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关键词： Congenital heart disease   Paediatric heart failure   Stem cell therapy  

摘要： Significant achievements in paediatric cardiology, surgical treatment and intensive care of congenital heart disease have drastically changed clinical outcomes for paediatric patients. Nevertheless, late-onset heart failure in children after staged surgeries still remains a serious concern in the medical community. Heart transplantation is an option for treatment;however, the shortage of available organs is a persistent problem in many developed countries. In order to resolve these issues, advanced technologies, such as innovative mechanical circulatory support devices and regenerative therapies, are strongly desired. Accumulated evidence regarding cell-based cardiac regenerative therapies has suggested their safety and efficacy in treating adult heart failure. Given that young children seem to have a higher regenerative capacity than adults, stem cell-based therapies appear a promising treatment option for paediatric heart failure as well. Based on the findings from past trials and studies, we present the potential of various different types of stem cells, ranging from bone marrow mononuclear cells to cardiosphere-derived stem cells for use in paediatric cell-based therapies. Here, we assess both the current challenges associated with cell-based therapies and novel strategies that may be implemented in the future to advance stem cell therapy in the paediatric population.

摘要： Autism,1 schizophrenia,2 attention-deficit/hyperactivity disorder,3 Alzheimer's disease4 and even anxiety5 may be manifestations of our microbial signature and an underlying intestinal dysbiosis. Human gut microbiota from autism spectrum disorder promotes behavioural symptoms in mice. 2 Yuan X, Kang Y, Zhou C, Huang XF, Song X. The gut microbiota promotes the pathogenesis of schizophrenia by multiple pathways. Gut microbiota and dietary patterns in children with attention deficit/hyperactivity disorder. [Extracted from the article]

关键词： Lymphatic malformations   Plastic bronchitis   Embolization   Lymphangiography  

摘要： Pulmonary lymphatic disorders are characterized by the presence of the abnormal lymphatic tissues in the thoracic cavity, presenting clinically as chylothorax, chylopericardium, chyloptysis, interstitial lung disease and plastic bronchitis. These conditions include: neonatal chylothorax, cardiac and non-cardiac plastic bronchitis, non-traumatic chylothorax, post congenital cardiac surgery chylothorax and complex lymphatic malformations. Recently developed lymphatic imaging techniques, such as intranodal lymphangiography and dynamic contrast enhanced magnetic resonance lymphangiography demonstrated abnormal pulmonary lymphatic flow from thoracic duct into pulmonary parenchyma as a pathophysiological mechanism of these diseases. Novel minimally invasive lymphatic interventions, such as thoracic duct embolization, interstitial lymphatic embolization and surgical lympho-venous anastomosis, provide an effective treatment of these conditions. (c) 2020 Elsevier Ltd. All rights reserved.

关键词： Rett syndrome   Autonomic dysregulation   Paediatric   Child   Brain injury   Emotional behavioural & autonomic dysregulation  

摘要： Rett Syndrome (RTT) is a complex neurodevelopmental disorder with autonomic nervous system dysfunction. The understanding of this autonomic dysregulation remains incomplete and treatment recommendations are lacking. By searching literature regarding childhood brain injury, we wanted to see whether understanding autonomic dysregulation following childhood brain injury as a prototype can help us better understand the autonomic dysregulation in RTT. Thirty-one (31) articles were identified and following thematic analysis the three main themes that emerged were (A) Recognition of Autonomic Dysregulation, (B) Possible Mechanisms & Assessment of Autonomic Dysregulation and (C) Treatment of Autonomic Dysregulation. We conclude that in patients with RTT (I) anatomically, thalamic and hypothalamic function should be explored, (II) sensory issues and medication induced side effects that can worsen autonomic function should be considered, and (III) diaphoresis and dystonia ought to be better managed. Our synthesis of data from autonomic dysregulation in paediatric brain injury has led to increased knowledge and a better understanding of its underpinnings, leading to the development of application protocols in children with RTT.

