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1.

001-es BibID:BIBFORM124893
035-os BibID:(scopus)85199625554 (wos)001276665500001
Első szerző:Aradi Zsófia (belgyógyász szakorvos)
Cím:Polyarthritis in Sjögren's Syndrome : Difficulties in Distinguishing Extraglandular Manifestation and Associated Rheumatoid Arthritis / Aradi Zsófia, Nagy Gábor, Horváth Ildikó Fanny, Antal-Szalmás Péter, Szántó Antónia
Dátum:2024
ISSN:2075-4418
Megjegyzések:Aim of the study was to investigate the demographic data and disease course characteristics of patients with Sjögren's syndrome (SS) and inflammatory joint pain of various origins and to search for factors that might help with the distinction of polyarthritis as an extraglandular manifestation and rheumatoid arthritis as an associated systemic autoimmune disorder. A total of 355 patients were retrospectively analyzed, 128 of whom served as controls (SS-C), while 159 had polyarthritis as an extraglandular symptom of Sjögren's syndrome (SS-pa) and 68 were diagnosed as having associated rheumatoid arthritis (SS-RA). The patients without any inflammatory joint manifestations were significantly older than the SS-pa patients, while, for the SS-RA group, the difference was not significant. The onset of joint pain appeared significantly earlier in the SS-RA patients. Regarding either extraglandular manifestations or associated autoimmune disorders, there were significant differences between the controls and both SS-pa and SS-RA groups, while no significant difference was found between the SS-pa and SS-RA groups. Thus, laboratory and imaging methods should be used to differentiate between the two conditions, but laboratory biomarkers are even more important for early diagnosis. A ROC curve analysis showed an acceptable diagnostic accuracy in differentiating between SS-pa and SS-RA patients using a binary logistic regression model, where highly positive rheumatoid factor (RF) and anti-cyclic citrullinated peptide (CCP) values, kidney involvement, and anti-Ro/SS-A positivity were shown to significantly raise the odds of having RA, whereas anti-La/SS-B positivity seemed to have a protective role, since it significantly decreased the odds of having it. Further biomarkers are needed to better classify SS patient cohorts with inflammatory joint pain of different origins and, consequently, different management requirements.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's syndrome
associated rheumatoid arthritis
polyarthritis
extraglandular manifestation
Megjelenés:Diagnostics. - 14 : 14 (2024), p. 1-12.-
További szerzők:Nagy Gábor (1974-) (laboratóriumi szakorvos, laboratóriumi hematológus és immunológus) Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Antal-Szalmás Péter (1968-) (laboratóriumi szakorvos) Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus)
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2.

