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Am J Gastroenterol 2021 May 13. Epub 2021 May 13.

Division of Hepatology, Bruce W Carter VA Medical Center, Miami, Florida, USA; Department of Medicine, Jackson Memorial Hospital, Miami, Florida, USA; Division of Digestive Health and Liver Diseases, University of Miami Miller School of Medicine, Miami, Florida, USA; Department of Health Behavior and Policy, Virginia Commonwealth University, Richmond, Virginia, USA; Division of Gastroenterology and Hepatology, University of Pennsylvania, Philadelphia, Pennsylvania, USA; Division of Gastroenterology and Hepatology, Corporal Michael J. Crescenz VA Medical Center, Philadelphia, Pennsylvania, USA; Section of Digestive Diseases, Yale School of Medicine, New Haven, Connecticut, USA; Division of Gastroenterology and Hepatology, VA Connecticut Healthcare System, West Haven, Connecticut, USA.

Introduction: Patients with cirrhosis and men have been under-represented in most studies examining the clinical benefit of response to ursodeoxycholic acid (UDCA) in primary biliary cholangitis (PBC). The aim of this study was to study the association of UDCA response and liver-related death or transplantation, hepatic decompensation, and hepatocellular carcinoma (HCC) in patients with PBC cirrhosis.

Methods: We conducted a retrospective cohort study of veterans, predominantly men, with PBC and compensated cirrhosis to assess the association of UDCA response with the development of all-cause and liver-related mortality or transplantation, hepatic decompensation, and HCC using competing risk time-updating Cox proportional hazards models. Read More

Ann Transl Med 2021 Apr;9(8):738

Institute of Molecular Pathobiochemistry, Experimental Gene Therapy and Clinical Chemistry, RWTH University Hospital Aachen, Aachen, Germany.

Primary sclerosing cholangitis (PSC) is a rare progressive cholangitis resulting in cirrhosis and cholangiocellular carcinoma. The pathogenesis is unclear and an effective medical therapy is not available. It is highly associated to ulcerative colitis for which recently a disturbance of the tight junction (TJ) barrier has been claimed as etiologic feature. Read More

Ann Transl Med 2021 Apr;9(8):733

Department of Pathology, Medical University of Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.

Liver biopsy is an essential and necessary element in the diagnosis and management of autoimmune hepatitis, and is of very special importance in the scoring system for diagnosis. Histopathology shows moderate to severe inflammatory infiltrates with abundant plasma cells in the enlarged portal tracts with interface hepatitis and moderate to severe necroinflammatory lesions in the lobules with lymphoplasmacytic reaction. Regeneration develops with rosette formation and regenerative nodules. Read More

Mol Immunol 2021 May 10;135:342-350. Epub 2021 May 10.

Department of Gastroenterology, The Second Affiliated Hospital of Kunming Medical University, Kunming, 650101 Yunnan Province, China; Yunnan Research Center for Liver Diseases, Kunming, 650101 Yunnan Province, China. Electronic address:

Tim-3, which is expressed on a variety of innate immune cells including NK cells, plays a key role in many autoimmune diseases. However, the immunomodulatory actions of Tim-3 on NK cells in primary biliary cholangitis (PBC) remain uncertain. Using a murine model of PBC we evaluated the expression of Tim-3 and its ligand Gal-9 in peripheral blood, liver, and spleen. Read More

Can Liver J 2019 9;2(1):31-44. Epub 2019 Jan 9.

Department of Medicine, University of Alberta, Edmonton, Alberta, Canada.

Background: Prior studies using reverse transcriptase inhibitors to treat a human betaretrovirus (HBRV) in patients with primary biliary cholangitis (PBC) resulted in a 21% reduction in alkaline phosphatase (ALP). Herein, we studied the safety and efficacy of combination tenofovir-emtricitabine (TDF/FTC) and lopinavir-ritonavir (LPRr) in PBC patients unresponsive to ursodeoxycholic acid (UDCA).

Methods: A double-blind randomized controlled trial was performed in patients on UDCA for 6 months or more with ALP levels greater than two-fold the upper limit of normal or bilirubin greater than the upper limit of normal. Read More

Sci Rep 2021 May 12;11(1):10134. Epub 2021 May 12.

Department of Medical Biology, Pomeranian Medical University, 70-111, Szczecin, Poland.

Primary sclerosing cholangitis (PSC) is commonly accompanied by ulcerative colitis (UC). MicroRNA-506 modulates expression of genes which are essential for sphingosine-mediated signaling pathway and intestinal mucosa protection. We investigated whether miR-506 and its target genes are involved in phenotypic presentations of colonic inflammation and/or neoplasia. Read More

Pharmacoepidemiol Drug Saf 2021 May 12. Epub 2021 May 12.

