15,200 results match your criteria Primary Biliary Cirrhosis


Liver Damage and Exposure to Toxic Concentrations of Endogenous Retinoids in the Pathogenesis of COVID-19 Disease: Hypothesis.

Viral Immunol 2021 May 13. Epub 2021 May 13.

Department of Ophthalmology and Pathology, University of Mississippi Medical Center, Jackson, Mississippi, USA.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has a marked tropism for the biliary tract; it damages the bile ducts and hepatocytes and can lead to liver decompensation, cirrhosis, and sepsis. The pathogenesis of liver damage and its association with damage to the lung, heart, and brain and to the other protean manifestations of COVID-19 disease are not fully understood. In particular, tissue damage from thinning and leaky blood vessels appears to result from an inflammatory response to the virus rather than the virus itself. Read More

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Cystatin C: best biomarker for acute kidney injury and estimation of glomerular filtration rate in childhood cirrhosis.

Eur J Pediatr 2021 May 12. Epub 2021 May 12.

Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, 110070, India.

The objective of the study was to evaluate the diagnostic and prognostic role of serum cystatin C, urinary neutrophil gelatinase-associated lipocalin (NGAL), and renal resistive index (RRI) in AKI among pediatric cirrhotics. The study included cirrhotic children under 18 years of age. AKI was diagnosed as per Kidney Diseases-Improving Global Outcomes (KDIGO) guidelines. Read More

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Cholangiocyte Organoids as a Cell Source for Biliary Repair.

Transpl Int 2021 May 11. Epub 2021 May 11.

Department of Pathology, Division of Experimental Pathology, School of Medicine, University of Pittsburgh, Pittsburgh, PA, USA.

Preoccupied with the astounding regenerative capabilities and metabolic importance of hepatocytes in the liver, the role of the resident "plumbers" of the liver, the cholangiocytes, can be overlooked in comparison. Cholangiocytes form the ductal network which makes up the biliary tree and transports bile from the liver to the gallbladder for secretion into the intestine during digestion. Chronic accumulation of bile in the liver is cytotoxic, causing fibrosis and eventually leads to cirrhosis and liver failure. Read More

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Practical approach for the diagnosis of biliary atresia on imaging, part 2: magnetic resonance cholecystopancreatography, hepatobiliary scintigraphy, percutaneous cholecysto-cholangiography, endoscopic retrograde cholangiopancreatography, percutaneous liver biopsy, risk scores and decisional flowchart.

Pediatr Radiol 2021 May 11. Epub 2021 May 11.

APHM, Equipe d'Accueil 3279 - IFR 125, Service d'Imagerie Pédiatrique et Prénatale, Hôpital Timone Enfants, Aix Marseille Univ, Marseille, France.

We aim to present a practical approach to imaging in suspected biliary atresia, an inflammatory cholangiopathy of infancy resulting in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. Left untreated or with failure of the Kasai procedure, biliary atresia progresses to biliary cirrhosis, end-stage liver failure and death within the first years of life. Differentiating biliary atresia from other nonsurgical causes of neonatal cholestasis is difficult as there is no single method for diagnosing biliary atresia and clinical, laboratory and imaging features of this disease overlap with those of other causes of neonatal cholestasis. Read More

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Laparoscopic partial liver resection for hepatocellular carcinoma arising from Fontan-associated liver disease: a case report.

Surg Case Rep 2021 May 10;7(1):115. Epub 2021 May 10.

Department of Hepato-Biliary-Pancreatic and Breast Surgery, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime, 791-0295, Japan.

Background: The Fontan procedure (FP) is a palliative surgery for functional single ventricle. The Fontan circulation maintains pulmonary circulation by a high central venous pressure, leading to chronic congestive liver. The number of patients diagnosed with hepatocellular carcinoma (HCC) arising from liver fibrosis and cirrhosis after FP is increasing. Read More

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Single-Cell RNA-seq Reveals Angiotensin-Converting Enzyme 2 and Transmembrane Serine Protease 2 Expression in TROP2 Liver Progenitor Cells: Implications in Coronavirus Disease 2019-Associated Liver Dysfunction.

Front Med (Lausanne) 2021 22;8:603374. Epub 2021 Apr 22.

Genome Institute of Singapore, Agency for Science, Technology and Research, Singapore, Singapore.

The recent coronavirus disease 2019 (COVID-19) pandemic is caused by severe acute respiratory syndrome coronavirus 2. COVID-19 was first reported in China (December 2019) and is now prevalent across the globe. Entry of severe acute respiratory syndrome coronavirus 2 into mammalian cells requires the binding of viral Spike (S) proteins to the angiotensin-converting enzyme 2 receptor. Read More

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Cartilage-hair Hypoplasia Complicated with Liver Cirrhosis Due to Chronic Intrahepatic Cholestasis: A Case Report.

