Novel Targets in Rare Liver Diseases in Childhood DOI Open Access

Stefan Bittmann

Applied Medical Research, Год журнала: 2024, Номер unknown, С. 1 - 11

Опубликована: Апрель 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.

Язык: Английский

Refining the Understanding of Autoimmune Liver Disease Mortality DOI Open Access

Lingyun Zhu,

Xingshi Hua,

Libin Zhan

и другие.

Liver International, Год журнала: 2025, Номер 45(3)

Опубликована: Фев. 20, 2025

ABSTRACT The comprehensive population‐based cohort study of autoimmune liver disease (AILD) mortality by Lasyte et al. provides valuable epidemiological insights. Several methodological perspectives merit consideration, including public database limitations, temporal trends and the role chronic kidney as an unmeasured covariate in risk assessment. These elements further enhance interpretation this robust AILD analysis.

Язык: Английский

Процитировано

0

Combination of hemochromatosis and primary biliary cholangitis DOI Creative Commons
Galiya R. Bicbavova, D. G. Belyakov, В. А. Ахмедов

и другие.

Experimental and Clinical Gastroenterology, Год журнала: 2025, Номер 9, С. 217 - 222

Опубликована: Март 6, 2025

A clinical case of an extremely rare morphologically verified combination primary biliary cholangitis and hemochromatosis in a patient is presented. brief description literature reference on these diseases are given from the standpoint modern approach to diagnostics, classification, manifestations treatment. The presented observation considers course disease onset present time, including stage self-induced cessation taking necessary medications seeking medical help, which led rapid progression with pronounced appearance typical color skin, xanthelasma, hepatosplenomegaly hepatocellular insufficiency. data laboratory instrumental examination conducted various clinics analyzed. It important note that liver cirrhosis as result has very unfavorable prognosis, treatment comprehensive.

Язык: Английский

Процитировано

0

Seladelpar for the Treatment of Primary Biliary Cholangitis DOI Creative Commons
Adonice Khoury, Jason Powell

Annals of Pharmacotherapy, Год журнала: 2025, Номер unknown

Опубликована: Март 12, 2025

Objective: To review the published data including pharmacology, efficacy, and safety of seladelpar, a peroxisome proliferator-activated receptor delta (PPARδ) agonist leading to Food Drug Administration (FDA) accelerated approval for treatment primary biliary cholangitis (PBC). Data sources: A PubMed (January 1, 1985 January 27, 2025) literature search was performed using terms MBX-8025, agonist, PBC. Other sources included Google Scholar National Institutes Health Clinical Trials Registry. Study selection extraction: All English-language evaluating pharmacokinetics, safety, efficacy seladelpar in PBC reviewed. synthesis: Seladelpar is first PPARδ that has shown significant reduction across biochemical response, alkaline phosphatase (ALP) normalization, pruritus as compared placebo while demonstrating tolerability. Relevance patient care clinical practice comparison existing drugs: While drug treatments are efficacious, there remains an unmet need due incomplete response many patients. Patients frequently suffer from symptoms, pruritus, impacting their quality life. could have beneficial role add-on therapy improving well alleviating pruritus. Conclusion: safe effective fills need. Seladelpar’s benefit predicted by improvement surrogate endpoints may confirmation traditional FDA approval.

Язык: Английский

Процитировано

0

Recompensation in Cirrhosis: Biomarkers and Strategies DOI

Wagner Ramírez-Quesada,

Edilmar Alvarado‐Tápias, Sarah Shalaby

и другие.

Seminars in Liver Disease, Год журнала: 2025, Номер unknown

Опубликована: Апрель 3, 2025

The onset of decompensation in advanced chronic liver disease (ACLD) is a hallmark natural history, with poor prognosis and significantly increased liver-related mortality. Etiological treatments for viral hepatitis or abstinence cirrhosis due to alcohol abuse have demonstrated that some patients experience partial complete clinical analytical improvement, stage termed “recompensation.” Although recompensation primarily defined clinically based on treatable etiologies, it still evolving conditions like metabolic dysfunction-associated steatotic (MASLD). Despite the need specific biomarkers hepatic recompensation, no been thoroughly studied this context. Biomarkers identified compensated ACLD (cACLD) following etiological treatment might be explored recompensation. pathophysiology mechanisms underlying remain unclear, understanding mechanism involved could help identify potential targets This review provides an update concept, examines existing data invasive non-invasive biomarkers, mainly cACLD after cure, raised explores future therapeutic process.