Department of Medicine, Division of Digestive Health and Liver Disease, University of Miami Miller School of Medicine, Miami, FL.

Purpose: Population-level studies on the treatment practices and comparative effectiveness of therapies in autoimmune hepatitis (AIH) are lacking due to the absence of validated methods to identify patients with AIH in large databases, such as administrative claims or electronic health records. This study ascertained the performance of International Classification of Diseases (ICD) codes for AIH, and developed and validated a novel algorithm that reliably identifies patients with AIH in health administrative data and claims.

Methods: This was a cross-sectional study of patients with ≥1 inpatient or ≥2 outpatient ICD codes for AIH between 2008-2019 at a single health system. Read More

Surg Today 2021 May 11. Epub 2021 May 11.

Department of Pediatric Surgery, West China Hospital of Sichuan University, #37 Guo-Xue-Xiang, Chengdu, 610041, Sichuan, China.

Purpose: To investigate the clinical characteristics of cystic biliary atresia (CBA) and evaluate the midterm follow-up outcomes after laparoscopic treatment.

Methods: We analyzed and compared data retrospectively on CBA patients (group A) and nonsyndromic type III biliary atresia (BA) patients (group B), who underwent laparoscopic Kasai portoenterostomy (LKPE) during the same period.

Results: There were no significant differences in operative time, conversion rate, or the incidence of any postoperative complications between groups A and B (P > 0. Read More

Surg Endosc 2021 May 11. Epub 2021 May 11.

Center for Advanced Endoscopy, Division of Gastroenterology, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, Rabb-Rose 101, Boston, MA, 02215, USA.

Background: The optimal timing of biliary drainage by endoscopic retrograde cholangiopancreatography (ERCP) for patients with acute cholangitis remains controversial. The aim of our study was to determine if ERCP performed within 6 or 12 h of presentation was associated with improved clinical outcomes.

Methods: Medical records for all patients with acute cholangitis who underwent ERCP at our institution between 2009 and 2018 were reviewed. Read More

Surg Endosc 2021 May 11. Epub 2021 May 11.

Department of Gastroenterology and Liver Diseases, Faculty of Medicine, Shaare Zedek Medical Center, Hebrew University of Jerusalem, Jerusalem, Israel.

Background: Dominant stricture (DS) is a main cause of complication among patients with Primary Sclerosing Cholangitis (PSC). Endoscopic treatment options include dilation and plastic stenting, each with its own benefits and drawbacks.

Aims: The aim of the current study is to assess the safety and efficacy of fully-covered metal stent (FCMS) in these patients. Read More

J Immunol Res 2021 23;2021:6890423. Epub 2021 Apr 23.

Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.

Background And Aims: Primary biliary cholangitis (PBC) is an autoimmune cholestatic liver disease. We found microRNA-34a (miR-34a), as the downstream gene of p53, was overexpressed in some of fibrogenic diseases. In this study, we sought to explore whether miR-34a plays a role in the fibrosis of PBC. Read More

Gan To Kagaku Ryoho 2021 Apr;48(4):572-574

Dept. of Surgery, Ikeda City Hospital.

During the follow‒up of Vater papillary adenoma, a 74‒year‒old man was admitted to our hospital with a chief complaint of upper abdominal pain and diagnosed as cholangitis with obstructive jaundice. Cholestasis had been considered to be caused by papillary adenoma, however, EUS exam showed continuous bile duct wall irregularity from papilla of Vater. So we diagnosed as papillary carcinoma with extension to the distal bile duct. Read More

HPB (Oxford) 2021 Apr 28. Epub 2021 Apr 28.

Division of Surgery, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.

Background: Resection for perihilar cholangiocarcinoma (pCCA) in primary sclerosing cholangitis (PSC) has been reported to lead to worse outcomes than resection for non-PSC pCCA. The aim of this study was to compare prognostic factors and outcomes after resection in patients with PSC-associated pCCA and non-PSC pCCA.

Methods: The international retrospective cohort comprised patients resected for pCCA from 21 centres (2000-2020). Read More

Sci Rep 2021 May 10;11(1):9820. Epub 2021 May 10.

Department of Radiology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117, Berlin, Germany.

Spatial heterogeneity of hepatic fibrosis in primary sclerosing cholangitis (PSC) in comparison to viral hepatitis was assessed as a potential new biomarker using MR elastography (MRE). In this proof-of-concept study, we hypothesized a rather increased heterogeneity in PSC and a rather homogeneous distribution in viral hepatitis. Forty-six consecutive subjects (PSC: n = 20, viral hepatitis: n = 26) were prospectively enrolled between July 2014 and April 2017. Read More

HPB (Oxford) 2021 Apr 27. Epub 2021 Apr 27.

