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Integrating molecular analyses with the 2021 WHO classification of adult pilocytic astrocytomas.
No full textPilocytic astrocytomas (PAs) are benign grade 1 gliomas according to the World Health Organization (WHO). They are common in children but rare in adults in whom they may have a worse prognosis. Pediatric PAs are usually associated with dysregulation of the mitogen-activated protein kinase (MAPK) pathway, often involving BRAF alterations such as the KIAA1549::BRAF (K-B) fusion or V600E mutation. We investigated the molecular characteristics of adult PA using gene-targeted next-generation sequencing and specific gene tests, including for K-B fusion, TERT promoter, and FGFR1 hotspot mutations. The most frequent molecular alterations detected involved the MAPK pathway, particularly affecting BRAF and NF1 genes (55%). The prevalence of the K-B fusion (>40%) was higher than previously reported, likely due to challenges in detecting it. We identified molecular alterations in some cases that raised the differential diagnosis of other tumor types, revealing limitations in the 2021 WHO classification for adult PA. After removing other diagnostic types that may mimic PA histology, no adult patients with a diagnosis of PA and K-B fusion died after more than 10 years of mean follow-up. These findings suggest that, similar to pediatric cases, PA in adults may be driven by a single molecular hit, where the K-B fusion is not related to poor outcome
POEM from A to Z: current perspectives.
No full text20.500.12530/87908The "third space endoscopy" or also called "submucosal endoscopy" is a reality we can transfer to our patients since 2010. Various modifications of the submucosal tunneling technique allow access to the submucosa or deeper layers of the gastrointestinal tract. In addition to peroral endoscopic myotomy for the treatment of achalasia, also called esophageal POEM, other variants have emerged that make it possible to treat different esophageal motility disorders, esophageal diverticula, subepithelial tumors of various locations, gastroparesis, reconnection of complete esophageal strictures or even thanks to exceptional endoscopists, pediatric disorders such as Hirschsprung's disease. Although some technical aspects are yet to be standardized, these procedures are becoming widespread worldwide and will likely become the standard treatment of these pathologies soon
Association between hypertension and selfperception of health status: Findings from a decade population-based survey in Spanish adults
No full textObjective
This study, conducted in the community setting, aimed to assess and discuss how a diagnosis of arterial hypertension affects self-perceived health status, examining the association with potential explanatory factors and comparing its impact with that of other chronic conditions.
Methods
Cross-sectional observational study using the 2011–2012 and 2017 Spanish National Health Surveys and the 2020 European Health Interview Survey for Spain as data sources. Health perception was categorised as very good, good, fair, bad, or very bad. The independent variables recorded demographic, social, clinical, and lifestyle information. The associations between variables were evaluated via a generalisation of an ordered logit model.
Results
A total of 66,168 subjects were included (21,007 in 2011, 23,089 in 2017, and 22,072 in 2020), 21.6% of whom were diagnosed with hypertension, 51.3% were women, and the average age was 48.24 (18.89) years. Around one in five people in the general population reported a “very good” health status. The probability of reporting a “very good” health condition decreased with a diagnosis of hypertension (6.2%; CI 95%: 3.1–9.3%) and hypertensive medication (4.5%; CI 95%: 1.8–7.3%). Such associations were independent of age, gender, social group, other chronic conditions or limitations, or various lifestyle habits. In contrast, no association was found with reporting a “bad” or “very bad” health status.
Conclusion
Being diagnosed with hypertension and prescription of antihypertensive medication are associated with a lower probability of reporting a “very good” health status, irrespective of other comorbidities or complications related to the diagnosis.S
Cost-effectiveness of endovascular thrombectomy for acute ischemic stroke with established large infarct in Germany: a decision tree and Markov model.
