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https://www.readbyqxmd.com/read/29112089/assessment-of-liver-disease-progression-in-cystic-fibrosis-using-transient-elastography
#1
Anne-Laure Gominon, Eric Frison, Jean-Baptiste Hiriart, Julien Vergniol, Haude Clouzeau, Raphael Enaud, Stephanie Bui, Michael Fayon, Victor de Ledinghen, Thierry Lamireau
OBJECTIVES: Cystic fibrosis related liver disease (CFLD) can develop silently in early life and approximately 10% of children with cystic fibrosis (CF) become cirrhotic before adulthood. Clinical, biological and ultrasound criteria used to define CFLD often reveal liver involvement at an advanced stage. The aim of this retrospective study was to assess the progression of liver stiffness measurement (LSM) in pediatric CF patients. METHODS: The change of LSM, expressed as kPa/year and %/year, was measured using transient elastography (TE, Fibroscan) in 82 CF children (median age: 6...
November 3, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28935574/taurocholate-induces-biliary-differentiation-of-liver-progenitor-cells-causing-hepatic-stellate-cell-chemotaxis-in-the-ductular-reaction-role-in-pediatric-cystic-fibrosis-liver-disease
#2
Katarzyna N Pozniak, Michael A Pearen, Tamara N Pereira, Cynthia S M Kramer, Priyakshi Kalita-De Croft, Sujeevi K Nawaratna, Manuel A Fernandez-Rojo, Geoffrey N Gobert, Janina E E Tirnitz-Parker, John K Olynyk, Ross W Shepherd, Peter J Lewindon, Grant A Ramm
Cystic fibrosis liver disease (CFLD) in children causes progressive fibrosis leading to biliary cirrhosis; however, its cause(s) and early pathogenesis are unclear. We hypothesized that a bile acid-induced ductular reaction (DR) drives fibrogenesis. The DR was evaluated by cytokeratin-7 immunohistochemistry in liver biopsies, staged for fibrosis, from 60 children with CFLD, and it demonstrated that the DR was significantly correlated with hepatic fibrosis stage and biliary taurocholate levels. To examine the mechanisms involved in DR induction, liver progenitor cells (LPCs) were treated with taurocholate, and key events in DR evolution were assessed: LPC proliferation, LPC biliary differentiation, and hepatic stellate cell (HSC) chemotaxis...
September 19, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28837442/diagnosis-follow-up-and-treatment-of-cystic-fibrosis-related-liver-disease
#3
Ivo P van de Peppel, Anna Bertolini, Johan W Jonker, Frank A J A Bodewes, Henkjan J Verkade
PURPOSE OF REVIEW: To provide an insight and overview of the challenges in the diagnosis, follow-up and treatment of cystic fibrosis-related liver disease (CFLD). RECENT FINDINGS: The variable pathophysiology of CFLD complicates its diagnosis and treatment. A 'gold standard' for CFLD diagnosis is lacking. Over the past years, new techniques to diagnose features of CFLD, such as transient elastography, have been investigated. Although most of these tests confirm cystic fibrosis-related liver involvement (CFLI), they are, however, not suitable to distinguish various phenotypical presentations or predict progression to clinically relevant cirrhosis or portal hypertension...
August 23, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28836688/src-kinase-inhibition-reduces-inflammatory-and-cytoskeletal-changes-in-%C3%AE-f508-human-cholangiocytes-and-improves-cftr-correctors-efficacy
#4
Romina Fiorotto, Mariangela Amenduni, Valeria Mariotti, Luca Fabris, Carlo Spirli, Mario Strazzabosco
CFTR, the channel mutated in cystic fibrosis (CF), is expressed by the biliary epithelium (i.e cholangiocytes) of the liver. Progressive clinical liver disease (CFLD) occurs in about 10% of CF patients and represents the third leading cause of death. Impaired secretion and inflammation contribute to CFLD, however the lack of human-derived experimental models has hampered the understanding of CFLD pathophysiology and the search for a cure. We have investigated the cellular mechanisms altered in human CF cholangiocytes using induced pluripotent stem cells (iPSC) derived from healthy controls and a ΔF508 CFTR patient...
July 24, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28753176/cystic-fibrosis-related-liver-disease-research-challenges-and-future-perspectives
#5
Dominique Debray, Michael R Narkewicz, Frank A J A Bodewes, Carla Colombo, Chantal Housset, Hugo R de Jonge, Johan W Jonker, Deirdre A Kelly, Simon C Ling, Thierry Poynard, Philippe Sogni, Michael Trauner, Peter Witters, Ulrich Baumann, Michael Wilschanski, Henkjan J Verkade
OBJECTIVES: Hepatobiliary complications are a leading cause of morbidity and mortality in cystic fibrosis (CF) patients. However, knowledge of the underlying pathological aspects and optimal clinical management is sorely lacking. METHODS: We provide a summary of the lectures given by international speakers at the ESPGHAN monothematic conference on CF-related liver disease (CFLD) held in Paris in January 2016, to discuss the status of our current knowledge of liver disease in CF patients, to define the critical areas that need to be addressed and to resolve actions to elucidate relevant mechanisms of disease to optimise future therapeutic options...