Department of Gastroenterological Surgery, Aichi Cancer Center Hospital, Kanokoden 1-1, Chikusa-ku, Nagoya, Japan.

Background: It is unclear whether hepaticojejunostomy with a continuous suture is suitable for patients with a non-dilated bile duct.

Methods: Medical records of patients who underwent pancreatoduodenectomy between 2003 and 2013 were retrospectively reviewed, focusing on the incidence of benign anastomotic stenosis and its relationship with suture method (continuous vs interrupted) and common hepatic duct size.

Results: Among 336 patients, 172 had a non-dilated (<8 mm) duct, and the remaining 164 had a dilated duct. Read More

Hepatology 2021 May 10. Epub 2021 May 10.

Institute of Liver Studies, MowatLabs, Department of Inflammation Biology, School of Immunology & Microbial Sciences, Faculty of Liver Sciences and Medicine, King's College London, London, UK.

Background And Aims: Genetic predisposition to autoimmune hepatitis (AIH) in adults is associated with possession of HLA class I (A*01, B*08) and class II alleles (DRB1*03, -04, -07 or -13), depending on geographic regions. Juvenile autoimmune liver disease (AILD) comprises AIH-1, AIH-2 and autoimmune sclerosing cholangitis (ASC), which are phenotypically different from their adult counterparts. We aimed to define the relationship between HLA profile and disease course, severity and outcome in juvenile AILD. Read More

J Laparoendosc Adv Surg Tech A 2021 May 7. Epub 2021 May 7.

Division of Hepato-biliary-pancreatic Surgery, Department of Surgery, Hospital de la Santa Creu i Sant Pau, Universitat Autonoma de Barcelona, Spain.

Biliary tree cysts (BTCs) represent an either localized or multifocal abnormal dilatation of the biliary tree, which entails an increased risk of acute cholangitis and cholangiocarcinoma (2.5%-16%). Its incidence in Western countries is ∼1/100. Read More

Cureus 2021 May 6;13(5):e14871. Epub 2021 May 6.

Medicine, College of Medicine and Medical Sciences, Arabian Gulf University, Manama, BHR.

Objectives The clinical course and progression of acute pancreatitis are poorly understood to date, necessitating more studies of clinical profiles during the disease. Moreover, understanding the etiologies and clinical presentations of acute pancreatitis (AP) in children can contribute to early diagnosis and, hence, earlier interventions. Therefore, this article aims to study the clinical profiles of children with acute pancreatitis (AP) in relation to complications and other variables. Read More

Clin Liver Dis (Hoboken) 2021 Apr 1;17(4):227-231. Epub 2021 May 1.

Department of Laboratory Medicine and Pathology Mayo Clinic Florida Jacksonville FL.

Intern Med 2021 May 7. Epub 2021 May 7.

Department of Internal Medicine, Nagahama City Kohoku Hospital, Japan.

Pediatr Hematol Oncol 2021 May 8:1-8. Epub 2021 May 8.

Faculty of Medicine, Department of Pediatric Immunology and Allergy, Ege University, İzmir, Turkey.

Mutations in the interleukin-21 receptor (IL-21R) gene are recently defined as primary immunodeficiency diseases. IL-21R defects result in combined immunodeficiency by affecting the functions of innate and adaptive immune system components.A six-year-old girl was admitted to our hospital with complaints of chronic diarrhea that started after the newborn period and generalized rash over the last three months. Read More

J Pediatr Surg 2021 Apr 9. Epub 2021 Apr 9.

Vietnam National Children Hospital, Hanoi, Vietnam.

Aims: In RCT of adults with decompensated cirrhosis, GCSF mobilizes hematopoietic stem cells HSC and improves short-term outcome. An FDA-IND for sequential Kasai-GCSF treatment in biliary atresia BA was approved. This phase 1 study examines GCSF safety in Kasai subjects. Read More

Int J Surg Case Rep 2021 Apr 30;82:105936. Epub 2021 Apr 30.

Department of Surgery, Shiga General Hospital, Moriyama, 5-4-30 Moriyama, Moriyama, Shiga 524-8524, Japan. Electronic address:

Introduction And Importance: The most common liver malignancies are hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and metastatic tumors. Hepatocellular carcinoma and intrahepatic cholangiocarcinoma may invade the portal vein (PV). An association between diffuse large B-cell lymphoma (DLBCL) and primary biliary cholangitis (PBC) remains unclear. Read More

Int J Clin Pract 2021 May 6:e14324. Epub 2021 May 6.