001-es BibID:BIBFORM124078
035-os BibID:(scopus)85203631691 (WoS)001312824900001
Első szerző:Barna Sándor (kutató orvos)
Cím:[131I]6β-Iodomethyl-19-norcholesterol SPECT/CT for the Lateralization of Mineralocorticoid Overproduction in Primary Aldosteronism / Barna Sandor, Sira Livia, Bhattoa Harjit Pal, Toth Laszlo, Czine Zsigmond, Szoboszlay Lilla, Nagy Edit B., Kepes Zita, Garai Ildiko, Bodor Miklos, Varga Jozsef, Nagy Endre V.
Dátum:2024
ISSN:2075-4418
Megjegyzések:Primary: aldosteronism is a frequent cause of secondary hypertension. With access to specialized care, an increasing number of patients with aldosteronism are being identified. Primary aldoste-ronism is treatable by adrenal surgery if aldosterone excess originates from one of the two, and not from both, adrenals. Bilateral hyperplasia requires lifelong mineralocorticoid receptor antago-nist treatment. Up till now, adrenal venous sampling (AVS) has been widely used to distinguish between one-sided and two-sided aldosterone overproduction and patient selection for surgery. AVS is an invasive technique, and the unsuccessful sampling of the right adrenal vein during AVS often prevents side comparison, making the AVS procedure useless. Molecular imaging using [131I]6?-iodomethyl-19-norcholesterol with SPECT CT imaging (SPECT/CT) may be a potential al-ternative. Methods: In 42 consecutive patients with confirmed primary aldosteronism, molecular imaging has been performed. After dexamethasone suppression of the non-affected adrenal tis-sue, 37 MBq [131I]6?-iodomethyl-19-norcholesterol was injected i.v., and SPECT/CT images were taken 7 days later. Based on the visual evaluation of the images by two nuclear medicine special-ists, patients with one-sided tracer accumulation underwent adrenalectomy. To identify a SPECT/CT parameter that best characterizes the side difference, the maximum counts and the mean counts of spherical VOIs were analyzed. Results: Of the 42 patients, 24 had one-sided aldos-terone overproduction by SPECT/CT. After surgical removal of the involved adrenal, all 24 pa-tients with SPECT/CT-identified unilateral aldosteronism achieved biochemical cure, defined as a normalized potassium level combined with an aldosterone-to-renin ratio ? 30. To identify the best measurable parameter of SPECT/CT side difference, the mean counts and maximum counts of a series of spherical VOIs of different diameters were analyzed. The ratio of the mean counts of 3 cm spherical VOIs of the right and left adrenal regions (lateralization index) was the best discrimi-nator; a ratio of ?1.29 was characteristic of one-sided disease, without overlap between the one-sided and two-sided patient groups. Conclusions: [131I]6?-iodomethyl-19-norcholesterol SPECT/CT with a count-based image interpretation and side-ratio calculation may be an equipol-lent non-invasive substitute for adrenal venous sampling in the lateralization of mineralocorticoid overproduction. It reliably identifies unilateral disease and facilitates patients' selection for surgical intervention. If confirmed by others, this functional imaging may replace AVS when lateralization is required for management decisions in primary aldosteronism
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
hypertension
primary aldosteronism
[131I]6?-iodomethyl-19-norcholesterol
SPECT/CT
Megjelenés:Diagnostics. - 14 : 17 (2024), p. 1-10.-
További szerzők:Sira Lívia (1973-) (endokrinológus) Bhattoa Harjit Pal (1973-) (laboratóriumi szakorvos) Tóth László (1971-) (patológus) Czine Zsigmond Szoboszlay Lilla Nagy Edit B. Képes Zita (1991-) (orvos) Garai Ildikó (1966-) (radiológus) Bodor Miklós (1969-) (belgyógyász, endokrinológus) Varga József (1955-) (fizikus) Nagy Endre V. (1957-) (belgyógyász, endokrinológus)
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3.

001-es BibID:BIBFORM114632
035-os BibID:(scopus)85172226742 (wos)001073257100001
Első szerző:Janka Tamás
Cím:The Value of Neutrophil-to-Lymphocyte Ratio to Identify Bacterial Infection and Predict Short-Term Mortality in Patients with Acutely Decompensated Cirrhosis / Tamás Janka, Dávid Tornai, Mária Papp, Zsuzsanna Vitális
Dátum:2023
ISSN:2075-4418
Megjegyzések:Liver cirrhosis patients are highly susceptible to infections, affecting survival, but current param-eters for detecting infection are not reliable enough in this population. We investigated ability of white blood cell (WBC), ?WBC, neutrophil and ?neutrophil count, neutrophil-to-lymphocyte (NLR) and ?NLR ratio, CRP, and PCT to identify infection and predict short-term mortality in liver cir-rhosis patients. We recruited 233 patients with liver cirrhosis hospitalized with acute decompen-sation (AD) who had an outpatient visit within 1 month (baseline laboratory data) and followed them for 90 days. Difference between laboratory values at baseline and the AD episode was defined as delta (?) values of the parameters. Delta values did not increase the diagnostic and predictive ability of investigated parameters. CRP was found to be the best diagnostic marker for infection in patients with cirrhosis. However, NLR seems to be superior for short-term mortality prediction, better than WBC. Distinguishing inflammations of different origin is a remaining clinical challenge in acutely decompensated cirrhosis. Based on our results NLR might be more suitable for predicting short-term mortality in patients with AD than WBC count currently included in CLIF-C AD score.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:Diagnostics. - 13 : 18 (2023), p. 1-14. -
További szerzők:Tornai Dávid (1989-) (hepatológia, biomarker kutatás) Papp Mária (1975-) (belgyógyász, gasztroenterológus) Vitális Zsuzsanna (1963-) (belgyógyász, gasztroenterológus)
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4.

