7,782 results match your criteria Primary Sclerosing Cholangitis


TREM-2 plays a protective role in cholestasis by acting as a negative regulator of inflammation.

J Hepatol 2022 Jun 21. Epub 2022 Jun 21.

Department of Liver and Gastrointestinal Diseases, Biodonostia Research Institute, Donostia University Hospital, University of the Basque Country (UPV-EHU), Donostia-San Sebastian, Spain; CIBERehd, Instituto de Salud Carlos III (ISCIII), Madrid, Spain; Department of Medicine, Faculty of Medicine and Nursing, University of the Basque Country, UPV/EHU, Leioa, Spain. Electronic address:

Background & Aims: Cholestatic liver injury triggers a wound healing response orchestrated by Kupffer cells (KCs), hepatic stellate cells (HSCs) and other immune cells that leads to peribiliary fibrosis and disease progression. Inflammation is central, particularly that mediated by bacterial components translocating from the gut to the liver, binding to toll-like receptors (TLRs). The triggering receptor expressed on myeloid cells-2 (TREM-2) inhibits TLR-mediated signaling and exerts a protective role in hepatocellular injury and carcinogenesis. Read More

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Gender and Autoimmune Liver Diseases: Relevant Aspects in Clinical Practice.

J Pers Med 2022 Jun 2;12(6). Epub 2022 Jun 2.

IRCCS Negrar, 37024 Verona, Italy.

Autoimmune liver diseases (AILDs) include autoimmune hepatitis, primary biliary cholangitis and primary sclerosing cholangitis. The etiologies of AILD are not well understood but appear to involve a combination of genetic and environmental factors. AILDs commonly affect young individuals and are characterized by a highly variable clinical course. Read More

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Clinical Utility of Personalized Serum IgG Subclass Ratios for the Differentiation of IgG4-Related Sclerosing Cholangitis (IgG4-SC) from Primary Sclerosing Cholangitis (PSC) and Cholangiocarcinoma (CCA).

J Pers Med 2022 May 24;12(6). Epub 2022 May 24.

Division of Gastroenterology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Korea.

Background: The differential diagnosis of immunoglobulin G4-sclerosing cholangitis (IgG4-SC) from primary sclerosing cholangitis (PSC) or cholangiocarcinoma (CCA) is important. In this study, we aimed to find the best combinations of serum IgG subclasses and IgG4 levels for differentiating IgG4-SC from PSC or CCA.

Methods: In total, 31 patients with IgG4-SC, 27 patients with PSC, and 40 patients with CCA were enrolled from 2003 to 2017 at a single tertiary referral center. Read More

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A Case of Hepatic Immunoglobulin G4-Related Disease Presenting as an Inflammatory Pseudotumor and Sclerosing Cholangitis.

Diagnostics (Basel) 2022 Jun 19;12(6). Epub 2022 Jun 19.

Department of Forensic Medicine, School of Medicine, Kyungpook National University, 680 Gukchaebosang-ro, Jung-gu, Daegu 41944, Korea.

An inflammatory pseudotumor is a benign disease characterized by tumor-like lesions consisting of inflammatory cells including plasma cells and fibrous tissue. Recently, some inflammatory pseudotumor cases proved to be a form of Immunoglobulin G4-related disease (IgG4-RD). This novel clinical entity, recognized as a fibroinflammatory condition, is characterized by lymphoplasmacytic infiltration with a predominance of IgG4-positive plasma cells, storiform fibrosis, and often elevated serum IgG4 concentrations. Read More

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Primary Biliary Cholangitis and Primary Sclerosing Cholangitis: Current Knowledge of Pathogenesis and Therapeutics.

Biomedicines 2022 May 31;10(6). Epub 2022 May 31.

Department of Internal Medicine, College of Medicine, Hallym University, Chuncheon-si 24252, Korea.

Cholangiopathies encompass various biliary diseases affecting the biliary epithelium, resulting in cholestasis, inflammation, fibrosis, and ultimately liver cirrhosis. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most important progressive cholangiopathies in adults. Much research has broadened the scope of disease biology to genetic risk, epigenetic changes, dysregulated mucosal immunity, altered biliary epithelial cell function, and dysbiosis, all of which interact and arise in the context of ill-defined environmental triggers. Read More

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Clinical course of inflammatory bowel disease and impact on liver disease outcomes in patients with autoimmune sclerosing cholangitis.