No full text20.500.12530/87913Recent studies, including the TENSION trial, support the use of endovascular thrombectomy (EVT) in acute ischemic stroke with large infarct (Alberta Stroke Program Early Computed Tomography Score (ASPECTS) 3-5). To evaluate the cost-effectiveness of EVT compared with best medical care (BMC) alone in this population from a German healthcare payer perspective. A short-term decision tree and a long-term Markov model (lifetime horizon) were used to compare healthcare costs and quality-adjusted life years (QALYs) between EVT and BMC. The effectiveness of EVT was reflected by the 90-day modified Rankin Scale (mRS) outcome from the TENSION trial. QALYs were based on published mRS-specific health utilities (EQ-5D-3L indices). Long-term healthcare costs were calculated based on insurance data. Costs (reported in 2022 euros) and QALYs were discounted by 3% annually. Cost-effectiveness was assessed using incremental cost-effectiveness ratios (ICERs). Deterministic and probabilistic sensitivity analyses were performed to account for parameter uncertainties. Compared with BMC, EVT yielded higher lifetime incremental costs (€24 257) and effects (1.41 QALYs), resulting in an ICER of €17 158/QALY. The results were robust to parameter variation in sensitivity analyses (eg, 95% probability of cost-effectiveness was achieved at a willingness to pay of >€22 000/QALY). Subgroup analyses indicated that EVT was cost-effective for all ASPECTS subgroups. EVT for acute ischemic stroke with established large infarct is likely to be cost-effective compared with BMC, assuming that an additional investment of €17 158/QALY is deemed acceptable by the healthcare payer
Upadacitinib for Induction of Remission in Pediatric Ulcerative Colitis: An International Multicenter Study.
No full text20.500.12530/87892Data on upadacitinib therapy in children with ulcerative colitis (UC) or unclassified inflammatory bowel disease (IBD-U) are scarce. We aimed to evaluate the effectiveness and safety of upadacitinib as an induction therapy in pediatric UC or IBD-U. In this multicenter retrospective study, children treated with upadacitinib for induction of remission of active UC or IBD-U from 30 centers worldwide were enrolled. Demographic, clinical, and laboratory data, as well as adverse events (AEs), were recorded at Week 8 post-induction. One hundred children were included (90 UC and 10 IBD-U, median age 15.6 [interquartile range 13.3-17.1] years). Ninety-eight were previously treated with biologic therapies, and 76 were treated with ≥2 biologics. At the end of the 8-week induction period, clinical response, clinical remission, and corticosteroid-free clinical remission (CFR) were observed in 84%, 62%, and 56% of the children, respectively. Normal C-reactive protein and fecal calprotectin (FC) Upadacitinib is an effective induction therapy for refractory pediatric UC and IBD-U. Efficacy should be weighed against the potential risks of AEs
The Natural History of Patients With Pre-Existing and De Novo Inflammatory Bowel Disease After Solid Organ Transplantation: EITOS Study of GETECCU.
No full text20.500.12530/87852Limited data are available on the outcome of inflammatory bowel disease (IBD) in patients with solid organ transplantation (SOT). We describe the natural history of pre-existing IBD and de novo IBD after SOT. This was a retrospective, multicenter study that included patients with pre-existing IBD at the time of SOT and patients with de novo IBD after SOT. The primary outcome was IBD progression, defined by escalation of medical treatment, surgical therapy, or hospitalization due to refractory IBD. Risk factors were identified using multivariate Cox proportional hazard analysis. A total of 177 patients (106 pre-existing IBD and 71 de novo IBD) were included. Most patients with pre-existing IBD (92.5%) were in remission before SOT. During follow-up, 32% of patients with pre-existing IBD had disease progression, with a median time between SOT and IBD progression of 2.2 (interquartile range, 1.3-4.6) years. In the de novo cohort, 55% of patients had disease progression with a median time to flare of 1.9 (interquartile range, 0.8-3.9) years after diagnosis. In the pre-existing IBD cohort, active IBD at the time of SOT (hazard ratio, 1.80; 95% confidence interval, 1.14-2.84; P = .012) and the presence of extraintestinal manifestations (hazard ratio, 3.10; 95% confidence interval, 1.47-6.54; P = .003) were predictive factors for IBD progression. One-third of patients with pre-existing IBD and about half of patients with de novo IBD have disease progression after SOT. Active IBD at the time of SOT and the presence of extraintestinal manifestations were identified as risk factors for IBD progression
The real-world use and effectiveness of avacopan in routine practice for the treatment of ANCA vasculitis. First experiences in Spain.
