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. Assessing life balance of European people with multiple sclerosis : a multicenter clinimetric study within the RIMS networkDaphne Kos, Sofie Ferdinand, Marijke Duportail, Isaline Eijssen, Sofie Schouteden, Lore Kerkhofs, Jelka Janša, Núria Filló, Kathleen M. Matuska, Heleen Beckerman, 2020, izvirni znanstveni članek Povzetek: Background Life balance is defined as “a satisfying pattern of daily activity that is healthful, meaningful, and sustainable to an individual within the context of his or her current life circumstances”. To assess life balance, the self-report instrument Life Balance Inventory (LBI) has been developed in the US. The aim of this study was to evaluate cross-cultural, construct validity and test-retest reliability of translated versions of the LBI in people with multiple sclerosis (MS) within different European cultures (Dutch, Flemish, Slovenian, and Spanish). Method The LBI was translated according to the principles of forward/backward translation and the cultural adaption process of patient-reported outcomes and evaluated in people with MS in each country/language area. LBI (score range 1–3; higher scores refer to better balance) was registered twice with an interval of 7 days to evaluate test-retest reliability using Intraclass Correlation Coefficients (ICCs) and Bland Altman analyses. To evaluate construct validity, Pearson correlations of the LBI with quality of life, fatigue, depression and self-efficacy were explored. Results The total sample (n = 313, 50 ± 11 years of age, MS duration 13 ± 8 years) consisted of five subsamples: Dutch (n = 81, 74% women, 54 ± 9.6 years of age), Flemish 1 (n = 42, 57% women, 49 ± 12 years), Flemish 2 (n = 105, 63% women, 50 ± 10.6 years), Slovenian (n = 48, 79% women, 44 ± 11.2 years) and Spanish (n = 37, 62% women, 47 ± 9.0 years). Baseline total LBI scores differed between subsamples (F(4, 312)=7.19, p < 0.001). ICC [95% CI] of total LBI was 0.88 [0.83–0.92] (Flemish 2), 0.65 [0.39–0.82] (Flemish 1), 0.55 [0.37–0.69] (Dutch), 0.45 [0.15–0.67] (Spanish) and 0.35 [0.07–0.59] (Slovenian). Systematic error was present in one sample; no proportional bias occurred. Correlations ranged from 0.05 to 0.55 for quality of life and self-efficacy, from −0.50 to 0.05 for fatigue and from −0.44 to −0.28 for depression, not fully supporting the hypotheses. Conclusion The study results provide limited support for test-retest reliability, cross-cultural and construct validity of the LBI in different European subsamples. Although LBI may serve as a supportive tool in goalsetting in rehabilitation, the current version of LBI is not recommended for (international) research purposes.
Ključne besede: cross-cultural validation, meaningful activities, patient reported outcome measure (prom), quality of life, rehabilitation
Objavljeno v DiRROS: 10.03.2026; Ogledov: 111; Prenosov: 48
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3. Association between regular physical activity during pregnancy and perinatal outcomes : a population-based cohort studyŠejla Heljezović, Miha Lučovnik, Ivan Verdenik, Darija Šćepanović, 2025, izvirni znanstveni članek Povzetek: Introduction: Regular physical activity during pregnancy is associated with several maternal and neonatal health benefits. However, most studies focus on specific forms of exercise in low-risk populations, limiting generalizability. Objective: To investigate associations between regular physical activity during singleton pregnancy and perinatal outcomes using a national perinatal registry. Methods: A population-based cohort study was conducted using data from the Slovenian National Perinatal Information System (2013–2022), which included 190,331 singleton pregnancies. Regular physical activity was defined as any form of physical activity at least twice weekly throughout pregnancy. The outcomes analyzed included preterm birth (<37 and <32 weeks), preeclampsia, gestational hypertension, gestational diabetes mellitus (GDM), pelvic girdle pain, small-for-gestational-age (SGA) and large-for-gestational-age (LGA) neonates, cesarean delivery, urinary incontinence postpartum, and Apgar scores < 7 at 5 minutes. Multivariate logistic regression adjusted for parity, maternal age, BMI, prepregnancy hypertension and diabetes, and gestational weight gain. Results: Regular physical activity during pregnancy was reported by 69 % of the women. It was significantly associated with reduced odds of preterm birth (<37 weeks, aOR 0.658; <32 weeks, aOR 0.393), preeclampsia (aOR 0.719), gestational hypertension (aOR 0.708), pelvic girdle pain (aOR 0.808), SGA (aOR 0.903), LGA (aOR 0.902) and low Apgar scores (aOR 0.638). Physically active women with GDM were less likely to require insulin (aOR 0.768). No significant associations were found with cesarean delivery or urinary incontinence. Conclusion: Regular physical activity during pregnancy, regardless of specific type, was associated with improved perinatal outcomes without safety concerns. These findings highlight the potential benefits of encouraging physical activity during pregnancy in diverse populations.