001-es BibID:BIBFORM128484
035-os BibID:(Scopus)86000504826 (WoS)001443305400001
Első szerző:Jóna Ádám (belgyógyász, hematológus)
Cím:Prognostication of Follicular Lymphoma : a Review of Prognostic Scores and Factors / Ádám Jóna, Evelin Kiss, Árpád Illés
Dátum:2025
ISSN:2075-4418
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:Diagnostics. - 15 : 5 (2025), p. 1-17. -
További szerzők:Kiss Evelin (1992-) (belgyógyász) Illés Árpád (1959-) (belgyógyász, haematológus, onkológus)
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5.

001-es BibID:BIBFORM126755
035-os BibID:(scopus)85211810776 (wos)001376955900001
Első szerző:Mezei Kincső (orvos)
Cím:Obesity : Friend or Foe in Sjögren's Syndrome Patients? / Mezei Kincső, Nagy Laura, Orosz Viktória, Aradi Zsófia, Bói Bernadett, Szántó Antónia
Dátum:2024
ISSN:2075-4418
Megjegyzések:Background/Objectives: In Sjögren's syndrome, exocrine glands are destructed in an autoimmune-mediated process. Obesity is known to influence a wide range of diseases. This study aimed to examine whether obesity has an impact on the disease course of our patients with Sjögren's syndrome. Methods: Out of the regularly followed-up patients, 125 were grouped based on their body mass index (BMI). Below a BMI of 25, they were listed as "non-obese" (n = 45), whereas above a BMI of 25, they were categorized as "obese" (n = 80). Demographic, laboratory, and immunological parameters; Sjögren's syndrome disease activity index; certain extraglandular manifestations; and treatment modalities were compared using biostatistical methods. Results: Among the examined cardiovascular and cerebrovascular co-morbidities, type 2 diabetes and hypertension were significantly more frequent in the obese group. Considering the associated further autoimmune disorders and extraglandular manifestations, in our patients, there were no significant differences between the two groups. Among laboratory parameters, gamma glutamil transferase, alanine transaminase, hemoglobin, hematocrit, lymphocyte rate, triglyceride, and c3 and c4 complement levels were significantly higher in the obese group, while the proportion of rheumatoid factor positivity and the neutrophil granulocyte rate were significantly lower. Immunoglobulin G, A, and M levels did not differ significantly between the two subsets. Obese patients needed steroid therapy significantly less frequently; however, statin therapy was remarkably more frequent in that group. Furthermore, the European League Against Rheumatism (EULAR) Sjögren's syndrome disease activity index (ESSDAI) was significantly lower in the group of overweight patients. Conclusions: Our results suggest that several immunological parameters of obese patients are more favorable compared to those with normal body weight. Behind that, we might suspect either the beneficial effect of statin therapy and/or the obesity paradox.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's syndrome
obesity
statin
obesity paradox
Megjelenés:Diagnostics. - 14 : 23 (2024), p. 1-11. -
További szerzők:Nagy Laura Orosz Viktória (1988-) (orvos) Aradi Zsófia (1992-) (belgyógyász szakorvos) Bói Bernadett Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus)
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6.

001-es BibID:BIBFORM106266
035-os BibID:(Scopus)85145924688 (WoS)000908868000001
Első szerző:Módis László (neurológus)
Cím:Central Nervous System Involvement in Primary Sjögren's Syndrome : Narrative Review of MRI Findings / Módis László V., Aradi Zsófia, Horváth Ildikó Fanny, Bencze János, Papp Tamás, Emri Miklós, Berényi Ervin, Bugán Antal, Szántó Antónia
Dátum:2023
ISSN:2075-4418
Megjegyzések:Central nervous system (CNS) involvement is one of the numerous extraglandular manifestations of primary Sjögren's syndrome (pSS). Moreover, neurological complaints precede the sicca symptoms in 25-60% of the cases. We review the magnetic resonance imaging (MRI) lesions typical for pSS, involving the conventional examination, volumetric and morphometric studies, diffusion tensor imaging (DTI) and resting-state fMRI. The most common radiological lesions in pSS are white matter hyperintensities (WMH), scattered alterations hyperlucent on T2 and FLAIR sequences, typically located periventricularly and subcortically. Cortical atrophy and ventricular dilatation can also occur in pSS. Whilst these conditions are thought to be more common in pSS than healthy controls, DTI and resting-state fMRI alterations demonstrate evident microstructural changes in pSS. As pSS is often accompanied by cognitive symptoms, these MRI alterations are expectedly related to them. This relationship is not clearly delineated in conventional MRI studies, but DTI and resting-state fMRI examinations show more convincing correlations. In conclusion, the CNS manifestations of pSS do not follow a certain pattern. As the link between the MRI lesions and clinical manifestations is not well established, more studies involving larger populations should be performed to elucidate the correlations.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's syndrome
central nervous system
magnetic resonance imaging
white matter hyperintensities
Megjelenés:Diagnostics. - 13 : 1 (2023), p. 1-18. -
További szerzők:Aradi Zsófia (1992-) (belgyógyász szakorvos) Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Bencze János (1991-) (orvos) Papp Tamás (1987-) (orvos) Emri Miklós (1962-) (fizikus) Berényi Ervin (1964-) (radiológus) Bugán Antal (1948-) (pszichológus, klinikai szakpszichológus, pszichoterapeuta) Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus)
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7.

