教学课程资源库 更多>>

关键词： clinical ethics   continuity of care   paediatric palliative care   quality of care  

摘要： Aim To explore and compare acute and long-term care professionals' perspectives about paediatric palliative care. Methods Focus group interviews were conducted in 2016-2017 with professionals from acute (Emergency Department, Intensive Care Unit) and long-term care (Complex Care Service, Palliative Care) teams. Results Fifty-eight participants were enrolled. Palliative care definitions were similar throughout groups: to provide active care early in the illness, focusing on the child as a whole and supporting families. Each group perceived a different role in the patient's illness trajectory, reflecting their own culture of care. They demonstrated important differences in their approach to palliative care. Disagreements regarding when or how to discuss goals of care were expressed. Acute care professionals reported discomfort when having to introduce these discussions for the first time, while long-term care professionals perceived negative judgements about their patients' quality of life by acute care teams during health events. Personalised care, communication with families and continuity of care were thought to be key elements to improve care. Conclusion Paediatric palliative care is well recognised throughout specialties, yet continuity of care is challenged by groups' roles and interventions in a patient's illness. A reflective and mutual clinical approach is needed to improve quality of care and professionals' satisfaction.

关键词： child   meta-analysis   obstructive sleep apnoea   rapid maxillary expansion  

摘要： Background Obstructive sleep apnoea (OSA) affects many children, and adenotonsillar hypertrophy is the most common cause of paediatric OSA. Aim Despite the growing treatment options, there is no comprehensive comparison of all interventions. We aimed to compare and rank the effectiveness of various treatments in a network meta-analysis. Design Literature was searched from inception to 13 May 2018 for paediatric OSA with adenotonsillar hypertrophy. The outcomes were the changes in apnoea-hypopnea index (AHI), oxyhaemoglobin desaturation index (ODI), and lowest arterial oxygen saturation (SaO(2)). Frequentist approach to network meta-analysis was used. Treatment hierarchy was summarized according to the surfaces under the cumulative ranking curves. Results Fourteen trials comprising 1064 paediatric OSA participants evaluating ten interventions (adenotonsillectomy, adenotonsillectomy + pharyngoplasty, adenotonsillotomy, antimicrobial therapy, steroids, leukotriene receptor antagonists [LTRAs], steroids + LTRAs, rapid maxillary expansion [RME], placebo, and no treatment) were identified for network meta-analysis. In terms of effectiveness in AHI reduction, surgical approach was still the most effective intervention than no treatment. RME was one of the most effective interventions to improve lowest SaO(2). No comparisons showed statistical significance in reducing ODI. Conclusions Irrespective of the intervention used, complete resolution of OSA was not achieved in most trials.

关键词： competency   learning curve   oesophagogastroduodenoscopy   paediatric endoscopy   training  

摘要： Background: The learning curve in paediatric oesophagogastroduodenoscopy (OGD) is unknown. Using >= 95% D2 (second part of the duodenum) intubation rates as a marker of technical competency, we conducted learning curve analyses to identify when trainees achieve competency in paediatric OGD. Factors associated with competency were also evaluated. Methods: This nationwide study analysed data from paediatric OGD procedures prospectively entered into the UK endoscopy training e-portfolio between 2014 and 2018. Moving average and learning curve cumulative summation analyses were performed to determine procedural numbers required to achieve >= 95% D2 intubation rates. Factors associated with D2 intubation were assessed using a multivariable binary logistic regression approach. Results: A total of 8929 procedures performed by 61 trainees were identified. These 61 trainees had recorded a mean of 124.6 procedures (range 22-571, interquartile range 165). By moving average analysis, 95% D2 intubation was achieved after 79 procedures. By learning curve cumulative summation analysis, 81.6% of trainees were competent after 100 procedures. Multivariable factors associated with unassisted procedural completion included: lifetime procedure count (P < 0.001), higher trainee seniority (P < 0.001), patient age (P = 0.002), outpatient status (P < 0.001), and attendance at a national Basic Skills OGD course (P = 0.011). Conclusions: This study demonstrates that, on average, 79 procedures in paediatric OGD are required to attain the competency outcome of >= 95% D2 intubation rates. By 100 procedures, 81.6% of our sample had achieved >= 95% D2 intubation. The minimum procedural count of 100 set by the UK and international training programmes can be used alongside existing objective assessment measures to safeguard competency within a training cohort.

摘要： Click for the corresponding questions to this CME article.

摘要： Background Paedeatric Zika remains an understudied topic. WHO and the Pan American Health Organization (PAHO) Zika case definitions have not been assessed in children. We aimed to characterise clinical profiles and evaluate the diagnostic performance of the WHO and PAHO case definitions in a large cohort of paediatric Zika cases. Methods From January, 2016 to February, 2017, encompassing the major 2016 Zika epidemic, participants in the Pediatric Dengue Cohort Study (PDCS) in Managua, Nicaragua, were encouraged to visit the study health centre at first indication of any illness. PDCS participants were aged 2-14 years, healthy at enrolment, and recruited before the initiation of the present study. Molecular and serological assays were used to test participants exhibiting any of four broad clinical profiles suspected of resulting from a symptomatic Zika virus infection. These clinical profiles were: fever and at least two of headache, retro-orbital pain, myalgia, arthralgia, rash, haemorrhagic manifestations, and leukopenia;fever and at least two of nausea or vomiting, rash, aches and pains, positive tourniquet test, leukopenia, and any dengue warning sign;undifferentiated fever without evident cause, with or without any other clinical finding;and afebrile rash with or without any other clinical finding. We characterised acute clinical findings (signs, symptoms, and complete blood counts) in both Zika cases and non-Zika cases. Findings We prospectively followed a cohort of about 3700 children, of which 1110 were deemed eligible for inclusion. Four participants with laboratory-confirmed Zika (three co-infections with dengue virus, one missing complete blood count data) and two participants who were non-Zika cases (missing complete blood count data) were excluded from analysis. We analysed 556 laboratory-confirmed Zika and 548 non-Zika cases. The WHO case definition captured 176 confirmed Zika cases, and the PAHO definition 109 confirmed Zika cases, who presented with t

