Hospital Universitari i Politècnic La Fe

Pancreatic adenocarcinoma (PDAC) is characterized by its poor prognosis. Cytology is essential for diagnostic confirmation and ancillary studies. The endoscopic ultrasound (EUS) set a precedent, facilitating cytological sampling through fine-needle aspiration (FNA), with a sensitivity of 93% and a specificity of 95%. In this work, we analyzed all EUS-guided pancreatic FNAs performed at our center over 2014-2025 and compared the cytological findings with the corresponding surgical specimen results. Observational, descriptive, and retrospective study of all EUS-guided pancreatic FNAs performed at our center (2014-January 2025) in patients ≥ 18 years and their corresponding surgical specimens. Samples were obtained using a linear echoendoscope and 25G needles, processed with Romanowsky and Papanicolaou stains, and cell blocks prepared for immunohistochemistry. Cytological diagnoses followed WHO criteria; histology from surgical specimens served as the reference standard. Statistical analysis was performed using SPSS v.22 with p < 0.05 considered significant. One hundred and sixty-three EUS-guided pancreatic FNAs were performed and 34 patients underwent surgical treatment (20.8%), 31 of them (91.2%) with a cytological diagnosis of malignancy. Thirty-three patients had malignant lesions (97%) in the surgical specimen. Cytology showed a sensitivity of 96.9%, a specificity of 100%, a positive predictive value of 100%, and a negative predictive value of 50%. Cyto-histological correlation was 91.2% with a κ = 0.654 (p < 0.001). Cytopathologists' expertise was statistically significant (p < 0.001). Cytology is highly reliable for confirming malignancy. Final management will be multidisciplinary.

Sustained seizure freedom without intolerable adverse events is the main goal of epilepsy treatment, but may be challenging to achieve for some patients, particularly following the failure of multiple anti-seizure medications (ASMs). A majority of days without seizures is another important clinical outcome that can lead to meaningful improvement in quality of life. The objective of this post-hoc analysis was to evaluate the percentage of seizure-free days by responder groups during the open-label extension (OLE) of study C017 with a focus on patients who achieved near-seizure freedom that was sustained during the duration of the study (≥ 90%-< 100% responders group). The study also evaluated retention rates and the impact of concomitant antiseizure medications. Study C017 (NCT01866111) was a double-blind, randomized, placebo-controlled, dose-response trial evaluating adults 18-70years old who were taking 1-3 concomitant ASMs. Participants completed the 18-week double-blind treatment period and entered the OLE. This post-hoc analysis quantified the proportion of seizure-free days by responder category and by number and type of concomitant ASMs. The occurrence and severity of treatment-emergent adverse events during the OLE were also reported. Sixty (16.9%) of the 354 study participants were≥90%-< 100% responders (median age 42.5years, 46.7% [n=28] female, median number of concomitant ASMs at baseline was 2). The study population also included 91 (25.7%) patients who were≥50%-< 75% responders, 62 (17.5%) patients who were≥75%-< 90% responders, and 13 (3.7%) patients who were 100% responders. The median duration of cenobamate exposure was 5.6years for the≥90%-< 100% responders; 1.8years for the non-responders; 5.5years for the≥50%-< 75% responders; 5.5years for the≥75%-< 90% responders; and 5.6years for the 100% responders. The percentage of seizure-free days during the entire OLE was 97.9% in the≥90%-< 100% responder group, compared to 93.5% in the≥75%-< 90% group and 83.9% in the≥50%-< 75% group. The number and mechanism of action of concomitant ASMs did not meaningfully impact the proportion of days without seizures. The percentage of days with seizures in the≥90%-< 100% responder group declined from 33.2% at baseline to 1.2% at Year 5. At Year 5, the≥90%-< 100% responder group had a high retention rate (90%). The most commonly occurring adverse events were dizziness, somnolence, and headache. Participants taking cenobamate who achieved≥90%-< 100% responder rates were seizure-free for nearly 98% of days during the 5years of the OLE. These results suggest that a high reduction of seizure burden can be achieved and maintained by many people with epilepsy taking cenobamate.