Clin Res Hepatol Gastroenterol 2022 Jun 18:101980. Epub 2022 Jun 18.

Department of Gastroenterology, King's College Hospital, London, UK; King's College London, UK.

Background & Aims: Autoimmune sclerosing cholangitis (ASC) is a childhood sclerosing cholangitis frequently associated with inflammatory bowel disease (IBD). We describe the IBD phenotype in ASC patients and associated liver disease outcomes.

Methods: Single centre retrospective observational review of ASC patients, with a control population of paediatric IBD. Read More

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Spatial transcriptomics identifies enriched gene expression and cell types in human liver fibrosis.

Hepatol Commun 2022 Jun 20. Epub 2022 Jun 20.

Norwegian PSC Research Center, Oslo University Hospital, Rikshospitalet, Oslo, Norway.

Liver fibrosis and cirrhosis have limited therapeutic options and represent a serious unmet patient need. Recent use of single-cell RNA sequencing (scRNAseq) has identified enriched cell types infiltrating cirrhotic livers but without defining the microanatomical location of these lineages thoroughly. To assess whether fibrotic liver regions specifically harbor enriched cell types, we explored whether whole-tissue spatial transcriptomics combined with scRNAseq and gene deconvolution analysis could be used to localize cell types in cirrhotic explants of patients with end-stage liver disease (total n = 8; primary sclerosing cholangitis, n = 4; primary biliary cholangitis, n = 2, alcohol-related liver disease, n = 2). Read More

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Digital single-operator video cholangioscopy improves endoscopic management in patients with primary sclerosing cholangitis-a retrospective observational study.

World J Gastroenterol 2022 May;28(20):2201-2213

Department of Medicine B (Gastroenterology, Hepatology, Endocrinology, Clinical Infectiology), University Hospital Muenster, Muenster 48149, Germany.

Background: Patients with primary sclerosing cholangitis (PSC) are at a high risk of developing cholestatic liver disease and biliary cancer, and endoscopy is crucial for the complex management of these patients.

Aim: To clarify the utility of recently introduced digital single-operator video cholangioscopy (SOVC) for the endoscopic management of PSC patients.

Methods: In this observational study, all patients with a history of PSC and in whom digital SOVC (using the SpyGlass DS System) was performed between 2015 and 2019 were included and retrospectively analysed. Read More

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Protective and aggressive bacterial subsets and metabolites modify hepatobiliary inflammation and fibrosis in a murine model of PSC.

Gut 2022 Jun 15. Epub 2022 Jun 15.

Division of Gastroenterology and Hepatology, University of North Carolina System, Chapel Hill, North Carolina, USA

Objective: Conflicting microbiota data exist for primary sclerosing cholangitis (PSC) and experimental models.

Goal: define the function of complex resident microbes and their association relevant to PSC patients by studying germ-free (GF) and antibiotic-treated specific pathogen-free (SPF) multidrug-resistant 2 deficient ( ) mice and microbial profiles in PSC patient cohorts.

Design: We measured weights, liver enzymes, RNA expression, histological, immunohistochemical and fibrotic biochemical parameters, faecal 16S rRNA gene profiling and metabolomic endpoints in gnotobiotic and antibiotic-treated SPF mice and targeted metagenomic analysis in PSC patients. Read More

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Immune checkpoint inhibitor-related cholangiopathy: Novel clinicopathological description of a multi-centre cohort.

Liver Int 2022 Jun 15. Epub 2022 Jun 15.

Department of Hepatology, King's college Hospital, London, UK.

Background And Aims: Cholestatic liver dysfunction is common in immune-related hepatitis (irH) during treatment with immune checkpoint inhibitors (CPI) for malignancy. We investigated the spectrum of bile duct injury and associated natural history in this cohort.

Method: Clinical, laboratory, radiological and histopathological data in patients with evidence of bile duct injury during CPI treatment from 2018 to 2020 was collected in three tertiary hospitals. Read More

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Anti-Proteinase 3 Antibodies as a Biomarker for Ulcerative Colitis and Primary Sclerosing Cholangitis in Children.

J Pediatr Gastroenterol Nutr 2022 Apr 24;74(4):463-470. Epub 2021 Nov 24.

Institute of Immunology, Technische Universität Dresden, Dresden, Germany.