July 27, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28575039/assessment-of-pathologic-increase-in-liver-stiffness-enables-earlier-diagnosis-of-cfld-results-from-a-prospective-longitudinal-cohort-study
#6
Victoria Klotter, Caroline Gunchick, Enno Siemers, Timo Rath, Helge Hudel, Lutz Naehrlich, Martin Roderfeld, Elke Roeb
About 30% of patients with Cystic Fibrosis (CF) develop CF-associated liver disease (CFLD). Recent studies have shown that transient elastography (TE), as a method to quantify liver stiffness, allows non-invasive diagnosis of CFLD in adults and children with CF. Within this study we aimed to prospectively identify patients at risk for development of CFLD by longitudinal analysis of liver stiffness and fibrosis scores in a 5-year follow-up. 36 pediatric and 16 adult patients with initial liver stiffness below the cut-off value indicative of CFLD (6...
2017: PloS One
https://www.readbyqxmd.com/read/28422310/adult-onset-cystic-fibrosis-liver-disease-diagnosis-and-characterization-of-an-underappreciated-entity
#7
COMPARATIVE STUDY
Christopher Koh, Sasan Sakiani, Pallavi Surana, Xiongce Zhao, Jason Eccleston, David E Kleiner, David Herion, T Jake Liang, Jay H Hoofnagle, Milica Chernick, Theo Heller
Cystic fibrosis (CF) liver disease (CFLD), a leading cause of death in CF, is mostly described in pediatric populations. Adult-onset CFLD lacks sufficient characterization and diagnostic tools. A cohort of CF patients without CFLD during childhood were followed for up to 38 years with serologic testing, imaging, and noninvasive fibrosis markers. Historical CFLD diagnostic criteria were compared with newly proposed CFLD criteria. Thirty-six CF patients were followed for a median of 24.5 years (interquartile range 15...
August 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27782957/correlation-of-transient-elastography-with-severity-of-cystic-fibrosis-related-liver-disease
#8
Amal Aqul, Maureen M Jonas, Sarah Harney, Roshan Raza, Gregory S Sawicki, Paul D Mitchell, Rima Fawaz
OBJECTIVES: The aim of the study was to evaluate whether liver stiffness measurement (LSM), determined by transient elastography, correlates with presence and severity of liver disease in children and young adults with cystic fibrosis (CF). METHODS: Subjects underwent LSM at routine CF visits. Presence and severity of cystic fibrosis liver disease (CFLD) was determined by clinical parameters. Subjects were classified as no CFLD, CFLD without portal hypertension (PHTN), and CFLD with PHTN...
April 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27629435/the-cystic-fibrosis-transmembrane-conductance-regulator-controls-biliary-epithelial-inflammation-and-permeability-by-regulating-src-tyrosine-kinase-activity
#9
Romina Fiorotto, Ambra Villani, Antonis Kourtidis, Roberto Scirpo, Mariangela Amenduni, Peter J Geibel, Massimiliano Cadamuro, Carlo Spirli, Panos Z Anastasiadis, Mario Strazzabosco
In the liver, the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) regulates bile secretion and other functions at the apical membrane of biliary epithelial cells (i.e., cholangiocytes). CF-related liver disease is a major cause of death in patients with CF. CFTR dysfunction affects innate immune pathways, generating a para-inflammatory status in the liver and other epithelia. This study investigates the mechanisms linking CFTR to toll-like receptor 4 activity. We found that CFTR is associated with a multiprotein complex at the apical membrane of normal mouse cholangiocytes, with proteins that negatively control Rous sarcoma oncogene cellular homolog (Src) activity...
December 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27445541/relevance-of-3d-cholangiography-and-transient-elastography-to-assess-cystic-fibrosis-associated-liver-disease
#10
C Lemaitre, S Dominique, E Billoud, M Eliezer, H Montialoux, M Quillard, G Riachi, E Koning, H Morisse-Pradier, G Savoye, C Savoye-Collet, O Goria
Background. Cystic fibrosis-associated liver disease (CFLD) is a major cause of death. The objective of our retrospective study was to describe the relevance of magnetic resonance imaging (MRI) and liver stiffness measurement (LSM) for CFLD evaluation. Methods. All cystic fibrosis adult patients evaluated by MRI and LSM were included. MR signs of portal hypertension (PHT), dysmorphia, or cholangitis were collected and LSM expressed in kPa and Metavir. Results. Of 25 patients, 52% had abnormal MRI. Median LSM was 5...