Ključne besede: pregnancy, physical activity, perinatal outcome
Objavljeno v DiRROS: 27.02.2026; Ogledov: 144; Prenosov: 54
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4. Editorial: genetic and immunological insights into angioedema without whealsMatija Rijavec, Maurizio Margaglione, Anastasios E. Germenis, 2026, drugi znanstveni članki Ključne besede: angioedema, biomarker, bradykinin, genetic, inflammatory mediators, outcome, real-world data, treatment
Objavljeno v DiRROS: 30.01.2026; Ogledov: 225; Prenosov: 140
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5. Association of preoperative smoking with the occurrence of postoperative pulmonary complications : a post hoc analysis of an observational study in 29 countriesGalina Dorland, W. Saadat, David M.P. van Meenen, Ary Serpa Neto, Michael Hiesmayr, 2025, izvirni znanstveni članek Povzetek: Introduction: While smoking has been consistently identified as a significant contributor to postoperative complications, the existing literature on its association with postoperative pulmonary complications remains conflicting. Aim: We examined the association of preoperative smoking with the occurrence of postoperative pulmonary complications (PPCs). Methods: Post hoc analysis of an observational study in 146 hospitals across 29 countries. We included patients at increased risk of PPCs, according to the Assess Respiratory Risk in Surgical Patients in Catalonia (ARISCAT) score (≥ 26 points). The primary endpoint was the occurrence of one or more predefined PPCs in the first five postoperative days, including unplanned postoperative need for supplementary oxygen, respiratory failure, unplanned need for invasive ventilation, ARDS, pneumonia and pneumothorax. Secondary endpoints included length of hospital stay and in–hospital mortality. We performed propensity score matching to correct for factors with a known association with postoperative outcomes. Results: Out of 2632 patients, 531 (20.2 %) patients were smokers and 2102 (79.8 %) non-smokers. At five days after surgery, 101 (19.0 %) smokers versus 404 (19.2) non–smokers had developed one or more PPCs (P = 0.95). Respiratory failure was more common in smokers (5.1 %) than non–smokers (3.0 %) (P = 0.02), while rates of other PPCs like need for supplementary oxygen, invasive ventilation, ARDS, pneumonia, or pneumothorax did not differ between the groups. Length of hospital stay and mortality was not different between groups. Propensity score matching did not change the findings. Conclusion: The occurrence of PPCs in smokers is not different from non–smokers.
Ključne besede: anaesthesia, intraoperative ventilation, smoking, postoperative outcome, postoperative pulmonary complications, PPCs, respiratory complications
Objavljeno v DiRROS: 23.12.2025; Ogledov: 477; Prenosov: 134
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6. Maternal fatigue after postpartum anemia treatment with intravenous ferric carboxymaltose vs. intravenous ferric derisomaltose vs. oral ferrous sulphate : a randomized controlled trialLea Bombač Tavčar, Hana Hrobat, Lea Gornik, Irena Preložnik Zupan, Marijana Vidmar Šimic, Polona Pečlin, Gorazd Kavšek, Miha Lučovnik, 2024, izvirni znanstveni članek Povzetek: (1) Background: Postpartum anemia is a common maternal complication and is recognized as a cause of impaired quality of life, reduced cognitive abilities, and fatigue. Efficient iron supplementation for the treatment of postpartum anemia is an essential component of high-quality maternal care. The optimal mode of iron supplementation has not been determined yet, whether oral or intravenous. The objective of this study was to compare postpartum anemia treatment with intravenous ferric carboxymaltose, intravenous ferric derisomaltose, and oral ferrous sulfate. (2) Methods: A single-center, open-label, randomized controlled trial. Women with hemoglobin < 100 g/L within 48 h postpartum were randomly allocated to receive intravenous ferric carboxymaltose, intravenous ferric derisomaltose, or oral ferrous sulfate. Intravenous iron was given in one or two doses, while ferrous sulfate was given as two 80 mg tablets once daily. The primary outcome was maternal fatigue measured by the Multidimensional Fatigue Inventory (MFI) six weeks postpartum. Hemoglobin, ferritin, and