Novel Targets in Rare Liver Diseases in Childhood DOI Open Access

Stefan Bittmann

Applied Medical Research, Journal Year: 2024, Volume and Issue: unknown, P. 1 - 11

Published: April 29, 2024

Rare liver diseases are highly diverse and driven by multiple mechanisms that not well understood. Classifying these is challenging, but the European Reference Network (ERN) on rare has proposed categorizing them as autoimmune, infectious, genetic/hereditary, vascular, neoplastic, others with unknown causes.

Language: Английский

Gut Microbiota-Derived Butyrate Induces Epigenetic and Metabolic Reprogramming in Myeloid-Derived Suppressor Cells to Alleviate Primary Biliary Cholangitis DOI
Rui Wang, Bo Li,

Bingyuan Huang

et al.

Gastroenterology, Journal Year: 2024, Volume and Issue: 167(4), P. 733 - 749.e3

Published: May 27, 2024

Language: Английский

Citations

15

Development of a promising PPAR signaling pathway-related prognostic prediction model for hepatocellular carcinoma DOI Creative Commons

Qingmiao Shi,

Yifan Zeng, Xue Chen

et al.

Scientific Reports, Journal Year: 2024, Volume and Issue: 14(1)

Published: Feb. 28, 2024

Abstract The peroxisome proliferator-activated receptor (PPAR) signaling pathway plays a crucial role in systemic cell metabolism, energy homeostasis and immune response inhibition. However, its significance hepatocellular carcinoma (HCC) has not been well documented. In our study, based on the RNA sequencing data of HCC, consensus clustering analyses were performed to identify PPAR pathway-related molecular subtypes, each which displaying varying survival probabilities infiltration status. Following, prognostic prediction model HCC was developed by using random forest method Cox regression analysis. Significant difference outcome, landscape, drug sensitivity pathological features observed between patients with different prognosis. Additionally, decision tree nomogram models adopted optimize model. Furthermore, robustness verified through single-cell RNA-sequencing data. Collectively, this study systematically elucidated that good predictive efficacy for HCC. These findings provide valuable insights further research personalized treatment approaches

Language: Английский

Citations

12

Hyodeoxycholic acid ameliorates cholestatic liver fibrosis by facilitating m6A-regulated expression of a novel anti-fibrotic target ETV4 DOI
Xiaoyong Xue, Runping Liu,

Yajie Cai

et al.

Journal of Hepatology, Journal Year: 2025, Volume and Issue: unknown

Published: Feb. 1, 2025

Language: Английский

Citations

1

Seladelpar treatment reduces IL-31 and pruritus in patients with primary biliary cholangitis DOI Creative Commons
Andreas E. Kremer, Marlyn J. Mayo, Gideon M. Hirschfield

et al.

Hepatology, Journal Year: 2023, Volume and Issue: 80(1), P. 27 - 37

Published: Dec. 20, 2023

Pruritus is a debilitating symptom for many people living with primary biliary cholangitis (PBC). In studies seladelpar, selective peroxisome proliferator-activated receptor-delta agonist, patients PBC experienced significant improvement in pruritus and reduction of serum bile acids. Interleukin-31 (IL-31) cytokine known to mediate pruritus, blocking IL-31 signaling provides relief pruritic skin diseases. This study examined the connection between seladelpar's antipruritic effects acid levels PBC.

Language: Английский

Citations

14

Seladelpar: First Approval DOI

Sheridan M. Hoy

Drugs, Journal Year: 2024, Volume and Issue: unknown

Published: Nov. 22, 2024

Language: Английский

Citations

5

Longitudinal outcomes of obeticholic acid therapy in ursodiol‐nonresponsive primary biliary cholangitis: Stratifying the impact of add‐on fibrates in real‐world practice DOI Creative Commons

Elena Góméz,

J.L. Montero, Ezequiel Molina

et al.

Alimentary Pharmacology & Therapeutics, Journal Year: 2024, Volume and Issue: 59(12), P. 1604 - 1615

Published: May 1, 2024

Summary Background Suboptimal response to ursodeoxycholic acid occurs in 40% of primary biliary cholangitis (PBC) patients, affecting survival. Achieving a deep (normalisation alkaline phosphatase [ALP] and bilirubin ≤0.6 upper limit normal) improves Yet, the long‐term effectiveness second‐line treatments remains uncertain. Aims To evaluate obeticholic (OCA) ± fibrates. Focusing on biochemical (ALP ≤1.67 times normal, with decrease at least 15% from baseline normal levels), normalisation ALP, remission (deep plus aminotransferase normalisation). Methods We conducted longitudinal, observational, multicentre study involving ursodeoxyccholic non‐responsive PBC patients (Paris‐II criteria) Spain Portugal who received OCA Results Of 255 median follow‐up was 35.1 months (IQR: 20.2–53). The whole cohort 47.2%, 61.4% 68.6% 12, 24 36 months. GLOBE‐PBC 5‐year UK‐PBC scores improved ( p < 0.001). Triple therapy (ursodeoxycholic fibrates) had significantly higher rates than dual = 0.001), including ALP normalisation, In multivariate analysis, triple remained independently associated 0.024), Adverse effects occurred 41.2% cases, leading 18.8% discontinuing OCA. Out 55 cirrhosis, 12 developed decompensation. All portal hypertension. Conclusion superior achieving therapeutic goals UDCA‐nonresponsive PBC. Decompensation linked pre‐existing

