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摘要： We read the article by Jahn I et al i with interest, which investigated the use of prescribing and resuscitation aids in paediatric emergency care.1 The authors highlighted some insightful points regarding the usage of such aids; we wished to evaluate and further their findings. 2020; 109 (9): 1 - 21. 2 Lyttle M, Magnus D, Kanani A, Kehler L, Parri N, Jones S. Variability in point of care ultrasound (POCUS) practices in paediatric emergency departments in the UK & Ireland; a PERUKI study. [Extracted from the article]

摘要： We thank Behzad-Noori D and Singh G for their letter to the editor1 commenting on our recent publication.2 We are pleased to see medical students interested in academic endeavour and note their multiple previous letters to other journals about survey design and validity. We again thank Behzad-Noori D and Singh G for their interest in our work and look forward to reading their own survey on app use in health care in a wider population of clinicians. REFERENCES 1 Behzad-Noori D, Singh G. Response to "Prescribing in a paediatric emergency: A PERUKI Survey of Prescribing and Resuscitation Aids". [Extracted from the article]

关键词： Rhabdomyosarcoma   Soft tissue sarcoma   Childhood cancer  

摘要： Background: Rhabdomyosarcoma (RMS) is the most common form of soft tissue sarcoma in children. We report the results of the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 study, which prospectively evaluated the reduction of chemotherapy in patients with embryonal RMS (ERMS) after initial surgery. Methods: Between October 2005 and December 2016, all patients with localised ERMS with an initial microscopically complete resection (IRS group I) with lymph node-negative (NO) were prospectively enrolled in the low-risk (n = 70, subgroup A;age < 10 years and tumour size <= 5 cm) or standard-risk group (n = 108, subgroup B;age >= 10 years or tumour size > 5 cm. Subgroup A received 8 courses of vincristine and dactinomycin (VA) for 22 weeks;subgroup B received 4 courses of VA with ifosfamide (IVA) and 5 courses of VA for 25 weeks. Results: The 5-year event-free survival (EFS) and overall survival (OS) were 90.8% (95% confidence interval [CI]: 85.0-94.4) and 95.7% (95% CI: 90.5-98.1), respectively (n = 178). The EFS and OS were 95.5% (95% CI: 86.8-98.5) and 100% (subgroupA), and 87.8% (95% CI: 79.3-93.0) and 93.0% (95% CI: 84.8-96.8)(subgroup B), respectively. Bearman stage 2 veno-occlusive disease (VOD) occurred in 4 very young patients. Conclusion: VA treatment for 8 courses was effective and well tolerated by the subgroup of patients with low-risk ERMS (group A). Four courses of IVA and 5 courses of VA instead of 9 courses of IVA also has very good results. Careful monitoring for liver toxicity is important in very young patients. (C) 2021 Elsevier Ltd. All rights reserved.

关键词： pediatric DRL   dose reference level   diagnostic reference level   computed tomography   DRL  

摘要： This study aims to review the existing literature on diagnostic reference levels (DRLs) in paediatric computed tomography (CT) procedures and the methodologies for establishing them. A comprehensive literature search was done in the popular databases such as PubMed and Google Scholar under the key words 'p(a)ediatric DRL', 'dose reference level', 'diagnostic reference level' and 'DRL'. Twenty-three articles originating from 15 countries were included. Differences were found in the methods used to establish paediatric CT DRLs across the world, including test subjects, reference phantom size, anatomical regions, modes of data collection and stratification techniques. The majority of the studies were based on retrospective patient surveys. The head, chest and abdomen were the common regions. The volume computed tomography dose index (CTDIvol) and dose-length product (DLP) were the dosimetric quantities chosen in the majority of publications. However, the size-specific dose estimate was a growing trend in the DRL concept of CT. A 16 cm diameter phantom was used by most of the publications when defining DRLs for head, chest and abdomen. The majority of the DRLs were given based on patient age, and the common age categories for head, chest and abdomen regions were 0-1, 1-5, 5-10 and 10-15 years. The DRL ranges for the head region were 18-68 mGy (CTDIvol) and 260-1608 mGy cm (DLP). For chest and abdomen regions the variations were 1.0-15.6 mGy, 10-496 mGy cm and 1.8-23 mGy, 65-807 mGy cm, respectively. All these DRLs were established for children aged 0-18 years. The wide range of DRL distributions in chest and abdomen regions can be attributed to the use of two different reference phantom sizes (16 and 32 cm), failure to follow a common methodology and inadequate dose optimisation actions. Therefore, an internationally accepted protocol should be followed when establishing DRLs. Moreover, these DRL variations suggest the importance of establish a national DRL for each cou

关键词： Deep learning   Dose prediction   Paediatric abdominal tumours   Patient referral   Proton therapy   Photon therapy  

