This report describes a unique constellation of accessory ossicles, highlighting their anatomical, clinical, and radiological significance. A 43-year-old female undergoing imaging for suspected fracture was evaluated using multi-detector computed tomography (MDCT) with 1.25 mm slice thickness. Multiplanar reconstructions (axial, coronal, sagittal) and three-dimensional volume-rendered images were analyzed. CT imaging revealed the coexistence of an os subfibulare and a tripartite os naviculare. Multiplanar and three-dimensional reconstructions confirmed the presence and configuration of variants. The combination of supernumerary bones and a multipartite ossicle represents an exceedingly uncommon anatomical presentation. This case illustrates an exceptional coexistence of multiple accessory ossicles, including an exceedingly rare tripartite os naviculare. Thorough radiological evaluation using MDCT and multiplanar reconstructions is essential for accurate identification and differentiation from fractures or other pathology.
BACKGROUND AND OBJECTIVE:Pulmonary alveolar proteinosis (PAP) is a rare disease of inappropriate alveolar surfactant accumulation. The study objective was to compare Greece-Türkiye-Cyprus cohorts, followed-up for 20 years. METHODS:Data were retrieved retrospectively by chart review. RESULTS:One hundred and thirty patients, Greece 39, Türkiye 87, Cyprus 4, were included; 115 (89%) autoimmune (a)PAP, 13 (10%) secondary, 1 (1%) hereditary. Abnormal anti-GM-CSF antibody titre was available in 86/115 patients, further analyzed for aPAP. Forty (46%) were male, with median (IQR) age at diagnosis of 40 (31-50) years. At median (IQR) duration of clinical follow-up of 39 (18-78) months, three patients died. Non-survivors had higher rates of cardiovascular disease [67% vs. 5%, p < 0.001], LTOT (67% vs. 13%, p = 0.001), pulmonary fibrosis [67% vs. 5%] and worse functional status at follow-up [FVC% pred 49 (29) vs. 87 (74-97); p = 0.026, DLCO% pred 21 (46) vs. 72 (58-82); p = 0.016]. aPAP patients were stratified by therapy group (no WLL-no i-GM-CSF, WLL alone, i-GMCSF alone and both WLL and iGM-CSF). WLL was more frequently performed in Türkiye and i-GMCSF alone in Greece (p = 0.006). All therapies were effective. No difference was detected on outcome when sole iGM-CSF was compared to WLL alone or in combination with iGM-CSF [deceased/alive 0%/100% vs. 7%/93%, p = 0.491]. Sole iGM-CSF significantly ameliorated clinical and functional parameters of disease severity [DLCO% (p = 0.013), SatO2 (p = 0.002), 6MWT (p = 0.026)]. Across all therapy groups, pulmonary fibrosis eliminated beneficial response to treatment. CONCLUSION:This first real-life comparison of aPAP cohorts originating from three countries showed the non-inferiority of sole iGM-CSF treatment. Fibrosis negatively affects response to treatment and survival.
