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• Pediatric BS usually presents as an incomplete form at onset and expresses the full disease phenotype over the disease course. • The rate of vascular involvement is reported to be 1.8 % to 21 %, although the real prevalence during is largely unknown. • This study directly compares demographical and clinical data regarding pediatric BS patients with and without thrombosis and reports the largest number of pediatric vascular BS cases. • In pediatric age, thrombosis could be observed at BS presentation in a significant proportion of cases and the most affected reported sites are cerebral sinuses. • In case of concomitant pustulosis and fever in BS patients, thrombosis development should be investigated. Vascular events account for a considerable burden of morbidity and mortality in Behçet syndrome (BS). Thrombosis occurs in 1.8–21 % pediatric BS patients, even if the real prevalence is still largely unknown. To report clinical features and outcomes of pediatric BS patients with thrombosis and to compare the demographic and clinical characteristics of BS patients with and without thrombosis. Retrospective data collection of BS patients with thrombosis (T +) included in the EUROFEVER registry. BS patients without thrombosis (T-), belonging to the same rheumatology units, were matched in a 2:1 ratio. 37 T + were compared to 74 T- patients. At onset, ICBD criteria fulfillment was higher in the T- group (p = 0.015). Caucasian patients were more often T-, Turkish patients were more frequent in T + group (p = 0.002). At onset, pustulosis was most frequently observed in the T- (p < 0.001) as well as gastrointestinal symptoms (p < 0.001) and ocular involvement (p = 0.022). Neurological symptoms were more often described in T + (p = 0.034). As for T +, thrombosis was reported at BS presentation in 8/37 (21.6 %). For the T + e patients who developed thrombosis later, oral aphthosis (p = 0.003), genital aphthosis (p = 0.014) were more frequently observed at BS onset, while pustulosis (p = 0.005) and fever (p = 0.043) coexisted with thrombosis. Thrombosis was mainly venous (26/37,70.3 %), involving the cerebral sinuses (21/37, 56.8 %). After thrombosis, 35/37 (94.6 %) T + patients received an immunomodulatory treatment compared with 16/29 (55.2 %) pre-thrombosis. A recurrence was reported in 6/31(19.4 %). Thrombosis was reported at BS presentation in one fifth of cases. Pustolosis and fever were more frequently concomitant to thrombosis. Sinus veins were the most frequent site. [ABSTRACT FROM AUTHOR]

Abstract Background Intensive care unit (ICU) admission of adults with cystic fibrosis (CF) is controversial because of poor outcome. This appraisal needs re-evaluation following recent changes in both CF management and ICU daily practice. Objectives were to determine long-term outcome of adults with CF admitted in ICU and to identify prognostic factors. Methods Retrospective multicenter study of 60 ICU hospitalizations for 42 adult CF patients admitted between 2000 and 2003. Reason for ICU admission, ventilatory support provided and one-year survival were recorded. Multiple logistic analysis was used to determine predictors of mortality. Results Prior to ICU admission, all patients (mean age 28.1 ± 8 yr) had a severe lung disease (mean FEV1 28 ± 12% predicted; mean PaCO2 47 ± 9 mmHg). Main reason for ICU hospitalization was pulmonary infective exacerbation (40/60). At admission, noninvasive ventilation was used in 57% of cases and was successful in 67% of patients. Endotracheal intubation was implemented in 19 episodes. Overall ICU mortality rate was 14%. One year after ICU discharge, 10 of the 28 survivors have been lung transplanted. Among recognized markers of CF disease severity, only the annual FEV1 loss was associated with a poor outcome (HR = 1.47 [1.18–1.85], p = 0.001). SAPSII (HR = 1.08 [1.03–1.12], p < 0.001) and endotracheal intubation (HR = 16.60 [4.35–63.34], p < 0.001) were identified as strong independent predictors of mortality. Conclusion Despite advanced lung disease, adult patients with CF admitted in ICU have high survival rate. Endotracheal intubation is associated with a poor prognosis and should be used as the last alternative. Although efforts have to be made in selecting patients with CF likely to benefit from ICU resources, ICU admission of these patients should be considered.

Résumé L’atteinte trachéo-bronchique touche 20 à 50 % des patients atteints de polychondrite atrophiante et peut représenter le mode d’entrée dans la maladie. Il convient de la rechercher lors du diagnostic et à chaque visite de suivi. La recherche d’une atteinte respiratoire doit inclure la réalisation d’un scanner thoracique avec des coupes réalisées en expiration et des épreuves fonctionnelles respiratoires. Ces examens complémentaires doivent être réalisés systématiquement au diagnostic, même chez les patients asymptomatiques, et ils doivent être répétés régulièrement au cours du suivi. L’indication de la fibroscopie doit rester très prudente et elle ne doit être réalisée que chez des patients symptomatiques par des endoscopistes experts. L’indication d’un geste local doit être posée avec grande prudence, après concertation pluridisciplinaire, en raison notamment des risques de perforation de la muqueuse trachéo-bronchique. Un dépistage précoce et la prise en charge des atteintes trachéo-bronchiques de la polychondrite atrophiante ont permis l’amélioration de leur pronostic, notamment depuis le développement de la fibroscopie interventionnelle. Tracheobronchial involvement affects 20 to 50% of patients with relapsing polychondritis and may reveal the disease. It should be looked for at the time of diagnosis and at each follow-up visit. The search for respiratory impairment must include a chest scan with dynamic expiratory computed tomography and pulmonary function tests. Those investigations should be routinely performed, even in asymptomatic patients, at the time of diagnosis, and repeated regularly during follow-up. The indication for bronchoscopy should be very cautious and it should be performed only by expert endoscopists in symptomatic patients. The indication of an endoscopic gesture, which may lead to perforation of the tracheobronchial mucosa, must be discussed by an expert endoscopist, after multidisciplinary evaluation. Early detection and management of tracheobronchial involvement in relapsing polychondritis has improved significantly its prognosis, especially since the development of interventional fiberoptic bronchoscopy. [ABSTRACT FROM AUTHOR]