transferrin saturation levels were analyzed as secondary outcomes. A Kruskal–Wallis test was used for group comparison (p < 0.05 significant). (3) Results: Three hundred women were included. The MFI score at six weeks postpartum did not differ between groups (median 38 (inter-quartile range (IQR) 29–47) in the ferric carboxymaltose group, median 34 (IQR 26–42) in the ferric derisomaltose group, and median 36 (IQR 25–47) in the ferrous sulfate group; p = 0.26). Participants receiving oral iron had lower levels of hemoglobin (135 (131–139) vs. 134 (129–139) vs. 131 (125–137) g/L; p = 0.008), ferritin (273 (198–377) vs. 187 (155–246) vs. 24 (17–37) µg/L; p < 0.001) and transferrin saturation (34 (28–38) vs. 30 (23–37) vs. 24 (17–37) %; p < 0.001) than those receiving ferric carboxymaltose or ferric derisomaltose. (4) Conclusions: Intravenous ferric carboxymaltose, intravenous ferric derisomaltose, and oral ferrous sulfate had similar impacts on maternal fatigue at six weeks postpartum despite improved laboratory parameters in the intravenous groups.
Ključne besede: postpartum anemia, fatigue, intravenous iron treatment, oral iron replacement, patient-centered outcome
Objavljeno v DiRROS: 01.12.2025; Ogledov: 762; Prenosov: 162
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7. Oral Coenzyme Q10 supplementation leads to better preservation of kidney function in steroid-resistant nephrotic syndrome due to primary Coenzyme Q10 deficiencyStefania Drovandi, Beata Lipska-Zietkiewicz, Fatih Ozaltin, Francesco Emma, Bora Gülhan, Olivia Boyer, Agnes Trautmann, Hong Xu, Tanja Kersnik-Levart, 2022, izvirni znanstveni članek Povzetek: Primary Coenzyme Q10 (CoQ(10)) deficiency is an ultra-rare disorder caused by defects in genes involved in CoQ(10) biosynthesis leading to multidrug-resistant nephrotic syndrome as the hallmark kidney manifestation. Promising early results have been reported anecdotally with oral CoQ(10) supplementation. However, the long-term efficacy and optimal prescription remain to be established. In a global effort, we collected and analyzed information from 116 patients who received CoQ(10) supplements for primary CoQ(10) deficiency due to biallelic pathogenic variants in either the COQ2, COQ6 or COQ8B genes. Median duration of follow up on treatment was two years. The effect of treatment on proteinuria was assessed, and kidney survival was analyzed in 41 patients younger than 18 years with chronic kidney disease stage 1-4 at the start of treatment compared with that of an untreated cohort matched by genotype, age, kidney function, and proteinuria. CoQ(10) supplementation was associated with a substantial and significant sustained reduction of proteinuria by 88% at 12 months. Complete remission of proteinuria was more frequently observed in COQ6 disease. CoQ(10) supplementation led to significantly better preservation of kidney function (5-year kidney failure-free survival 62% vs. 19%) with an improvement in general condition and neurological manifestations. Side effects of treatment were uncommon and mild. Thus, our findings indicate that all patients diagnosed with primary CoQ(10) deficiency should receive early and life-long CoQ(10) supplementation to decelerate the progression of kidney disease and prevent further damage to other organs.
Ključne besede: coenzyme Q10, deficiency, supplementation therapy, end-stage kidney disease, ESKD, genetic kidney disease, hereditary, kidney survival, outcome, proteinuria reduction
Objavljeno v DiRROS: 21.11.2025; Ogledov: 347; Prenosov: 175
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8. Patient-reported outcome measures in atopic dermatitis and chronic urticaria are underused in clinical practiceIvan Cherrez-Ojeda, Jean Bousquet, Ana M. Giménez-Arnau, Kiran V Godse, Dorota Krasowska, Joanna Bartosińska, Paulina Szczepanik-Kułak, Bartłomiej Wawrzycki, Pavel Kolkhir, Anastasiia Allenova, Mitja Košnik, 2024, izvirni znanstveni članek Povzetek: Background
Patient-reported outcome measures (PROMs) are validated and standardized tools that complement physician evaluations and guide treatment decisions. They are crucial for monitoring atopic dermatitis (AD) and chronic urticaria (CU) in clinical practice, but there are unmet needs and knowledge gaps regarding their use in clinical practice.