Intern Med 2021 May 7. Epub 2021 May 7.

Department of Gastroenterology, Internal Medicine, Kitasato University School of Medicine, Japan.

We herein report a rare case of cartilage-hair hypoplasia (CHH) complicated with liver cirrhosis. A 20-year-old Japanese man with CHH was found incidentally to have liver cirrhosis and an esophageal varix. This patient had been treated for infections due to immunodeficiency since early childhood. Read More

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Myositis with Anti-mitochondrial Antibody Type 2 with Diplopia and Ptosis.

Intern Med 2021 May 7. Epub 2021 May 7.

Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Japan.

Anti-mitochondrial antibody type 2 is a diagnostic marker of primary biliary cirrhosis and complicates myositis. Myositis with anti-mitochondrial antibody type 2 is clinically characterized by slowly progressive limb, cardiac, and respiratory muscle weakness as well as serum creatinine kinase elevations. However, there has been few cases with eye symptoms. Read More

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Incidence of Hepatocellular Carcinoma in Patients with Non-alcoholic Fatty Liver Disease: A Systematic Review, Meta-analysis, and Meta-regression.

Clin Gastroenterol Hepatol 2021 May 6. Epub 2021 May 6.

Division of Abdominal and Transplantation Surgery, Department of Surgery, Geneva University Hospitals and Faculty of Medicine, Geneva, Switzerland; Hepato-pancreato-biliary Centre, Geneva University Hospitals and Faculty of Medicine, Geneva, Switzerland. Electronic address:

Background & Aims: Nonalcoholic fatty liver disease (NAFLD) may be a risk factor for hepatocellular carcinoma (HCC), but the extent of this association still needs to be addressed. Pooled-incidence rates of HCC across the disease spectrum of NAFLD have never been estimated by meta-analysis.

Methods: In this systematic review, we searched Web of Science, Embase, Pubmed, and the Cochrane library from January 1, 1950 through July 30, 2020. Read More

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Impact of serum autotaxin level correlating with histological findings in biliary atresia.

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

Department of Pediatric Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan.

Purpose: Portoenterostomy is the standard treatment for biliary atresia (BA) that reduces jaundice in two thirds of cases. However, progressive liver fibrosis is common, leading to cirrhosis in most patients. Autotaxin is a new marker for the progression of hepatic fibrosis. Read More

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Response to Lleo and Hirschfield.

Hepatology 2021 May 3. Epub 2021 May 3.

Division of Gastroenterology and Hepatology, University of Pennsylvania, Philadelphia, PA, USA.

We thank Lleo and Hirschfield for their comments on our manuscript exploring the association of male sex and liver-related mortality in patients with Primary Biliary Cholangitis (PBC) and cirrhosis . As they correctly point out, prior studies looking at the association of sex with PBC were limited by relatively low numbers of males in the cohort, and particularly those with cirrhosis . Read More

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Letter to the Editor: are we confident that PBC liver-related mortality is higher in males?

Hepatology 2021 May 3. Epub 2021 May 3.

Toronto Centre for Liver Disease, Division of Gastroenterology and Hepatology, University of Toronto, Toronto, ON, Canada.

We read with interest the recent communication reporting the prognostic impact of male sex in patients with primary biliary cholangitis (PBC) and well-compensated cirrhosis. The work raises important questions for clinicians managing PBC. Read More

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Endogenous Opioid Levels Do Not Correlate With Itch Intensity and Therapeutic Interventions in Hepatic Pruritus.

Front Med (Lausanne) 2021 14;8:641163. Epub 2021 Apr 14.

Department of Medicine 1, University Hospital Erlangen and Translational Research Center, Friedrich-Alexander-University Erlangen-Nürnberg, Erlangen, Germany.

Chronic pruritus affects up to 70% of patients with immune-mediated hepatobiliary disorders. Antagonists of the μ-opioid receptor (MOR) and agonists of the κ-opioid receptor (KOR) are used to treat hepatic itch, albeit with limited success. An imbalance between ligands of MOR and KOR receptors has recently been suggested as a potential mechanism of hepatic pruritus. Read More

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Effect of Vitamin D Supplementation on Vitamin D Level and Bone Mineral Density in Patients With Cirrhosis: A Randomized Clinical Trial.

Am J Gastroenterol 2021 Apr 29. Epub 2021 Apr 29.