Язык: Английский

Процитировано

0

TMEM132C and LIPE protein molecules drive synovial hyperplasia via the PPARγ signaling axis: Mechanistic insights into core pathogenic proteins in rheumatoid arthritis DOI

Fangyue Cheng,

Zhen Dai, Jinling Zhang

и другие.

International Journal of Biological Macromolecules, Год журнала: 2025, Номер unknown, С. 143027 - 143027

Опубликована: Апрель 1, 2025

Язык: Английский

Процитировано

0

Xiaohuang Qudan decoction alleviates ANIT-induced cholestatic liver injury by inhibiting the JAK2/STAT3 pathway and regulating TH17/Treg DOI
Zheng Tan, Haiyong Chen,

Zhiqin Ye

и другие.

Chinese Journal of Natural Medicines, Год журнала: 2025, Номер 23(4), С. 457 - 470

Опубликована: Апрель 1, 2025

Язык: Английский

Процитировано

0

Are Gender Differences Important for Autoimmune Liver Diseases? DOI Creative Commons
Annarosa Floreani, Daniela Gabbia, Sara De Martin

и другие.

Life, Год журнала: 2024, Номер 14(4), С. 500 - 500

Опубликована: Апрель 12, 2024

Gender Medicine has had an enormous expansion over the last ten years. Autoimmune liver diseases include several conditions, i.e., autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), sclerosing (PSC), and conditions involving or tree overlapping with AIH, as well IgG4-related disease. However, little is known about impact of sex in pathogenesis natural history these conditions. The purpose this review to provide update gender disparities among by reviewing data published from 1999 2023. epidemiology been changing years, due amelioration knowledge their diagnosis, pathogenesis, treatment. clinical collected so far support existence differences diseases. Notably, could be longer than that which now known, problems being initiated even at a pediatric age. Moreover, disparity observed during onset complications related end-stage disease, including cancer incidence. there still important debate researchers With review, we would like emphasize urgency basic science research increase our understanding

Язык: Английский

Процитировано

3

Multi-omics and multi-stages integration identified a novel variant associated with silicosis risk DOI

Chunmeng Jin,

Xiaobo Tao,

Wendi Zhang

и другие.

Archives of Toxicology, Год журнала: 2024, Номер 98(9), С. 2907 - 2918

Опубликована: Май 29, 2024

Язык: Английский

Процитировано

3

Unique metabolomics characteristics for distinguishing cirrhosis related to different liver diseases: A systematic review and meta-analysis DOI

Liu Yang,

Fang Wang,

Sijia Liu

и другие.

Diabetes & Metabolic Syndrome Clinical Research & Reviews, Год журнала: 2024, Номер 18(6), С. 103068 - 103068

Опубликована: Июнь 1, 2024

Язык: Английский

Процитировано

3

Bile Acids-Based Therapies for Primary Sclerosing Cholangitis: Current Landscape and Future Developments DOI Creative Commons
Stefano Fiorucci,

Ginevra Urbani,

Cristina Di Giorgio

и другие.

Cells, Год журнала: 2024, Номер 13(19), С. 1650 - 1650

Опубликована: Окт. 4, 2024

Primary sclerosing cholangitis (PSC) is a rare, chronic liver disease with no approved therapies. The ursodeoxycholic acid (UDCA) has been widely used, although there evidence that the use of UDCA delays time to transplant or increases survival. Several candidate drugs are currently being developed. largest group these new agents represented by FXR agonists, including obeticholic acid, cilofexor, and tropifexor. Other target bile metabolism ASTB/IBAP inhibitors fibroblasts growth factor (FGF)19 analogues. Cholangiocytes, epithelial duct cells, play role in PSC development. Recent studies have revealed cells undergo downregulation GPBAR1 (TGR5), receptor involved bicarbonate secretion immune regulation. Additional under evaluation PPARs (elafibranor seladelpar), anti-itching such as MAS-related G-protein–coupled receptors antagonists, anti-fibrotic immunosuppressive agents. Drugs targeting gut bacteria pathways also investigation, given strong link between microbiota.

Язык: Английский

Процитировано

3