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摘要： Key factors in these reductions have been the roll-out and scale-up of vaccine coverage against Haemophilus influenzae type b (Hib) and pneumococcal infections (pneumococcal conjugate vaccine [PCV]), improved childhood nutrition and feeding practices, and declines in household air pollution. According to the GBD 2017 Lower Respiratory Infections Collaborators,3 improved nutrition-related factors and reductions in household air pollution were the main contributors to impressive reductions in mortality in India. A 2016 study reported that the odds of low birthweight was 10·6 times higher in the northwest than in the southwest of Nigeria,7 and the 2018 Demographic and Health Survey, which gathered data from national household surveys, report vaccine coverage to range from 73% for PCV and 74% for Hib in Abuja state to 6% for PCV and 7% for Hib in Sokoto state.8 These estimates highlight the need for caution in extrapolating broader conclusions on the roles of different interventions and the public health gains made across heterogeneous countries with little subnational data.

关键词： Paediatric   Ureteroscopy   RIRS   Complications   Success   Urolithiasis  

摘要： Purpose To report the outcomes of paediatric ureteroscopy (URS) for stone disease from a specialist endourology centre in the UK. Ureteroscopy for management of stone disease has increased worldwide and is now being done more commonly in the paediatric age group. Methods Data were analysed retrospectively from a database maintained between April 2010 and May 2018. Consecutive patients <= 16 years of age undergoing semi-rigid or flexible URS for stone disease were included. Stone size and stone-free rate (SFR) were routinely assessed using an ultrasound (USS) and/or plain KUB XR. Complications were graded according to the Clavien-Dindo classification and recorded within 30 days post-procedure and readmissions within 90 days after the procedure were also captured. Results Over the 8-year period between April 2010 and April 2018, 81 patients with a mean age of 8.8 years (range 18 months-16 years) and a male to female ratio 1:1.1 underwent 102 procedures (1.28 procedure/patient to be stone free). Of the 81 patients, 29 (35.8%) had comorbidities, with 26 (32%) having multiple comorbidities. The mean (+/- SEM) single and overall stone size was 9.2 mm (+/- 0.48, range 3-30 mm) and 11.5 mm (+/- 0.74, range 4-46 mm) respectively, with 22 (27.1%) having multiple stones. Thirty-five (34.7%) had stent in situ pre-operatively. The stone location was in the ureter (26.6%), lower pole (35.4%), and renal pelvis (16.5%), with 22/81(27%) having multiple stones and 21/102 (20.5%) where a ureteral access sheath (UAS) was used. With a mean hospital stay of 1.2 days, the initial and final SFR was 73% and 99%, respectively, and 61/102 (60%) had ureteric stent placed at the end of the procedure. While there were no intra-operative complications, the readmission rate was less than 1% and there were only three early complications recorded. This included a case each of prolonged admission for pain control (grade I), urinary retention (grade II) and post-operative sepsis requiring a brief ITU

关键词： acute generalized exanthematous pustulosis   aetiopathogenesis   children   drug reaction with eosinophilia and systemic symptoms   hypersensitivity drug reactions   lymphocyte transformation tests   paediatrics   severe cutaneous adverse reactions   Stevens-Johnson syndrome   toxic epidermal necrolysis  

摘要： Background Severe cutaneous adverse reactions (SCARs) are delayed-type hypersensitivity reactions to drugs including as follows: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS), Stevens-Johnson syndrome (SJS), Toxic Epidermal Necrolysis (TEN) and Acute Generalized Exanthematous Pustulosis (AGEP). Incidence, triggers and management of SCARs have not been investigated in large-scale epidemiological studies on children. Objective The aim of our study was to collect epidemiological, clinical and aetiological data from children with SCARs referred to our tertiary care paediatric hospital of Florence. Methods From 2010 to 2018 charts of children with diagnosis of SCAR were reviewed, and data collected during the acute phase and/or the subsequent allergy evaluation. Patients underwent patch tests, intradermal tests and lymphocyte transformation tests. All children were investigated for infectious diseases. Results Incidence of SCARs in hospitalized children was 0.32% over a 9-year period. Fifty-four children were enrolled (31 M;23 F;median age 6.5 years): 17 cases of DRESS, 30 SJS, 3 TEN, 2 AGEP, 1 linear immunoglobulin A bullous disease (LABD) and 1 pemphigus. Twenty-eight out of 54 patients underwent drug allergy investigations, and 50% of them resulted positive. Combining clinical history and results of allergy work-up, 74% SCARs seem to be caused by drugs, 18.6% by both drugs and infections, 3.7% by infections, and 3.7% remained idiopathic. No deaths occurred. Conclusions In this study, SCARs incidence is in line with literature data. Drugs were most commonly the leading cause. Management of SCARs requires cooperation among professional figures for an early diagnosis and a prompt treatment. Mortality rate seems to be lower in children.