No full text20.500.12530/87849ANCA-associated vasculitis (AAV) is a group of chronic diseases with relapses that associate organic damage because of the disease and its treatment. Avacopan is a new treatment indicated for AAV. We present the first experiences with avacopan in Spain as part of an Early Access program. Patients with AAV who started avacopan between June 2022 and September 2023 were included. For comparison, a historical cohort of patients diagnosed with AAV around the same time and treated without avacopan was also included. Twenty-nine patients treated with avacopan were analysed. Twelve patients (41.4%) were male, and median age was 56 years. Most patients were ANCA MPO positive (21/29, 72.4%). The most frequently affected organ was the kidney (23/29, 79.31%), with a mean estimated glomerular filtration rate (eGFR) of 23.2 ml/min. Mean follow-up was 456.8 (±181.7) days with a remission rate of 86.2%. eGFR increased from 23.2 (11.2) to 38.38 (18.55) ml/min after 12 months of diagnosis. Two patients had adverse events related to avacopan (severe neutropenia and a gastrointestinal affectation), 13 infections were reported and one death. Patients treated with avacopan received a significantly lower cumulative dose of prednisone at 6 and 12 months (P-values of 0.02 and The combination of avacopan with standard immunosuppressive therapy presents a good safety profile and provides added value by contributing to the control of AAV activity, increase GFR and removal of steroids
Three-year survival follow-up of patients with gastrointestinal cancer treated during the COVID-19 pandemic in Spain: data from the PANDORA-TTD20 study.
No full text20.500.12530/87858The initial SARS-CoV-2 pandemic wave in Spain in 2020 precipitated significant paradigm shifts in gastrointestinal oncology patient management. This study captures the "Zeitgeist" of this period by analyzing adaptive strategies, treatment modifications, and survival outcomes, leveraging a 3-year follow-up perspective to extract insights from this unprecedented experience. We conducted a multicenter, retrospective cohort study utilizing the RETUD-TTD registry, encompassing 703 patients across 19 Spanish centers in April 2020. We evaluated alterations in clinical practice, therapeutic approaches, coronavirus disease 2019 (COVID-19)-related impacts, and patient survival. A Bayesian hierarchical model was employed to identify potential regional-specific frailties. The peak of the pandemic in April 2020 catalyzed substantial shifts in oncological care delivery. Outpatient consultations decreased by 13%, with a notable selection bias toward cases with more favorable prognostic indicators. Multidisciplinary tumor board discussions were significantly curtailed (eg, mean monthly colorectal cancer cases discussed was reduced from 40 to 23), compromising qualitative care measures. This occurred concurrently with an average of over 3 oncologists per center on medical leave. Contrary to initial concerns, the healthcare system demonstrated remarkable resilience. The majority of patients received standard-of-care therapies with regulatory approval, albeit with regimen modifications in 15% of cases. These adaptations included extended dosing intervals, dose intensity modulations, and transitions to oral formulations while maintaining unexpectedly stable long-term survival outcomes. The Bayesian frailty model detected minimal unmeasured prognostic factors related to geographic location, and the type of pandemic-induced adaptation did not significantly impact survival. The model revealed that coronavirus disease 2019's impact was less pronounced than other core prognostic variables. The decentralized Spanish healthcare system exhibited substantial robustness in managing pre-pandemic diagnosed gastrointestinal malignancies, despite asymmetrical, and occasionally severe organizational disruptions. The insights gleaned from this experience could inform future crisis preparedness strategies and optimize care provision during subsequent public health emergencies
Evaluating the Benefit of Home Support Provider Services for Positive Airway Pressure Therapy in Patients With Obstructive Sleep Apnea: Protocol for an Ambispective International Real-World Study.