Persistent left superior vena cava is a congenital variation of the thoracic venous system. Although it is often asymptomatic, its presence can complicate procedures such as central venous access, cardiac device implantation, and cardiothoracic surgery. Reported prevalence data are inconsistent and predominantly derived from populations with underlying cardiac pathology. This study seeks to provide a robust, evidence-based estimate of the prevalence of persistent left superior vena cava in populations not preselected for congenital heart disease. Additionally, it aims to quantify its principal anatomical configurations, including the laterality of the superior vena cava and the presence or absence of a bridging brachiocephalic vein. A systematic search was conducted using PubMed, Google Scholar, Scopus, and Web of Science. Studies documenting the prevalence of persistent left superior vena cava in populations not selected on the basis of congenital heart disease criteria were included. Statistical analysis was carried out using random-effects meta-analytic models. Eighteen studies (n = 169,835 individuals) were included. The pooled prevalence of persistent left superior vena cava was 0.61
Abstract:Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease characterized by abnormal accumulation of surfactant in alveoli. Pathogenetically, in aPAP, the presence of granulocyte macrophage-colony stimulating factor (GM-CSF) autoantibodies hinders physiological binding of GM-CSF to its receptor, disrupting terminal differentiation of alveolar macrophages and the activation GM-CSF-PU.1-PPARG1-ABCG1 axis, resulting in a primary reduction in cholesterol efflux from alveolar macrophages and a secondary reduction in surfactant clearance through macrophages from the alveolar surface. APAP is the most common, accounting for more than 90 to 95% of all patients included under the PAP term, which encompasses and classifies all forms of PAP according to etiopathogenetic mechanisms, as primary, secondary, congenital, and unclassified. APAP is worldwide distributed with an estimated prevalence fluctuating between 7.0 and 9.7 cases/million and an annual incidence of 1.65, affecting middle-aged men and women. Clinical manifestation may be gradual and insidious, mainly manifesting with progressive dyspnea, but the natural history is variable, since some patients stabilize for a long period, while others progress to respiratory failure and death; in a minority, spontaneous resolution may be observed, while some develop lung and/or systemic infections, and rarely pulmonary fibrosis. Until recently, whole lung lavage (WLL) was universally accepted as the gold-standard therapeutic modality in aPAP. However, after considerable progress in the past 25 years and the publication of several positive studies, replacing the use of inhaled-GM-CSF as the standard of care for aPAP and conceding WLL a rescue option is becoming more and more concrete. In conclusion, aPAP is the classic paradigm of a scientific disease progressing from the "bench-to-bedside," since several discoveries made in the laboratory setting have become necessary to clarify its pathogenetic mechanisms, define diagnostic tools, and implement new therapeutic modalities, which established the disease as treatable and fully reversible, literally, moving patients from "hell to heaven."
Developmental venous anomalies (DVAs) are benign congenital veins that collect normal brain drainage into a single outlet. Cerebral cavernous malformations (CMs) are clusters of thin-walled capillary cavities prone to bleeding. When both lesions coexist, the DVA's altered venous pressure and flow can promote CM formation or rupture. Detecting a DVA abutting an otherwise unexplained intracerebral hemorrhage can therefore raise suspicion of an occult CM as a likely cause, a clue which may be invaluable for daily clinical practice. The main focus of this review is to acknowledge the hallmark imaging appearances of DVAs and CMs, as well as their coexistence, explore the clinical consequences of mixed lesions, and emphasize that recognizing their partnership is vital for an accurate, timely diagnosis and appropriately targeted management.
Biallelic SERPINA1 Q0 variants lead to early-onset emphysema even in nonsmokers, mandating early-life preventive and therapeutic measures. ZZ patients' lungs on shut-off transcription strategies equating them to "null" variants should also be protected. https://bit.ly/4mIlRL9.
Background: Protracted bacterial bronchitis (PBB) and bronchiectasis share common clinical and microbiological features, but direct comparative data in children are limited. Objectives: To compare bronchoalveolar lavage (BAL) microbiology between pediatric PBB and bronchiectasis and identify predictors of lower airway and polymicrobial infections. Methods: We retrospectively reviewed children diagnosed with PBB or bronchiectasis at a tertiary center (January 2019-June 2025) who underwent both high-resolution computed tomography of the chest and bronchoscopy with BAL within a 6-month period. Multivariable logistic regression was used to identify predictors of lower airway and polymicrobial infections, adjusting for age, gender, tracheomalacia/bronchomalacia, asthma, and Bhalla score. Results: Among 135 children (85 with PBB, 50 with bronchiectasis), those with bronchiectasis were older (median 7.8 vs. 4.2 years, p < 0.001), while comorbidities showed statistically non-significant differences. The prevalence of lower airway infection was high (PBB 81.2%, bronchiectasis 74.0%; p = 0.330). Pathogen distribution demonstrated statistically non-significant differences between groups after adjustment, with Haemophilus influenzae being the most common pathogen in both groups. Moraxella catarrhalis was more frequent in PBB in unadjusted analysis (21.2% vs. 8.0%; p = 0.045), but this difference did not persist after adjustment. Polymicrobial infection occurred in 25.9% of PBB and 16.0% of bronchiectasis cases (p = 0.180). In regression analyses, younger age independently predicted polymicrobial infection (adjusted OR 0.81, 95% CI 0.69-0.95). Conclusions: BAL microbiology showed statistically non-significant differences between PBB and bronchiectasis, supporting the concept of a disease continuum. Younger children were more prone to polymicrobial infection. These findings support early targeted antibiotic therapy and close clinical surveillance to prevent progression to irreversible airway damage.