Objecctive
We investigated the global real-world use of AD and CU PROMs in allergology and dermatology clinics as well as their associated local and regional networks.
Methods
Across 72 specialized allergy and dermatology centers and their local and regional networks, 2,534 physicians in 73 countries completed a 53-item questionnaire on the use of PROMs for AD and CU.
Results
Of 2,534 physicians, 1,308 were aware of PROMs. Of these, 14% and 15% used PROMs for AD and CU, respectively. Half of physicians who use PROMs do so only rarely or sometimes. Use of AD and CU PROM is associated with being female, younger, and a dermatologist. The Patient-Oriented Scoring Atopic Dermatitis Index and Urticaria Activity Score were the most common PROMs for AD and CU, respectively. Monitoring disease control and activity are the main drivers of the use of PROMs. Time constraints were the primary obstacle to using PROMs, followed by the impression that patients dislike PROMs. Users of AD and CU PROM would like training in selecting the proper PROM.
Conclusions
Although PROMs offer several benefits, their use in routine practice is suboptimal, and physicians perceive barriers to their use. It is essential to attain higher levels of PROM implementation in accordance with national and international standards.
Ključne besede: allergy, atopic dermatitis, chronic urticaria, dermatology, patient reported outcome measures
Objavljeno v DiRROS: 02.07.2025; Ogledov: 543; Prenosov: 316
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9. Usage prevalence of angioedema patient-reported outcome measures : results from the UCARE and ACARE PROMUSE studyIvan Cherrez-Ojeda, Jean Bousquet, Ana M. Giménez-Arnau, Kiran V Godse, Dorota Krasowska, Joanna Bartosińska, Paulina Szczepanik-Kułak, Bartłomiej Wawrzycki, Pavel Kolkhir, Anastasiia Allenova, Mitja Košnik, 2024, drugi znanstveni članki Ključne besede: allergy, angioedema, measures, results of tests, patient reported outcome measures
Objavljeno v DiRROS: 02.07.2025; Ogledov: 535; Prenosov: 503
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10. Gut microbiome in cancer : the next big opportunity for better patient outcomes?Jure Povšin, Timotej Sotošek, Metka Novak, Barbara Breznik, 2025, pregledni znanstveni članek Povzetek: The gut microbiome, a diverse community of microorganisms in the human body, plays an important role in maintaining health and influences various processes such as digestion, immunity, and protection against pathogens. A person's unique gut microbiome, shaped by factors such as birth method, diet, antibiotics, and lifestyle, contributes to bodily functions such as nutrient metabolism, drug processing, and im-mune regulation. Changes in the gut microbiome are associated with a predisposition to cancer and can influence the effectiveness of cancer treatments. Dysbiosis in the gut microbiome can lead to inflammation, tumor development, and metastasis, highlight-ing its importance in cancer research and prevention. The gut microbiota significantly influences cancer development and treatment outcomes. Certain bacteria enhance the effects of therapies such as cyclophosphamide and contribute to the body's im-mune response against tumors. Microbes produce anti-cancer molecules and probiotic compounds, making them potential tools in cancer prevention and treatment. Future research aims to develop targeted antibiotics and explore fecal microbiota transfer to selectively manipulate the microbiota for improved cancer treatment. Due to genetic and physiological similarities, mouse models are invaluable in biomedical research. However, because the gut microbiome of humans and mice and the composition of the tumor microenvironment differ, direct comparison between these two models can be challenging in research. Bridging these gaps is crucial for comparative medicine, especially in cancer research where the microbiome plays an important role in treat-ment outcomes. One important area where the gut microbiome could offer potential new treatment options is in primary brain tumors such as gliomas. To date, there are no long-lasting effective treatments for this type of cancer, but research in mouse models shows a link between tumor progression and response to treatment with changes in the gut microbiome. Overall, the gut microbiome and its modulation represent an opportu-nity for more efficient future cancer treatment.
Ključne besede: gut microbiome, cancer, treatment outcome, tumor models, glioma
Objavljeno v DiRROS: 03.04.2025; Ogledov: 976; Prenosov: 438
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