Objectives: Anti-neutrophil cytoplasmic antibody (ANCA) directed against proteinase 3 (PR3) is a marker for granulomatosis with polyangiitis, but is also found in patients with inflammatory bowel disease (IBD), mainly ulcerative colitis (UC). The aim of our study was to investigate ANCA and PR3-ANCA in paediatric IBD.

Methods: We tested 326 paediatric IBD patients and 164 controls for anti-Saccharomyces cerevisiae antibodies (ASCA), ANCA (indirect immunofluorescence, IIF) and PR3-ANCA (chemiluminescence immunoassay). Read More

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Can dysplasia surveillance be better targeted in ulcerative colitis by using faecal calprotectin?

Scand J Gastroenterol 2022 Jun 13:1-8. Epub 2022 Jun 13.

Department of Gastroenterology, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.

In the inflammatory bowel diseases, chronic inflammation predisposes to dysplasia and colorectal carcinoma, leading to the need of surveillance colonoscopies. The most-used marker of colonic inflammation is faecal calprotectin. Its correlation with endoscopic and histological findings is well-documented. Read More

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Intrahepatic cholangiocarcinoma: Typical features, uncommon variants, and controversial related entities.

Authors:
Yoh Zen

Hum Pathol 2022 Jun 10. Epub 2022 Jun 10.

Institute of Liver Studies, King's College Hospital, London SE5 9RS, UK. Electronic address:

Pathologists play a central role in the diagnosis and classification of intrahepatic cholangiocarcinoma (iCCA). iCCA is currently classified into small- and large-duct types. Small-duct iCCA is characterized by a mass-forming gross appearance, mucus-poor ductule-like histology, and frequent association with chronic parenchymal liver diseases (e. Read More

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Tetrahydroxylated bile acids improve cholestatic liver and bile duct injury in the Mdr2 mouse model of sclerosing cholangitis via immunomodulatory effects.

Hepatol Commun 2022 Jun 12. Epub 2022 Jun 12.

Hans Popper Laboratory of Molecular Hepatology, Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria.

Bile salt export pump (Bsep) (Abcb11) mice are protected from acquired cholestatic injury due to metabolic preconditioning with a hydrophilic bile acid (BA) pool with formation of tetrahydroxylated bile acids (THBAs). We aimed to explore whether loss of Bsep and subsequent elevation of THBA levels may have immunomodulatory effects, thus improving liver injury in the multidrug resistance protein 2 (Mdr2) (Abcb4) mouse. Cholestatic liver injury in Mdr2 Bsep double knockout (DKO), Mdr2 , Bsep , and wild-type mice was studied for comparison. Read More

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Prevalence of inflammatory bowel disease in patients with primary sclerosing cholangitis: A systematic review and meta-analysis.

Liver Int 2022 Jun 11. Epub 2022 Jun 11.

State Key Laboratory of Cancer Biology, National Clinical Research Center for Digestive Diseases and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China.

Background And Aim: Previous studies have established an association between primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD), including Crohn's disease and ulcerative colitis (UC). The disease burden of IBD in PSC patients was not well estimated. The study aimed to quantify the pooled prevalence of IBD in PSC and to investigate whether subtypes of PSC and sex influence the prevalence of IBD. Read More

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IL-31 Levels Correlate with Pruritus in Patients with Cholestatic and Metabolic Liver Diseases and is FXR Responsive in NASH.

Hepatology 2022 Jun 10. Epub 2022 Jun 10.

Gilead Sciences, Inc., Foster City, CA, USA.

Background: Pruritus is associated with multiple liver diseases, particularly those with cholestasis, but the mechanism remains incompletely understood. Our aim was to evaluate serum IL-31 as a putative biomarker of pruritus in clinical trials of an farnesoid X receptor (FXR) agonist, cilofexor, in patients with nonalcoholic steatohepatitis (NASH), primary sclerosing cholangitis (PSC), and primary biliary cholangitis (PBC) RESULTS: Serum IL-31 was measured in clinical studies of cilofexor in NASH, PSC, and PBC. In patients with PSC or PBC, baseline IL-31 was elevated compared to NASH patients and healthy volunteers (HVs). Read More

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Interplay between Mast Cells and Regulatory T Cells in Immune-Mediated Cholangiopathies.

Int J Mol Sci 2022 May 24;23(11). Epub 2022 May 24.

Centre for Liver and Gastrointestinal Research & NIHR Birmingham Liver Biomedical Research Unit, Institute of Biomedical Research, Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham B15 2TT, UK.