Department of Infectious Diseases and Clinical Microbiology, Inonu University Faculty of Medicine, 44280, Malatya, Turkey.

Aim: To analyze developing infections after living donor hepatectomy (LDH) in living liver donors (LLDs).

Methods: Demographic and clinical charecteristics of 1106 LLDs were retrospectively analysed in terms of whether postoperative infection development. Therefore, LLDs were divided into two group: with (n=190) and without (n=916) antimicrobial agent use. Read More

World J Hepatol 2021 Apr;13(4):472-482

Division of Gastroenterology and Hepatology, SUNY Upstate Medical University and Syracuse VA Medical Center, Syracuse, NY 13210, United States.

Background: There is minimal objective data regarding adverse events related to endoscopic retrograde cholangio-pancreatography (ERCP) in patients with cirrhosis compared to those without cirrhosis and even fewer data comparing complications among cirrhosis patients based on severity of cirrhosis.

Aim: To determine if patients with cirrhosis are at increased risk of adverse events related to ERCP: mainly pancreatitis, bleeding, perforation, cholangitis, and mortality; And to see if higher Child-Pugh (CP) score and Model for End-Stage Liver Disease (MELD) score are associated with higher post-ERCP complications.

Methods: We performed a retrospective analysis of 692 patients who underwent ERCP and analyzed the impact of cirrhosis etiology, gender, type of sedation used during procedure, interventions performed, and co-morbidities on the rate of complications in cirrhosis patients as compared to non-cirrhosis patients. Read More

World J Hepatol 2021 Apr;13(4):456-471

Department of General Surgery, Tan Tock Seng Hospital, Singapore 308433, Singapore.

Background: Acute cholangitis (AC) is a disease spectrum with varying extent of severity. Age ≥ 75 years forms part of the criteria for moderate (Grade II) severity in both the Tokyo Guidelines (TG13 and TG18). Aging is associated with reduced physiological reserves, frailty, and sarcopenia. Read More

BMJ Case Rep 2021 May 6;14(5). Epub 2021 May 6.

Gastroenterology, Showa University Fujigaoka Hospital, Yokohama, Japan.

Although prednisolone, granulocyte/monocyte apheresis, calcineurin inhibitor and anti-tumour necrosis factor (TNF) therapy are generally used, no treatment strategy for inflammatory bowel disease complicated with pyoderma gangrenosum (PG) has been established yet. Herein, we present the case of a 29-year-old man with ulcerative colitis (UC) complicated with primary sclerosing cholangitis. When UC relapsed and PG developed, prednisolone and granulocyte/monocyte apheresis were used; however, their therapeutic effects were deemed insufficient. Read More

Surgery 2021 May 3. Epub 2021 May 3.

Second Department of Surgery, School of Medicine, Wakayama Medical University, Wakayama, Japan.

Background: Pancreaticoduodenectomy without subsequent nasogastric tube management has not been widely adopted due to delayed gastric emptying, the specific and frequent morbidity associated with this surgical procedure. We assessed the feasibility of pancreaticoduodenectomy without use of nasogastric tubes and the risk factors for subsequent nasogastric tube reinsertion.

Methods: We retrospectively reviewed 465 patients who underwent pancreaticoduodenectomy at a single institution between 2010 and 2019. Read More

Diagn Pathol 2021 May 6;16(1):41. Epub 2021 May 6.

Department of Pathology, University of Helsinki and Helsinki University Hospital, Haartmaninkatu 3, 00290, Helsinki, Finland.

Background: The objective was to build a novel method for automated image analysis to locate and quantify the number of cytokeratin 7 (K7)-positive hepatocytes reflecting cholestasis by applying deep learning neural networks (AI model) in a cohort of 210 liver specimens. We aimed to study the correlation between the AI model's results and disease progression. The cohort of liver biopsies which served as a model of chronic cholestatic liver disease comprised of patients diagnosed with primary sclerosing cholangitis (PSC). Read More

Semin Liver Dis 2021 May 6. Epub 2021 May 6.

Section of Digestive Diseases, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut.

Translational studies in human cholestatic diseases have for years been hindered by various challenges, including the rarity of the disorders, the difficulty in obtaining biliary tissue from across the spectrum of the disease stage, and the difficulty culturing and maintaining primary cholangiocytes. Organoid technology is increasingly being viewed as a technological breakthrough in translational medicine as it allows the culture and biobanking of self-organizing cells from various sources that facilitate the study of pathophysiology and therapeutics, including from individual patients in a personalized approach. This review describes current research using biliary organoids for the study of human cholestatic diseases and the emerging applications of organoids to regenerative medicine directed at the biliary tree. Read More