- Hepatobiliary involvement is a hallmark in cystic fibrosis (CF), as the causative CF Transmembrane Conductance Regulator (CFTR) defect is expressed in the biliary tree. However, bile acid (BA) compositions in regard to pancreatic insufficiency, which is present at an early stage in about 85% of CF patients, have not been satisfactorily understood.
We assess the pattern of serum BAs in people with CF (pwCF) without CFTR modulator therapy in regard to pancreatic insufficiency and the CFTR genotype.
In 47 pwCF, 10 free and 12 taurine- and glycine-conjugated BAs in serum were prospectively assessed.
Findings were related to genotype, pancreatic insufficiency prevalence (PIP)-score, and hepatic involvement indicated by serum liver enzymes, as well as clinical and ultrasound criteria for CF-related liver disease.
Serum concentrations of total primary BAs and free cholic acid (CA) were significantly higher in pwCF with higher PIP-scores (p = 0.025, p = 0.009, respectively). Higher total BAs were seen in pwCF with PIP-scores >= 0.88Hepatobiliary involvement is a hallmark in cystic fibrosis (CF), as the causative CF Transmembrane Conductance Regulator (CFTR) defect is expressed in the biliary tree. However, bile acid (BA) compositions in regard to pancreatic insufficiency, which is present at an early stage in about 85% of CF patients, have not been satisfactorily understood.
We assess the pattern of serum BAs in people with CF (pwCF) without CFTR modulator therapy in regard to pancreatic insufficiency and the CFTR genotype.
In 47 pwCF, 10 free and 12 taurine- and glycine-conjugated BAs in serum were prospectively assessed.
Findings were related to genotype, pancreatic insufficiency prevalence (PIP)-score, and hepatic involvement indicated by serum liver enzymes, as well as clinical and ultrasound criteria for CF-related liver disease.
Serum concentrations of total primary BAs and free cholic acid (CA) were significantly higher in pwCF with higher PIP-scores (p = 0.025, p = 0.009, respectively). Higher total BAs were seen in pwCF with PIP-scores >= 0.88 (p = 0.033) and with pancreatic insufficiency (p = 0.034).
Free CA was higher in patients with CF-related liver involvement without cirrhosis, compared to pwCF without liver disease (2.3-fold, p = 0.036). pwCF with severe CFTR genotypes, as assessed by the PIP-score, reveals more toxic BA compositions in serum. Subsequent studies assessing changes in BA homeostasis during new highly effective CFTR-modulating therapies are of high interest.…
MetadatenAuthor details: | Harold Tabori, Jochen Schneider, Stefan LüthORCiDGND, Carlos Zagoya, Anton Barucha, Thomas Lehmann, Eberhard Kauf, Astrid Barth, Jochen G. MainzORCiDGND |
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DOI: | https://doi.org/10.3390/ijms232012436 |
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ISSN: | 1422-0067 |
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Pubmed ID: | https://pubmed.ncbi.nlm.nih.gov/36293293 |
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Title of parent work (English): | International journal of molecular sciences |
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Publisher: | MDPI |
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Place of publishing: | Basel |
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Publication type: | Article |
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Language: | English |
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Date of first publication: | 2022/10/18 |
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Publication year: | 2022 |
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Release date: | 2024/08/26 |
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Tag: | CF liver disease; bile acid; biliary; cystic fibrosis; hepatic; high performance liquid chromatography |
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Volume: | 23 |
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Issue: | 20 |
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Article number: | 12436 |
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Number of pages: | 16 |
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Funding institution: | Medizinische Hochschule Brandenburg (MHB) fund; Deutsche; Forschungsgemeinschaft (DFG) |
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Organizational units: | Humanwissenschaftliche Fakultät / Strukturbereich Kognitionswissenschaften / Department Sport- und Gesundheitswissenschaften |
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DDC classification: | 6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit |
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Peer review: | Referiert |
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Publishing method: | Open Access / Gold Open-Access |
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| DOAJ gelistet |
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License (German): | CC-BY - Namensnennung 4.0 International |
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