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关键词： behavioural treatment   childhood obesity treatment   implementation   parental education   personalised medicine  

摘要： Aim To investigate the implementation of a plan of action for treatment of childhood obesity, and the effect after 2 years of treatment. Methods Children aged 6-12.9 years who started obesity treatment between 2008 and 2015 in a paediatric clinic in Stockholm County were included. The treatment model included staff education and support and group activities for parents and children separately followed by individual sessions to a multidisciplinary team. The main outcome was change in body mass index standard deviation score (BMI SDS), in comparison to a matched control group. Results In the intervention group, 1334 children (52% boys) with an average age of 9.3 years and BMI SDS of 2.7 and 3012 children in the control group were included. The intervention group decreased their BMI SDS more after two years compared with the control group, (-0.31 vs -0.23, P < .001). Younger age and higher BMI SDS at treatment initiation and families that completed the group sessions (all P < .001) had greater decreases in BMI SDS after 2 years. Sex did not affect the outcome. Conclusion Even though the treatment in the control group was effective, the implementation of the action plan yielded a better treatment response compared with the control group.

摘要： A polyphenotypic malignant paediatric brain tumour presenting a MN1-PATZ1 fusion, no epigenetic similarities with CNS High-Grade Neuroepithelial Tumour with MN1 Alteration (CNS HGNET-MN1) and related to PATZ1-fused sarcomas Central nervous system high-grade neuroepithelial tumour with I MN1 i alteration (CNS HGNET-MN1) is a brain tumour methylation class that has recently been identified, based on (epi)genetic profiles of a large CNS-PNETs cohort [1]. In addition, the tumour exhibited a polyphenotypic immunoprofile, a feature described in CNS HGNET-MN1 [4] but also pointing toward other tumour entities, such as atypical teratoid rhabdoid tumour or I CIC-NUTM1 i sarcoma [5]. [Extracted from the article]

关键词： inflammation   outcome and process assessment   health care   cytokines  

摘要： Background Current data suggest that COVID-19 is less frequent in children, with a milder course. However, over the past weeks, an increase in the number of children presenting to hospitals in the greater Paris region with a phenotype resembling Kawasaki disease (KD) has led to an alert by the French national health authorities. Methods Multicentre compilation of patients with KD in Paris region since April 2020, associated with the detection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ('Kawa-COVID-19'). A historical cohort of 'classical' KD served as a comparator. Results Sixteen patients were included (sex ratio=1, median age 10 years IQR (4.7 to 12.5)). SARS-CoV-2 was detected in 12 cases (69%), while a further three cases had documented recent contact with a quantitative PCR-positive individual (19%). Cardiac involvement included myocarditis in 44% (n=7). Factors prognostic for the development of severe disease (ie, requiring intensive care, n=7) were age over 5 years and ferritinaemia >1400 mu g/L. Only five patients (31%) were successfully treated with a single intravenous immunoglobulin (IVIg) infusion, while 10 patients (62%) required a second line of treatment. The Kawa-COVID-19 cohort differed from a comparator group of 'classical' KD by older age at onset 10 vs 2 years (p<0.0001), lower platelet count (188 vs 383 G/L (p<0.0001)), a higher rate of myocarditis 7/16 vs 3/220 (p=0.0001) and resistance to first IVIg treatment 10/16 vs 45/220 (p=0.004). Conclusion Kawa-COVID-19 likely represents a new systemic inflammatory syndrome temporally associated with SARS-CoV-2 infection in children. Further prospective international studies are necessary to confirm these findings and better understand the pathophysiology of Kawa-COVID-19.

关键词： Cone beam computed tomography (CBCT)   Paediatric   Clinical indication   Dental radiology  

摘要： Introduction Cone beam computed tomography (CBCT) is used across all dental specialties and has a number of advantages compared to 2D images. The SEDENTEXCT guidelines provide a number of indications for the use, however there are currently no specific guidelines for paediatric dentistry. The aim of this study was to assess current practice of CBCT imaging within paediatric dental departments in England, audit compliance of CBCT justifications against the standards set by SEDENTEXCT and assess whether the use of CBCT affected the treatment plan for each individual patient. Methods From the retrospective analysis of CBCT examinations taken over a 4-year period across three dental hospitals in the north of England, the following data were collected: age at the time of exposure, clinical indication, region of interest (ROI) and diagnostic findings. Clinical notes were also used to identify whether the CBCT had an effect on the final treatment plan. Results A total of 335 CBCT examinations were performed, mean age: 11 years. The number of CBCTs increased each year with a twofold increase in the first 2 years. The main clinical indication in 46% of CBCT examinations was the assessment of localised developing dentition, 68% were in the upper anterior sextant and 61% of CBCT exams were in the mixed dentition age group. The investigations were justified in 100% of the cases. Conclusion The quantity of CBCT examination in paediatric dental patients is increasing to assist treatment planning but more often to enable improved surgical planning.

