International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients

Objectives. The aims of this study were to describe the clinical features of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) and identify distinct phenotypes in a large cohort of patients from different countries. Methods. We established a web-based multicentre cohort through an international collaboration within the periodic fevers working party of the Pediatric Rheumatology European Society (PReS). The inclusion criterion was a diagnosis of PFAPA given by an experienced paediatric rheumatologist participating in an international working group on periodic fever syndromes. Results. Of the 301 patients included from the 15 centres, 271 had pharyngitis, 236 cervical adenitis, 171 oral aphthosis and 132 with all three clinical features. A total of 228 patients presented with additional symptoms (131 gastrointestinal symptoms, 86 arthralgias and/or myalgias, 36 skin rashes, 8 neurological symptoms). Thirty-one patients had disease onset after 5 years and they reported more additional symptoms. A positive family history for recurrent fever or recurrent tonsillitis was found in 81 patients (26.9%). Genetic testing for monogenic periodic fever syndromes was performed on 111 patients, who reported fewer occurrences of oral aphthosis or additional symptoms. Twenty-four patients reported symptoms (oral aphthosis and malaise) outside the flares. The CRP was >50 mg/l in the majority (131/190) of the patients tested during the fever. Conclusion. We describe the largest cohort of PFAPA patients presented so far. We confirm that PFAPA may present with varied clinical manifestations and we show the limitations of the commonly used diagnostic criteria. Based on detailed analysis of this cohort, a consensus definition of PFAPA with better-defined criteria should be propose

Arthrite juvénile idiopathique (forme polyarticulaire avec facteur rhumatoïde positif - étude multicentrique rétrospective de 26 cas)

LYON1-BU Santé (693882101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Polychondrite atrophiante à début pédiatrique

LYON1-BU Santé (693882101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Comparing maize management and coexistence means between GM and non GM maize in two french regions

We compared how coexistence in maize at the farm level was i) envisioned in the east of France where GM have never been really cropped, and ii) managed in the south of France where GM maize was cropped in 2006 and 2007. Surveys in respectively 27 and 23 contrasted farms were made in maize-rich sub-regions with high maize pest pressure. Sampling farms for diversity, we used semi-directive interviews with two different protocols for the 2 regions. After data analysis, we identified factors explaining the choice of GM or NGM maize cropping like: presence of maize specialty crops (no GM), presence of other technical scope of progress for maize yield (no GM), perception of corn borer as a risk (more GM) or return time of maize (more GM when maize is frequent). We also identified the different coexistence means preferred according to the region: buffer zone in the South of France with its large fields and sowing period shifts in the East of France where available sowing days are plenty in the spring. We also found out that the results of the first year of testing the GM crop may be the main factor explaining GM maize adoption or abandonment. We will use these results to build a multi-criteria model of maize allocation in a small region

Comparing maize management and coexistence means between GM and non GM maize in two french regions

We compared how coexistence in maize at the farm level was i) envisioned in the east of France where GM have never been really cropped, and ii) managed in the south of France where GM maize was cropped in 2006 and 2007. Surveys in respectively 27 and 23 contrasted farms were made in maize-rich sub-regions with high maize pest pressure. Sampling farms for diversity, we used semi-directive interviews with two different protocols for the 2 regions. After data analysis, we identified factors explaining the choice of GM or NGM maize cropping like: presence of maize specialty crops (no GM), presence of other technical scope of progress for maize yield (no GM), perception of corn borer as a risk (more GM) or return time of maize (more GM when maize is frequent). We also identified the different coexistence means preferred according to the region: buffer zone in the South of France with its large fields and sowing period shifts in the East of France where available sowing days are plenty in the spring. We also found out that the results of the first year of testing the GM crop may be the main factor explaining GM maize adoption or abandonment. We will use these results to build a multi-criteria model of maize allocation in a small region

Life cycle assessment of multi-step versus one-step coating processes using oil or bio-based resins

