Serum bile acids in cystic fibrosis patients – glycodeoxycholic acid as a potential marker of liver disease

Drzymała-Czyż, Sławomira; Dziedzic, Krzysztof; Szwengiel, Artur; Krzyżanowska-Jankowska, Patrycja; Nowak, Jan; Nowicka, Agata; Aringazina, Raisa; Drzymała, Sylwia; Kashirskaya, N.; Walkowiak, Jarosław

doi:10.1016/j.dld.2021.06.034

Cited by 16 publications

(16 citation statements)

References 49 publications

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“…Moreover, cirrhotic pwCF revealed, as expected, lower CA levels than the CFLI w / o LC group. A similar pattern was observed by Drzymała et al [ 24 ], reporting lower CA levels in patients with liver cirrhosis compared to those without liver involvement. Compared to the CFLI w / o LC group, this appears to be a consequence of the impaired hepatic bile synthesis in cirrhosis.…”

Section: Discussionsupporting

confidence: 88%

“…Similarly, Azer and colleagues found high levels of CA to be associated with progression of hepatic injury [ 28 ]. More recently, Drzymała et al [ 24 ] observed higher CA concentrations in serum from pwCF compared to healthy subjects. In addition, the authors found CA concentrations to be higher in patients with some degree of liver involvement, including cirrhosis, than in pwCF without a diagnosis of liver disease.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, the identification of non-CFTR genetic polymorphism SERPINA1 Z allele was mentioned as a risk factor of liver disease in CF [ 23 ]. More recently, only one study has explored the association between BA concentrations in serum and the degree of liver involvement (LI) in pwCF, wherein it is suggested that serum deoxycholic acid and its glycine conjugate have the potential to serve as biomarkers to differentiate between pwCF with non-cirrhotic LI and pwCF with no detectable liver disease [ 24 ]. Nevertheless, there is a lack of studies investigating the relationship between CFTR genotype/phenotype and BAs observed in pwCF.…”

Section: Introductionmentioning

confidence: 99%

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Elevated Levels of Toxic Bile Acids in Serum of Cystic Fibrosis Patients with CFTR Mutations Causing Pancreatic Insufficiency

Tabori

Schneider²,

Zagoya³

et al. 2022

IJMS

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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 ≥0.88 (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.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Elevated Levels of Toxic Bile Acids in Serum of Cystic Fibrosis Patients with CFTR Mutations Causing Pancreatic Insufficiency

Tabori

Schneider²,

Zagoya³

et al. 2022

IJMS

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“…To investigate potential correlations between the gut microbiome and bile acids in BA, we calculated Spearman correlation coefficients and selected the 50 most abundant microorganisms for analysis. As shown in Figure 3B, Eubacterium_halli_group, Erysipelatoclostridium, Ruminococcus_gnavus_group, Fusicatenibacter, Dorea, and Monoglobus were all highly positively correlated with GDCA (all p < 0.01), a predictive marker for liver damage (Drzymała-Czyżet al, 2021). Ruminococcus_gnavus_group and Fusicatenibacter also showed a highly significant positive correlation with TDCA (all p < 0.01), Bifidobacterium showed a significant negative correlation with conjugated bile acids (GCA, TCA, TCDCA, and GCDCA; all p < 0.05), and Rothia was negatively correlated with secondary bile acids (DCA and LCA; both p < 0.05).…”

Section: Association Between Disease-related Fecal Microbiota Fecal B...mentioning

confidence: 85%

Comprehensive Analysis of Gut Microbiota and Fecal Bile Acid Profiles in Children With Biliary Atresia

Yang

Shen

Wang

et al. 2022

Front. Cell. Infect. Microbiol.

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BackgroundBiliary atresia (BA) is the most common cholestatic liver disease in neonates. Herein, we aimed at characterizing the gut microbiota and fecal bile acid profiles of BA patients, defining the correlations between them, and evaluating the relationship between the clinical pathogenesis and changes in the gut microbiota and bile acid profiles.MethodsA total of 84 fecal samples from BA patients (n = 46) and matched healthy controls (HCs, n = 38) were subjected to sequencing by 16S rRNA gene amplification, and fecal bile acid were analyzed by targeted metabolomics.FindingsCompared with the controls, a structural separation of the intestinal flora of BA patients was uncovered, which was accompanied by changes in the composition of fecal bile acids. In the BA group, Actinobacillus, Monoglobus, and Agathobacter were enriched in patients without cholangitis (p < 0.05). Selenomonadaceae and Megamonas were more abundant in patients without recurrent cholangitis episodes (p < 0.05), while Lachnospiraceae and Ruminococcaceae were enriched in patients with multiple recurrences of cholangitis (p < 0.05). Postoperative jaundice clearance was associated with Campylobacter and Rikenellaceae (p < 0.05), and tauroursodeoxycholic acid was associated with jaundice clearance (p < 0.001).ConclusionBA patients are characterized by different compositions of gut microbiota and bile acids, and their interaction is involved in the process of liver damage in BA, which may be closely related to the occurrence of postoperative cholangitis and jaundice clearance.

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“…It is worthy of mention that in this study, we found that SC06 decreased ibuprofen and increased glycodeoxycholic acid and 7-dehydrocholesterol in ob/ob mice. 7-Dehydrocholesterol can be catalyzed to cholesterol by 7-dehydrocholesterol reductase, which is a final step in the biogenesis of cholesterol; 64 glycodeoxycholic acid (GDCA) is a primary bile acid, and its concentration impacts liver diseases, such as cystic fibrosis; 65 both of them could be involved in bile acid metabolism. Compelling evidence has revealed that altered gut microbiota and metabolites may have significant impacts on bile acid metabolism in the host.…”

Section: Discussionmentioning

confidence: 99%

Bacillus amyloliquefaciens SC06 alleviates the obesity of ob/ob mice and improves their intestinal microbiota and bile acid metabolism

Zeng

Zhou

et al. 2022

Food Funct.

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Serum bile acids in cystic fibrosis patients – glycodeoxycholic acid as a potential marker of liver disease

Cited by 16 publications

References 49 publications

Elevated Levels of Toxic Bile Acids in Serum of Cystic Fibrosis Patients with CFTR Mutations Causing Pancreatic Insufficiency

Elevated Levels of Toxic Bile Acids in Serum of Cystic Fibrosis Patients with CFTR Mutations Causing Pancreatic Insufficiency

Comprehensive Analysis of Gut Microbiota and Fecal Bile Acid Profiles in Children With Biliary Atresia

Bacillus amyloliquefaciens SC06 alleviates the obesity of ob/ob mice and improves their intestinal microbiota and bile acid metabolism

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