Neuroblastoma (NBL) is the most common extra-cranial tumour in childhood. It can present as an abdominal mass, but is usually metastatic at diagnosis so the symptomatology can be varied. Nephroblastoma, also more commonly known as a Wilms tumour, is the commonest renal tumour in childhood and more typically presents as abdominal pathology with few constitutional symptoms, although rarely haematuria can be a presenting feature. The pathophysiology and clinical aspects of both tumours including associated risk factors and pathologies are discussed. Oncogenetics and chromosomal abnormalities are increasingly recognised as important prognostic indicators and their impact on initial management is considered. Imaging plays a pivotal role in terms of diagnosis and recent imaging advances mean that radiology has an increasingly crucial role in the management pathway. The use of image defined risk factors in neuroblastoma has begun to dramatically change how this tumour is characterised pre-operatively. The National Wilms Tumour Study Group have comprehensively staged Wilms tumours and this is reviewed as it impacts significantly on management. The use of contrast-enhanced MRI and diffusion-weighted sequences have further served to augment the information available to the clinical team during initial assessment of both neuroblastomas and Wilms tumours. The differences in management strategies are outlined. This paper therefore aims to provide a comprehensive update on these two common paediatric tumours with a particular emphasis on the current crucial role played by imaging.
Background Recently, two randomized controlled trials demonstrated the benefit of mechanical thrombectomy performed between 6 and 24 h in acute ischemic stroke. The current economic evidence is supporting the intervention only within 6 h, but extended thrombectomy treatment times may result in better long-term outcomes for a larger cohort of patients. Aims We compared the cost-utility of mechanical thrombectomy in addition to medical treatment versus medical treatment alone performed beyond 6 h from stroke onset in the UK National Health Service (NHS). Methods A cost-utility analysis of mechanical thrombectomy compared to medical treatment was performed using a Markov model that estimates expected costs and quality-adjusted life years (QALYs) over a 20-year time horizon. We present the results of three models using the data from the DEFUSE 3 and DAWN trials and evidence from published sources. Results Over a 20-year period, the incremental cost per QALY of mechanical thrombectomy was $1564 (£1219) when performed after 12 h from onset, $5253 (£4096) after 16 h and $3712 (£2894) after 24 h. The probabilistic sensitivity analysis demonstrated that thrombectomy had a 99.9% probability of being cost-effective at the minimum willingness to pay for a QALY commonly used in the UK. Conclusions The results of this study demonstrate that performing mechanical thrombectomy up to 24 h from acute ischemic stroke symptom onset is still cost-effective, suggesting that this intervention should be implemented by the NHS on the basis of improvement in quality of life as well as economic grounds.
Background Although some national recommendations for the role of radiology in a polytrauma service exist, there are no European guidelines to date. Additionally, for many interdisciplinary guidelines, radiology tends to be under-represented. These factors motivated the European Society of Emergency Radiology (ESER) to develop radiologically-centred polytrauma guidelines. Results Evidence-based decisions were made on 68 individual aspects of polytrauma imaging at two ESER consensus conferences. For severely injured patients, whole-body CT (WBCT) has been shown to significantly reduce mortality when compared to targeted, selective CT. However, this advantage must be balanced against the radiation risk of performing more WBCTs, especially in less severely injured patients. For this reason, we recommend a second lower dose WBCT protocol as an alternative in certain clinical scenarios. The ESER Guideline on Radiological Polytrauma Imaging and Service is published in two versions: a full version (download from the ESER homepage, https://www.eser-society.org) and a short version also covering all recommendations (this article). Conclusions Once a patient has been accurately classified as polytrauma, each institution should be able to choose from at least two WBCT protocols. One protocol should be optimised regarding time and precision, and is already used by most institutions (variant A). The second protocol should be dose reduced and used for clinically stable and oriented patients who nonetheless require a CT because the history suggests possible serious injury (variant B). Reading, interpretation and communication of the report should be structured clinically following the ABCDE format, i.e. diagnose first what kills first.
Prader-Willi syndrome (PWS) is the most common genetic obesity syndrome, with associated learning difficulties, neuroendocrine deficits, and behavioural and psychiatric problems. As the life expectancy of individuals with PWS increases, there is concern that alterations in brain structure associated with the syndrome, as a direct result of absent expression of PWS genes, and its metabolic complications and hormonal deficits, might cause early onset of physiological and brain aging.In this study, a machine learning approach was used to predict brain age based on grey matter (GM) and white matter (WM) maps derived from structural neuroimaging data using T1-weighted magnetic resonance imaging (MRI) scans. Brain-predicted age difference (brain-PAD) scores, calculated as the difference between chronological age and brain-predicted age, are designed to reflect deviations from healthy brain aging, with higher brain-PAD scores indicating premature aging.Two separate adult cohorts underwent brain-predicted age calculation. The main cohort consisted of adults with PWS (n = 20; age mean 23.1 years, range 19.8–27.7; 70.0% male; body mass index (BMI) mean 30.1 kg/m2, 21.5–47.7; n = 19 paternal chromosome 15q11–13 deletion) and age- and sex-matched controls (n = 40; age 22.9 years, 19.6–29.0; 65.0% male; BMI 24.1 kg/m2, 19.2–34.2) adults (BMI PWS vs. control P = .002). Brain-PAD was significantly greater in PWS than controls (effect size mean ± SEM +7.24 ± 2.20 years [95% CI 2.83, 11.63], P = .002). Brain-PAD remained significantly greater in PWS than controls when restricting analysis to a sub-cohort matched for BMI consisting of n = 15 with PWS with BMI range 21.5–33.7 kg/m2, and n = 29 controls with BMI 21.7–34.2 kg/m2 (effect size +5.51 ± 2.56 years [95% CI 3.44, 10.38], P = .037). In the PWS group, brain-PAD scores were not associated with intelligence quotient (IQ), use of hormonal and psychotropic medications, nor severity of repetitive or disruptive behaviours. A 24.5 year old man (BMI 36.9 kg/m2) with PWS from a SNORD116 microdeletion also had increased brain PAD of 12.87 years, compared to 0.84 ± 6.52 years in a second control adult cohort (n = 95; age mean 34.0 years, range 19.9–55.5; 38.9% male; BMI 28.7 kg/m2, 19.1–43.1).This increase in brain-PAD in adults with PWS indicates abnormal brain structure that may reflect premature brain aging or abnormal brain development. The similar finding in a rare patient with a SNORD116 microdeletion implicates a potential causative role for this PWS region gene cluster in the structural brain abnormalities associated primarily with the syndrome and/or its complications. Further longitudinal neuroimaging studies are needed to clarify the natural history of this increase in brain age in PWS, its relationship with obesity, and whether similar findings are seen in those with PWS from maternal uniparental disomy.
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