ArticlePloS one2013
Serum proteome profiling identifies novel and powerful markers of cystic fibrosis liver disease.
Article in PloS one, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
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Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 35 citations in OpenAlex.
- Non-invasive Diagnostic Tests in Cystic Fibrosis-Related Liver Disease: A Diagnostic Test Accuracy Network Meta-Analysis.Frontiers in medicine · 2021Pooled it
- Soluble endoglin as a marker of clinically significant portal hypertension in patients with cirrhosis.Scientific reports · 2026Article
- Age-Dependent Systemic Regulation of C1q/TNF-Related Protein 3 and Progranulin in Patients with Cystic Fibrosis: Biomarkers or Therapeutic Targets?Biomedicines · 2026Article
- Unveiling the Impact of BMP9 in Liver Diseases: Insights into Pathogenesis and Therapeutic Potential.Biomolecules · 2024Review
- TGF-β superfamily co-receptors in cancer.Developmental dynamics : an official publication of the American Association of Anatomists · 2022Review
- Impact of Altered Gut Microbiota and Its Metabolites in Cystic Fibrosis.Metabolites · 2021Review
- Role of transient elastography and APRI in the assessment of pediatric cystic fibrosis liver disease.Canadian liver journal · 2021Article
- Soluble Endoglin as a Potential Biomarker of Nonalcoholic Steatohepatitis (NASH) Development, Participating in Aggravation of NASH-Related Changes in Mouse Liver.International journal of molecular sciences · 2020Article
- CFTR dysfunction increases endoglin and TGF-β signaling in airway epithelia.Physiological reports · 2019Article
- Hepatitis C Virus Core Protein Modulates Endoglin (CD105) Signaling Pathway for Liver Pathogenesis.Journal of virology · 2017Article
- 2D-DIGE as a strategy to identify serum biomarkers in Mexican patients with Type-2 diabetes with different body mass index.Scientific reports · 2017Article
- Article
- Features of Severe Liver Disease With Portal Hypertension in Patients With Cystic Fibrosis.Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association · 2016Article
- Hepatic and Splenic Acoustic Radiation Force Impulse Shear Wave Velocity Elastography in Children with Liver Disease Associated with Cystic Fibrosis.BioMed research international · 2015Article
- Cystic fibrosis related liver disease--another black box in hepatology.International journal of molecular sciences · 2014Review
- Endoglin in liver fibrogenesis: Bridging basic science and clinical practice.World journal of biological chemistry · 2014Review
- Identification of neutrophil activation markers as novel surrogate markers of CF lung disease.PloS one · 2014Article
- Article
- Derivative component analysis for mass spectral serum proteomic profiles.BMC medical genomics · 2014Article
- Liver disease in cystic fibrosis: an update.Hepatitis monthly · 2013Review
Corrections and comments
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
aimsCystic Fibrosis associated liver disease (CFLD) develops in approximately 30% of CF patients. However, routine sensitive diagnostic tools for CFLD are lacking. Within this study, we aimed to identify new experimental biomarkers for the detection of CFLD.
methods45 CF patients were included in the study and received transient elastography. Differential regulation of 220 different serum proteins was assessed in a subgroup of patients with and without CFLD. Most interesting candidate proteins were further quantified and validated by ELISA in the whole patient cohort. To assess a potential relation of biomarker expression to the degree of hepatic fibrosis, serum biomarkers were further determined in 18 HCV patients where liver histology was available.
results43 serum proteins differed at least 2-fold in patients with CFLD compared to those without liver disease as identified in proteome profiling. In ELISA quantifications, TIMP-4 and Endoglin were significantly up-regulated in patients with CFLD as diagnosed by clinical guidelines or increased liver stiffness. Pentraxin-3 was significantly decreased in patients with CFLD. Serum TIMP-4 and Endoglin showed highest values in HCV patients with liver cirrhosis compared to those with fibrosis but without cirrhosis. At a cut-off value of 6.3 kPa, transient elastography compassed a very high diagnostic accuracy and specificity for the detection of CFLD. Among the biomarkers, TIMP-4 and Endoglin exhibited a high diagnostic accuracy for CFLD. Diagnostic sensitivities and negative predictive values were increased when elastography and TIMP-4 and Endoglin were combined for the detection of CFLD.
conclusionsSerum TIMP-4 and Endoglin are increased in CFLD and their expression correlates with hepatic staging. Determination of TIMP-4 and Endoglin together with transient elastography can increase the sensitivity for the non-invasive diagnosis of CFLD.
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