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关键词： Paediatric oncology   Targeted drugs   Preclinical research   Clinical development   Systematic review  

摘要： Background: Children with cancer are in urgent need of new therapies, as approximately 25% of patients experience a relapse and 20% succumb to their disease. Moreover, the majority of survivors suffer from clinically relevant health problems. Repurposing of targeted agents developed for adult indications could provide novel therapeutic options for paediatric cancer patients. To prioritise targeted drugs for paediatric clinical development, we applied a systematic review methodology to develop a Target Actionability Review (TAR) strategy. These TARs assess the strength and completeness of published preclinical proof-of-concept (PoC) data by structured critical appraisal of and summarising the available scientific literature for a specific target (pathway) and the associated drugs in paediatric tumours. Methods: A sensitive literature search in PubMed was performed and relevant papers were identified. For each paper, the individual experimental findings were extracted, marked for paediatric tumour type and categorised into nine separate PoC data modules. Each experimental finding was scored for experimental outcome and quality independently by two reviewers;discrepancies were assessed by a third reviewer and resolved by adjudication. Scores corresponding to one PoC module were merged for each tumour type and visualised in a heat map matrix in the publicly available R2 data portal [***]. Results and conclusions: To test our TAR methodology, we conducted a pilot study on MDM2 and TP53. The heat map generated from analysis of 161 publications provides a rationale to support drug development in specific paediatric solid and brain tumour types. Furthermore, our review highlights tumour types where preclinical data are incomplete or lacking and for which additional preclinical testing is advisable. (C) 2020 The Authors. Published by Elsevier Ltd.

关键词： Congenital scoliosis   Lumbosacral hemivertebrae   Hemivertebrae resection   Short segmental fusion   Paediatric  

摘要： Purpose To evaluate the clinical and radiological outcomes for patients who undergone posterior-only lumbosacral hemivertebrectomy with short fusion with minimum two year follow-up. Methods From 2005 to 2016, a consecutive series of 16 paediatrics scoliosis due to lumbosacral hemivertebrae were included in this study. They were treated by one stage posterior hemivertebrae resection with bilateral or unilateral short fusion. Coronal and sagittal parameters and pelvic obliquity were measured at pre-operatively, immediate post-operatively and at final follow-up. The outcome and efficacy of the correction were investigated. Results The mean age was 10.4 +/- 3.4 years (3-15 years). The mean follow-up period was 38.8 +/- 16.2 months (24-79 months). The mean segmental scoliosis was 35.4 +/- 9.2 and 7.7 +/- 5.4 pre-operatively and post-operatively (78.4% correction rate) and 8.2 +/- 5.0 (77%) at the latest follow-up. The compensatory coronal curve of 28.6 +/- 16.1 was spontaneously corrected to 8.0 +/- 8.4 in post-operatively 12.0 +/- 8.4 at final follow-up. Trunk shift was significantly improved on both coronal (RTS 86.1%) and sagittal plane (68.7%) after the surgery and kept stable during the follow-up. Sacral tilt of 14.2 +/- 5.3 was significantly improved to 4.7 +/- 3.6 at final follow-up. There was no significant difference between bilateral and unilateral instrumentation groups (P > 0.05). One case had implants failure, and the incidence rate is 6.3%. Conclusions Early posterior hemivertebrectomy with short fusion is effective in the treatment of lumbosacral hemivertebrae. It can achieve good coronal curve correction, sacral tilt, and trunk shift improvement. No neurological complications were found. Importantly, it can prevent secondary structural deformities and potentially save fusion level.

摘要： The article informs that bipolar disorder (BPD) begins in childhood and suggests for an accurate and standardized diagnosis. Topics include rise in diagnosis among children who have symptoms such as elated mood, increased irritability and energy; and need of investigation of potential clinical costs of the current approach.

