US is increasingly performed in Crohn disease (CD) in children as a first line imaging modality. It reduces the use of other more invasive examinations such as endoscopy, CT or contrast enema. We describe bowel ultrasonography technique, normal bowel appearances on US and pathological patterns in CD. We discuss the current role and limitations of bowel US in CD in children including diagnosis, extent of disease, assessment of disease activity, follow-up and detection of complications. The diagnostic accuracy of US is discussed according to the literature and compared to other imaging modalities. US is currently used for screening in children with the suspicion of inflammatory bowel disease (IBD) with a good negative predictive value. In follow-up, US has a role in monitoring medical treatment by evaluating disease activity, extent of disease and for detecting complications.

Progressive cystic fibrosis (CF) lung disease is the main cause of mortality in CF patients. CF lung disease starts in early childhood. With current standards of care, respiratory function remains largely normal in children and more sensitive outcome measures are needed to monitor early CF lung disease. Chest CT is currently the most sensitive imaging modality to monitor pulmonary structural changes in children and adolescents with CF. To quantify structural lung disease reliably among multiple centres, standardisation of chest CT protocols is needed. SCIFI CF (Standardised Chest Imaging Framework for Interventions and Personalised Medicine in CF) was founded to characterise chest CT image quality and radiation doses among 16 participating European CF centres in 10 different countries. We aimed to optimise CT protocols in children and adolescents among several CF centres. A large variety was found in CT protocols, image quality and radiation dose usage among the centres. However, the performance of all CT scanners was found to be very similar, when taking spatial resolution and radiation dose into account. We conclude that multicentre standardisation of chest CT in children and adolescents with CF can be achieved for future clinical trials.

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Adding extracranial internal carotid artery evaluation via the submandibular window to transcranial Doppler sonograpy allowed us to detect 10.3% patients at risk for extracranial internal carotid arteriopathy. Further studies are needed to evaluate the prognosis of these anomalies.

Our data do not support biological screening at the time of a TIVAD insertion. We emphasise the contribution of a medical history of venous thromboembolism and prospective Doppler-US for identifying asymptomatic catheter venous thrombosis to select patients who may benefit from biological screening and possible anticoagulant therapy.

Objectives JDM and juvenile overlap myositis represent heterogeneous subtypes of juvenile idiopathic inflammatory myopathy (JIIM). Chronic evolution can occur in up to 60% of cases, and morbidity/mortality is substantial. We aimed to describe the clinical, biological, histological and type I IFN status in JIIM associated with anti-melanoma differentiation-associated protein 5 (anti-MDA5) autoantibodies at presentation (group 1) in comparison with other JIIM (group 2). Methods This was a retrospective and prospective study of patients with JIIM ascertained from three French paediatric rheumatology reference centres between 2013 and 2019. Muscle biopsies were reviewed. Type I interferon pathway activity was assessed by dosage of IFNα serum protein and the expression of IFN-stimulated genes. Results Sixty-four patients were included, 13 in group 1 (54% JDM and 46% juvenile overlap myositis) and 51 in group 2 (76% JDM and 24% juvenile overlap myositis). Group 1 patients demonstrated more arthritis, skin ulcerations, lupus features and interstitial lung disease, and a milder muscular involvement. Serum IFNα levels were higher in group 1 than 2, and decreased after treatment or improvement in both groups. Outcome was similar in both groups. Unconventional treatment (more than two lines) was required in order to achieve remission, especially when skin ulceration was reported. Conclusion This study indicates a higher frequency of arthritis, skin ulcerations and interstitial lung disease, but milder muscular involvement, in JIIM with positive anti-MDA5 autoantibodies compared with other JIIM. Our data support an important role of systemic IFNα in disease pathology, particularly in the anti-MDA5 auto-antibody-positive subgroup. In severe and refractory forms of JIIM, IFNα may represent a therapeutic target.

Acute lung injury (ALI), including its most serious form called acute respiratory distress syndrome (ARDS), is a devastating disease that can occur at any age. ALI/ARDS accounts for only 5-8% of admissions to pediatric intensive care units (PICUs) but is fatal in 30-60% of cases. International multicenter prospective studies are needed to better understand pediatric ALI/ARDS. However, a reproducible definition of ALI/ARDS is crucial to ensure that study populations are homogeneous. We designed a retrospective review to test the inter-observer variability of chest radiograph interpretation for presence of the American-European Consensus Conference (AECC) radiographic criterion for ALI/ARDS. The medical files of 24 children ventilated for ALI/ARDS in our PICU between January 1993 and December 2002 were reviewed. Five pediatric radiologists and five pediatric intensivists interpreted one frontal chest radiograph (FCR) per patient taken on the day of ALI/ARDS diagnosis. Each reader indicated whether the radiograph showed the AECC radiographic criterion for ALI/ARDS. Data analysis involved comparing each reader to all the others based on the raw agreement and Kappa coefficient (kappa). Features in the 24 patients were consistent with earlier studies. Global inter-observer agreement beyond chance was fair (kappa = 0.29 +/- 0.02) among the five radiologists (kappa = 0.26 +/- 0.05) and among the five intensivists (kappa = 0.29 +/- 0.05). Thus, considerable inter-observer variability occurred in assessing the radiographic criterion for ALI/ARDS, as previously shown in adults. Given the low incidence of ALI/ARDS in children, this variability may have a large impact in studies of pediatric ALI/ARDS.

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