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关键词： myoclonic absences   atypical absences with atonic component   encephalopathy related to status epilepticus during slow sleep (ESES)   negative myoclonus  

摘要： Objective. Epileptic myoclonus (EM) is reported in many paediatric epilepsies from neonatal period to adolescence. Myoclonus can be the only seizure type or may occur among others, independently or in combination as a single ictal event. We report two children presenting with absences associated with myoclonus, predominating on one side, in a setting of two different types of absence seizures and two different electro-clinical syndromes. Methods. Patients were explored with long-duration video-EEG coupled to surface EMG polygraphy. EEG was visually analysed and complemented by jerk-locked back-averaging. Results. Two types of seizure, encompassing myoclonus and absence, were identified: myoclonic absences in the context of epilepsy with myoclonic absences and atypical absences with atonic component (negative myoclonus) in the context of encephalopathy related to status epilepticus during slow sleep (ESES). In the latter case, rhythmic upper limb jerking, mimicking positive myoclonus, corresponded to recovery of muscular tone after each negative myoclonus. Significance. Due to the rhythmic recovery of muscle tone, subsequent rhythmic negative myoclonus may exhibit a similar clinical picture to that of rhythmic positive myoclonus. Video-EEG recording coupled to EMG polygraphy is essential in order to precisely characterize motor manifestations during seizures with myoclonus.

关键词： congenital cardiac catheter intervention   congenital heart disease   paediatric cardiac catheterisation   paediatric cardiology  

摘要： There has been marked innovation and progress in the techniques, technologies and outcomes in the field of paediatric and congenital cardiac catheter intervention over the past 4 decades. In the modern era, it is now possible to treat many forms of congenital heart disease through minimally invasive percutaneous therapies, with associated reduction in morbidity for this group of patients. This article provides an overview of current practice and developments in this field, with particular focus on the interventional procedures commonly performed in the paediatric population. © 2020

关键词： Abstract   Medical conference   Paediatrics   Publishing  

摘要： Background The proportion of abstracts presented at medical conferences that are subsequently published is a potentially useful surrogate for the quality of the material presented. The mean publication percentage for paediatric conference abstracts reported in the literature is 39%. The publication of abstracts presented at the Irish Paediatric Association's (IPA) annual conference have not previously been explored. Aim To identify the subsequent publication proportion for abstracts presented at the IPA annual conference and to identify factors associated with a higher likelihood of publication. Methods As 95% of publications occur within 5 years of conference presentation, abstracts from the 2008 to 2012 IPA conferences were selected for analysis. A PubMed/Medline search was conducted using the author's names and, if required, abstract keywords. For comparability with previous studies, articles were deemed published if they were full journal articles, contained at least one similar author and reported similar outcomes. Results Over the 5-year study period, 584 IPA abstracts were presented. The percentage of abstracts published was 19.7%. One hundred and fifteen articles were published in 45 different journals;31 (27%) of these were published in the Irish Medical Journal. The percentage of abstracts published was significantly higher for oral presentations (23% vs. 15%;p = 0.012), university-associated abstracts (31% vs. 16%;p < 0.001) and interventional studies (52% vs. 18%;p < 0.001). On multivariate analysis, only university association and interventional studies remained significantly associated with publication. Conclusion The percentage of IPA abstracts that were published was low when compared internationally. Further analysis is required to explore the reasons underpinning this.

关键词： Medulloblastoma   Radiotherapy   Adverse effects   Craniospinal   Irradiation  

摘要： Along with surgery, radiation therapy (RT) remains an essential option to cure patients suffering from medulloblastoma. However, its long-term adverse effects, particularly due to craniospinal irradiation (CSI), which is necessary to eradicate microscopic spread, are a limiting factor. The most frequent sequelae involve neurocognitive and endocrine impairment, which occurs in nearly all patients. Recent progress achieved through genetic and molecular biology offers the possibility to better stratify patients according to risk factors such as age, post-resection tumour residue and metastasis. Thus, new therapeutic studies assess the possibility to reduce radiation dose and/or radiation field size for patients with the most favourable prognosis. New radiotherapy techniques are also used such as Intensity-Modulated Radiotherapy (IMRT), tomotherapy and proton therapy, which aim at reducing the dose delivered to normal tissue. Conventional photon-based therapy has a relatively high exit dose in contrast with proton therapy which causes less damage to surrounding healthy tissue. It is noteworthy that each technique requires a long follow-up in order to prove that late effects could be reduced without compromising survival rates. Dosimetric comparison theoretically suggests that proton therapy may be the superior method for CSI in terms of late effects, but further research is needed to firmly establish this. Whatever the technique used, the great complexity of CSI requires discipline and expertise along with an external quality control online before the first RT session. (c) 2018 Elsevier Masson SAS. All rights reserved.