2016: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
https://www.readbyqxmd.com/read/27297203/long-term-ursodeoxycholic-acid-therapy-does-not-alter-lithocholic-acid-levels-in-patients-with-cystic-fibrosis-with-associated-liver-disease
#11
Carla Colombo, Andrea Crosignani, Gianfranco Alicandro, Wujuan Zhang, Arianna Biffi, Valentina Motta, Fabiola Corti, Kenneth D R Setchell
OBJECTIVE: To evaluate the fasting and postprandial serum bile acid composition in patients with cystic fibrosis-associated liver disease (CFLD) after chronic administration of ursodeoxycholic acid (UDCA) (20 mg/kg/day). The aim was to specifically focus on the extent of biotransformation of UDCA to its hepatotoxic metabolite, lithocholic acid, because of recent concerns regarding the safety of long-term, high-dose UDCA treatment for CFLD. STUDY DESIGN: Twenty patients with CFLD (median age 16 years, range: 2...
October 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/26806442/longitudinal-transient-elastography-measurements-used-in-follow-up-for-patients-with-cystic-fibrosis
#12
Stephanie Van Biervliet, Hugo Verdievel, Saskia Vande Velde, Ruth De Bruyne, Danny De Looze, Xavier Verhelst, Anja Geerts, Eddy Robberecht, Hans Van Vlierberghe
Cystic fibrosis-related liver disease (CFLD) is diagnosed using a combination of criteria. Transient elastography (TE), an ultrasonographic method to evaluate liver stiffness, can differentiate patients with and without liver disease. This retrospective study (2007-2013) aimed to detect developing CFLD using consequent TE measurements. All cystic fibrosis patients with TE measurements between 2007 and 2013 (n = 150, median age 17 (9-24) y) were included, of which 118 had a median of three (range, 2-4) measurements with an interval of 1 (1-2) y...
April 2016: Ultrasound in Medicine & Biology
https://www.readbyqxmd.com/read/26609528/hepatic-and-splenic-acoustic-radiation-force-impulse-shear-wave-velocity-elastography-in-children-with-liver-disease-associated-with-cystic-fibrosis
#13
Teresa Cañas, Araceli Maciá, Rosa Ana Muñoz-Codoceo, Teresa Fontanilla, Patricia González-Rios, María Miralles, Gloria Gómez-Mardones
Liver disease associated with cystic fibrosis (CFLD) is the second cause of mortality in these patients. The diagnosis is difficult because none of the available tests are specific enough. Noninvasive elastographic techniques have been proven to be useful to diagnose hepatic fibrosis. Acoustic radiation force impulse (ARFI) imaging is an elastography imaging system. The purpose of the work was to study the utility of liver and spleen ARFI Imaging in the detection of CFLD. Method. 72 patients with cystic fibrosis (CF) were studied and received ARFI imaging in the liver and in the spleen...
2015: BioMed Research International
https://www.readbyqxmd.com/read/26254836/baseline-ultrasound-and-clinical-correlates-in-children-with-cystic-fibrosis
#14
MULTICENTER STUDY
Daniel H Leung, Wen Ye, Jean P Molleston, Alexander Weymann, Simon Ling, Shruti M Paranjape, Rene Romero, Sara Jane Schwarzenberg, Joseph Palermo, Estella M Alonso, Karen F Murray, Bruce C Marshall, Averell H Sherker, Marilyn J Siegel, Rajesh Krishnamurthy, Roger Harned, Boaz Karmazyn, John C Magee, Michael R Narkewicz
OBJECTIVE: To investigate the relationship between abdominal ultrasound findings and demographic, historical, and clinical features in children with cystic fibrosis (CF). STUDY DESIGN: Children age 3-12 years with CF without known cirrhosis, were enrolled in a prospective, multicenter study of ultrasound to predict hepatic fibrosis. Consensus ultrasound patterns were assigned by 3 radiologists as normal, heterogeneous, homogeneous, or cirrhosis. Data were derived from direct collection and US or Toronto CF registries...