关键词： MAP Client   Bone reconstruction   Paediatric   Statistical shape modelling   Bone morphing  

摘要： In biomechanical simulations, generic linearly scaled musculoskeletal anatomies are commonly used to represent children, often neglecting or oversimplifying subject-specific features that may affect model estimates. Inappropriate bone sizing may influence joint angles due to erroneous joint centre identification. Alternatively, subject-specific image-based musculoskeletal models allow for more realistic representations of the skeletal system. To this end, statistical shape modelling (SSM) and morphing techniques may help to reconstruct bones rapidly and accurately. Specifically, the musculoskeletal atlas project (MAP) Client, which employs magnetic resonance imaging (MRI) and/or motion capture data to inform SSM and nonrigid morphing techniques, proved able to accurately reconstruct adult pelvis and femur bones. Nonetheless, to date, the above methods have never been applied to paediatric data. In this study, pelvis, femurs and tibiofibular bones of 18 typically developing children were reconstructed using the MAP Client. Ten different combinations of SSM and morphing techniques, i.e. pipelines, were developed. Generic bone geometries from the gait2392 OpenSim model were linearly scaled for comparisons. Jaccard index, root mean square distance error and Hausdorff distance were computed to quantify reconstruction accuracy. For the pelvis bone, colour maps were produced to identify areas prone to inaccuracies and hip joint centres (HJC) location was compared. Finally, per cent difference between MRI- and MAP-measured left-to-right HJC distances was computed. Pipelines informed by MRI data, alone or in combination with motion capture data, accurately reconstructed paediatric lower limb bones (i.e. Jaccard index>0.8). Scaled OpenSim geometries provided the least accurate reconstructions. Principal component-based scaling methods produced size-dependent results, which were worse for smaller children.

关键词： Business and industry   Health care   Medical research  

关键词： COVID-19   Targeted disease modifying anti-rheumatic drugs   Rheumatic diseases   Epidemiology   Pediatric rheumatology  

摘要： Objectives: To investigate the incidence of COVID-19 in a cohort of adult and paediatric patients with rheumatic diseases receiving targeted biologic and synthetic disease modifying anti-rheumatic drugs (tDMARDs) and to explore the possible effect of these treatments in the clinical expression of COVID-19. Methods: A cross-sectional study comprising of a telephone survey and electronic health records review was performed including all adult and paediatric patients with rheumatic diseases treated with tDMARDs in a large rheumatology tertiary centre in Barcelona, Spain. Demographics, disease activity, COVID-19 related symptoms and contact history data were obtained from the start of the 2020 pandemic. Cumulative incidence of confirmed cases (SARS-CoV-2 positive PCR test) was compared to the population estimates for the same city districts from a governmental COVID-19 health database. Suspected cases were defined following WHO criteria and compared to those without compatible symptoms. Results: 959 patients with rheumatic diseases treated with tDMARDs were included. We identified 11 confirmed SARS-CoV-2 positive cases in the adult cohort and no confirmed positive cases in the paediatric cohort. COVID-19 incidence rates of the rheumatic patient cohort were very similar to that of the general population [(0.48% (95% CI 0.09 to 0.87%)] and [0.58% (95% CI 0.56 to 0.60%)], respectively. We found significant differences in tDMARDs proportions between the suspected and non-suspected cases (p=0.002). Conclusion: Adult and paediatric patients with rheumatic diseases on tDMARDs do not seem to present a higher risk of COVID-19 or a more severe disease outcome when compared to general population. (C) 2020 Elsevier Inc. All rights reserved.

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摘要： This expert Consensus Statement from the Pediatric Continuous Renal Replacement Therapy (PCRRT) workgroup presents recommendations for the management of hyperammonaemia requiring kidney replacement therapy in paediatric populations. Additional studies are needed to strengthen these recommendations, which will be reviewed every 2 years. Hyperammonaemia in children can lead to grave consequences in the form of cerebral oedema, severe neurological impairment and even death. In infants and children, common causes of hyperammonaemia include urea cycle disorders or organic acidaemias. Few studies have assessed the role of extracorporeal therapies in the management of hyperammonaemia in neonates and children. Moreover, consensus guidelines are lacking for the use of non-kidney replacement therapy (NKRT) and kidney replacement therapies (KRTs, including peritoneal dialysis, continuous KRT, haemodialysis and hybrid therapy) to manage hyperammonaemia in neonates and children. Prompt treatment with KRT and/or NKRT, the choice of which depends on the ammonia concentrations and presenting symptoms of the patient, is crucial. This expert Consensus Statement presents recommendations for the management of hyperammonaemia requiring KRT in paediatric populations. Additional studies are required to strengthen these recommendations.