Language: Английский

Citations

4

The impact of deep response to ursodeoxycholic acid in primary biliary cholangitis – should it be the new clinical standard? DOI

Adrielly Martins,

Cynthia Levy

Current Opinion in Gastroenterology, Journal Year: 2025, Volume and Issue: unknown

Published: Jan. 6, 2025

Purpose of review This explores the emerging concept “deep response” in primary biliary cholangitis (PBC), defined by normalization biochemical markers, particularly alkaline phosphatase and bilirubin. It examines its potential as a new standard for disease management implications long-term patient outcomes, health policies, clinical decision-making. Recent findings studies suggest that achieving deep response significantly improves outcomes some patients with PBC. In particular, significant complication-free survival gain was observed among who at baseline were high risk progression. However, limitations data variability populations pose challenges universal adoption this standard. Summary Deep represents promising optimizing PBC management, offering measurable goals clinicians potentially improved patients. further research is necessary to better define appropriate thresholds, understand risks overprescribing, identify subgroups are most likely benefit from strategy. A balanced, patient-centered approach incorporating into comprehensive could improve care high-risk

Language: Английский

Citations

0

The Role of Ursodeoxycholic Acid Administration During the COVID‐19 Pandemic: A Questionnaire Survey DOI Creative Commons
Cheng Zhou, Ran Jia,

Jinqiu Yang

et al.

Canadian Journal of Infectious Diseases and Medical Microbiology, Journal Year: 2025, Volume and Issue: 2025(1)

Published: Jan. 1, 2025

In December 2022, China classified COVID-19 as a category B infectious disease. This ended 2 years of close epidemiological surveillance COVID-19. The objective this questionnaire was to assess the infection status in pandemic since Henan Province, China, and prevalence people who were taking ursodeoxycholic acid (UDCA) during period. We distributed questionnaires patients attending gastroenterology clinic at First Affiliated Hospital University Chinese Medicine. lasted for 3 weeks total 660 collected, which number UDCA 70. is first investigation into rate among those time pandemic. Our results showed that overall 71.43% (n = 50), with 10% 7) asymptomatic infections, significantly lower than 85.42% 504) 6.27% 37) rates respondents did not take. administration trend toward reducing infection, but difference statistically significant when compared shorter durations medication use. While less 30% participants remained uninfected study period, indicating potential protective effect, it important note complete prevention SARS-CoV-2 by observed.

Language: Английский

Citations

0

The role of circular RNAs in autoimmune diseases: Potential diagnostic biomarkers and therapeutic targets DOI Creative Commons
Xin’ai Li, Junhui Wang, Peng Wang

et al.

The FASEB Journal, Journal Year: 2025, Volume and Issue: 39(2)

Published: Jan. 28, 2025

Abstract With the emergence of high‐quality sequencing technologies, further research on transcriptomes has become possible. Circular RNA (circRNA), a novel type endogenous molecule with covalently closed circular structure through “back‐splicing,” is reported to be widely present in eukaryotic cells and participates mainly regulating gene protein expression various ways. It becoming hotspot non‐coding field. CircRNA shows close relation several varieties autoimmune diseases (AIDs) both physiological pathological level could potentially used clinically terms diagnosis treatment. Here, we focus reviewing importance circRNA AIDs, aim establishing new biomarkers providing insights into understanding role functions AIDs. Specific signaling pathways how RNAs are regulated AIDs will also illustrated this review.

Language: Английский

Citations

0

Recent advances in diagnosis and management of chronic cholestatic liver diseases: expert consensus DOI Open Access

Shrikant Mukewar

International Journal of Research in Medical Sciences, Journal Year: 2025, Volume and Issue: 13(2), P. 967 - 976

Published: Jan. 30, 2025

Chronic cholestatic liver diseases (CCLD), primarily including primary biliary cholangitis (PBC) and sclerosing (PSC), are characterized by impaired bile flow, leading to systemic complications such as pruritus, jaundice, fat soluble vitamin deficiencies progressive damage. Recent advances in diagnostics, particularly the use of non-invasive tests like FIB-4, APRI transient elastography, have significantly improved early detection assessment fibrosis. Ursodeoxycholic acid (UDCA) remains cornerstone treatment for PBC, effectively improving biochemical markers delaying disease progression. In contrast, options PSC remain limited. Emerging therapies targeting synthesis gut microbiota modulation under investigation, offering potential future solutions PSC. Indian clinical setting, other causes intrahepatic cholestasis, alcoholic (ALD) drug-induced injury (DILI), more prevalent. ALD with cholestasis is seen 10-30% patients, while DILI, often driven tuberculosis medications complementary alternative medicines, accounts a significant proportion cases. Infectious hepatitis A fibrosing C patients post-liver transplantation also contribute burden. Experts recommend ongoing UDCA conditions, regular fibrosis assessments, further research into new pharmacological agents both PBC

Language: Английский

Citations

0