关键词： Paediatric   Adenotonsillectomy   Obstructive sleep apnoea   Epidemiology   Australia   New Zealand  

摘要： Aims: To review the contributions of Australian and New Zealand research on adenotonsillectomy for the treatment of symptoms of obstructed breathing during sleep (sleep disordered breathing, SDB) in children. Methods: A search of the scientific literature was conducted using the MEDLINE (Ovid), PubMed and Scopus databases in August 2020. The following search string was used: (tonsillectomy OR adenoidectomy OR adenotonsillectomy) AND (paediatric OR child) AND (Australia OR New Zealand). A focused internet search was additionally conducted on Google to identify grey literature. Results: Researchers from Australia and New Zealand have made important contributions to the understanding and improvement of adenotonsillectomy (AT), including its epidemiology, cost, surgical techniques and pen-operative safety. Rates of AT have fluctuated over the years, becoming the most common paediatric surgery today, with SDB becoming the most common indication. Research in Australia and New Zealand has also focussed on the impact of AT on quality of life, and behaviour, neurocognition and cardiovascular sequelae. Conclusions: Australian and New Zealand researchers have played a significant role in understanding the epidemiology and improving the safety of AT. There are promising directions in research still to come, including better understanding of the reasons for geographical variation in surgery rates, developing more efficient pre-operative risk assessment tools and alternative treatment options for mild OSA. (C) 2020 Elsevier B.V. All rights reserved.

关键词： griseofulvin   laboratory abnormalities   monitoring   Terbinafine   tinea capitis  

摘要： Griseofulvin and terbinafine are considered effective first-line therapies for tinea capitis (TC). Haematological dyscrasias and hepatic injury are possible adverse effects with both drugs. There is a debate in the literature regarding the necessity of laboratory monitoring during griseofulvin and terbinafine treatment. We aimed at assessing the prevalence and severity of haematological and hepatic laboratory test abnormalities in a paediatric cohort of African immigrants in Tel-Aviv with TC who were treated with Terbinafine or Griseofulvin. We conducted a retrospective study of all TC cases diagnosed and treated at the paediatric dermatology clinic, Tel-Aviv Medical centre, between June 2013 and March 2019. Epidemiologic, clinical and laboratory data were collected. Our cohort included 321 patients of whom 225 (70%) were treated with Griseofulvin and 96 (30%) with Terbinafine. We identified a total of 64 (20%) patients with haematological or hepatic laboratory test abnormalities that in most cases (96.3%) were considered as mild. No difference in laboratory abnormalities prevalence was identified between the griseofulvin and terbinafine groups (21.3% and 16.6%, respectively). Only one patient treated with Griseofulvin revealed significantly increased levels of hepatic aminotransferases that required discontinuation of treatment. Mild elevation in hepatic transaminases is relatively common among paediatric patients treated with systemic antifungal treatment for TC. However, significant laboratory abnormalities are extremely rare and may be diagnosed and addressed early through periodic laboratory tests monitoring.

关键词： IL‐   10   IL‐   10R   inflammatory bowel diseases   mucosal homeostasis   pathology   ulcerative colitis  

摘要： Interleukin-10 (IL-10) is a key anti-inflammatory cytokine. We aimed to assess IL-10 and IL-10 receptor (IL-10R) expression in the gut, and determine whether these patterns are altered in patients with ulcerative colitis (UC). Formalin-fixed paraffin-embedded rectal and transverse colon sections were collected from three groups of patients: (a) control subjects with normal colonoscopy and without history of inflammatory bowel disease;(b) UC patients with extensive colitis or pancolitis (E3/E4 phenotype);and (c) UC patients with limited distal disease (E1/E2 phenotype;n = 8-10 subjects per group). Immunohistochemistry (IHC) was performed to assess expression patterns of IL-10, IL-10R1 and IL-10R2, and was correlated with clinical, endoscopic and histologic severity indices among patients. A trend towards increased IL-10 expression was noted in rectal biopsies of patients with active UC, compared with controls. Moreover, IL-10 levels were significantly increased in transverse colon biopsies of patients with extensive/pancolitis, compared with control subjects and patients with limited distal disease. Rectal IL-10R1 and IL-10R2 levels were comparable between control subject and patients with active UC. However, transverse colon IL-10R1 levels were significantly higher in patients with E3/E4 colitis, compared with controls. Finally, we found no correlation between clinical, endoscopic and histologic severity of inflammation among UC patients and IL-10, IL-10R1 or IL-10R2 expression in rectal sections. Mucosal expression patterns of IL-10 and IL-10R, evaluated by IHC, were overall similar between control subjects and patients with active UC. Given IL-10's anti-inflammatory properties, additional studies are required to determine whether signalling through the IL-10R is altered among these patients.

摘要： Background Diagnostic uncertainty is the subjective perception of an inability to provide an accurate explanation of the patient's health problem or that a label is missing or incorrect. While recently explored in youth with chronic pain and families, this is the first study to investigate diagnostic uncertainty from the perspectives of physicians. Methods Individual, semi-structured interviews were conducted with 16 paediatricians who assess and/or treat youth who experience complex chronic pain. Interviews explored paediatricians' perceptions, beliefs and confidence regarding the assessment and management of chronic pain in youth and how they manage uncertainty regarding the diagnosis. Interviews were analysed using inductive reflexive thematic analysis. Results Analyses generated one prominent theme: 'drawing a line in the sand'. Within this theme, physicians discussed uncertainty as inherent to their role treating youth with chronic pain. The metaphor of 'drawing a line in the sand' was used to describe a process of identifying a point at which physicians no longer sought a new diagnosis for the child's pain or continued diagnostic investigations. This line was influenced by numerous factors, which are highlighted through four subthemes: physician training, experience and mentorship;individual patient and family factors;perceived reassurance of diagnostic investigations;and the broader social context and implications. Conclusions How physicians manage diagnostic uncertainty must be understood, as it is likely to critically impact how a diagnosis of chronic pain is communicated, the diagnostic investigations undertaken, the wait time to receiving a diagnosis, and ultimately youths' pain experiences.