EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation

Objectives To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schonlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria.Methods The preliminary Vienna 2005 consensus conference, which proposed preliminary criteria for paediatric vasculitides, was followed by a EULAR/PRINTO/PRES-supported validation project divided into three main steps. Step 1: retrospective/prospective web-data collection for HSP, c-PAN, c-WG and c-TA, with age at diagnosis <= 18 years. Step 2: blinded classification by consensus panel of a subgroup of 280 cases (128 difficult cases, 152 randomly selected) enabling expert diagnostic verification. Step 3: Ankara 2008 Consensus Conference and statistical evaluation (sensitivity, specificity, area under the curve, kappa-agreement) using as 'gold standard' the final consensus classification or original treating physician diagnosis.Results A total of 1183/1398 (85%) samples collected were available for analysis: 827 HSP, 150 c-PAN, 60 c-WG, 87 c-TA and 59 c-other. Prevalence, signs/symptoms, laboratory, biopsy and imaging reports were consistent with the clinical picture of the four c-vasculitides. A representative subgroup of 280 patients was blinded to the treating physician diagnosis and classified by a consensus panel, with kappa-agreement of 0.96 for HSP (95% CI 0.84 to 1), 0.88 for c-WG (95% CI 0.76 to 0.99), 0.84 for c-TA (95% CI 0.73 to 0.96) and 0.73 for c-PAN (95% CI 0.62 to 0.84), with an overall. of 0.79 (95% CI 0.73 to 0.84).Conclusion EULAR/PRINTO/PRES propose validated classification criteria for HSP, c-PAN, c-WG and c-TA, with substantial/almost perfect agreement with the final consensus classification or original treating physician diagnosis.

Preliminary Criteria for Global Flares in Childhood-Onset Systemic Lupus Erythematosus

Objective. To develop widely acceptable preliminary criteria of global flare for childhood-onset systemic lupus erythematosus (cSLE).Methods. Pediatric rheumatologists (n = 138) rated a total of 358 unique patient profiles with information about the cSLE flare descriptors from 2 consecutive visits: patient global assessment of well-being, physician global assessment of disease activity (MD-global), health-related quality of life, anti-double-stranded DNA antibodies, disease activity index scores, protein: creatinine (P:C) ratio, complement levels, and erythrocyte sedimentation rate (ESR). Based on 2,996 rater responses about the course of cSLE (baseline versus followup), the accuracy (sensitivity, specificity, and area under the receiver operating characteristic curve) of candidate flare criteria was assessed. An international consensus conference was held to rank these candidate flare criteria as per the American College of Rheumatology recommendations for the development and validation of criteria sets.Results. The highest-ranked candidate criteria considered absolute changes (Delta) of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) or British Isles Lupus Assessment Group (BILAG), MD-global, P:C ratio, and ESR; flare scores can be calculated (0.5 x Delta SLEDAI + 0.45 x Delta P:C ratio + 0.5 x Delta MD-global + 0.02 x Delta ESR), where values of >= 1.04 are reflective of a flare. Similarly, BILAG-based flare scores (0.4 x Delta BILAG + Delta 0.65 x Delta P:C ratio + 0.5 + Delta MD-global + 0.02 x Delta ESR) of >= 1.15 were diagnostic of a flare. Flare scores increased with flare severity.Conclusion. Consensus has been reached on preliminary criteria for global flares in cSLE. Further validation studies are needed to confirm the usefulness of the cSLE flare criteria in research and for clinical care.

New recurrent deletions in the PPAR gamma and TP53 genes are associated with childhood myelodysplastic syndrome

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Presence of mutans streptococci and Candida spp. in dental plaque/dentine of carious teeth and early childhood caries

This study determined the presence of mutans streptococci and Candida spp. in supragingival. dental plaque and infected dentine of caries-free children, with early childhood caries and caries. Pooled samples of dental plaque and infected dentine were collected from 56 children aged 1-5 years, which were divided into 3 groups: early childhood caries (ECC); caries and caries-free. Infected dentine was collected in ECC and caries groups to compare the frequency of these microorganisms in the collected sites. The samples were inoculated in SB20 and SA medium, for mutans streptococci and Candida spp., respectively, and incubated at 37 degrees C for 48 h. Colony growth was verified and the identification was performed by biochemical tests and CHROMagar Candida. Fisher's test or chi-square (chi(2)) were applied (p = 0.05). The more prevalent species were S. mutans and Candida albicans in ECC (85.4% and 60.4%, respectively), independently of the sample site. S. mutans only was significantly associated with carious teeth, whether in early childhood caries or not. However, the frequency of C. albicans in ECC was higher when compared to caries and caries-free groups. There is a significant association between the presence of C. albicans and early childhood caries. (c) 2006 Elsevier Ltd. All rights reserved.

