Browsing by Author "Pruner, I. (36350119000)"

[No abstract available]

[No abstract available]

Objectives: To assess in patients with systemic sclerosis (SSc) the concentration of different subpopulations of circulating extracellular vesicles (EVs) and their association with the progression of interstitial lung disease (PF-ILD). Methods: The prospective study included 59 SSc cases, 54% with interstitial lung disease (ILD). Plasma levels of EVs were analysed with flow cytometry and labelled as endothelial (EEVs), platelet (PEVs), leucocyte (LEVs), and EVs, expressing ICAM1, TF, or HMGB1. The presence of ILD was defined by HRCT. Lung functional tests were done every 3–6 months over a 3-year follow-up period. PF-ILD was defined as ≥10% decline of FVC % from baseline, or ≥5–9% along with a decline in DLCO of ≥15%. Results: At baseline, 32/59 SSc patients had ILD, with a median disease duration of 3 years, and 38% were therapy naïve. In ILD patients, increased levels of all investigated EVs were found in respect to SSc patients without ILD (p < 0.05). Therapy naïve ILD cases had altered only ICAM1 + EVs compared to treated (p < 0.05). Multivariate regression analysis (MR) showed an independent association of PEVs (OR 1.004, 95% CI 1.001–1.01) and ICAM1 + EVs (OR 1.3, 95% CI 1.1–1.5) with ILD. During the follow-up period, 12/32 ILD patients developed PF-ILD, and in this group, the levels of all explored EVs were elevated compared to those without PF-ILD (p < 0.05). In an ROC analysis, all EVs showed a good ability to identify PF-ILD patients (p < 0.05). Cox MR confirmed the independent predictive value of ICAM1 + EVs (HR 1.1, 95% CI 1.01–1.1) with SSc PF-ILD. Conclusion: Circulating EV levels are increased in SSc and correlate with ILD. In particular, ICAM1 + EVs may be a novel biomarker of PF-ILD, identifying SSc patients at high risk of progression who may require early aggressive treatment. Based on our results, the role of EVs in the pathogenesis and progression of ILD should be investigated further. Copyright © 2025 Colic, Pruner, Damjanov, Antovic, Sefik-Bukilica, Cerinic and Antovic.

Essentials Prothrombin Belgrade mutation leads to antithrombin resistance. Clinical and biochemical phenotypes in a large family with this mutation were investigated. In carriers, we detected decreased factor II activity and increased endogenous thrombin potential. Prothrombin Belgrade mutation represents a strong prothrombotic risk factor. Summary: Background The recently reported c.1787G>A mutation in the prothrombin gene leads to Arg596Gln replacement in the protein molecule (prothrombin Belgrade). This substitution impairs binding of antithrombin to thrombin and results in inherited thrombophilia, known as antithrombin resistance. Objectives We aimed to elucidate the clinical and biochemical characteristics of thrombophilia associated with antithrombin resistance in a large Serbian family with the prothrombin Belgrade mutation. Patients and methods Nineteen family members were investigated, among whom 10 were carriers of the c.1787G>A mutation. In all subjects the clinical phenotype was determined and laboratory investigations of hemostatic parameters were performed. Results Six out of the 10 mutation carriers developed thromboembolic events, mainly deep venous and mesenteric vein thrombosis. The median age of the first thrombotic event was 26.5 (12–41) years, whereas the incidence rate of first thrombosis was 2.2% per year. In all mutation carriers prothrombin activity was significantly decreased in comparison with non-carriers, clearly distinguishing each group. However, the presence of the mutation did not affect the prothrombin antigen level in plasma. The endogenous thrombin potential was significantly increased in all carriers in comparison with non-carriers, indicating the presence of blood hypercoagulability. Interestingly, levels of D-dimer and the F1+2 fragment were similar in both groups. Conclusions Although rare, the prothrombin Belgrade mutation represents strong thrombophilia with early onset of thrombosis in the investigated family. According to our results, decreased prothrombin activity may be a simple screening test for detection of this mutation in thrombotic patients. © 2017 International Society on Thrombosis and Haemostasis