CHRONOS is a multicenter, retrospective, cohort study involving acute graft-versus-host disease (aGvHD) adult patients with gastrointestinal (GI) symptoms, steroid- and ruxolitinib refractory, who initiated third-line therapy between 30 May 2019 and 30 September 2024. Primary endpoints were all-organ overall response rate (ORR) and GI-specific ORR (GI-ORR) around 28 days after treatment initiation. Secondary endpoints included duration of response, real-world progression-free survival (rwPFS) of underlying malignancy, and overall survival (OS). Fifty-nine patients from 16 sites in Europe were included. On Day 28, ORR was 36% (95% CI: 24-49%), GI-ORR was 37% (95% CI: 25-51%); 29% (95% CI: 11-49%) of responders lost response within 30 days, and 52% (95% CI: 29-72%) within 90 days. Median rwPFS and median OS were both 86 days (95% CI: 54-128 days). Median OS was higher in responders than in non-responders (186 versus 45 days). Over the 12-month follow-up period, 41 patients died, mainly due to aGvHD progression (n = 25), and infectious complications (n = 9). Within 3 months, Grade ≥ 2 infectious events occurred in 51% of patients; Grade 3-4 thrombocytopenia and neutropenia in 64% and 32%, respectively. These findings demonstrate limited effectiveness of third-line therapy in this cohort of steroid- and ruxolitinib-refractory aGvHD patients with GI symptoms.

In this retrospective single-center study, 109 patients with CTEPH underwent DECT, CBCT, and DSA within a 3-month interval. A standardized vascular obstruction score was applied independently to each modality. Linear regression models were constructed to assess associations with mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance (PVR), cardiac output (CO), and cardiac index (CI), quantified by adjusted R². Score distributions were compared using Friedman and Wilcoxon tests, and interobserver agreement was assessed with Cohen's κ. DSA demonstrated the highest degree of association with mPAP and PVR among the evaluated modalities (adjusted R² = 0.089 and 0.126), followed by DECT (0.075 and 0.098) and CBCT (0.050 and 0.062). DSA also correlated with CO and CI. Mean obstruction scores differed significantly across modalities (p < 0.001), with DECT yielding higher values than CBCT (p < 0.001) and DSA (p < 0.001). Interobserver agreement was highest for CBCT (κ = 0.76) and DECT (κ = 0.74), and lowest for DSA (κ = 0.57). None of the modalities correlated significantly with NYHA class or 6MWD. A unified morphologic vascular obstruction score applied across DECT, CBCT, and DSA demonstrates reproducible associations with invasive hemodynamic parameters in CTEPH. While not a replacement for right heart catheterization, it provides a standardized framework for multimodality assessment and may support methodological integration across imaging modalities. This study presents a systematic application of a unified vascular obstruction scoring system across dual-energy CT, cone-beam CT, and digital subtraction angiography in patients with chronic thromboembolic pulmonary hypertension. The results demonstrate significant correlations with invasive hemodynamic parameters, with dual-energy CT and cone-beam CT providing higher reproducibility than angiography. These findings support the use of a standardized scoring framework to enable consistent multimodality assessment, improve reproducibility in structured multimodality imaging assessment, and facilitate cross-institutional comparisons in chronic thromboembolic pulmonary hypertension.

Ionizing radiation (IR) exposure poses a significant biomedical challenge in clinical, occupational, and emergency contexts, highlighting the urgent need for effective medical countermeasures against acute radiation syndrome (ARS) and delayed effects of radiation exposure (DEARE). Depending on the timing of administration, radiation countermeasures are classified as radioprotectors, radiomitigators, or therapeutics. Among these, radiomitigators offer a critical advantage by attenuating IR-induced damage when administered after exposure, thereby expanding their applicability in unanticipated radiation incidents. This review provides an overview of the pathophysiological mechanisms underlying IR-induced injury and summarizes the current FDA-approved radiation countermeasures. It then focuses on radiomitigators that have demonstrated efficacy in preclinical animal models, together with available evidence from clinical studies, emphasizing their translational potential for both emergency preparedness and oncological settings. We examine routes of administration and key mechanisms of action, including modulation of oxidative and nitrosative stress, enhancement of DNA damage response pathways, preservation of mitochondrial function, regulation of inflammatory and immune signaling, attenuation of fibrotic remodeling, maintenance of vascular integrity, and promotion of tissue regeneration and repair. Finally, challenges associated with clinical translation and strategies to optimize radiomitigators for the management of radiation-induced injury are discussed. By integrating these insights and consolidating existing knowledge, this review aims to guide basic and clinical research toward more effective radiomitigative strategies and combination therapies to improve survival, limit tissue damage, and preserve long-term quality of life in individuals exposed to IR.