关键词： sickle cell disease   acute kidney injury   Epidemiology   hydroxycarbamide   paediatric  

摘要： Renal disease is a common complication experienced by patients with sickle cell disease (SCD), though the epidemiology of acute kidney injury (AKI) in paediatric patients and its impact on long-term renal outcomes is unclear. We utilized the Pediatric Health Information System (PHIS) to identify inpatient encounters of paediatric patients with SCD admitted for vaso-occlusive pain crisis (VOC). Overall, 1 center dot 4% of patients experienced at least one episode of AKI and 2 center dot 5% of admissions were complicated by AKI. Patients with at least one episode of AKI were more likely to be adolescents or young adults at the time of their initial admission, had increased rates of admission to the ICU, longer lengths of stay, increased costs of hospitalization, increased risk of readmission and increased rates of SCD-related comorbidities. Generalized estimating equation modelling demonstrated that increasing age, history of hypertension, history of haematuria and history of chronic kidney disease were associated with increased odds of developing AKI, though hydroxycarbamide use (OR 0 center dot 64, 95% CI 0 center dot 44-0 center dot 94) was protective. Episodes of AKI during hospitalization in children with SCD are associated with increased morbidity and utilization of hospital resources. Increasing the use of hydroxycarbamide may decrease the likelihood of this complication.

关键词： paediatric abusive head trauma   non-accidental head injury   physical abuse   baby   infant   healthcare professional   instrument development  

摘要： This two-stage study aimed to develop and validate a paediatric abusive head trauma (AHT) awareness questionnaire for healthcare professionals. In stage one, an item pool was created for the AHT awareness questionnaire. Four experts assessed the content validity. Test-retest reliability and internal consistency were examined using a pilot study of 24 healthcare professionals. In stage two, 302 healthcare professionals from a medical centre in southern Taiwan were recruited to establish the questionnaire's psychometric properties. The final version of the questionnaire comprised 81 items, including demographics, attitudes towards childrearing and four sub-concepts on AHT awareness (infant crying, soothing skills, clinical manifestations and consequences of AHT, and risk factors of AHT). The content validity of the questionnaire was satisfactory with a score of 0.91 to 1.0 for the scale content validity index. The test-retest reliability was 0.51-0.71. Cronbach's alphas for the final sample were 0.52-0.93 for the four subscales. Exploratory factor analysis extracted two factors as risk factors of AHT with a total explained variance of 55.9 per cent. This questionnaire is useful in understanding AHT awareness with identified aspects among healthcare professionals and other professionals working in child protection fields. Items and subscales of the AHT awareness questionnaire could act as a reference guide for future training programmes. 'This two-stage study aimed to develop and validate a paediatric abusive head trauma (AHT) awareness questionnaire for healthcare professionals' Key Practitioner Messages We developed and validated a questionnaire to assess healthcare professionals' awareness of paediatric AHT. This is the first instrument developed to measure healthcare professionals' awareness of paediatric AHT with acceptable psychometric properties for use in Taiwan. The questionnaire can be used to understand healthcare professionals' awareness of paediatric AHT.

关键词： 儿科学   眼科学   小儿疾病   斜视   诊疗  

ISBN: (纸本)9787536974623

摘要： 本书是“美国2019-2020基础与临床科学教程（BCSC）”的第6册图书，由美国眼科学会（AA0）组织编写，是国际眼科联盟（WOC）和中国医学考试网都推荐全世界的眼科医生和专业研究生学习的眼科临床教程。主要介绍了视网膜、玻璃体等基础的眼部解剖学和生理学知识，斜视和小儿葡萄膜炎、青光眼、白内障等眼部疾病的检查、诊断与治疗，视力矫正等。图书涵盖面广、内容权威。除了大量清晰的病理图片、临床学习视频外，还配有同步测试题及译者的主要参考资料，非常适合自学使用。

关键词： airway management   airway obstruction   dilatation   health information systems   patient safety   treatment outcome  

