1. Long-term outcome after combined or sequential liver and kidney transplantation in children with infantile and juvenile primary hyperoxaluria type 1Sebastian Loos, Markus J. Kemper, Kaja Schmaeschke, Uta Herden, Lutz Fischer, Bernd Hoppe, Tanja Kersnik-Levart, Enke Grabhorn, Raphael Schild, Jun Oh, 2023, izvirni znanstveni članek Povzetek: Introduction: Combined or sequential liver and kidney transplantation (CLKT/SLKT) restores kidney function and corrects the underlying metabolic defect in children with end-stage kidney disease in primary hyperoxaluria type 1 (PH1). However, data on long-term outcome, especially in children with infantile PH1, are rare. Methods: All pediatric PH1-patients who underwent CLKT/SLKT at our center were analyzed retrospectively. Results: Eighteen patients (infantile PH1 n=10, juvenile PH1 n = 8) underwent transplantation (CLKT n=17, SLKT n = 1) at a median age of 5.4 years (1.5–11.8). Patient survival was 94% after a median follow-up of 9.2 years (6.4–11.0). Liver and kidney survival-rates after 1, 10, and 15 years were 90%, 85%, 85%, and 90%, 75%, 75%, respectively. Age at transplantation was significantly lower in infantile than juvenile PH1 (1.6 years (1.4–2.4) vs. 12.8 years (8.4–14.1), P = 0.003). Median follow-up was 11.0 years (6.8–11.6) in patients with infantile PH1 vs. 6.9 years (5.7–9.9) in juvenile PH1 (P = 0.15). At latest follow-up kidney and/or liver graft loss and/or death showed a tendency to a higher rate in patientswith infantile vs. juvenile PH1 (3/10 vs. 1/8, P=0.59). Discussion: In conclusion, the overall patient survival and long-term transplant outcome of patients after CLKT/SLKT for PH1 is encouraging. However, results in infantile PH1 tended to be less optimal than in patients with juvenile PH1.
Ključne besede: hyperoxaluria, infantile, juvenile, transplantation, outcome
Objavljeno v DiRROS: 10.03.2026; Ogledov: 130; Prenosov: 105
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2. ESR essentials: juvenile idiopathic arthritis : what every radiologist needs to know: practice recommendations by the European Society of Paediatric RadiologySílvia Costa Dias, Celine Habre, Pier Luigi Di Paolo, Paola d’Angelo, Thomas A. Augdal, Oskar W. Angenete, Damjana Ključevšek, 2026, pregledni znanstveni članek Povzetek: Juvenile Idiopathic Arthritis (JIA) is a major contributor to chronic diseases, affecting around 1–2 in 1000 children under the age of 16. With modern treatments, the morbidity has been reduced; however, there is increasing evidence that many, if not most, children with JIA will have a chronic disease with ongoing activity into adulthood. Many studies discuss the possibility of an early window of opportunity in which patients have the best chance of responding to therapy, thereby underscoring the importance of timely and appropriate imaging. Children typically present at 4–5 years of age with one or more stiff and painful joints. If JIA is suspected, the child should undergo an ultrasound of the involved joint(s), performed by a radiologist with experience in paediatric imaging. If this is normal, with no abnormal laboratory tests and low clinical suspicion of JIA, no further imaging is required. If there is inconsistency between ultrasound and clinical findings, then they should proceed to MRI, including intravenous contrast, of the involved joint. Additional radiographs, or low-dose CT for the axial joints to examine for potential destructive change, deformation, or growth abnormalities, should be considered. In children presenting with monoarthritis, bacterial infection must be ruled out.