Department of Gastroenterology and Human Nutrition Unit, All India Institute of Medical Sciences, New Delhi, India; Department of Clinical Nutrition, Institute of Liver and Biliary Sciences, New Delhi, India; Department of Cardiac Biochemistry, All India Institute of Medical Sciences, New Delhi, India; Department of Biostatistics, All India Institute of Medical Sciences, New Delhi, India.

Introduction: In patients with cirrhosis, highly prevalent vitamin D deficiency and low bone mineral density (BMD) increase the burden of disease, and role of vitamin D supplementation is not clear. So, our aim was to determine the effect of vitamin D supplementation on vitamin D level and BMD in patients with cirrhosis.

Methods: Patients with cirrhosis (18-60 years) of any etiology were enrolled. Read More

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The Management of Cholestatic Liver Diseases: Current Therapies and Emerging New Possibilities.

J Clin Med 2021 Apr 18;10(8). Epub 2021 Apr 18.

Clinic of Gastroenterology and Hepatology, Università Politecnica delle Marche, 60126 Ancona, Italy.

Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are two chronic cholestatic liver diseases affecting bile ducts that may progress to biliary cirrhosis. In the past few years, the increasing knowledge in the pathogenesis of both diseases led to a growing number of clinical trials and possible new targets for therapy. In this review, we provide an update on the treatments in clinical use and summarize the new drugs in trials for PBC and PSC patients. Read More

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Mesenchymal Stromal Cell Therapy in Novel Porcine Model of Diffuse Liver Damage Induced by Repeated Biliary Obstruction.

Int J Mol Sci 2021 Apr 21;22(9). Epub 2021 Apr 21.

Biomedical Center, Faculty of Medicine in Pilsen, Charles University, Alej Svobody 76, 323 00 Pilsen, Czech Republic.

In liver surgery, biliary obstruction can lead to secondary biliary cirrhosis, a life-threatening disease with liver transplantation as the only curative treatment option. Mesenchymal stromal cells (MSC) have been shown to improve liver function in both acute and chronic liver disease models. This study evaluated the effect of allogenic MSC transplantation in a large animal model of repeated biliary obstruction followed by partial hepatectomy. Read More

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Pathological Study of Facial Eczema (Pithomycotoxicosis) in Sheep.

Animals (Basel) 2021 Apr 9;11(4). Epub 2021 Apr 9.

Departamento de Sanidad Animal, Facultad de Veterinaria, Universidad de León, C/Prof. Pedro Cármenes s/n, E-24071 León, Spain.

Facial eczema (FE) is a secondary photosensitization disease of farm ruminants caused by the sporidesmin A, produced in the spores of the saprophytic fungus . This study communicates an outbreak of ovine FE in Asturias (Spain) and characterizes the serum biochemical pattern and the immune response that may contribute to liver damage, favoring cholestasis and the progression to fibrosis and cirrhosis. Animals showed clinical signs of photosensitivity, with decrease of daily weight gain and loss of wool and crusting for at least 6 months after the FE outbreak. Read More

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GOOD QUALITY OF LIFE AFTER MORE THAN A DECADE OF LIVING DONOR LIVER TRANSPLANTATION.

Arq Gastroenterol 2021 Jan-Mar;58(1):10-16

Serviço de Transplante Hepático, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brasil.

Background: Receptors of living donor liver transplantation (LDLT) have higher rate of postoperative biliary and vascular complications that may reduce posttransplant quality of life (QOL) due to the need of invasive and repetitive treatments.

Objective: The purpose of our study is to assess the various aspects of QOL of receptors undergoing LDLT after 10 years of transplantation and to identify potential factors that might be associated with impaired QOL.

Methods: Data of all patients with more than 10 years of LDLT were retrospectively evaluated. Read More

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[Risk and control management of endoscopic retrograde cholangiopancreatography in patients with liver cirrhosis].

Authors:
X M Li L G Lu X B Cai

Zhonghua Gan Zang Bing Za Zhi 2021 Mar;29(3):209-212

Department of Gastroenterology, Shanghai General Hospital, Shanghai Jiaotong University School of Medicine, Shanghai 200080, China.

Endoscopic retrograde cholangiopancreatography (ERCP) is the most direct and effective method for the diagnosis and treatment of biliary and pancreatic diseases. Compared with surgery, ERCP has the advantages of minimal trauma, shorter surgery time, fewer complications, and shorter hospital stay. Liver cirrhosis, as the advanced stage of liver disease, has reduced tolerance to surgical stresses, and complications such as hepatic encephalopathy, esophagogastric varices, and coagulation dysfunction may occur during the decompensated stage, which poses a challenge to ERCP, and thus increase the intraoperative risk and postoperative complications. Read More

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Correlation Between Portal Pressure and Indocyanine Green Retention Rate is Unaffected by the Cause of Cirrhosis: A Prospective Study.