Background: Immunocompromised patients, particularly those with humoral immune deficiencies or receiving B-cell-targeted therapies, are at increased risk of persistent SARS-CoV-2 infection, a condition often underrecognized and lacking standardized treatment. Methods: We present a case series of patients with persistent SARS-CoV-2 infection and underlying humoral immunodeficiency, treated at the General University Hospital "Attikon" from February 2023 to September 2024. Persistent infection was defined by prolonged symptoms, compatible imaging findings, and RT-PCR positivity beyond 21 days. All patients received combination antiviral therapy with remdesivir and nirmatrelvir/ritonavir, and intravenous immunoglobulin (IVIG), using a structured diagnostic and therapeutic algorithm. Results: Eleven patients (55% male), median age 56 [IQR 50-66] years, were included. Seven (64%) had hematologic malignancy, 10 (91%) received anti-CD20 therapy, and 6 (55%) had both. Median symptom duration before diagnosis was 63 [58-135] days. Ten (91%) experienced recurrent symptoms; one (9%) had progressive symptoms with severe respiratory failure requiring high-flow nasal cannula. Persistent infection was confirmed via bronchoscopy with bronchoalveolar lavage in 6 patients (55%). Prior to diagnosis, 5 patients (45%) required one hospitalization, 1 (9%) was hospitalized twice, and 2 (18%) had more than two hospitalizations. Following combination therapy, 10 (91%) achieved complete response at 180-day follow-up. Conclusions: The proposed diagnostic and therapeutic algorithm combining remdesivir, nirmatrelvir/ritonavir, and IVIG enhanced diagnostic value and therapeutic outcomes in this high-risk population.
Objective:Lung involvement in paediatric rheumatic diseases significantly impacts morbidity and mortality. High-resolution computed tomography (HRCT) is a sensitive diagnostic tool but raises concerns about radiation exposure. Spirometry, a non-invasive and accessible test, is often used to assess pulmonary function, but its accuracy in detecting lung disease in newly diagnosed pediatric rheumatic conditions remains uncertain. This study evaluates spirometry's reliability, using HRCT as the reference standard. Methods:Patients suspected of lung involvement due to respiratory symptoms or disease prevalence underwent HRCT and pulmonary assessment. A retrospective review of HRCT scans was conducted for 22 pediatric patients diagnosed with rheumatic disease (January 2021-December 2023), all of whom had pathological findings. HRCT was performed using a paired end-inspiratory and forced-expiratory protocol with 1-mm collimation. Radiological findings, including parenchymal opacities, ground-glass opacities, reticular patterns, honeycombing, parenchymal bands, bronchiectasis, peribronchial wall thickening, and air trapping, were assessed. Spirometric values-percent predicted Forced Expiratory Volume in 1 second (ppFEV1), Forced Vital Capacity (ppFVC), and FEV1/FVC ratio-were collected for patients with acceptable flow-volume curves. Results:Seventeen of 22 patients (77.3%) had technically acceptable spirometry. Compared to HRCT, spirometry demonstrated a sensitivity of 29.4%. Conclusion:Despite its excellent positive predictive value, spirometry's low sensitivity suggests it may miss early lung involvement in paediatric rheumatic diseases. HRCT remains the preferred diagnostic tool to ensure accurate detection and management, despite radiation concerns.
