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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.

摘要： Size mismatch between donor and recipient in solid organ transplantation can add significant morbidity and mortality to the recipient (1). However due to the scarcity of both deceased donors, as well as suitable living donors, discrepancies between the size and weight of the donor, compared with the recipient are often a necessity, if solid organ transplantation is to proceed in a particular individual.◆ (This article is protected by copyright. All rights reserved.)

关键词： aplastic anaemia   eltrombopag   childhood   immunosuppressive therapy  

关键词： autoimmune hepatitis   paediatric   children  

摘要： Objectives: To describe the clinical characteristics, biochemical and histological features, outcomes and predictors of prognosis of children with autoimmune hepatitis (AIH) from a paediatric centre in South Africa. Methods: Thirty-nine children diagnosed with AIH at Red Cross War Memorial Children's Hospital between 2005 and 2015 were included. Relevant patient's data were retrieved from the hospital's medical records and database. Liver biopsy slides were reviewed. Ethical approval was obtained. Data were analysed using SPSS. Results: Females were 29 (74%). Mean age at presentation was 7.2763.35 years and the mean follow-up was 4.5 +/- 2.4 years. Jaundice was present in 97% of patients at presentation. An acute presentation was observed in 26 (67%) even though cirrhosis was detected in 22 (56%). Autoantibody screening was completed in 35 patients, 20 (57%) were AIH-1, 1 (3%) was AIH-2 and 14 (40%) were seronegative AIH. Of the 25 patients who underwent magnetic resonance cholangiography 17 (68%) had associated autoimmune sclerosing cholangitis. The remission rate was 79%. However, 11 children relapsed later. One child required liver transplantation and one demised. Seronegative and seropositive patients have comparable characteristics and outcomes. While a higher alanine transaminase (ALT) level at presentation is a significant predictor of remission, a lower ALT level and cirrhosis are significant risk factors for unfavourable outcome. Overall survival rate was 97%. Conclusion: AIH responds well to therapy with excellent survival. Hence, it should be considered in any child presenting with viral screen negative hepatitis and start therapy timeously to prevent disease progression.

关键词： adverse event   burnout   child   children's nursing   nurse   paediatric   professional well-being   quality of care   safety   safety culture   work environment  

摘要： Aim To explore Italian paediatric nurses' reported burnout and its relationship to their perceptions of safety and adverse events. Design A cross-sectional study using the RN4CAST@IT-Ped database with a web-based survey design. Methods The RN4CAST@IT-Ped questionnaire was used to collect data in 2017. This comprised three main components: three dimensions (22 items) of the Maslach Burnout Inventory including emotional exhaustion, depersonalization and personal accomplishment. Participants also scored an overall grade of patient safety and estimated the occurrence of adverse clinical events. Results Nurses (N = 2,243) reported high levels of burnout. Most rated clinical safety as high. The risk of adverse events ranged from 1.3-12.4%. The degree of burnout appeared to influence the perception of safety and adverse events. Conclusion The association between nurses' burnout and perceptions of higher rates of adverse events and reduced safety in clinical practice is an important finding. However, it is unclear whether this was influenced by a negative state of mind, and whether reduced safety and increased adverse events negatively influenced nurses' well-being, thus leading to burnout. Regardless, the association between nurses' burnout and these quality concepts needs further exploration to examine the effect, if any, on burnout and safety, and identify supportive mechanisms for nurses. Impact The association between reported burnout and perception of safety and risk of adverse events in Italian paediatric nurses has been reported for the first time. Nurses reporting burnout are at greater risk of intensely negative perceptions of clinical safety and adverse events. This is an important finding as perceptions can influence practice and behaviours. Quality measures in children's clinical environments need to go beyond obvious indicators to examine nurses' well-being as this also influences quality and safety.

关键词： drug interactions   amphotericin b   systemic mycosis   hematopoietic stem cell transplantation   azoles   child   hematologic neoplasms   pediatrics   safety   infections   cytomegalovirus   mortality   engraftment   toxic effect   risk reduction   coinfection   allogeneic hematopoietic stem cell transplant   mold   antifungal prophylaxis   candida   adverse event   amphotericin b liposomal  

摘要： Background: Primary antifungal prophylaxis in paediatric allogeneic HSCT recipients is mainly based on azoles, which can have related toxicity and drug interactions. Low-dose liposomal amphotericin B (L-AmB) is an attractive intravenous alternative because of its low toxicity and lower risk of interactions. Objectives: To evaluate the effectiveness and safety of L-AmB (1 mg/kg/day) for primary antifungal prophylaxis in pre-engraftment paediatric HSCT patients. Patients and methods: Retrospective, observational study including all consecutive patients aged <= 18 years who underwent HSCT and received antifungal prophylaxis with intravenous L-AmB (1 mg/kg/day, from day #1 to 48 h before discharge) between January 2012 and December 2016. Results: In total, 125 HSCT procedures in 118 patients were included, median age 7.2 years (IQR 4.2-11.5). Haematological malignancies were the main underlying condition (63.6%), and 109 (87.2%) were considered at high risk for invasive fungal infection (IFI). Ten patients (7.7%), all high risk, developed breakthrough IFI (three Candida spp., seven invasive mould infections) and tended to have higher overall mortality. The only statistically significant risk factor for IFI was cytomegalovirus co-infection. Adverse events, all grade I, occurred in 25 (20%), requiring L-AmB withdrawal in one case. Overall survival at 30 days was 99.2%. At study completion, one patient had died of IFI. Conclusions: The incidence of breakthrough IFI was comparable to that of previous reports, with a very low rate of significant toxicity. Thus, prophylactic L-AmB may be a safe, effective option for antifungal prophylaxis in the pre-engraftment phase for children undergoing HSCT, even those at high risk.