World J Surg 2021 Apr 23. Epub 2021 Apr 23.

Department of Visceral Surgery, Lausanne University Hospital and University of Lausanne, Rue du Bugnon 46, 1011, Lausanne, Switzerland.

Background: Accurate estimation of the hepatic functional reserve before liver resection is important to avoid post-hepatectomy liver failure (PHLF). The aim of the present study was to evaluate the association of indocyanine green retention test with portal pressure by the cause of cirrhosis (non-viral vs. viral) and assessed postoperative outcomes including incidence of PHLF in patients with viral and non-viral cirrhosis. Read More

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COVID-19-Induced Bile Duct Casts and Cholangitis: A Case Report.

Cureus 2021 Apr 19;13(4):e14560. Epub 2021 Apr 19.

Gastroenterology, Virginia Mason Medical Center, Seattle, USA.

Coronavirus disease 2019 (COVID-19) infection can lead to various complications involving all of the major organ systems. Gastrointestinal manifestations such as nausea, vomiting, and diarrhea are commonly associated with this condition. Biliary complications from COVID-19 constitute an area of active research. Read More

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Validation of functional liver imaging scores (FLIS) derived from gadoxetic acid-enhanced MRI in patients with chronic liver disease and liver cirrhosis: the relationship between Child-Pugh score and FLIS.

Eur Radiol 2021 Apr 21. Epub 2021 Apr 21.

Department of Radiology, Biomedical Research Institute, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, South Korea.

Objectives: To validate the functional liver imaging score (FLIS) for prediction of hepatic function in gadoxetic acid-enhanced MRI.

Methods: We retrospectively identified 134 patients (88 men, 46 women; mean age, 58.8 years) between January 2015 and December 2018 with the following inclusion criteria: patients diagnosed with liver cirrhosis or chronic liver disease (CLD) who underwent gadoxetic acid-enhanced MRI. Read More

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Insight into Bile Duct Reaction to Obstruction from a Three-dimensional Perspective Using ex Vivo Phase-Contrast CT.

Radiology 2021 Apr 20:203967. Epub 2021 Apr 20.

From the School of Biomedical Engineering and Technology, Tianjin Medical University, No. 22 Qixiangtai Rd, Tianjin 300070, China (W.J.L., X.H.X., L.L.Q., C.H.H.); Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China (X.Y.Z.); Beijing Key Laboratory of Translational Medicine in Liver Cirrhosis and National Clinical Research Center of Digestive Disease, Beijing, China (X.Y.Z.); and the Second Department of Gastroenterology, Qingdao Municipal Hospital, Qingdao, China (D.D.H.).

Background Biliary obstruction leads to an increase in biliary pressure within the biliary system, which induces the morphologic adaptation of the biliary tree. Purpose To observe and to quantify the morphologic characteristics of the adaptation in a bile duct ligation rat model and verify it in patients with biliary atresia in a three-dimensional (3D) manner using x-ray phase-contrast CT. Materials and Methods A bile duct ligation model was induced in 40 male Sprague-Dawley rats, which were divided into five groups: the control group (no ligation) and groups 2, 4, 6, and 8 weeks after bile duct ligation (eight animals in each group). Read More

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Paricalcitol inhibits oxidative stress-induced cell senescence of the bile duct epithelium dependent on modulating Sirt1 pathway in cholestatic mice.

Free Radic Biol Med 2021 Apr 16;169:158-168. Epub 2021 Apr 16.

Department of Cell Biology, School of Medicine, Taizhou University, Taizhou, PR China. Electronic address:

Background: Clinical studies indicate that vitamin D receptor (VDR) expression is reduced in primary biliary cirrhosis patient livers. However, the mechanism by which activated VDR effect cholestatic liver injury remains unclear.

Methods: Mice were injected intraperitoneally with the VDR agonist paricalcitol or a vehicle 3 days prior to bile duct ligation (BDL) and for 5 or 28 days after surgery. Read More

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Differentiation of clinical patterns and survival outcomes of hepatocellular carcinoma on hepatitis B and nonalcoholic fatty liver disease.

J Chin Med Assoc 2021 Apr 12. Epub 2021 Apr 12.

Department of Gastroenterology, Ren-Ai Branch, Taipei City Hospital, Taipei, Taiwan, ROC Department of Pathology, Ren-Ai Branch, Taipei City Hospital, Taipei, Taiwan, ROC Department of Obstetrics and Gynecology, Taipei Medical University Hospital, Taipei, Taiwan, ROC University of Taipei, Taipei, Taiwan, ROC.