关键词： altmetric   bibliometrics   dimensions   impact factor   paediatric dentistry  

摘要： Background Traditional metrics have been extensively used to evaluate the scientific performance. Despite being widespread accepted, citation-based metrics are not able to describe the social impact of research. A diverse metric, Altmetric, was proposed to overcome those limitations. Aim This study aims to analyse the social impact of research in the field of paediatric dentistry and to assess if a correlation exists between the JCR citations, the AAS score, and the recently released Dimensions citation count. Design A bibliometric study was conducted on the four journals related to Paediatric Dentistry listed in the JCR from 2014 to 2017. Descriptive statistics was used to describe the articles and the journals. Pearson's correlation analysis was used to explore the relationship among JCR citations, AAS, and Dimensions. Results The percentage of articles with an AAS presents a huge variability and was significantly higher in the International Journal of Paediatric Dentistry. In our sample, the correlation between the JCR citation count and the AAS was poor in the years 2014, 2015, and 2016 and low in 2017. The correlation between JCR citation and Dimensions citation count was strong. Conclusions The social impact of research in paediatric dentistry can be increased. Dimensions could be an alternative to the JCR. Both the editors and the researcher should change their vision and facilitate the access to research information to scholar and non-scholar audiences.

关键词： Appendiceal neuroendocrine neoplasms   Locoregional lymph node metastases   Prophylactic right hemicolectomy  

摘要： Background There are no clear histopathological parameters determining the risk of lymph node (LN) metastases and appropriateness of completion prophylactic right hemicolectomy (RHC) in patients with appendiceal neuroendocrine neoplasms (ANENs). Materials and methods The PubMed, Cochrane Library, Embase, Web of Science and SCOPUS databases were searched up to November 2018. Quality/risk of bias was assessed using the Newcastle-Ottawa Scale (NOS). Results A total of 526 articles were screened. In 11 adult and 3 paediatric studies, 602 and 77 unique patients, respectively, with ANEN and undergoing RHC, were included. The rate of LN metastases for a cutoff size >10 mm was 48.6% (vs 12.1% for lesions <10 mm) among adult patients, with an odds ratio (OR) of 4.8 (95% CI, 1.5-15.8). For 20 mm size cutoff, these figures were 61% (vs 28.2% for lesions <20 mm) with an OR of 3.2 (95% CI, 1.3-7.8). Vascular-, lymph vessel- and perineural invasions were identified as predictive factors for LN metastases in adult patients. In paediatric patients, there were no strong morphological predictors for LN metastases. The 10-year disease-specific survival (DSS) for adult patients without LN metastases was 99.2% vs 95.6% in patients with LN (OR: 0.2;95% CI, 0.02-2.4). The complication rate of prophylactic RHC was 11.4%. Conclusions This meta-analysis demonstrates that tumour size >20 mm as well as >10 mm and/or vascular-, lymph vessel- and perineural invasions are associated with increased risk for LN metastases in adult patients with ANEN. The prognostic value of LN positivity remains to be determined in further studies with long-term follow-up.

关键词： children   diary   paediatric intensive care   parents   social support   survey methods  

摘要： Aim The aim of the study was to explore the incidence, use, and scope of patient diaries in paediatric intensive care units (PICUs) in the United Kingdom and Ireland. Design This was an electronic survey sent to 30 PICUs in the United Kingdom and Ireland. Results All PICUs (n = 30) responded, and 43% (n = 13) offered diaries. For those units that did not supply diaries, the reasons given were concerns around the legal and professional implication of using diaries. Parental/carer consent to use a diary was obtained informally (79%, n = 11), and once there was agreement to provide a diary to parents, diaries were usually started immediately (72%, n = 12). Parents were the main contributors to the diaries (94%, n = 17), and the diaries were populated with photographs (94%, n = 15), drawings (100%, n = 16), and stickers (94%, n = 15). The reasons for offering diaries were to fill gaps in memories, to engage with families, and to explain what has happened in lay language. The owner of the diary was reported to be the family (82%, n = 14) and the child (12%, n = 2). Conclusions The use of patient diaries is an evolving intervention in paediatric intensive care settings in the United Kingdom and Ireland. This national survey has provided a clearer picture of how this intervention is used in the United Kingdom and Ireland. PICU patient diaries are used in a significant number of units, and how these are used is relatively standardized, although in some different ways from general ICUs. Relevance to clinical practice This survey provides a baseline for future exploration, understanding, and promotion of patient diaries, as a well evaluated tool for the critically ill child and his or her family.