Ključne besede: child, arthritis, juvenile, diagnostic imaging, ultrasonography, magnetic resonance imaging
Objavljeno v DiRROS: 19.12.2025; Ogledov: 469; Prenosov: 164
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3. Systemic auto-inflammatory manifestations in patients with spondyloarthritisCarla Gaggiano, Mojca Zajc Avramovič, Antonio Vitale, Nina Emeršič, Jurgen Sota, Nataša Toplak, Stefano Gentileschi, Valeria Caggiano, Maria Tarsia, Gašper Markelj, Tina Vesel, Anja Koren Jeverica, Tadej Avčin, 2024, izvirni znanstveni članek Povzetek: Objectives. – (1) characterizing a group of spondyloarthritis (SpA) patients with systemic autoinflammatory symptoms (S-SpA); (2) comparing SpA features with and without auto-inflammatory symptoms; (3) comparing the auto-inflammatory features of S-SpA and Still’s disease (SD). Methods. – Retrospective observational study. Clinical data of adult and pediatric patients with S-SpA, SD or SpA were collected retrospectively and analyzed. Results. – Forty-one subjects with S-SpA, 39 with SD and 42 with SpA were enrolled. The median latency between systemic and articular manifestations in S-SpA was 4.4 (IQR: 7.2) years. S-SpA and SpA had similar frequency of peripheral arthritis and enthesitis (N.S.), while tenosynovitis was more frequent(P = 0.01) and uveitis less frequent (P < 0.01) in S-SpA. MRI showed signs of sacroiliac inflammation and damage in both S-SpA and SpA equally (N.S.). S-SpA patients had less corner inflammatory lesions (P < 0.05) and inflammation at the facet joints (P < 0.01), more interspinous enthesitis (P = 0.01) and inter-apophyseal capsulitis (P < 0.01). Compared to SD, S-SpA patients had lower-grade fever (P < 0.01), less rash (P < 0.01) and weight loss (P < 0.05), but more pharyngitis (P < 0.01), gastrointestinal symptoms (P < 0.01) and chest pain (P < 0.05). ESR, CRP, WBC, ANC, LDH tested higher in SD (P < 0.01). Resolution of systemic symptoms was less frequent in S-SpA than SD on corticosteroid (P < 0.01) and methotrexate (P < 0.05) treatment. When considering all SD patients, a complete response to corticosteroids in the systemic phase significantly reduced the likelihood of developing SpA (OR = 0.06, coefficient −2.87 [CI: −5.0 to −0.8]). Conclusions. – SpA should be actively investigated in patients with auto-inflammatory manifestations, including undifferentiated auto-inflammatory disease and SD
Ključne besede: spondyloarthritis, febrile spondyloarthritis, Still’s disease, systemic juvenile idiopathic arthritis, adult-onset Still’s disease, auto-inflammatory diseases
Objavljeno v DiRROS: 10.12.2025; Ogledov: 479; Prenosov: 163
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5. Influenza vaccine uptake in juvenile idiopathic arthritis during the COVID-19 pandemic : a multi-centre cross-sectional study by PRES vaccination working partyDespoina Maritsi, Noa Alpert, Maša Bizjak, Amit Ziv, Barbora Balažiová, Nataša Toplak, 2025, drugi znanstveni članki Povzetek: Background: Children with rheumatic diseases are at risk for contracting severe influenza and COVID-19 and are thus targeted for these vaccination. Objectives: To assess the influenza (flu) vaccination rate in children with Juvenile Idiopathic Arthritis (JIA), investigate families’ attitudes towards the influenza vaccine, and the effect of the COVID-19 pandemic on flu vaccine uptake. Methods: This multi-centre, cross-sectional study was conducted across 9 countries. JIA caregivers completed an anonymous questionnaire about their children’s influenza vaccination, including the 2019–2020 and 2020–2021 seasons, including knowledge, and perceptions regarding influenza vaccination. Results: Based on responses from 655 JIA caregivers, 152 children (23.2%) received influenza vaccinations in the 2020–2021 season, representing a significant rise from 18.6% in the previous season (p < 0.01). The likelihood of vaccination was higher among employed/self-employed caregivers compared to unemployed (28.2% and 29.9% vs. 13.9%), and those with tertiary education versus elementary (28% vs. 9.7%), both p < 0.01. Concerns of children’s vulnerability to SARS-CoV-2 and severe COVID-19 disease due to JIA were prevalent (51.3% and 85.3% respectively), with 51.3% supporting COVID-19 vaccination. Caregivers who previously vaccinated their children for influenza showed a greater inclination towards SARS-CoV-2 vaccination (73.4% and 79.5%, p < 0.01). Conclusions: Families of children with JIA reported an increasing flu vaccine uptake and a high intention for COVID- 19 vaccine administration. Previous vaccination behavior was shown as a significant predictor of future behaviour. Strengthening health education may address fears and lead to better vaccine coverage against both influenza and SARS-CoV-2 in children with JIA and other inflammatory rheumatic diseases.
Ključne besede: influenza vaccine, juvenile idiopathic arthritis, JIA, COVID-19, vaccination, children
Objavljeno v DiRROS: 09.12.2025; Ogledov: 430; Prenosov: 209
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6. Safety, long-term effectiveness, and immunogenicity of varicella vaccination in children with juvenile idiopathic arthritis treated with biologic therapyMaša Bizjak, Jakob Peterlin, Tadej Avčin, Miroslav Petrovec, Alojz Ihan, Mojca Zajc Avramovič, Gašper Markelj, Tina Vesel, Veronika Osterman, Jerneja Ahčan, Helena Mole, Katja Dejak Gornik, Alenka Biteznik, Sara Jevnikar, Larisa Janžič, Miha Bajc, Andreja Nataša Kopitar, Nataša Toplak, 2025, izvirni znanstveni članek Povzetek: Objective: To evaluate safety, long-term effectiveness and immunogenicity of varicella vaccination in children with JIA, treated with biologic disease-modifying antirheumatic drugs (bDMARDs). Methods: This is a prospective case-control study. VZV-naive patients with JIA on selected bDMARDs (TNFi, IL-6 and IL-1 inhibitors), who were at risk for contracting varicella, had stable disease and normal values of immunoglobulins and lymphocyte populations, were vaccinated against varicella. Adverse events (AEs) and disease activity were followed after vaccination. VZV-specific humoral (VZV-IgG) and cell-mediated immunity (VZV-CMI) were measured at predetermined time points after vaccination by Liaison and intracellular cytokine staining, respectively. Two healthy control (HC) groups comprised 52 healthy children after varicella vaccination and 69 healthy children after varicella infection. Results: 17 patients were vaccinated against varicella (12 on TNFi, 4 on IL-6 inhibitors and 1 on IL-1 inhibitor), of whom 14 patients received both the first and second dose on bDMARDs. No vaccine-strain infections or other serious AEs occurred after vaccination. Disease activity increased in 3/17 (18 %) patients following vaccination. Four out of 17 (24 %) patients developed mild breakthrough varicella (BV) 4 months-4.5 years after vaccination, and none of the HC. Fourteen out of 17 (82 %) patients and 50/52 (96 %) vaccinated HC were seropositive after second vaccination and 8/11 (72 %) patients and 42/43 (98 %) vaccinated HC developed VZV-CMI, which persisted longer compared to VZV-IgG. Patients presented lower antibody levels compared to HC. The rate of VZV-IgG decline was comparable between patients and HC after vaccination or infection. Five patients received the third vaccine dose due to primary or secondary vaccine failure, and none of them developed BV. Conclusions: Varicella vaccination was safe and largely immunogenic in our cohort of JIA patients treated with bDMARDs. Although the vaccination was not always fully effective, it prevented severe disease in all vaccinated patients.