摘要： Objective: Dose prediction using deep learning networks prior to radiotherapy might lead tomore efficient modality selections. The study goal was to predict proton and photon dose distributions based on the patient-specific anatomy and to assess their clinical usage for paediatric abdominal tumours. Material and methods: Data from 80 patients with neuroblastoma or Wilms' tumour was included. Pencil beam scanning (PBS) (5 mm/ 3%) and volumetric-modulated arc therapy (VMAT) plans (5 mm) were robustly optimized on the internal target volume (ITV). Separate 3-dimensional patch-based U-net networks were trained to predict PBS and VMAT dose distributions. Doses, planning-computed tomography images and relevant optimization masks (ITV, vertebra and organs-at-risk) of 60 patients were used for training with a 5-fold cross validation. The networks' performance was evaluated by computing the relative error between planned and predicted dose-volume histogram (DVH) parameters for 20 inference patients. In addition, the organs-at-risk mean dose difference between modalities was calculated using planned and predicted dose distributions (Delta D-mean = D-vmAT-D-PBS). Two radiation oncologists performed a blind PBS/VMAT modality selection based on either planned or predicted Delta D-mean. Results: Average DVH differences between planned and predicted dose distributions were <=vertical bar 6%vertical bar for both modalities. The networks classified the organs-at-risk D-mean difference as a gain (Delta D-mean > 0) with 98% precision. An identical modality selection based on planned compared to predicted Delta D(mean )was made for 18/20 patients. Conclusion: Deep learning networks for accurate prediction of proton and photon dose distributions for abdominal paediatric tumours were established. These networks allowing fast dose visualisation might aid in identifying the optimal radiotherapy technique when experience and/or resources are unavailable. (C) 2020 The Authors. Published by E

关键词： Whole-body imaging   Child   Diffusion magnetic resonance imaging   Hodgkin disease   Neoplasm staging  

摘要： Objectives To assess the concordance of whole-body MRI (WB-MRI) and an FDG-PET/CT-based reference standard for the initial staging in children with Hodgkin lymphoma (HL) Methods Children with newly diagnosed HL were included in this prospective, multicentre, international study and underwent WB-MRI and FDG-PET/CT at staging. Two radiologists and a nuclear medicine physician independently evaluated all images. Discrepancies between WB-MRI and FDG-PET/CT were assessed by an expert panel. All FDG-PET/CT errors were corrected to derive the FDG-PET/CT-based reference standard. The expert panel corrected all reader errors in the WB-MRI DWI dataset to form the intrinsic MRI data. Inter-observer agreement for WB-MRI DWI was calculated using overall agreement, specific agreements and kappa statistics. Concordance for correct classification of all disease sites and disease stage between WB-MRI (without DWI, with DWI and intrinsic WB-MRI DWI) and the reference standard was calculated as primary outcome. Secondary outcomes included positive predictive value, negative predictive value and kappa statistics. Clustering within patients was accounted for using a mixed-effect logistic regression model with random intercepts and a multilevel kappa analysis. Results Sixty-eight children were included. Inter-observer agreement between WB-MRI DWI readers was good for disease stage (kappa= 0.74). WB-MRI DWI agreed with the FDG-PET/CT-based reference standard for determining disease stage in 96% of the patients versus 88% for WB-MRI without DWI. Agreement between WB-MRI DWI and the reference standard was excellent for both nodal (98%) and extra-nodal (100%) staging. Conclusions WB-MRI DWI showed excellent agreement with the FDG-PET/CT-based reference standard. The addition of DWI to the WB-MRI protocol improved the staging agreement.

关键词： Kawasaki disease   Myocarditis   Paediatric vascularitis   COVID-19   SARS-COV-2  

摘要： Myocarditis and Kawasaki disease are common but usually distinct diseases in children. During the coronavirus pandemic (COVID-19), reports of a new form of myocarditis with clinical features of Kawasaki appeared. We investigated the place of this new disease in the spectrum encompassing Kawasaki disease and myocarditis. Thirty two consecutive children referred to our centre for a suspicion of Kawasaki or a diagnosis of myocarditis were included and eventually divided into four groups: 11 Kawasaki diseases, 6 Kawasaki syndromes (children with another diagnosis), 7 myocarditis without Kawasaki clinical feature and 7 myocarditis with incomplete Kawasaki clinical features. All were treated with immunoglobulins except those of the myocarditis group. The survival rate was 91%. The 7 children with myocarditis and clinical features of incomplete Kawasaki were all positive for SARS-CoV-2. They had a transient myocardial failure with a favourable course and none had coronary artery disease. Conclusion: Every COVID-19 child within our population had a mild to severe myocarditis and presented with fever plus two or three Kawasaki clinical features. Short-term evolution was good for these children. This new disease seems to fill the gap between isolated myocarditis and Kawasaki disease. What is Known: A new paediatric disease close to Kawasaki disease appeared during the COVID-19 pandemic What is New: center dot In our population,children presented with fever,vivid Kawasaki clinical features(although the Kawasaki syndrome was always incomplete)and a myocarditis without coronary abnormalities. center dot The new disease fills the gap between paediatric myocarditis and Kawasaki disease but its prognosis is much better.

