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Maxillary arch dimensions, occlusion assessment and space conditions in patients with cleft palate in the period of deciduous dentition - a retrospective study
Full text linkPurpose The aim of this study was to evaluate the maxillary arch dimensions, dentoalveolar relationships and spacing conditions in patients with cleft palate in comparison with the control group. Methods The subjects consisted of 31 children with cleft palate only (CP) aged 5.5 ± 0.51 (20 with a cleft of hard and soft palate (SHPC group) and 11 with a cleft of soft palate only (SHP group)). In the control group 30 subjects had a normal occlusion at age 5. Maxillary arch dimensions, dentoalveolar relationship according to the Huddard Bodenham index (HBI) and space conditions were compared with the control group. Results In all variables of maxillary arch dimensions except for total arch height the control group exhibited greater values. Most of the significant differences were on account of differences between the control and the SHPC group, with only three comparisons yielding significant results when comparing the two groups of children with clefts (SHPC vs SPC, IV-IV central: pDunnett T3= 0.0002, 95%CIDifference=-9.9-(-3.18)V-V distopalatal cusps: pDunnett T3= 0.0002, 95%CIDifference=-9.97-(-3.17)Total arch length: pDunnett T3= 0.0014, 95%CIDifference=1.74-7.85). The three groups differed significantly in anterior HBI only (HKruskal-Wallis=15.56, p=0.0067). The spacing conditions in both jaws were also shown to be significantly dependent on the group of subjects studied (Upper jaw: χ2omnibus= 16.79, p=0.0018lower jaw: χ2omnibus= 13.75, p=0.0102). Conclusions The growth of the upper dental arch at the age of five is impeded in participants with CP in comparison to a control group. It is important to assess the effect of cleft subtypes on growth and development to get a better understanding
Interleukin-6 extraction ratios during prolonged CytoSorb hemoadsorption depend on procedural blood flow
Full text linkBackground: Hemoadsorption with CytoSorb is a novel treatment for cytokine release syndrome, but there are few published data on the rate of cytokine removal with prolonged use. Here, we report a prospective observational study of IL-6 extraction ratios with prolonged CytoSorb use. Methods: A secondary analysis was conducted on a prospective observational cohort study involving patients treated with CytoSorb. Blood samples for IL-6 were taken before treatment, after 30 min, and every 6 h of treatment at three sites: (1) before the adsorber, (2) between the adsorber and dialyzer, and (3) after the dialyzer. The extraction ratios of the adsorber were then calculated. Results: We included 21 dialysis circuits performed in 15 critically ill patients, mainly those with cytokine storm because of septic shock. The median extraction ratio of IL-6 after 30 min was 26% (interquartile range, IQR 18-37%). The ratio decreased to 10% (6-21%) after 6 h and remained between 9-16% for up to 24 h (with a low number of circuits used beyond 12 h). Extraction ratios were similar in circuits with high (>1,000 ng/L) and low baseline IL-6 levels. On the contrary, in circuits with high blood flow (≥200 mL/min, i.e., intermittent hemodialysis), the extraction ratio was very low (median 6%) at 6 h and negligible thereafter, whereas the circuits with lower blood flow (<200 mL/min) maintained an extraction ratio of 20% for up to 12 h. Conclusion: We observed a significant reduction in the IL-6 extraction ratio within 6 h in circuits with high blood flow, whereas circuits with lower blood flow maintained an adequate extraction ratio for up to 12 h. Recent consensus recommendations on an 8-12 h exchange interval should mainly be applied to continuous dialysis methods, whereas in intermittent hemodialysis, the exchanges should be more frequent
Dataset on microclimate properties in sinkholes of Dinaric beech forests (Slovenia) a decade after the silvicultural treatment
No full textInformation on the funders/programmes/projects that made the data collection possible: ARIS J4-4542/22-04480L Natural regeneration processes in beech forests after disturbance, ARIS research core funding P4-0107 Forest ecology, biology and technology, ARIS post-doc project Z4-4543 Long-term changes of forest vegetation caused by global and local environmental change drivers, the project Development of research infrastructure for the international competitiveness of the Slovenian RRI space – RI-SI-LifeWatch, financed by the Republic of Slovenia, Ministry of Education, Science and Sport and the European Union from the European Regional Development Fund. The forest treatment experiment was supported by ManFor CBD 2010-2015 Life Environment Project LIFE09 ENV/IT/000078
A case report and a literature review of Myeloid/Lymphoid Neoplasm with Eosinophilia and PCM1::JAK2 rearrangement representing as B-cell acute lymphoblastic leukemia B-ALL
