Purpose: This study evaluates the prognostic significance of pleural effusion (PE) in COVID-19 patients across thirteen centers in Germany, aiming to clarify its role in predicting clinical outcomes. Methods: In this retrospective analysis within the RACOON project (Radiological Cooperative Network of the COVID-19 pandemic), 1183 patients (29.3 % women, 70.7 % men) underwent chest CT to assess PE. We investigated PE's association with 30-day mortality, ICU admission, and the need for mechanical ventilation. Results: PE was detected in 31.5 % of patients, showing a significant correlation with 30-day mortality (47.5 % in non-survivors vs. 27.3 % in survivors, p < 0.001), with a hazard ratio of 2.22 (95% CI 1.65-2.99, p < 0.001). No significant association was found between PE volume or density and mortality. ICU admissions were noted in 46.8 % of patients, while mechanical ventilation was required for 26.7 %. Conclusion: Pleural effusion is present in a significant portion of COVID-19 patients and independently predicts increased 30-day mortality, underscoring its value as a prognostic marker. Its identification, irrespective of volume or density, should be a priority in radiological reports to guide clinical decision-making.

Thoracolumbar stump ribs are one of the essential indicators of thoracolumbar transitional vertebrae or enumeration anomalies. While some studies manually assess these anomalies and describe the ribs qualitatively, this study aims to automate thoracolumbar stump rib detection and analyze their morphology quantitatively. To this end, we train a high-resolution deep learning model for rib segmentation using nnUNet and achieve significant improvements over existing models (Dice score 0.997 vs. 0.779, p-value < 0.01). In addition, we employ a novel iterative algorithm and piecewise linear interpolation to estimate rib length, achieving a success rate of 98.2%. When analyzing morphological features, we show that stump ribs articulate more posteriorly at the vertebrae ($-19.2\pm 3.8$ vs. $-13.8\pm 2.5$ mm, p-value < 0.01), are thinner ($260.6\pm 103.4$ vs. $563.6\pm 127.1$${\mathrm{mm}}^2$, p-value < 0.01), and are oriented more downwards and sideways within the first centimeters in contrast to full-length ribs. We show that with partially visible ribs, these features can achieve an F1-score of 0.84 and an AUC of 0.98 in differentiating stump ribs from regular ones. We publish the model weights and masks for public use.

Objective: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive motor neuron degeneration. Fused in sarcoma (FUS)‐associated juvenile ALS (jALS) represents a distinct and aggressive subgroup with rapid deterioration and poor prognosis. Certain FUS mutations have been associated with comorbid intellectual disability, suggesting neurodevelopmental involvement. We compared FUS‐jALS with adult‐onset FUS‐ALS cases (aALS) to evaluate the association between premorbid cognitive impairment, genetic and clinical features incorporating neuroimaging data. Methods: Patients with genetically confirmed FUS‐ALS were classified as jALS (onset < 25 years) or aALS (onset ≥ 25 years). Neuropsychological assessment used Mehrfachwahl‐Wortschatz‐Test (MWT) for verbal IQ, and the Edinburgh Cognitive and Behavioral ALS Screen (ECAS), with cognitive impairment classified according to Strong criteria. Volumetric analysis was conducted on structural MRI and FDG‐PET data. Results: All three jALS (P525L [ n = 2], H517_Q519del [ n = 1]) showed rapid progression with early severe clinical events. Neuropsychological assessment revealed global cognitive deficits (ALS‐ci) with widespread dysfunction beyond typical ALS‐specific patterns and reduced verbal IQ, pointing towards premorbid cognitive impairment. aALS demonstrated slower progression and were predominantly cognitively unimpaired (ALS‐ni) or showed an ALS‐specific impairment. Neuroimaging revealed distinct patterns: jALS cases demonstrated posterior cortical atrophy and hypometabolism on FDG‐PET, while aALS showed largely preserved brain volumes and limbic‐subcortical hypometabolism. Interpretation: Specific FUS mutations (P525L, H517_Q519del) predispose to jALS with severe progression and premorbid cognitive impairments, supporting a genotype–phenotype association. Posterior cortical findings suggest neurodevelopmental delay rather than disease‐related neurodegeneration. Genetic FUS screening may be warranted in patients with intellectual disability and motor signs, given emerging targeted therapies.

Eine wichtige primäre Ursache der Tracheomegalie ist das Mounier-Kuhn-Syndrom. Das Mounier-Kuhn-Syndrom ist eine seltene, chronische Tracheomegalie, die durch eine pathologische Erweiterung des Tracheadiameters bis zu 3,0 cm gekennzeichnet ist. Zusätzlich sind eine Erweiterung des rechten Hauptbronchus über 2,0 cm und des linken Hauptbronchus über 1,8 cm zu beobachten. Die genaue Ursache dieser Erkrankung ist noch nicht vollständig geklärt, jedoch werden primär-genetische und sekundäre Aspekte diskutiert, einschließlich möglicher Verbindungen zu anderen Erkrankungen wie dem Ehlers-Danlos-Syndrom, Cutis laxa oder dem Marfan-Syndrom (Chandran et al. BMJ Case Rep 2021; 14 (01): e239876). Die Erkrankung kann in jedem Lebensalter auftreten, wird jedoch häufig in der dritten Lebensdekade diagnostiziert, wobei Männer etwa achtmal häufiger betroffen sind als Frauen (Krustin E et al. Respiratory medicine 2013; 107: 1822–1828). Patienten präsentieren sich typischerweise mit rezidivierenden Infektionen und (Belastungs-) Dyspnoe, können aber auch asymptomatisch sein. Es ist eine Ausdünnung der glatten Muskulatur, insbesondere longitudinaler Anteile sowie von elastischen Fasern zu beobachten. Aufgrund dessen kann es zur Protrusion von nicht-muskulären Segmenten zwischen transversal verlaufenden Muskelzügeln kommen, welche die Trachealknorpel miteinander verbinden, sodass divertikuläre Ausstülpungen entstehen (Katz I et al., Am J Roentgenol Radium Ther Nucl Med. 1962; 88: 1084–1094). Das Mounier-Kuhn-Syndrom kann in drei Untertypen unterteilt werden, die verschiedene Merkmale aufweisen. Typ I beschreibt eine symmetrische Erweiterung der Trachea und Bronchien. Beim Typ II kommt es zu einer tendenziell exzentrischen Erweiterung mit Divertikeln. Im Typ III zeigen sich Divertikel insbesondere entlang des distalen Bronchus (Krustin E et al. Respiratory medicine 2013; 107: 1822–1828). Received: 27 May 2024 Accepted after revision: 17 October 2024 Article published online: 12 November 2024 © 2024. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany