Recomendaciones para el diagnóstico y tratamiento del adenocarcinoma ductal de páncreas

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Eculizumab in paroxysmal nocturnal hemoglobinuria with Budd-Chiari syndrome progressing despite anticoagulation

Abstract Paroxysmal nocturnal hemoglobinuria (PNH) is a progressive, life-threatening disorder characterized by chronic intravascular hemolysis caused by uncontrolled complement activation. Hepatic vein thrombosis (Budd-Chiari syndrome) is common in PNH patients. This case report describes the response to eculizumab (a humanized monoclonal antibody that inhibits terminal complement activation) in a 25-year-old male with progressive liver function deterioration despite standard anticoagulation therapy and transjugular intrahepatic porto-systemic shunt. The patient presented with anemia, severe thrombocytopenia, headache, abdominal pain, and distention. He was diagnosed with PNH, cerebral vein thrombosis, and Budd-Chiari syndrome. Despite adequate anticoagulation, diuretic administration, and placement of a transjugular shunt, additional thrombotic events and progressive liver damage were observed. Eculizumab therapy was initiated, resulting in rapid blockade of intravascular hemolysis, increased platelet counts, ascites resolution, and liver function recovery, all of which are presently sustained. Since starting eculizumab the patient has had no further thrombotic events and his quality of life has dramatically improved. This is the first report to confirm the role of complement-mediated injury in the progression of Budd-Chiari syndrome in a patient with PNH. This case shows that terminal complement blockade with eculizumab can reverse progressive thromboses and hepatic failure that is unresponsive to anticoagulation therapy and suggests that early initiation of eculizumab should be included in the therapeutic regimen of patients with PNH-related Budd-Chiari syndrome.</p

Rol de la TC multicorte en las hernias diafragmáticas: Ensayo iconográfico Role of Multislice Computed Tomography in the evaluation of diaphragmatic hernias: Pictorial essay

Las hernias diafragmáticas consisten en la migración de estructuras abdominales hacia el tórax a través de un defecto del diafragma. Ellas pueden tener origen congénito (de Morgagni y de Bochdalek) o adquirido, incluyendo las traumáticas o no traumáticas (del hiato, defectos diafragmáticos posteriores). Debido a que en algunas hernias diafragmáticas está indicada la reparación quirúrgica, los métodos de diagnóstico por imágenes cumplen un rol fundamental. La TC multicorte, con su capacidad multiplanar y posibilidad de efectuar cortes finos, nos permite valorar y caracterizar adecuadamente el defecto diafragmático y sus complicaciones. En este ensayo iconográfico realizamos un breve repaso de la embriología y anatomía del diafragma, revisamos los distintos tipos de hernias diafragmáticas y la utilidad de la TC multicorte.<br>Diaphragmatic hernias are characterized by the migration of abdominal structures into the chest through a diaphragmatic defect. These may have either a congenital etiology (e.g., Morgagni and Bochdalek), or an acquired etiology, including traumatic and nontraumatic hernias (hiatal, posterior diaphragmatic defects). Since a surgical repair is indicated in certain types of hernias, imaging diagnostic methods play a key role. Multislice Computed Tomography (MSCT) allows multiplanar views and thin section evaluation, thus providing a useful tool for the assessment and characterization of the diaphragmatic defect and its complications. In this pictorial essay we briefly review the diaphragm anatomy and embryology, the different types of diaphragmatic hernias and the role of MSCT

Global Variation in Magnetic Resonance Imaging Quality of the Prostate

Background High variability in prostate MRI quality might reduce accuracy in prostate cancer detection. Purpose To prospectively evaluate the quality of MRI scanners taking part in the quality control phase of the global PRIME (Prostate Imaging Using MRI ± Contrast Enhancement) trial using the Prostate Imaging Quality (PI-QUAL) standardized scoring system, give recommendations on how to improve the MRI protocols, and establish whether MRI quality could be improved by these recommendations. Materials and Methods In the prospective clinical trial (PRIME), for each scanner, centers performing prostate MRI submitted five consecutive studies and the MRI protocols (phase I). Submitted data were evaluated in consensus by two expert genitourinary radiologists using the PI-QUAL scoring system that evaluates MRI diagnostic quality using five points (1 and 2 = nondiagnostic; 3 = sufficient; 4 = adequate, 5 = optimal) between September 2021 and August 2022. Feedback was provided for scanners not achieving a PI-QUAL 5 score, and centers were invited to resubmit new imaging data using the modified protocol (phase II). Descriptive comparison of outcomes was made between the MRI scanners, feedback provided, and overall PI-QUAL scores. Results In phase I, 41 centers from 18 countries submitted a total of 355 multiparametric MRI studies from 71 scanners, with nine (13%) scanners achieving a PI-QUAL score of 3, 39 (55%) achieving a score of 4, and 23 (32%) achieving a score of 5. Of the 48 (n = 71 [68%]) scanners that received feedback to improve, the dynamic contrast-enhanced sequences were those that least adhered to the Prostate Imaging Reporting and Data System, version 2.1, criteria (44 of 48 [92%]), followed by diffusion-weighted imaging (20 of 48 [42%]) and T2-weighted imaging (19 of 48 [40%]). In phase II, 36 centers from 17 countries resubmitted revised studies, resulting in a total of 62 (n = 64 [97%]) scanners with a final PI-QUAL score of 5. Conclusion Substantial variation in global prostate MRI acquisition parameters as a measure of quality was observed, particularly with DCE sequences. Basic evaluation and modifications to MRI protocols using PI-QUAL can lead to substantial improvements in quality. Clinical trial registration no. NCT04571840 Published under a CC BY 4.0 license. Supplemental material is available for this article. See also the editorial by Almansour and Chernyak in this issue