Immune-mediated cholangiopathies are characterised by the destruction of small and large bile ducts causing bile acid stasis, which leads to subsequent inflammation, fibrosis, and eventual cirrhosis of the liver tissue. A breakdown of peripheral hepatic immune tolerance is a key feature of these diseases. Regulatory T cells (Tregs) are a major anti-inflammatory immune cell subset, and their quantities and functional capacity are impaired in autoimmune liver diseases. Read More

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Outruling cholangiocarcinoma in patients with primary sclerosing cholangitis wait-listed for liver transplantation: A report on the Irish national experience.

Surgeon 2022 Jun 6. Epub 2022 Jun 6.

Department of Hepatobiliary and Transplant Surgery, St. Vincent's University Hospital, Elm Park, Dublin 4, Ireland.

Background: The presence of diffuse biliary stricturing in Primary Sclerosing Cholangitis (PSC) makes the diagnosis of early Cholangiocarcinoma (CCA) in this context difficult. A finding of incidental CCA on liver explant is associated with poor oncological outcomes, despite this; there remains no international consensus on how best to outrule CCA in this group ahead of transplantation. The objectives of this study were to report the Irish incidence of incidental CCA in individuals with PSC undergoing liver transplantation, and to critically evaluate the accuracy of diagnostic modalities in outruling CCA in our wait-listed PSC cohort. Read More

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Value of Liver Function Tests in Cirrhosis.

Authors:
Praveen Sharma

J Clin Exp Hepatol 2022 May-Jun;12(3):948-964. Epub 2021 Nov 14.

Department of Gastroenterology, Sir Ganga Ram Hospital, New Delhi, India.

The liver comprises both parenchymal and non-parenchymal cells with varying functions. Cirrhosis is often complicated by the development of portal hypertension and its associated complications. Hence, assessment of liver in cirrhosis should include assessment of its structural, function of both hepatic and non-hepatic tissue and haemodynamic assessment of portal hypertension. Read More

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November 2021

IgG4-Related Sclerosing Cholangitis with Cholecystitis Mimicking Cholangiocarcinoma: A Case Report.

J Clin Exp Hepatol 2022 May-Jun;12(3):1012-1016. Epub 2021 Nov 16.

Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.

IgG4-related diseases are rare multisystem disorders involving salivary glands, retroperitoneum, pancreas, biliary tract, and liver. Isolated biliary strictures and gall bladder involvement are rare in such patients, and presentation with cholangitis and weight loss can misguide the diagnosis toward malignancy. Here, we report an interesting case of IgG4-related biliary stricture with gall bladder involvement, presented with cholangitis and weight loss. Read More

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November 2021

Chemoprevention of Colitis-Associated Dysplasia or Cancer in Inflammatory Bowel Disease.

Gut Liver 2022 Jun 7. Epub 2022 Jun 7.

Division of Gastroenterology, Changhua Christian Hospital, Changhua, Taiwan.

The association between inflammatory bowel disease and colorectal cancer is well known. Although the overall incidence of inflammatory bowel disease has declined recently, patients with this disease still have a 1.7-fold increased risk of colorectal cancer. Read More

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Persistent Cholestatic Injury and Secondary Sclerosing Cholangitis in COVID-19 Patients.

Arch Pathol Lab Med 2022 Jun 3. Epub 2022 Jun 3.

Liver Center and Gastrointestinal Division (Wilechansky, Chung) at the Massachusetts General Hospital, Boston, Massachusetts.

Context.–: Coronavirus disease 2019 (COVID-19) has been associated with liver injury, and a small subset of patients recovering from severe disease have shown persistent markedly elevated liver biochemistries for months after infection.

Objective. Read More

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Epidemiology and outcomes of primary sclerosing cholangitis: an Australian multicentre retrospective cohort study.

Hepatol Int 2022 Jun 3. Epub 2022 Jun 3.

Gastroenterology and Hepatology Department, The Alfred Hospital, 55 Commercial Rd, Melbourne, 3004, Australia.

Background And Aims: Little is known regarding the epidemiology and outcomes of patients with primary sclerosing cholangitis (PSC) in Australia. We, therefore, evaluated the epidemiology and clinical outcomes of PSC in a large cohort of Australian patients and compared these to the general population.

Methods: We conducted a multicentre, retrospective cohort study of PSC patients at nine tertiary liver centers across three Australian states, including two liver transplant centers. Read More

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How genetic risk contributes to autoimmune liver disease.