Introduction: Long-term lung sequelae in severe COVID-19 survivors, as well as their treatment, are poorly described in the current literature. Objective: To investigate lung fibrotic sequelae in survivors of severe/critical COVID-19 pneumonia and their fate according to a "non-interventional" approach. Methods: Prospective study of the above COVID-19 survivors after hospital discharge from March 2020 to October 2022. Re-evaluation lasted 3-12 months and included chest HRCT, PFTs, dyspnea, and overall health evaluation by modified Medical Research Council (mMRC) and St. George's Respiratory Questionnaire (SGRQ), respectively. Results: In this study, 198 patients (61.1% male) with a median age of 57 years (IQR 49-66). After 3 months, 187 (94.4%) patients were assessed; after 6 months, 82 (41.1%) patients were assessed; and after 12 months, 16 (8%) patients were assessed. At each time point, a significant reduction was observed in the extent of COVID-19-associated opacities (p < 0.001 and p = 0.002) and of parenchymal bands (p = 0.014 and p = 0.025). Persisting fibrotic-like changes were observed in 18 (9%) patients (apical findings in 2 patients, fibrotic non-specific interstitial pneumonia-like changes in 14 patients, minimal fibrotic changes in 2 patients). At 3 months, the predicted median FVC% was 93% (80-100%) and the predicted DLCO% was 65% (58-78%) with a statistically significant improvement at 6 months in both (p = 0.001). Moreover, 81.1% had mMRC ≤ 1 and the median SGRQ was 11.65 [0-24.3] with a significant reduction at 6 months in both dyspnea (p < 0.001) and SGRQ (p = 0.027) persisting at 12 months. Conclusions: This prospective study, including only survivors of severe/critical COVID-19 pneumonia, documented the significant improvement in all imaging, functional, and clinical parameters by applying the "non-interventional" approach. These data do not indicate any post-COVID-19 severe/critical pneumonia and "epidemic of widespread pulmonary fibrosis".
Height velocity is considered a key auxological tool to monitor growth, but updated height velocity growth charts are lacking. We aimed to derive new French height velocity growth charts by using a big-data approach based on routine measurements. We extracted all growth data of children aged 1 month–18 years from the electronic medical records of 42 primary care physicians, between 1 January 1990 and 8 February 2018, throughout the French metropolitan territory. We derived annual and biannual height velocity growth charts until age 15 years by using the Lambda-Mu-Sigma method. These new growth charts were compared to the 1979 French and 2009 World Health Organisation (WHO) ones. New height velocity growth charts were generated with 193 124 and 209 221 annual and biannual values from 80 204 and 87 260 children, respectively, and showed good internal fit. Median curves were close to the 1979 French or 2009 WHO ones, but SD curves displayed important differences. Similar results were found with the biannual height velocity growth charts. We produced new height velocity growth charts until age 15 years by using a big-data approach applied to measurements routinely collected in clinical practice. These updated growth charts could help optimise growth-monitoring performance.
Introduction: Lung involvement in pediatric rheumatic diseases significantly impacts morbidity and mortality. High-resolution computed tomography (HRCT) is a sensitive diagnostic tool for detecting lung involvement; however, it raises concerns regarding radiation exposure. Spirometry, a non-invasive and widely accessible test, is often used to assess pulmonary function, but its accuracy in identifying lung disease in children with newly diagnosed rheumatic conditions remains uncertain. This study evaluates the reliability of spirometry, using HRCT as the reference standard, in detecting early lung involvement in this population. Methods: At our institution, all patients suspected of having lung involvement—either due to respiratory symptoms or the high prevalence of respiratory involvement associated with their specific disease—underwent an HRCT scan and pulmonary assessment. A retrospective review of HRCT scans was conducted for 22 pediatric patients diagnosed with rheumatic disease between January 2021 and December 2023, all of whom exhibited pathological findings in their HRCT scans. HRCT scans were conducted with a paired end-inspiratory and forced-expiratory protocol using 1-mm collimation. Radiological findings, including parenchymal opacities, ground-glass opacities (GGOs), reticular patterns, honeycombing, parenchymal bands, bronchiectasis, peribronchial wall thickening, and air trapping were assessed. Spirometric values, including percent predicted values for Forced Expiratory Volume in 1 second (ppFEV1), Forced Vital Capacity (ppFVC), and the FEV1/FVC ratio, were collected for all patients who had an acceptable flow-volume curve Results: Of the 22 patients enrolled, 17 (77.3%) had technically acceptable spirometry results. Using HRCT as the reference standard, spirometry yielded a sensitivity of 29.4%. Conclusion: Although spirometry demonstrated excellent positive predictive value, its low sensitivity indicates that it may miss early lung involvement in children with newly diagnosed rheumatic diseases. These findings reinforce HRCT as the preferred diagnostic tool in this population, despite concerns about radiation exposure, to ensure accurate detection and management of lung involvement.
