Humanos , Injeções Intra-Articulares , Masculino , Éteres Metílicos/administração & dosagem , Medição da Dor , Pais , Pacientes , Sevoflurano
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