No full text20.500.12530/87908Adherence and persistence to positive airway pressure (PAP) therapy are key factors for positive health outcomes. Home support providers participate in the home implementation and follow-up of PAP therapy for patients with obstructive sleep apnea (OSA). In Europe, home support provider service levels are country (or area) specific, resulting in differences in content and frequency of patient interactions. However, no robust evaluation of the impact of these differences on clinical and patient outcomes has been performed. The AWAIR study aims to evaluate and compare the impact of different home support provider service levels on PAP adherence and persistence in 4 European countries. This real-world, ambispective, cohort study-conducted in France, Belgium, Spain, and Portugal-will recruit adults with OSA who started PAP therapy between 2019 and 2023 and were followed by an Air Liquide Healthcare home support provider. Given the large number of eligible participants (around 150,000), the study will use a decentralized and digital approach. A patient video will present the study objectives and the participation process. A secure electronic solution will be used to manage patient information and consent, as well as to administer a web-based questionnaire. Retrospective data, collected during routine patient follow-up by home support providers, include the level of service and device data, notably PAP use. Prospective data collected using an electronic patient-reported outcome tool include health status, OSA-related factors, patient-reported outcomes including quality of life and symptoms, OSA and PAP literacy, patient-reported experience, and satisfaction with PAP therapy and service. Hierarchical models, adjusted for preidentified confounding factors, will be used to assess the net effect of home support provider services on PAP adherence and persistence while minimizing real-world study biases and considering the influence of country-level contextual factors. We hypothesize that higher levels of home support provider services will be positively associated with adherence and persistence to PAP therapy. As of December 2024, the study has received approval in France, Portugal, and 2 regions of Spain. The study began enrollment in France in October 2024. Results are expected in the second quarter of 2025. The AWAIR study has a unique design, leveraging an unprecedented number of eligible participants, decentralized technologies, and a real-world comparative methodology across multiple countries. This approach will highlight intercountry differences in terms of patient characteristics, PAP adherence, and persistence, as well as patient-reported outcomes, patient-reported experiences, and satisfaction with the home service provider. By assessing the added value of home support provider services, the results will support best practices for patient management and for decision-making by payers and authorities. PRR1-10.2196/65840
Assessment of the diagnostic utility of the electroencephalogram in pediatric emergencies
No full text20.500.12530/87871Introduction: We analyze the diagnostic utility of urgent EEG (electroencephalogram) performed in children under 16 years of age in our center. Material and methods: Descriptive, retrospective, observational study of consecutive patients from 0 to 16 years of age, who underwent an urgent EEG for any reason, from January to December 2022. Results: Of the 388 patients, 70 were children: 37 (52.85%) women, and 33 (47.14%) men. Average age: 6.27 +/- 4.809. Of the 70 patients, 6 (8.57%) had previous epilepsy. Reasons for consultation: 17 febrile seizures, 10 first focal seizures, 10 first TCG seizures, 6 paroxysmal episodes, 6 absences, 3 myoclonus of extremities, 3 syncope, 2 SE, 2 visual alterations, 2 low level of consciousness, 2 cyanosis, 2 suspected meningitis or encephalitis, 1 choking, 1 atypical headache, 1 chorea, 1 presyncope, 1 language delay. Of the 70 patients, 47 had a normal EEG (67.14%). Of the 47 patients with a normal EEG, 10 were diagnosed with epilepsy, and 3 of them began receiving antiepileptic treatment upon discharge. None of the patients with suspected syncope or paroxysmal disorder (17 patients, 24.28%) had EEG abnormalities. Of the 17 patients with atypical febrile seizures, 3 had EEG abnormalities. Conclusions: A third of the EEG records performed in the Emergency Department showed alterations, probably due to the time taken. Almost half of the patients with suspected epilepsy or EE showed EEG abnormalities, which confirmed the diagnosis in these cases and encouraged the clinician to start drug treatment. No case with a high suspicion of epilepsy was dismissed due to the normality of the EEG recording in our series. No patient diagnosed with syncope or paroxysmal disorder had EEG abnormalities. Nearly a quarter of patients with atypical febrile seizures showed EEG abnormalities. We barely register cases of status epilepticus, probably due to the degree of complexity of our center. (c) 2024 Sociedad Espanola de Neurolog& imath;a. Published by Elsevier Espana, S.L.U. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/ 4.0/)