Nusinersen was the first disease modifying treatment approved for 5q spinal muscular atrophy (SMA). Long-term results of broad populations, particularly for adolescents and adults, remain limited. We conducted a population-based, ambispective observational study of all SMA patients living in the Valencian Community (Spain) between September 2017 and December 2022 and follow-up until December 2025. Demographic, clinical and motor outcomes using revised SMA Functional Composite Score (SMA-FCR) were collected. Patients were classified as responders or non-responders. The risk for nusinersen discontinuation was assessed with a Bayesian model, and SMA-FCR trajectories with mixed linear regression. Of 72 patients included, 18 were &lt;12 years old (all treated with nusinersen) and 54 were ≥12 years (28 treated; 26 untreated) at the baseline visit. After a median of 7 years, all patients &lt;12 years were classified as responders versus 68% of patients ≥12 years. Discontinuation rates were 11% in children compared to75% in the older cohort. In patients ≥12 years, reasons for discontinuation included: treatment burden (71%), and loss(53%) or lack of benefit (43 %). Lower baseline SMA-FCR (expEstimate= 0.84 [0.718,0.93], prob:1) and older age (expEstimate=1.028 [1.011,1.055], prob:1) independently predicted higher discontinuation risk. Sustained nusinersen treatment was independently associated with SMA-FCR increase, while untreated and discontinued patients showed slight deterioration over time. In this long-term population-based study, nusinersen use and persistence was high in children but declined significantly after age 12 due to treatment burden and limited efficacy. However, a proportion of adolescents and adults (those younger and with higher baseline function) experienced sustained benefit.

Periprosthetic joint infection (PJI) is a devastating complication of total hip arthroplasty (THA) that has major clinical and financial implications. Despite CDCR being the accepted gold standard for chronic PJI, serious complications, such as extensive bone loss and protracted rehabilitation, have led to the investigation of PTCR as an alternative treatment strategy for chronic PJI. According to the PICOS framework, a systematic review and meta-analysis were performed to assess the clinical efficacy of partial 2-stage exchange in chronic PJI with a stable femoral component. There were no time or language limits during the search for relevant articles in PubMed, Embase, Scopus, and Cochrane Library databases. Data extraction and quality assessment were performed according to the MINORS criteria by 2 independent reviewers. Statistical analysis, including heterogeneity and sensitivity analyses, was performed using R Studio software with the appropriate meta-analytical packages. 13 studies, including 239 patients, were analysed. When the data were pooled, MDCT showed an 88% rate of complete eradication with homogeneity (I² = 0%). Secondary traits included 12% re-operations, 8% deaths, and 1% fractures. Infectious and systemic complications were observed in 13% and 3% of patients, respectively. Implant stability was present in 89% of cases, with significant functional improvement, with a mean Harris Hip Score of 37.77 at the final follow-up. Treatment failure, defined as infection recurrence or the need for chronic antibiotic suppression, was observed in 14% of cases, and sensitivity analysis excluding zero-event studies showed a 17% failure rate. Partial 2-stage exchange results in favorable eradication of infection, may reduce bone stock loss, and optimises functional outcomes. However, concerns remain regarding its efficacy against resistant strains and proper patient selection criteria. Well-designed comparative studies with longer follow-up periods are needed to establish treatment algorithms and confirm long-term clinical results.PROSPERO registration number CRD42024599183.