关键词： diagnosis   lncRNA FBXL19‐   AS1   paediatric acute myeloid leukemia   prognosis  

摘要： Background Long non-coding RNAs (lncRNA) have emerged as novel clinical biomarkers and therapeutic targets for various tumors because of their disease- and stage-restricted expression. lncRNA FBXL19 antisense RNA 1 (FBXL19-AS1) expression has been confirmed to be up-regulated in several tumors. However, its expression and effects in paediatric acute myeloid leukemia (AML) have not been elucidated. Methods Serum FBXL19-AS1 expression was determined in 137 AML patients compared to 43 healthy controls ( < 0.01). Results Using receiver operating characteristic curve analysis, we observed that serum FBXL19-AS1 provided the highly diagnostic performance for the detection of AML (AUC = 0.841, < 0.001). We also examined the association between serum FBXL19-AS1 expression and clinicopathological factors, finding that its high expression was associated with French-American-British classification ( = 0.011) and cytogenetics ( = 0.021). Survival assays with the Kaplan-Meier method revealed that the overall survival ( = 0.0088) and disease-free-survival ( = 0.0027) of AML patients with high serum FBXL19-AS1 levels were distinctly shorter compared to those with low serum FBXL19-AS1 levels. More importantly, Multivariate analysis identified serum FBXL19-AS1 overexpression as an independent unfavorable prognostic factor for both overall survival and disease-free-survival of AML patients. Conclusions Overall, our findings revealed that high expression of serum FBXL19-AS1 might be useful as a novel prognostic and diagnostic biomarker for AML patients.

关键词： Inflammatory Bowel Disease   COVID-19   Paediatrics   lockdown   telemedicine   immunosuppression  

摘要： Background: IBD management has been significantly affected during the COVID-19 lockdown with potential clinical issues. Aims: The aim of this study was to analyse the impact of COVID-19 pandemic on the Italian paediatric IBD cohort. Methods: This was a multicentre, retrospective, cohort investigation including 21 different Italian IBD referral centres. An electronic data collection was performed among the participating centres including: clinical characteristics of IBD patients, number of COVID-19 cases and clinical outcomes, disease management during the lockdown and the previous 9 weeks. Results: 2291 children affected by IBD were enrolled. We experienced a significant reduction of the hospital admissions [604/2291 (26.3%) vs 1281/2291 (55.9%);p < 0.001]. More specifically, we observed a reduction of hospitalizations for new diagnosis (from n = 44 to n = 27) and endoscopic re-evaluations (from n = 46 to n = 8). Hospitalization for relapses and surgical procedures remained substantially unchanged. Biologic infusions did not significantly vary [393/2291 (17.1%) vs 368/2291 (16%);p = 0.3]. Telemedicine services for children with IBD were activated in 52.3% of the centres. In 42/2291(1.8%) children immunosuppressive therapies were adapted due to the concurrent COVID-19 pandemic. Conclusion: Due to the several limitations of the lockdown, cares for children with IBD have been kept to minimal standards, giving priorities to the urgencies and to biologics' infusions and implementing telemedicine services. (C) 2020 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

摘要： Background Antibiotics are widely used and inaccurate or inappropriate prescription of antibiotics causes a significant increase in the prevalence of multidrug-resistant bacterial infections among children. This research aimed to study antibiotic prescriptions in hospitalised paediatric patients and to determine the prevalence of inappropriate antimicrobial use and the main types of prescribing errors. Methods After obtaining the Ethics Committee approval, screening was conducted among 535 patients admitted to the Department of Pediatrics at Haydarpasa Numune Training and Research Hospital in the period from 01 January 2016 to 31 December 2016 who had been treated with an antibiotic. Patients' demographics, diagnosis and antibiotic therapy details were collected using a standardised case report form and assessed by a clinical pharmacologist and an infectious disease specialist regarding the convenience and accurateness of prescription of antibiotics. Results Out of 535 antibiotic prescriptions, single antibiotics were used inappropriately in 216 (56.10%) of the patients and there were 39 (26%) unnecessary antibiotic combinations. Most of the errors were made in the dose frequency (55.69%), followed by indication (25.88%), administration route (16.08%) and dosage (2.67%). Conclusions The results of our study show that a high level of antibiotics in the paediatric clinic was misprescribed. Inappropriate usage increases the chances of microbial resistance and the cost of treatment. Precautions should be taken in this regard.