Effective communication and collaboration among healthcare professionals are crucial for delivering high-quality patient care. Interdepartmental miscommunication poses a significant challenge to healthcare systems, potentially undermining the quality of healthcare services provided. In the same manner, communication barriers between referring physicians and radiologists can specifically affect radiology services and patient outcomes. This article attempts to put the spotlight on the ever-present chronic challenges of this issue and prompt readers to recognize the relevant potential pitfalls in their daily clinical practice. Practical solutions are explored and proposed, which should be tailored to the specific needs and issues that each individual institution may face.
The most common acute infection and leading cause of death in children worldwide is pneumonia. Clinical and laboratory tests essentially diagnose community-acquired pneumonia (CAP). CAP can be caused by bacteria, viruses, or atypical microorganisms. Imaging is usually reserved for children who do not respond to treatment, need hospitalisation, or have hospital-acquired pneumonia. This review discusses the imaging findings for acute CAP complications and the diagnostic role of each imaging modality. Pleural effusion, empyema, necrotizing pneumonia, abscess, pneumatocele, pleural fistulas, and paediatric acute respiratory distress syndrome (PARDS) are acute CAP complications. When evaluating complicated CAP patients, chest radiography, lung ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) can be used, with each having their own pros and cons. Imaging is usually not needed for CAP diagnosis, but it is essential for complicated cases and follow-ups. Lung ultrasound can supplement chest radiography (CR), which starts the diagnostic algorithm. Contrast-enhanced computed tomography (CECT) is used for complex cases. Advances in MRI protocols make it a viable alternative for diagnosing CAP and its complications.
Lung involvement in rheumatic diseases is a major determinant of patient morbidity and mortality; however, its diagnosis depends on the detection methods used. We compared the incidence of pulmonary involvement in newly diagnosed treatment-naive patients with rheumatic diseases, as assessed by pulmonary function tests (PFTs) and chest high-resolution computed tomography (HRCT). This retrospective study reviewed the symptoms, spirometry results, and HRCT scans of 22 newly diagnosed, treatment-naïve patients and age-matched controls from the University General Hospital “Attikon”, Athens, Greece, between January 2021 and December 2023. Correlations between clinical findings and spirometry parameters were tested. HRCT scans revealed lung abnormalities in all patients, including peribronchial wall thickening (61.5%), ground glass opacities (59.1%), parenchymal bands (54.5%), air-trapping (50%), reticular patterns (45.5%), bronchiectatic changes (40.9%), and parenchymal opacities (31.8%). PFTs, performed in 17/22 (77.3%) patients, revealed definite pathology in 5 patients (restrictive: N=4; obstructive: N=1). No association was found between spirometric indices and clinical parameters. The results indicate a higher prevalence of abnormal chest CT than of PFT abnormalities, suggesting that HRCT is more sensitive in detecting early lung involvement in pediatric patients with rheumatic disease and should be considered in the routine evaluation of these patients.
A novel automated multi-classification approach is proposed for the anticipation of lung abnormalities using chest X-ray and CT images. The study leverages a publicly accessible dataset with an insufficient and unbalanced number of images, addressing this issue by employing the data augmentation approach DCGAN to balance the dataset. Various preprocessing procedures are applied to improve features and reduce noise in lung pictures. As the base for the model, the vision trans-former and convolution-based compact convolutional transformer (CCT) model is utilized. To determine the best model configuration, an ablation study is performed on the original CCT model using a CT scan dataset with image dimensions of 32 x 32. Following that, this model is trained on the X-ray dataset to evaluate performance on an entirely other modality. The performances are compared to six pre-trained models with 32x32 images. While traditional models achieved modest performance, with test accuracies ranging from 43% to 77% and 49% to 73% requiring lengthy training times, the suggested model performed exceptionally well, obtaining test accuracies of 99.77% and 95.37% for CT and X-ray, respectively with a short training duration of 10-12 and 40-42 seconds/epoch. Robustness is demonstrated through the progressive reduction of the number of training images, with findings indicating that the model maintains good performance even on a reduced dataset. An explainable AI technique Grad-CAM is used to explain the model’s judgment. Grad-CAM-based color visualization is shown to explain model assessments and help health specialists make quick, confident decisions. This study used image preprocessing and deep learning techniques to detect lung anomalies, and it addressed the challenges of training time and computational complexity.