关键词： Desmoid tumour   beta-catenin   CTNNB1   Gardner syndrome   Medical therapy   Radiotherapy   Surgery   Patient advocacy groups   SPAEN  

摘要： Desmoid tumor (DT;other synonymously used terms: Desmoid-type fibromatosis, aggressive fibromatosis) is a rare and locally aggressive monoclonal, fibroblastic proliferation characterised by a variable and often unpredictable clinical course. Previously surgery was the standard primary treatment modality;however, in recent years a paradigm shift towards a more conservative management has been introduced and an effort to harmonise the strategy amongst clinicians has been made. We present herein an evidence-based, joint global consensus guideline approach to the management of this disease focussing on: molecular genetics, indications for an active treatment, and available systemic therapeutic options. This paper follows a one-day consensus meeting held in Milan, Italy, in June 2018 under the auspices of the European Reference Network for rare solid adult cancers, EURACAN, the EuropeanOrganisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group (STBSG) as well as Sarcoma Patients EuroNet (SPAEN) and The Desmoid tumour Research Foundation (DTRF). The meeting brought together over 50 adult and pediatric sarcoma experts from different disciplines, patients and patient advocates from Europe, North America and Japan. (C) 2019 The Authors. Published by Elsevier Ltd.

摘要： Paediatric palliative care (PPC) is an emerging specialty that aims at improving the quality of life for patients and their families. PPC is complex, and fundamentally different from adult palliative care in that it involves rare conditions, high-tech care, children in diverse developmental stages and complex proxy decision-making. [Extracted from the article]

关键词： brain tumour   cancer predisposition   constitutional mismatch repair   familial adenomatous polyposis   genetic syndrome   Gorlin syndrome   Li-Fraumeni syndrome   neoplasia   neurofibromatosis   rhabdoid tumours   tuberous sclerosis complex  

摘要： Cancer in childhood is a disorder of growth and development. Up to 10% of patients diagnosed with cancer during childhood have a known underlying genetic predisposition syndrome. Affected individuals usually have multisystem involvement from the underlying syndrome and certain syndromes are associated with development of characteristic tumours with sites of predilection within the neuraxis. For the healthcare professionals involved with paediatric patients it is important to have basic knowledge of the cancer susceptibility syndromes. A holistic multidisciplinary approach is required for the overall management of the syndrome itself with specific recommendations for imaging surveillance and genetic counselling based on the pattern of inheritance and the relative risk of developing a tumour. Appropriate knowledge of these syndromes will help paediatricians manage and refer patients at risk to specialist neuro-oncology centres. A typical brain tumour diagnosis can also indicate certain underlying genetic disorders and examples of such tumours include optic pathway glioma, choroid plexus carcinoma and subependymal giant cell astrocytoma. A detailed family history can be helpful in identifying at risk patients and families as the typical clinical signs associated with the genetic condition are often not fully apparent in young children. This article focuses on well-known genetic diagnoses associated with or predisposing to childhood brain tumours. In some instances, the brain tumour diagnosis subsequently leads to the diagnosis of an underlying genetic syndrome. © 2019 Elsevier Ltd

关键词： Nonunion   Paediatric femur   Infection   External fixator   Ilizarov  

摘要： Purpose Nonunion of femur fractures is a devastating disabling complication which is rare in children. The purpose of this study was to report the outcomes of treating infected femur nonunions in children by the Ilizarov fixator in one stage. Patients and methods The study included 13 patients with unilateral infected nonunion of the femur with an average age of 9.1 years. The nonunion duration averaged 10.69 months. Ten cases were draining nonunions, and three patients had quiescent sinuses. Associated problems include shortening in all cases (mean 3.5 cm), joint stiffness (9 cases), and angular deformity (7 cases). The quiescent cases were treated by bloodless monofocal compression-distraction. Four draining cases were treated by debridement and compression with relengthening through nonunion site. The remaining six cases were treated by bifocal technique. Results The mean follow-up duration was 60.15 months. External fixation period averaged 5.3 months. Successful union was achieved in all patients. Recurrences of infection occurred in two cases including one with refracture and another one with late pathological fracture. Other complications included pin tract infections, one delayed union, two residual angular deformities, and 6 cm residual shortening in one patient. ASAMI bone results were excellent (8 patients), good (3 patients), fair (one patient), and poor (one patient). The functional results were excellent (9 cases), good (3cases), and fair (one case). Conclusions The Ilizarov method provided a viable treatment option for treating paediatric infected femur nonunions in single stage of management with infection control in most cases and satisfactory outcomes.