摘要： Objectives To assess the safety and efficacy in routine clinical practice of balloon dilatation procedures in the treatment of paediatric airway stenosis. Design Observational data collection in prospective online research database. Setting Acute NHS Trusts with ENT department undertaking complex paediatric airway work. Participants Children (<18) undergoing balloon dilatation treatment for airway stenosis. Main outcome measures Airway diameter, complications, hospital resource usage. Results Fifty-nine patients had 133 balloon procedures during 128 visits to 10 hospitals. Sixty-nine (52%) of balloon procedures were conducted with a tracheostomy. Intra-operative Cotton-Myer grade decreased in 43 (57%). The mean pre-balloon subglottic diameter was 4.2 [95% CI: 3.8 to 4.5] mm, and its rate of increase was 0.8 [0.5 to 1.2] mm per year modelled on 30 patients' long-term data. As the primary treatment of stenosis, the procedural success rate of balloon dilatation (n = 52) was 65% (22% with tracheostomy, 88% without tracheostomy), and 71% as an adjunct to open reconstructive surgery (n = 7). In the 64 hospital visits where a balloon procedure was conducted with a tracheostomy in place, only one in-hospital complication (lower respiratory tract infection) occurred. For those without a tracheostomy in place, in-hospital complications occurred in seven of 64 balloon hospital visits, all related to ongoing or worsening stenosis. Six out-of-hospital complications were deemed related to ongoing or worsening stenosis following the procedure, and two complications were a combination of lower respiratory infection and ongoing or worsening stenosis. Conclusions Balloon dilation increases the size of the airway intraoperatively and is associated with long-term increase in airway diameter. Safety outcomes mostly relate to ongoing or worsening stenosis and are more common in patients without a tracheostomy.

摘要： Osteomyelitis (OM) is a major cause of morbidity in sickle cell disease (SCD) patients (Thanni, [14]; Tarer I et al. i , [13]) with I Staphylococcus aureus i and the I Salmonella i species being the most frequently isolated microorganisms (Burnett I et al. i , [4]; Thanni, [14]). Ten patients were discharged on oral antibiotics: cefuroxime ( I N i = 5), levofloxacin plus rifampicin ( I N i = 3) or flucloxacillin ( I N i = 2). In the non-MDR group, complications were intraosseous abscesses ( I N i = 2) and chronic osteomyelitis ( I N i = 1). However, other Gram-negative bacilli (GNB) (18-19-6%) ranked almost equal to I S. aureus i (Burnett I et al. i , [4]; Thanni, [14]), similar to recent data from GNB bacteraemia in SCD patients (14-26%) (Williams I et al. i , [15]). [Extracted from the article]

摘要： The author discusses the growing interest in long-stay patients in paediatric intensive care. Topics mentioned include the emergence of the term 'long-stay patient' in the 1980s, the definition of chronic critical illness in adults, and the use of an integrated family system approach to providing family-centred care.

关键词： Children   Crohn's disease   Healthcare   Ulcerative colitis  

摘要： Transition care in inflammatory bowel disease is increasingly recognized as challenging given the inherent differences between paediatric and adult health care models, disease characteristics and treatment strategies. Transition is a dynamic process involving adolescents and young adults that are moving from a paediatric to an adult health care setting, and it should be flexible, continually updated and tailored to each patient. The implementation of a transition clinic is essential given the increasing incidence of the paediatric population with inflammatory bowel disease and the lifelong impact of this disease. The key question is when and how to structure transition according to the adolescent's clinical, psychosocial, educational needs and expectations to ensure continuity of care. In the attempt to improve the management of transition in inflammatory bowel disease and address the wide gap between adult and child care, we provide an update of the transition clinic and we propose a "treat to target" approach in transition to facilitate an effective and successful transition programme. In the changing landscape of the treatment of inflammatory bowel disease, further studies are necessary to determine the role of the transition clinic in determining the choice and strategy of therapy and its monitoring and the adoption of newer strategies such as biomarkers guided treating to target. (C) 2020 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.