Background: The main etiologies of hepatocellular carcinoma (HCC) were often hepatitis B virus (HBV) or C and alcohol, rarely autoimmune and biliary diseases. Nonalcoholic fatty liver disease (NAFLD) has been an emerging role that could lead to chronic liver disease, nonalcoholic steatohepatitis, cirrhosis, and eventually HCC in recent years. The aim of our study is to investigate and compare the clinical features of HCC in NAFLD and HBV patients, including age, gender, cirrhosis, liver function tests, largest tumor size and cancer stage at the time of diagnosis. Read More

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Acute severe autoimmune hepatitis with anti-rods and rings autoantibodies; literature first evidence.

Gastroenterol Hepatol Bed Bench 2021 ;14(1):89-94

Clinical Investigation Laboratory, ASST Bergamo Est, Seriate, Bergamo, Italy.

Autoimmune hepatitis (AIH) was defined as a progressive, chronic inflammatory autoimmune liver disease (ALD). The diagnosis of AIH requires the presence of characteristic clinical and laboratory features, and the exclusion of other clinical conditions that cause chronic hepatitis and cirrhosis. AIH can have an acute onset that mimics an acute viral or toxic hepatitis or an acute severe (fulminant, ASF) presentation that satisfies criteria for acute liver. Read More

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January 2021

Transcriptional regulator BOB.1: Molecular mechanisms and emerging role in chronic inflammation and autoimmunity.

Autoimmun Rev 2021 Apr 20;20(6):102833. Epub 2021 Apr 20.

CHU Nantes, Université de Nantes, Inserm, Centre de Recherche en Transplantation et Immunologie, UMR 1064, ITUN, Nantes, France.

Lymphocytes constitute an essential and potent effector compartment of the immune system. Therefore, their development and functions must be strictly regulated to avoid inappropriate immune responses, such as autoimmune reactions. Several lines of evidence from genetics (e. Read More

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A ZFYVE19 gene mutation associated with neonatal cholestasis and cilia dysfunction: case report with a novel pathogenic variant.

Orphanet J Rare Dis 2021 Apr 14;16(1):179. Epub 2021 Apr 14.

Postgraduate School of Pediatrics Department of Medicine, Surgery and Dentistry "Scuola Medica Salernitana", University of Salerno, Via Allende, 84081, Baronissi, (SA), Italy.

Background: ZFYVE19 (Zinc Finger FYVE-Type Containing 19) mutations have most recently been associated to a novel type of high gamma-glutamyl transpeptidase (GGT), non-syndromic, neonatal-onset intrahepatic chronic cholestasis possibly associated to cilia dysfunction. Herein, we report a new case with further studies of whole exome sequencing (WES) and immunofluorescence in primary cilia of her cultured fibroblasts which confirm the observation.

Results: A now 5-year-old girl born to clinically healthy consanguineous Moroccan parents was assessed at 59 days of life due to severe cholestatic jaundice with increased serum bile acids and GGT, and preserved hepatocellular synthetic function. Read More

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Impact of sarcopenia on clinical outcomes of patients undergoing simultaneous liver and kidney transplantation: a cohort study.

Clin Res Hepatol Gastroenterol 2021 Apr 10:101692. Epub 2021 Apr 10.

APHP, Unité Médicale de Transplantation Hépatique Hôpital Pitié Salpêtrière, Boulevard de l'Hôpital 47-83, 75013 Paris, France; Sorbonne Université, INSERM, Centre de recherche Saint-Antoine (CRSA), Paris, France; Sorbonne Université, INSERM, Institute of Cardiometabolisme and Nutrition (ICAN), Paris, France.

Background: The impact of sarcopenia in patients undergoing simultaneous liver and kidney transplantation (SLKT) has not been fully delineated. The aim of this single-centre-cohort-study was to evaluate the impact of sarcopenia on the clinical outcomes.

Methods: Between 2003 and 2018, 79 patients underwent SLKT. Read More

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OptBand: optimization-based confidence bands for functions to characterize time-to-event distributions.

Authors:
T Chen S Tracy H Uno

Lifetime Data Anal 2021 Apr 13. Epub 2021 Apr 13.

Department of Data Science, Dana-Faber Cancer Institute, Boston, USA.

Classical simultaneous confidence bands for survival functions (i.e., Hall-Wellner, equal precision, and empirical likelihood bands) are derived from transformations of the asymptotic Brownian nature of the Nelson-Aalen or Kaplan-Meier estimators. Read More

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