001-es BibID:BIBFORM083015
035-os BibID:(WoS)000513129500033 (Scopus)85078461475
Első szerző:Molnár Csaba (pathológus)
Cím:High Genetic Diversity and No Evidence of Clonal Relation in Synchronous Thyroid Carcinomas Associated with Hashimoto's Thyroiditis : a Next-Generation Sequencing Analysis / Molnár Csaba, Bádon Emese Sarolta, Mokánszki Attila, Mónus Anikó, Beke Lívia, Győry Ferenc, Nagy Endre, Méhes Gábor
Dátum:2020
ISSN:2075-4418
Megjegyzések:The close association between pre-existing Hashimoto's thyroiditis and thyroid cancer is well established. The simultaneous occurrence of multiple neoplastic foci within the same organ suggests a common genotoxic effect potentially contributing to carcinogenesis, the nature of which is still not clear. Next-generation sequencing (NGS) provides a potent tool to demonstrate and compare the mutational profile of the independent neoplastic foci. Our collection of 47 cases with thyroid carcinoma and Hashimoto's thyroiditis included 14 with at least two tumorous foci. Detailed histological analysis highlighted differences in histomorphology, immunoprofile, and biological characteristics. Further, a 67-gene NGS panel was applied to demonstrate the mutational diversity of the synchronic tumors. Significant differences could be detected with a wide spectrum of pathogenic gene variants involved (ranging between 5 and 18, cutoff >5.0 variant allele frequencies (VAF)). Identical gene variants represented in both synchronous tumors of the same thyroid gland were found in only two cases (BRAF and JAK3 genes). An additional set of major driver mutations was identified at variable allele frequencies in a highly individual setup suggesting a clear clonal independence. The different BRAF statuses in coincident thyroid carcinoma foci within the same organ outline a special challenge for molecular follow-up and therapeutic decision-making.
Tárgyszavak:Orvostudományok Elméleti orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:Diagnostics. - 10 : 1 (2020), p. 1-11. -
További szerzők:Bádon Emese Sarolta (1991-) (orvos) Mokánszki Attila (1983-) (molekuláris biológus Ph.D hallgató) Mónus Anikó Beke Lívia (1971-) (orvosdiagnosztikai laboratóriumi analitikus) Győry Ferenc (1969-) (kardiológus) Nagy Endre V. (1957-) (belgyógyász, endokrinológus) Méhes Gábor (1966-) (patológus)
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8.

001-es BibID:BIBFORM114463
035-os BibID:(Scopus)85172253351
Első szerző:Obajed Al-Ali Nóra (okleveles orvosdoktor)
Cím:One step back from bedside to the bench - How do different arterial stiffness parameters behave in relation to peripheral resistance? / Nóra Obajed Al-Ali, Sára Rebeka Tóth, László Váróczy, László Imre Pinczés, Pál Soltész, Zoltán Szekanecz, György Kerekes
Dátum:2023
ISSN:2075-4418
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
arterial stiffness
cardiovascular risk
elasticity parameters
distensibility coefficient
pulse wave velocity
oscillometry
applanation tonometry
Megjelenés:Diagnostics. - 13 (2023), p. 1-14. -
További szerzők:Tóth Sára Váróczy László (1974-) (belgyógyász, haematológus) Pinczés László Imre (1990-) (hematológus) Soltész Pál (1961-) (belgyógyász, kardiológus) Szekanecz Zoltán (1964-) (reumatológus, belgyógyász, immunológus) Kerekes György (1973-) (belgyógyász, kardiológus, angiológus)
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9.