关键词： Paediatric intubation   Neonates   Videolaryngoscopy   Direct laryngoscopy   Meta-analysis   Randomized controlled trial  

摘要： Objective: Anatomical and physiological differences in paediatric and adult airways make intubation of paediatric patients a challenge. This study aimed to compare the efficacy and safety of video laryngoscopy (VL) to direct laryngoscopy (DL) on intubation outcomes in paediatric patients. Design: Systematic review and meta-analysis. Setting: Operating room. Patients: Paediatric patients who needed tracheal intubation. Intervention: Video laryngoscopy or direct laryngoscopy. Measurements: Electronic searches in PubMed, Embase, and the Cochrane Library were performed to identify relevant randomized controlled trials published through January 2020. Outcomes included time to incubate, intubation failure at first attempt, Cormack-Lehane laryngeal view grade, intubation difficulty scale (IDS), percentage of glottic opening score (POGO), optimal external laryngeal manipulation (OLEM), and complications. Relative risks and weighted mean difference (WMD), with 95% CI, were employed to calculate summary results using a random-effects model. Main results: Overall, 27 trials including 2461 paediatric patients were analysed. Children with video laryngoscopy intubation required longer time to incubate than direct laryngoscopy intubation (WMD 3.41, 95% CI: 1.29-5.53, P = 0.002), whereas infants receiving video laryngoscopy and direct laryngoscopy intubation experienced similar time to incubate (WMD 1.72, 95% CI: - 1.09-4.54, P = 0.230). No significant differences were observed on intubation failure at first attempt between video laryngoscopy and direct laryngoscopy incubations in children and infants, respectively. Video laryngoscopy improved the POGO and intubation trauma but not Cormack-Lehane laryngeal view grade, IDS, external laryngeal manipulation, hoarseness, or oxygen desaturafion. Conclusions: Compared with direct laryngoscopy intubation, there were no benefits for paediatric patients with video laryngoscopy on time to incubate and failure at first attempt, but there were

关键词： chimeric antigen receptor T cell   acute lymphoblastic leukaemia   paediatric   adoptive T‐   cell therapy  

摘要： Over the last decade, chimeric antigen receptor (CAR)-T cell therapy has emerged as a promising treatment modality for relapsed/refractory B-cell malignancies in both children and adults. As an adoptive immune therapy, CAR-T cells have the potential to overcome disease that is resistant to chemo- and radiotherapy as well as represent a viable option for those who have already reached toxicity ceilings with standard therapies. CD19-directed CAR-T cell products have been licensed for use in paediatric B-cell acute lymphoblastic leukaemia that is refractory, in relapse post-transplant or in second or later relapse. Many challenges remain, rightly resulting in a heavily-mined research field. These include mitigating short-term immune-mediated toxicity, maintaining durability of responses, broadening treatment accessibility and extending its applicability to other malignant settings. In this review, dedicated to marking 60 years since the establishment of the British Society for Haematology, we will focus on the contribution of our community towards the success of CD19-directed CAR-T cell therapy in children. We will put current practice in CAR-T cell therapy into the context of future challenges to be addressed in order for it to fulfil its "game-changing" therapeutic potential.

关键词： Paediatric cardiology   training   congenital cardiology  

摘要： The recommendations of the Association for European Paediatric and Congenital Cardiology for basic training in paediatric and congenital cardiology required to be recognised as a paediatric cardiologist by the Association for European Paediatric and Congenital Cardiology are described below. Those wishing to achieve more advanced training in particular areas of paediatric cardiology should consult the training recommendations of the different Association for European Paediatric and Congenital Cardiology Working Groups available on the Association for European Paediatric and Congenital Cardiology website (***) and the respective publications 1-6. The development of training requirements is the responsibility of the Educational Committee and the Association for European Paediatric and Congenital Cardiology Council in collaboration with the Working Groups of the Association for European Paediatric and Congenital Cardiology. Trainees should be exposed to all aspects of general paediatric and congenital cardiology from fetal life to adolescence and adulthood. Centres performing generalised and specialised work in paediatric and congenital cardiology should be committed to deliver postgraduate training. At each training institute, trainers should be appointed to supervise and act as mentors to the trainees. Association for European Paediatric and Congenital Cardiology will provide basic teaching courses to supplement the training process.