Ključne besede: varicella vaccination, juvenile idiopathic arthritis, biologic therapy, anti-cytokine therapy
Objavljeno v DiRROS: 13.11.2025; Ogledov: 365; Prenosov: 190
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7. Different patterns of inter-annual variability in mean vessel area and tree-ring widths of beech from provenance trials in Slovenia and HungaryJožica Gričar, Domen Arnič, Luka Krajnc, Peter Prislan, Gregor Božič, Marjana Westergren, Mátyás Csaba, Hojka Kraigher, 2024, izvirni znanstveni članek Povzetek: International provenance trials of ecologically and economically important tree species are crucial to deciphering the influence of environmental factors and intraspecific variability on tree growth and performance under climate change to guide assisted gene flow and assisted migration of tree provenances and species. In this context, we compared inter-annual trends in tree-ring widths (carbon sequestration potential) and vessel characteristics (conductivity optimisation) of four beech provenances in two international provenance trials, one in Slovenia (Kamenski hrib, a core beech growing site) and one in Hungary (Bucsuta, a marginal beech site) in 2009–2019. We found different patterns of inter-annual variability in mean vessel area and tree-ring widths among provenances and sites, pointing to diverse genetic background and environmental influence on these two wood-anatomical traits. The average values of the vessel area varied less between provenances at Kamenski hrib than at Bucsuta. Weather conditions differently affected tree-ring width and mean vessel area. Furthermore, the length of the period of response of vessel area to the analysed weather conditions differed in summer and winter periods. The differences in the mean vessel area within the tree ring were more pronounced in the weather-wise extreme years, regardless of the provenance. Consistent with previous studies, we confirmed that site conditions affect the climate sensitivity of trees, which is more pronounced at marginal sites or in extreme years. The findings on how different environmental conditions affect the radial growth of young beech trees of different origin are very important for future forest management.
Ključne besede: Fagus sylvatica, quantitative wood anatomy, common gardens, intraspecific variation, juvenile period, weather conditions
Objavljeno v DiRROS: 03.12.2024; Ogledov: 901; Prenosov: 1212
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8. Tree rings, wood density and climate–growth relationships of four Douglas-fir provenances in sub-Mediterranean SloveniaLuka Krajnc, Jožica Gričar, Jernej Jevšenak, Polona Hafner, Robert Brus, 2022, izvirni znanstveni članek Povzetek: Tree rings, wood density and the climate–growth relationship of four Douglas-fir provenances were analysed separately for the juvenile and adult phases. Four provenances were selected from an existing IUFRO provenance trial planted in 1971 based on their diameter at breast height and vitality. Increment cores were extracted from individual trees, on which we measured tree-ring widths (RW), earlywood widths (EWW) and latewood widths (LWW). Wood density was assessed in standing trees using resistance drilling. The climate–growth correlations were calculated between provenance chronologies of RW, EWW, LWW and latewood share, and the day-wise aggregated Standardised Precipitation-Evapotranspiration Index (SPEI). The analysis was done separately for the juvenile and mature phases of growth. Provenances 1064 (Jefferson) and 1080 (Yelm) exhibited larger annual radial increments than provenances 1028 (Merrit) and 1089 (Cathlamet). The two provenances with the highest annual radial increment in the juvenile phase did not exhibit the same trend in the adult phase. In all provenances, RW, and consequently EWW and LWW, were wider in the juvenile than in adult phase. The share of latewood was in all cases higher in juvenile wood than in mature wood. All four provenances had similar wood densities in both analyzed growth phases. Our analysis showed that when selecting the most promising provenance for planting, possible changes in relative growth rate from the juvenile to adult phase need to be considered.
Ključne besede: Pseudotsuga menziesii, SPEI, juvenile phase, adult phase, latewood share, resistance drilling
Objavljeno v DiRROS: 21.11.2022; Ogledov: 1439; Prenosov: 928
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