Authors:
David Ellinghaus

Semin Immunopathol 2022 Jun 1. Epub 2022 Jun 1.

Institute of Clinical Molecular Biology (IKMB), Kiel University and University Medical Center Schleswig-Holstein, Rosalind-Franklin-Str. 12, 24105, Kiel, Germany.

Genome-wide association studies (GWAS) for autoimmune hepatitis (AIH) and GWAS/genome-wide meta-analyses (GWMA) for primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) have been successful over the past decade, identifying about 100 susceptibility loci in the human genome, with strong associations with the HLA locus and many susceptibility variants outside the HLA locus with relatively low risk. However, identifying causative variants and genes and determining their effects on liver cells and their immunological microenvironment is far from trivial. Polygenic risk scores (PRSs) based on current genome-wide data have limited potential to predict individual disease risk. Read More

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Allograft liver failure awaiting liver transplantation in Japan.

J Gastroenterol 2022 Jul 1;57(7):495-504. Epub 2022 Jun 1.

Department of Pediatric Surgery and Transplantation, Kumamoto Rosai Hospital, Kumamoto, Japan.

Background: Following liver transplantation (LT), allograft liver failure can be developed by various causes and requires re-LT. Hence, this study aimed to clarify the characteristics and prognostic factors of patients with allograft liver failure awaiting deceased donor LT (DDLT) in Japan.

Methods: Of the 2686 DDLT candidates in Japan between 2007 and 2016, 192 adult patients listed for re-LT were retrospectively enrolled in this study. Read More

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Recent discoveries in microbiota dysbiosis, cholangiocytic factors, and models for studying the pathogenesis of primary sclerosing cholangitis.

Open Med (Wars) 2022 13;17(1):915-929. Epub 2022 May 13.

Department of Surgery, Guangzhou First People's Hospital, No. 1 Panfu Road, Yuexiu District, Guangzhou, Guangdong 510180, People's Republic of China.

Primary sclerosing cholangitis (PSC) is a cholangiopathy caused by genetic and microenvironmental changes, such as bile homeostasis disorders and microbiota dysbiosis. Therapeutic options are limited, and proven surveillance strategies are currently lacking. Clinically, PSC presents as alternating strictures and dilatations of biliary ducts, resulting in the typical "beaded" appearance seen on cholangiography. Read More

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A case report of drug-induced liver injury due to the infliximab biosimilar CT-P13 on switching from original infliximab in a patient with Crohn's disease.

Ther Adv Drug Saf 2022 24;13:20420986221100118. Epub 2022 May 24.

Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan.

Inflammatory bowel diseases (IBDs) are chronic immune disorders of unclear etiology. Tumor necrosis factor (TNF) inhibitors are effective for IBD treatment and are cost-effective because they reduce hospital admissions and are associated with fewer surgery requirements and a better quality of life in IBD patients. A large number of clinical trials of infliximab biosimilar (CT-P13) have suggested that the administration of biosimilars provides high efficacy and safety similar to that of the originators, with a lower cost, so switching from the original to a biosimilar is considered an acceptable treatment. Read More

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Challenges and opportunities in achieving effective regulatory T cell therapy in autoimmune liver disease.

Semin Immunopathol 2022 May 31. Epub 2022 May 31.

Centre for Liver and Gastrointestinal Research & NIHR Birmingham Liver Biomedical Research Unit, Institute of Biomedical Research, Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, B15 2TT, UK.

Autoimmune liver diseases (AILD) include autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). These immune-mediated liver diseases involve a break down in peripheral self-tolerance with largely unknown aetiology. Regulatory T cells (Treg) are crucial in maintaining immunological tolerance. Read More

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Fecal microbiota and bile acids in IBD patients undergoing screening for colorectal cancer.

Gut Microbes 2022 Jan-Dec;14(1):2078620

Gastroenterology Department, Sorbonne University, INSERM, Centre de Recherche Saint-Antoine, CRSA, AP-HP, Saint Antoine Hospital, Paris, France.

Due to the potential role of the gut microbiota and bile acids in the pathogenesis of both inflammatory bowel disease (IBD) and sporadic colorectal cancer, we aimed to determine whether these factors were associated with colorectal cancer in IBD patients. 215 IBD patients and 51 non-IBD control subjects were enrolled from 10 French IBD centers between September 2011 and July 2018. Fecal samples were processed for bacterial 16S rRNA gene sequencing and bile acid profiling. Read More

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