Atmospheric pollution effects on childhood health: an environmental study in the Paraíba Valley

OBJETIVOS: estimar as correlações entre os níveis de poluentes atmosféricos e o número de internações por doença respiratória em crianças, em hospital universitário. MÉTODOS: trata-se de um estudo ecológico de série temporal. Os dados sobre poluentes atmosféricos foram obtidos junto à CETESB e os dados sobre internação hospitalar, junto ao SAME do Hospital Universitário de Taubaté, Brasil. O estudo se refere a dados e internação do ano de 2001. Para estudar as correlações dos valores dos poluentes entre si e entre as internações, utilizou-se da técnica de correlação de Pearson. Foram estimados os riscos relativos para internação por doença respiratória comparando os quartis dos agentes poluentes com os valores do primeiro quartil. A significância estatística adotada foi alfa = 5%. RESULTADOS: foram internadas 158 crianças com doença respiratória no ano de 2001 (30% do total de internações no ano). Os poluentes estiveram correlacionados entre si e houve correlação positiva, entre o número de internação e dióxido de enxofre e material particulado; houve aumento de 25% no risco de internação comparando o quarto quartil e o primeiro quartil. CONCLUSÕES: houve correlação positiva entre número de internações por doenças respiratórias e poluentes atmosféricos.

p53 gene analysis in childhood B non - Hodgkin's lymphoma

CONTEXTO: Alterações do gene supressor de tumor p53, como mutações e deleções, são lesões genéticas encontradas com maior freqüência nas neoplasias humanas, incluindo câncer de mama, pulmão e cólon. Entre as malignidades hematológicas, o gene 53 é freqüentemente mutado no linfoma de Burkitt, sendo detectadas mutações em 30-40% das amostras tumorais e em 70% das linhagens celulares. OBJETIVO: Analisar as alterações do gene p53 em crianças com linfoma não-Hodgkin de origem B. TIPO DE ESTUDO: Estudo descritivo. LOCAL: Centro de Oncologia Terciário. PARTICIPANTES: O estudo analisou 12 pacientes com linfoma não-Hodgkin B classificados como linfoma de Burkitt. A análise de possíveis mutações do gene p53 foi realizada pela técnica de PCR-SSCP dos exons 5, 6 ,7 e 8/9 do gene. RESULTADOS: Um padrão anormal de migração foi observado em quatro pacientes (33.3%), em um paciente no exon 6 e em três no exon 7. Os casos positivos incluíam dois pacientes que evoluíram para o óbito por progressão da doença. CONCLUSÃO: Esses resultados preliminares sugerem que as alterações do gene p53 são freqüentes em crianças com linfoma de Burkitt e podem contribuir para patogênese ou progressão da doença.

Myelodysplastic syndrome in childhood: report of two cases with deletion of chromosome 4 and the Philadelphia chromosome

We report two pediatric patients with unclassified myelodysplastic syndrome (MDS) by the French-American-British (FAB) group. Both cases had clinical and hematological peculiarities, which had not been described yet. The cytogenetic alterations were 4q deletion and the Philadelphia (Ph) chromosome which appeared at different moments of the disease. One patient showed the Ph chromosome at disease transformation and the other at diagnosis. The different breakpoints at 4q and the presence of Ph could be a marker of this form of MDS. The association of clinical and hematological findings suggests the possibility of a new group of pediatric MDS. (C) 2002 Elsevier B.V. Ltd. All rights reserved.

Association of free amino acids with caries experience and mutans streptococci levels in whole saliva of children with early childhood caries

Objective: The aim of the present study was to identify the free amino acid content in whole saliva of children with (CE) and without early childhood caries (CF) (ECC), correlating these findings with caries experience and mutans streptococci (MS) levels in saliva.Design: Seventy-eight healthy children, both genders, 6-71 months age, were selected to participate in the study. Following examination for dmft scores calculation, unstimulated whole saliva was collected from all 78 participants, stored at -80 degrees C, and used for amino acid analysis, on a Biochem 20 plus amino acid analyzer. Stimulated whole saliva was collected from 52 children, transported, diluted and plated on MSB agar medium for detection of MS in cfu/mL.Results: Forty different free amino acids were identified in whole saliva, with great variation in their concentration. A statistically significant relation was found between caries experience and the presence of free proline and glycine. While proline (p = 0.0182) was more frequently absent in the CF group, the absence of glycine (p = 0.0397) was more often observed in the CE group. In the presence of higher levels of MS, free glycine reduced the risk of experiencing dental caries (p = 0.0419). Conversely, the presence of proline was found to increase the risk of experiencing the disease (p = 0.0492).Conclusions: The presence of free proline and absence of free glycine in children with ECC, highly contaminated with MS, increased the chances of experiencing dental caries in the present population. Further studies are needed to better understand this phenomenon. (C) 2008 Elsevier Ltd. All rights reserved.