October 2015: Journal of Pediatrics
https://www.readbyqxmd.com/read/26223427/aspartate-aminotransferase-to-platelet-ratio-and-fibrosis-4-as-biomarkers-in-biopsy-validated-pediatric-cystic-fibrosis-liver-disease
#15
Daniel H Leung, Mahjabeen Khan, Charles G Minard, Danielle Guffey, Louise E Ramm, Andrew D Clouston, Gregory Miller, Peter J Lewindon, Ross W Shepherd, Grant A Ramm
UNLABELLED: Up to 10% of cystic fibrosis (CF) children develop cirrhosis by the first decade. We evaluated the utility of two simple biomarkers, aspartate aminotransferase to platelet ratio index (APRI) and FIB-4, in predicting degree of fibrosis in pediatric CF liver disease (CFLD) validated by liver biopsy. In this retrospective, cross-sectional study, 67 children with CFLD had dual-pass liver biopsies and 104 age- and sex-matched CF children without liver disease (CFnoLD) had serum to calculate APRI and FIB-4 collected at enrollment...
November 2015: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/25855877/noninvasive-methods-including-transient-elastography-for-the-detection-of-liver-disease-in-adults-with-cystic-fibrosis
#16
Matthew D Sadler, Pam Crotty, Linda Fatovich, Stephanie Wilson, Harvey R Rabin, Robert P Myers
BACKGROUND: Liver disease is the third leading cause of mortality in patients with cystic fibrosis (CF). However, detection of CF-associated liver disease (CFLD) is challenging. OBJECTIVE: To evaluate the diagnostic performance of noninvasive methods for the detection of CFLD with a focus on transient elastography (TE). METHODS: Patients at the Adult CF Clinic of Calgary and Southern Alberta (n=127) underwent liver stiffness measurement (LSM) by TE using the FibroScan (FS, Ecosens, France) M probe; aspartate aminotransferase to platelet ratio index (APRI) and FibroTest (FT) scores were also calculated...
April 2015: Canadian Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/25680200/cholic-acid-induces-a-cftr-dependent-biliary-secretion-and-liver-growth-response-in-mice
#17
Frank A J A Bodewes, Marcel J Bijvelds, Willemien de Vries, Juul F W Baller, Annette S H Gouw, Hugo R de Jonge, Henkjan J Verkade
The cause of Cystic fibrosis liver disease (CFLD), is unknown. It is well recognized that hepatic exposure to hydrophobic bile salts is associated with the development of liver disease. For this reason, we hypothesize that, CFTR dependent variations, in the hepatic handling of hydrophobic bile salts, are related to the development CFLD. To test our hypothesis we studied, in Cftr-/- and control mice, bile production, bile composition and liver pathology, in normal feeding condition and during cholate exposure, either acute (intravenous) or chronic (three weeks via the diet)...
2015: PloS One
https://www.readbyqxmd.com/read/25625579/circulating-micrornas-as-noninvasive-diagnostic-biomarkers-of-liver-disease-in-children-with-cystic-fibrosis
#18
Naomi L Cook, Tamara N Pereira, Peter J Lewindon, Ross W Shepherd, Grant A Ramm
OBJECTIVES: Cystic fibrosis liver disease (CFLD), resulting from progressive hepatobiliary fibrosis, causes significant morbidity and mortality in up to 20% of children with cystic fibrosis (CF). Both pathogenesis and early detection of CFLD are elusive. Current diagnostic procedures to detect early CFLD and stage fibrosis severity are inadequate. Recent studies highlight a role for microRNAs (miRNAs) in the pathogenesis of many diseases and have suggested that serum miRNAs could be used as diagnostic biomarkers...
February 2015: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/25097709/liver-disease-in-cystic-fibrosis
#19
REVIEW
Natalia Kobelska-Dubiel, Beata Klincewicz, Wojciech Cichy
Cystic fibrosis-associated liver disease (CFLD) affects ca. 30% of patients. The CFLD is now considered the third cause of death, after lung disease and transplantation complications, in CF patients. Diagnostics, clinical assessment and treatment of CFLD have become a real challenge since a striking increase of life expectancy in CF patients has recently been observed. There is no elaborated "gold standard" in the diagnostic process of CFLD; clinical evaluation, laboratory tests, ultrasonography and liver biopsy are used...
2014: Przegla̜d Gastroenterologiczny
https://www.readbyqxmd.com/read/25093717/cystic-fibrosis-related-liver-disease-another-black-box-in-hepatology
#20
REVIEW
Katharina Staufer, Emina Halilbasic, Michael Trauner, Lili Kazemi-Shirazi
Due to improved medical care, life expectancy in patients with cystic fibrosis (CF) has veritably improved over the last decades. Importantly, cystic fibrosis related liver disease (CFLD) has become one of the leading causes of morbidity and mortality in CF patients. However, CFLD might be largely underdiagnosed and diagnostic criteria need to be refined. The underlying pathomechanisms are largely unknown, and treatment strategies with proven efficacy are lacking. This review focuses on current invasive and non-invasive diagnostic standards, the current knowledge on the pathophysiology of CFLD, treatment strategies, and possible future developments...
2014: International Journal of Molecular Sciences
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