摘要： Background Pembrolizumab is approved for the treatment of advanced cancer in adults;however, no information is available on safety and efficacy in paediatric patients. We aimed to establish the recommended phase 2 dose of pembrolizumab and its safety and antitumour activity in advanced paediatric cancer. Methods KEYNOTE-051 is an ongoing phase 1-2 open-label trial. In this interim analysis, children aged 6 months to 17 years were recruited at 30 hospitals located in Australia, Brazil, Canada, France, Germany, Israel, Italy, South Korea, Sweden, the UK, and the USA. Patients with melanoma or a centrally confirmed, PD-L1-positive, relapsed or refractory solid tumour or lymphoma, and a Lansky Play/Karnofsky Performance status score of 50 or higher, received intravenous pembrolizumab at an initial dose of 2 mg/kg every 3 weeks. Pharmacokinetics and dose-limiting toxicities were used to establish the recommended phase 2 dose, and the safety and antitumour activity of this dose were assessed. Primary endpoints were determination of dose-limiting toxicities at the maximum administered dose, safety and tolerability, and the proportion of patients with objective response to pembrolizumab for each tumour type according to the Response Evaluation Criteria in Solid Tumours version 1.1 or the International Neuroblastoma Response Criteria. Safety and efficacy were assessed in all treated patients who received at least one dose of pembrolizumab. Separate reporting of the cohort of patients with relapsed or refractory classical Hodgkin lymphoma was a post-hoc decision. The data cutoff for this interim analysis was Sept 3, 2018. This trial is still enrolling patients and is registered with ***, number NCT02332668. Findings Of 863 patients screened between March 23, 2015, and Sept 3, 2018, 796 had tumours that were evaluable for PD-L1 expression (278 [35%] were PD-L1-positive);155 eligible patients were enrolled and 154 had at least one dose of pembrolizumab. The media

关键词： epigenetics   acute myeloid leukaemia   Polycomb   paediatric  

摘要： Comprehensive cataloguing of the acute myeloid leukaemia (AML) genome has revealed a high frequency of mutations and deletions in epigenetic factors that are frequently linked to treatment resistance and poor patient outcome. In this review, we discuss how the epigenetic mechanisms that underpin normal haematopoiesis are subverted in AML, and in particular how these processes are altered in childhood and adolescent leukaemias. We also provide a brief summary of the burgeoning field of epigenetic-based therapies, and how AML treatment might be improved through provision of better conceptual frameworks for understanding the pleiotropic molecular effects of epigenetic disruption.

关键词： asthma   cystic fibrosis   primary ciliary dyskinesia  

摘要： We should be measuring peripheral blood eosinophil count and sensitization to aeroallergens to target patients who will benefit from inhaled steroids, rather than putting them in tap water.[7] More biologicals are emerging, largely directed at TH-2-driven asthma. There are analogies between CF and PCD diagnostics; in both, most cases can be diagnosed based on simple tests if the diagnosis is considered, but in each disease there is an increasing armamentarium of sophisticated diagnostics. Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators. Treatment of chronic asthma with prednisolone significance of eosinophils in the sputum. [Extracted from the article]

关键词： Prosthetic knee   biomechanical modelling   amputee   gait analysis   stance control  

摘要： The 2-axes Automatic Stance Phase Lock (ASPL) stance control mechanism has been demonstrated to improve adult amputees' mobility but has yet to be developed for the paediatric population. The overall objective for this work was to characterize the ASPL control mechanism with biomechanical modelling and design a 2-axes ASPL prosthetic knee joint suitable for children between the ages of 6 and 12 years. Paediatric anthropometric data and ASPL control mechanism performance characteristics established from adult ASPL knee users were utilized to develop paediatric-appropriate configurations of the ASPL stance control mechanism. Additional predefined design criteria were also included in the detailed knee design. Developed prototypes of the knee joint, Children-ASPL (CASPL) knee, were clinically validated using a single-subject cross-over study design, to assess control mechanism and overall knee functions. Faster walking speed, longer step and stride length with the CASPL knee suggest potential improvements in overall walking performance. The participant also felt confident walking with the CASPL knee and perceived the locking mechanism to be stable. Stemming from the findings here, future design revisions are aimed to improve the performance of the current prototype, including reliability of knee lock disengagement and performance of the swing phase control mechanism.