001-es BibID:BIBFORM124654
035-os BibID:(scopus)85206580621 (wos)001331719000001
Első szerző:Orosz Mónika (Szülész-nőgyógyász)
Cím:Pre-Conception Androgen Levels and Obstetric Outcomes in Polycystic Ovary Syndrome : a Single-Center Retrospective Study / Mónika Orosz, Fanni Borics, Dávid Rátonyi, Beáta Vida, Szilvia Csehely, Attila Jakab, Luca Lukács, Rudolf Lampé, Tamás Deli
Dátum:2024
ISSN:2075-4418
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
polycystic ovary syndrome
in vitro fertilization
gestational diabetes mellitus
androgen levels
pre-pregnancy hormone levels
obstetric outcomes
Megjelenés:Diagnostics. - 14 (2024), p. 1-15. -
További szerzők:Borics Fanni (1995-) (orvos) Rátonyi Dávid (1996-) (Szülész-nőgyógyász) Vida Beáta (1994-) (szülész-nőgyógyász) Csehely Szilvia (1990-) (szülész-nőgyógyász szakorvos) Jakab Attila (1964-) (szülész-nőgyógyász, endokrinológus) Lukács Luca (1995-) (hallgató) Lampé Rudolf (1983-) (szülész-nőgyógyász) Deli Tamás (1979-) (szülész-nőgyógyász, endokrinológus szakorvos)
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10.

001-es BibID:BIBFORM108355
035-os BibID:(WoS)000933828200001 (Scopus)85147861701
Első szerző:Rákóczi Éva (klinikai szakorvos)
Cím:Evaluation of the Efficacy of BBIBP-CorV Inactivated Vaccine Combined with BNT62b2 mRNA Booster Vaccine / Éva Rákóczi, Gusztáv Magócs, Sára Kovács, Béla Nagy, Gabriella Szűcs, Zoltán Szekanecz
Dátum:2023
ISSN:2075-4418
Megjegyzések:Abstract: Objectives: In this prospective study, SARS-CoV??2 spike protein specific total immunoglobulin (Ig) levels were analyzed before and after BNT162 b2 mRNA booster vaccination in individuals previously administered with two doses of BBIBP-CorV vaccine in comparison to immunized participants with three doses of BNT162 b2 vaccination. Methods: Sixty-one Caucasian volunteers (39 females, 22 males) vaccinated by BBIBP-CorV were included (mean age: 63.9 years). Sixty-one patients (41 females, 20 males) as controls were vaccinated with BNT162b2 (mean age: 59.9 years). Both groups received the third booster BNT162b2 vaccine. Total anti-SARS-CoV??2 S1-RBD Ig levels were measured by an immunoassay (Roche Diagnostics) and their calculated ratios after/before booster dose were compared between the two groups. Results: At baseline, significantly lower anti-SARS-CoV??2 S1-RBD total antibody levels were determined after initial immunization by two doses of inactivated BBIBP-CorV compared to BNT62b2 mRNA vaccine (p < 0.001). After BNT162b2 boosters, similarly high total Ig levels were detected in both the heterologous (27,195 [15,604?42,754] BAU/mL, p < 0.001) and the homologous booster cohort (24,492 [13,779??42,671] BAU/mL, p < 0.001) compared to baseline. Hence, the ratio of after/before total Ig levels was significantly higher with heterologous vs homologous immunization (p < 0.001). Conclusion: To address the concept that basic BBIBP-CorV vaccination is not as effective as BNT162b, we analyzed the effect of heterologous vaccination with BNT162b2. Our results suggest that BNT162b2 can successfully boost the effects of two-dose BBIBP-CorV vaccination.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
COVID-19
BBIBP-CorV
BNT62b2
booster
vaccine
Megjelenés:Diagnostics. - 13 : 3 (2023), p. 1-12. -
További szerzők:Magócs Gusztáv Kovács Sára Nagy Béla Jr. (1980-) (labordiagnosztikai szakorvos) Szűcs Gabriella (1963-) (belgyógyász, allergológus és klinikai immunológus, reumatológus) Szekanecz Zoltán (1964-) (reumatológus, belgyógyász, immunológus)
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11.