Full text linkMyeloid/Lymphoid Neoplasms with Eosinophilia and Tyrosine Kinase Gene Fusions (MLN-eo-TK) represent a distinct and heterogeneous group of hematologic malignancies characterized by recurrent gene fusions involving tyrosine kinases, such as PDGFRA, PDGFRB, FGFR1, JAK2, FLT3, ETV::ABL1 and other partner genes/variants. Among these, gene rearrangements involving PCM1::JAK2 are rare and may present diagnostic challenges, particularly when manifesting as acute lymphoblastic leukemia (ALL). We describe a case of a patient who presented with B-cell acute lymphoblastic leukemia (B-ALL) with a JAK2 rearrangement. After the induction therapy, strong myeloid proliferation in bone marrow without evidence of residual lymphoblasts was observed and JAK2 rearrangement was recognized to be a consequence of translocation t(89)(p22p24)] resulting in PCM1::JAK2 fusion. This finding indicated the presence of an underlying chronic myeloid/lymphoid neoplasm, meeting criteria for MLN-eo-TK. Following an inadequate response to standard chemotherapy, salvage regimens incorporating targeted agents (JAK2 and BCL-2 inhibitors) and allogeneic bone marrow transplantation were administered, all of which unfortunately resulted in short-lived clinical benefit. The case highlights the importance of distinguishing de novo lymphoid malignancies from MLN-eo-TK, especially when JAK2 rearrangements are detected. Recognition of the clonal myeloid component during or after lymphoid-directed therapy has important diagnostic and therapeutic implications, supporting the use of targeted JAK2 inhibition in addition to standard chemotherap
Reaching the therapeutic ceiling in IBD
Full text linkThe term Advanced Combination Treatment (ACT) involves the combination of at least two biologics or the use of a biologic with a small molecule drug, each with different mechanisms of action. This narrative review evaluates the current evidence supporting ACT in inflammatory bowel disease (IBD), focusing on preclinical studies, real-world evidence, and randomized controlled trials. A systematic review of randomized controlled trials has concluded that ACT significantly improves clinical outcomes, without significant safety concerns in patient with IBD. However, variability in trial designs and the lack of standardized outcome measures have led to initiatives aimed at mitigating these issues through a clear expert consensus. While the evidence for ACT in IBD is compelling, substantial challenges remain in standardizing treatment protocols and ensuring long-term safety. In the meantime, the use of ACT in clinical practice remains off-label and requires careful consideration of patient-specific factors. Future clinical trials should consider robust biomarkers for patient selection and leverage mechanistic insights to select combination components
Atypical presentation of cerebral amyloid angiopathy in a 42-year-old man with recurrent lobar hemorrhages and neuropsychiatric symptoms
Full text linkBackground: Cerebral amyloid angiopathy (CAA) is a small- and medium-vessel cerebrovascular disease characterized by β-amyloid accumulation within cortical and leptomeningeal arterial walls. Although it most commonly manifests in older individuals as spontaneous lobar hemorrhage, uncommon cases have been reported in younger patients, especially those with risk factors such as previous head trauma, genetic predisposition, or prior exposure to cadaveric dura. Case Description: We describe a diagnostically challenging case of suspected CAA in a 42-year-old man with a history of multiple traumatic brain injuries, prior neurosurgical interventions, neuropsychiatric symptoms, and late-onset seizures. Importantly, during treatment for head trauma in early adulthood, he received a cadaveric dura transplant. Over subsequent years, he developed recurrent lobar hemorrhages and progressive cognitive and behavioral changes, raising concern for an atypical, possibly iatrogenic, form of CAA. Conclusion: This case highlights the need to consider CAA – even in younger patients – when recurrent lobar hemorrhages and neuropsychiatric symptoms occur in the context of relevant risk factors such as previous head trauma or cadaveric dura exposure. We believe that such exposure may underlie this patient’s gradual but persistent neurological and cognitive decline
New treatments in haemophilia and laboratory testing
Full text linkMožnosti zdravljenja hemofilije so v zadnjih letih močno napredovale. Nove oblike zdravljenja s koncentrati s podaljšanim delovanjem in nefaktorsko zdravljenje omogočajo bolnikom drug način in/ali podaljšan interval med odmerki. Laboratorijsko spremljanje zdravljenja je za obravnavo bolnikov s hemofilijo ključno. Najpogosteje uporabljena metoda za ugotavljanje aktivnosti faktorjev VIII in IX je enostopenjska metoda z aktiviranim parcialnim tromboplastinskim časom. V specializiranih laboratorijih aktivnost faktorjev določajo tudi s kromogeno metodo. Izbor metode in vrste reagenta lahko močno vplivata na rezultat merjenja. Pomembne razlike (> 35 %) med metodama so opisane za modificirane koncentrate rekombinantnih faktorjev s podaljšano življenjsko dobo in pri genskem zdravljenju hemofilije. Preventivno zdravljenje z bispecifičnimi protitelesi zahteva uvedbo več dodatnih diagnostičnih metod pri bolnikih z inhibitorji in brez. Za laboratorijsko spremljanje zmanjšanja aktivnosti naravnih antikoagulantov še ni jasnih smernic. Laboratorij mora natančno vedeti, kakšno obliko zdravljenja bolnik prejema, saj le izbor ustrezne enostopenjske in/ali kromogene metode, ovrednotene za posamezno učinkovino, omogoča primerno prilagojeno zdravljenje hemofilije A ali B