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Amal Aqul, Maureen M Jonas, Sarah Harney, Roshan Raza, Gregory S Sawicki, Paul D Mitchell, Rima Fawaz
OBJECTIVE: To evaluate whether liver stiffness measurement (LSM), determined by transient elastography (TE), correlates with presence and severity of liver disease (LD) in children and young adults with cystic fibrosis (CF). METHODS: Subjects underwent LSM at routine CF visits. Presence and severity of CFLD was determined by clinical parameters. Subjects were classified as no CFLD, CFLD without portal hypertension (PHTN), and CFLD with PHTN. LSM was compared to AST/platelet ratio index (APRI) as a correlate to severity of CFLD...
October 25, 2016: Journal of Pediatric Gastroenterology and Nutrition
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
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
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
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
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
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
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
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
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
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
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
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
Marion Rowland, Cliona Gallagher, Charles G Gallagher, Risteárd Ó Laoide, Gerard Canny, Anne Marie Broderick, Jennifer Drummond, Peter Greally, Dubhfeasa Slattery, Leslie Daly, Noel G McElvaney, Billy Bourke
BACKGROUND: Liver disease is an important complication in CF. AIMS: To determine if CFLD is a risk factor for mortality in CF, and which baseline characteristics predict all-cause mortality. METHODS: Irish children with CFLD, and their age and gender matched controls were enrolled at baseline and reviewed after 10years to determine which characteristics predict mortality. RESULTS: 72/84 (85.71%) participants were followed, (mean age Cases 21...
January 2015: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Ioana Mihaiela Ciucă, Liviu Pop, Liviu Tămaş, Sorina Tăban
Cystic fibrosis (CF) is the most frequent monogenic genetic disease, autosomal recessive transmitted, characterized by an impressive clinical polymorphism and appreciative fatal prospective. Liver disease is the second non-pulmonary cause of death in cystic fibrosis, which, with increasing life expectancy, became an important management problem. Predisposing factors like male gender, pancreatic insufficiency, meconium ileus and severe mutation are incriminated to influence the occurrence of cystic fibrosis associated liver disease (CFLD)...
2014: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
R H J Bandsma, M A Bozic, J A Fridell, M H Crull, J Molleston, Y Avitzur, Y Mozer-Glassberg, R P Gonzalez-Peralta, M Hodik, A Fecteau, M de Angelis, P Durie, V L Ng
BACKGROUND: Diabetes is associated with increased morbidity and mortality in patients with cystic fibrosis (CF). While liver transplantation is well established for CF-related liver disease (CFLD), the role of simultaneous liver-pancreas transplantation is less understood. METHODS: We polled 81 pediatric transplantation centers to identify and characterize subjects who had undergone simultaneous liver-pancreas transplantation and obtain opinions about this procedure in CFLD...
July 2014: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Lisette Leeuwen, Annabel K Magoffin, Dominic A Fitzgerald, Marco Cipolli, Kevin J Gaskin
OBJECTIVE: To identify the incidence and outcomes of cholestasis and meconium ileus (MI) in infants with cystic fibrosis (CF). DESIGN: Retrospective cohort study. SETTING: Single-centre study. PATIENTS: From January 1986 to December 2011, 401 infants with CF (69 with MI) presented to our centre. MAIN OUTCOME MEASUREMENTS: (1) incidence of cholestasis, (2) identification of risk factors for cholestasis, (3) association between the presence of cholestasis and MI and the development of clinically significant CF-associated liver disease (CFLD) defined as multilobular cirrhosis with portal hypertension...
May 2014: Archives of Disease in Childhood
Paraskevi Panagopoulou, Maria Fotoulaki, Aristidis Nikolaou, Sanda Nousia-Arvanitakis
BACKGROUND: Optimal nutritional status (NS) in cystic fibrosis (CF) is associated with better lung function and increased overall survival. This study estimated the prevalence of malnutrition and obesity among CF patients in a tertiary center. METHODS: In a cross-sectional study of 68 CF patients (33 female; 37 children/adolescents) weight, height, body composition, respiratory function (% of the predicted forced expiratory volume in 1 s; FEV1%pred ) and serum lipids were measured; body mass index (BMI), BMI standard deviation score (BMI-SDS) and BMI percentiles were calculated; Pseudomonas colonization, pancreatic insufficiency, diabetes mellitus (CFDM), liver disease (CFLD) and genotype were recorded; NS was classified according to the 2005 Cystic Fibrosis Foundation (CFF) criteria...
February 2014: Pediatrics International: Official Journal of the Japan Pediatric Society
Lisette Leeuwen, Dominic A Fitzgerald, Kevin J Gaskin
The survival of patients with cystic fibrosis (CF) has progressively increased over recent decades, largely attributable to early diagnosis through newborn screening and advances in nutritional and respiratory care. As the life expectancy of patients with CF has improved, non-respiratory complications such as liver disease have become increasingly recognized. Biochemical derangements of liver enzymes in CF are common and may be attributed to a number of specific hepatobiliary abnormalities. Among them, Cystic Fibrosis-associated Liver Disease (CFLD) is clinically the most significant hepatic complication and is believed to have a significant impact on morbidity and mortality...
March 2014: Paediatric Respiratory Reviews
Timo Rath, Lisa Hage, Marion Kügler, Katrin Menendez Menendez, Reinhart Zachoval, Lutz Naehrlich, Richard Schulz, Martin Roderfeld, Elke Roeb
BACKGROUND AND AIMS: Cystic Fibrosis associated liver disease (CFLD) develops in approximately 30% of CF patients. However, routine sensitive diagnostic tools for CFLD are lacking. Within this study, we aimed to identify new experimental biomarkers for the detection of CFLD. METHODS: 45 CF patients were included in the study and received transient elastography. Differential regulation of 220 different serum proteins was assessed in a subgroup of patients with and without CFLD...
2013: PloS One
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