International audienc

Self-stratifying fire retardant coatings

International audienceThe design of innovative coatings with multifunctional properties is very challenging. Self-stratification can be an alternative as it is an eco-efficient process allowing the formation, in only one application, of a multi-layer film gathering the following properties: adhesion to a substrate, a functional property such as flame retardancy and weathering protective properties and potentially many others. This effective and economical concept thus allows reducing the number of steps to coat a substrate, while providing a coating with equivalent or better performances than a common three layers process (primer-functional coating-topcoat), also reducing the amount of energy used, the pollution and waste generation1.For self-stratification to occur, liquid coatings must contain at least two partially incompatible thermosetting and thermoplastic polymers dissolved in a common solvent blend2. In this work, an innovative self-stratifying coating based on epoxy (DGEBA)/silicone blend has been developed and applied on polycarbonate substrate. Perfect self-stratification was evidenced by microscopic analysis coupled with X-ray mappings3 (Figure 1). Flame retardant additives (2-10 wt.-%) were added to the formulation and the fire properties were evaluated via the determination of the Limiting Oxygen Index (LOI), UL94 and via Mass Loss Calorimetry (MLC). Some of the coatings allowed the formation of a protective barrier against fire and led to V0 rating at UL94 test and an increase of 28% to 35% in oxygen at LOI test. However, the increase in more stringent regulations on toxicological and environmental aspects now leads to the need of “green” flame retardant self-stratifying coatings. In this work, a “greener” self-stratifying coating with bio-based epoxy resin and specific solvent blend replacing xylene (Figure 2) will be presented and fully commented

MISS questionnaire in French version: a good tool for children and parents to assess methotrexate intolerance

International audienceThe aim of this study was to assess the relevance for children and parents to use the French-validated version of the methotrexate intolerance severity score (MISS), a measure of methotrexate intolerance for children suffering from juvenile idiopathic arthritis. The French-version MISS was developed following the "Guidelines for the process of cross-cultural adaptation of self-report measures." The new version was tested in families of children with juvenile idiopathic arthritis who completed the questionnaire twice at a 2-week interval. Item correlations, Cronbach's alpha, and kappa coefficients were computed to evaluate acceptability, internal consistency, and reproducibility. A culturally acceptable version to French was obtained. A total of 71 individuals were included from May 2015 to November 2015. The results show very good acceptability: good response rate (80%), few missing data (\textless1%) and good understanding of parents and children. The inter-item, dimension-item, and inter-dimension correlations were satisfactory (except for "vomiting" items-other items correlation). Cronbach's alpha coefficient was well higher than the usually recommended value of 0.6. The results of validity of internal and external consistencies were satisfactory. We also found good agreement between the test-retest for every family. The empirical discriminative cut-off point of 3 showed a sensitivity of 86% and a specificity of 83%. The MISS questionnaire is quick to complete, easy to use. It can be completed by children or their parents with no significant difference. This validated French-version MISS can help study prevalence and risk factors of methotrexate intolerance, better detect this intolerance, and provide better support for patients on long-term treatment

Transition de la médecine pédiatrique à la médecine pour adultes dans les maladies autoimmunes et autoinflammatoires rares

La commission « Transition » de la filière de santé des maladies auto-immunes et auto-inflammatoires rares a développé des outils et émis des recommandations pour la prise en charge des adolescents et jeunes adultes atteints de ces maladies chroniques évoluant souvent par poussées, durant la période de transition de la médecine pédiatrique vers la médecine pour adultes. L’enjeu de l’adhésion du jeune patient à la poursuite de son parcours de soin dans le système de santé des adultes rend particulièrement importante l’alliance thérapeutique avec son médecin pédiatre puis son médecin pour adultes. Le groupe de travail issu de cette commission a ainsi mené une enquête sur la façon dont les médecins et les jeunes patients perçoivent leur relation durant la période de la transition du suivi médical

Building a transitional care checklist in rheumatology: A Delphi-like survey

tObjectives: To design a transitional care checklist to be used by and facilitate the work of health pro-fessionals in providing transitional care for children with a chronic rheumatologic disease and theirfamilies.Methods: A Delphi-like study among an international expert panel was carried out in four steps: (1)a working group of 6 specialists established a draft; (2) a web-survey among a panel of internationalexperts evaluated it; (3) a 2-day consensus conference with an expert panel discussed items not reachingagreement; (4) a web-survey among the panel of international experts with the list of reformulated items.Results: The first draft of the checklist included 38 items in 3 phases of transition and 5 age groups.Thirty-three international experts evaluated the checklist reaching ≥ 80% agreement for 26 items and≤ 80% for 12. The consensus conference of 12 experts discussed and redefined the 12 items. Twenty-five international experts filled out the web-survey and all items reached a minimum of 80% agreementexcept one. The final checklist was reached.Conclusions: This Delphi-like study defined what themes should be included and at what age they needto be addressed with patients with a chronic rheumatology disease and their families during transition.This checklist reached a strong international and interdisciplinary consensus while examining transitionin a broad way. It should now be spread widely to health professionals to be used by all those who carefor adolescents aged ≥ 12 years at times of transition. It could be transposed to most chronic conditions.Recommendations for further research are given