001-es BibID:BIBFORM094040
Első szerző:Szekanecz Zoltán (reumatológus, belgyógyász, immunológus)
Cím:Rheumatoid arthritis diagnosis with antimutated citrullinated vimentin ELISA by Orgentec Diagnostika / Szekanecz Zoltán, Gabriella Lakos
Dátum:2008
ISSN:1753-0059 1753-0067
Megjegyzések:Background: There is a need for highly sensitive and specific laboratory tests for the early diagnosis of rheumatoid arthritis (RA). Anticitrullinated protein/peptide antibodies are produced in the sera of RA patients. Objective: To assess and discuss the diagnostic value of Orgentec antimutated citrullinated vimentin (anti-MCV) ELISA and to compare this diagnostic to commercially available anticyclic citrullinated peptide (anti-CCP) tests. Methods: The evaluation is based on our recently published study on the comparison of anti-MCV and anti-CCP2 tests, as well as on published studies. Results/conclusion: In established RA, the anti-MCV ELISA exerts a specificity comparable with anti-CCP2. Numerous recent studies suggest that anti-MCV may have higher sensitivity, however there are also some publications stating the opposite. In early RA, anti-MCV may be a better predictor of disease activity and radiological progression than anti-CCP tests.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
anricyclic citrullinared pepride
anrirnutated citrullinared vimenrin
rheumatoid arthritis
Megjelenés:Expert opinion on medical diagnostics. - 2 : 9 (2008), p. 1083-1090. -
További szerzők:Lakos Gabriella (1963-) (laboratóriumi szakorvos, transzfúziológus, immunológus)
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12.

001-es BibID:BIBFORM132230
Első szerző:Vén Péter László
Cím:Longitudinal Comparison of Currently Used Risk Scores for Prognostication of Primary Sclerosing Cholangitis (PSC) in a Hungarian Bicenter PSC Cohort / Ven Peter Laszlo, Tornai David, Toth Bence, Vitalis Zsuzsanna, Tornai Istvan, Tornai Tamas, Par Gabriella, Papp Maria
Dátum:2025
ISSN:2075-4418
Megjegyzések:Abstract Background/Objectives: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with limited epidemiological data from Central?Eastern Europe. This study characterized a Hungarian PSC cohort, comparing patients with and without inflammatory bowel disease (IBD), and longitudinally evaluated the predictive efficacy of established prognostic scores (Mayo Risk Score, Amsterdam-Oxford Model [AOM], UK-PSC short/long). Methods: Data from 135 PSC patients (median diagnosis age 31 years, 57.7% male) were collected yearly at two Hungarian centers, with a median follow-up of 8.8 years. Outcomes included liver transplantation (LT) and liver-related death. Prognostic value of baseline clinical scores was assessed for 2-, 5-, 8-, and 10-year composite outcome. Results: PSC-IBD patients (54.1%) were younger with higher baseline Mayo and AOM scores, and had increased rates of colorectal carcinoma (8.22% vs. 0.00%) and liver transplantation (26.03% vs. 9.68%) within 10 years than PSC-only patients. There were no differences in liver-related mortality or composite outcomes between the groups. All prognostic scores showed good short-term predictive ability for poor outcomes (AUROC at 2 years: 0.858?0.958), which diminished over time (AUROC at 10 years: 0.708?0.756). The AOM demonstrated the most consistent performance. Persistent alkaline phosphatase (ALP) elevation (?2.2?ULN) 2 years post-diagnosis, despite ursodeoxycholic acid therapy, strongly predicted 10-year adverse outcomes (HR: 3.927, p < 0.001), outperforming formal scoring systems (HR: 2.688?1.522). Conclusions: While PSC-IBD patients had more CRC and liver transplantation, overall transplantation-free survival was similar to PSC-only patients. Prognostic utility of current scores declines with longer follow-up; AOM was most stable. Sustained ALP elevation is a robust long-term prognostic indicator.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
longitudinal assessment
prognostic scores
cholestatic disease
autoimmune liver disease (AILD)
inflammatory bowel disease (IBD)
epidemiology
composite outcome
Megjelenés:Diagnostics. - 15 : 17 (2025), p. 1-15. -
További szerzők:Tornai Dávid (1989-) (hepatológia, biomarker kutatás) Tóth Bence (1995-) (általános orvos, belgyógyász szakorvos) Vitális Zsuzsanna (1963-) (belgyógyász, gasztroenterológus) Tornai István (1954-) (belgyógyász, gasztroenterológus) Tornai Tamás István (1984-) (belgyógyász) Pár Gabriella Papp Mária (1975-) (belgyógyász, gasztroenterológus)
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