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cystic fibrosis liver disease

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https://www.readbyqxmd.com/read/28814334/abernethy-malformation-associated-with-caroli-s-syndrome-in-a-patient-with-a-pkhd1-mutation-a-case-report
#1
Xiao-Xiao Mi, Xiao-Guang Li, Zi-Rong Wang, Ling Lin, Chun-Hai Xu, Jun-Ping Shi
BACKGROUND: Abernethy malformation is a rare congenital anomaly characterised by the partial or complete absence of the portal vein and the subsequent development of an extrahepatic portosystemic shunt. Caroli's disease is a rare congenital condition characterised by non-obstructive saccular intrahepatic bile duct dilation. Caroli's disease combined with congenital hepatic fibrosis and/or renal cystic disease is referred to - Caroli's syndrome. The combination of Abernethy malformation and Caroli's syndrome has not been reported previously...
August 16, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28759700/interventions-for-treating-intrahepatic-cholestasis-in-people-with-sickle-cell-disease
#2
REVIEW
Arturo J Martí-Carvajal, Cristina Elena Martí-Amarista
BACKGROUND: Sickle cell disease is the most common hemoglobinopathy occurring worldwide and sickle cell intrahepatic cholestasis is a complication long recognized in this population. Cholestatic liver diseases are characterized by impaired formation or excretion (or both) of bile from the liver. There is a need to assess the clinical benefits and harms of the interventions used to treat intrahepatic cholestasis in people with sickle cell disease. This is an update of a previously published Cochrane Review...
July 31, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28754453/pathophysiologic-implications-of-innate-immunity-and-autoinflammation-in-the-biliary-epithelium
#3
REVIEW
Mario Strazzabosco, Romina Fiorotto, Massimiliano Cadamuro, Carlo Spirli, Valeria Mariotti, Eleanna Kaffe, Roberto Scirpo, Luca Fabris
The most studied physiological function of biliary epithelial cells (cholangiocytes) is to regulate bile flow and composition, in particular the hydration and alkalinity of the primary bile secreted by hepatocytes. After almost three decades of studies it is now become clear that cholangiocytes are also involved in epithelial innate immunity, in inflammation, and in the reparative processes in response to liver damage. An increasing number of evidence highlights the ability of cholangiocyte to undergo changes in phenotype and function in response to liver damage...
July 25, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28753176/cystic-fibrosis-related-liver-disease-research-challenges-and-future-perspectives
#4
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/28738321/serum-phospholipid-fatty-acid-composition-in-cystic-fibrosis-patients-with-and-without-liver-cirrhosis
#5
Sławomira Drzymała-Czyż, Mariusz Szczepanik, Patrycja Krzyżanowska, Monika Duś-Żuchowska, Andrzej Pogorzelski, Ewa Sapiejka, Paweł Juszczak, Aleksandra Lisowska, Berthold Koletzko, Jarosław Walkowiak
BACKGROUND/AIMS: Cystic fibrosis (CF) liver disease is the third most frequent cause of death in CF patients. Although it alters fatty acid (FA) metabolism, data concerning the profile of FA in CF patients with liver cirrhosis is lacking. This study aimed to assess the FA composition of serum phospholipids in CF patients with and without liver cirrhosis. METHODS: The study comprised 25 CF patients with liver cirrhosis and 25 without it. We assessed Z-scores for body height and weight, lung function, exocrine pancreatic sufficiency and colonization with Pseudomonas aeruginosa...
July 22, 2017: Annals of Nutrition & Metabolism
https://www.readbyqxmd.com/read/28668410/focal-lesions-in-cirrhosis-not-always-hcc
#6
Maxime Ronot, Marco Dioguardi Burgio, Yvonne Purcell, Romain Pommier, Giuseppe Brancatelli, Valérie Vilgrain
Even though most hepatocellular carcinomas (HCC) develop in the setting of cirrhosis, numerous other focal liver lesions and pseudolesions may be encountered. The role of the radiologist is therefore to differentiate these lesions from HCC to avoid under- and overdiagnosis. There are several ways of classifying these lesions: those which predate the development of fibrosis and cirrhosis (cystic lesions, hemangioma), those related to or a consequence of cirrhosis (regenerative nodules, dysplastic nodules, focal fibrosis, peribiliary cysts, shunts, or even cholangiocarcinoma), and those related to the underlying cause of chronic liver disease (lymphoma)...
August 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/28648506/the-role-of-glis3-in-thyroid-disease-as-part-of-a-multisystem-disorder
#7
REVIEW
P Dimitri
Congenital hypothyroidism is the most common hereditary endocrine disorder. In a small number of cases, mutations have been identified that are associated with maldevelopment and maldescent of the thyroid. Some of these mutations present as syndromes with a multisystem phenotype such as NKX2-1, PAX8, and FOXE. The association of permanent neonatal diabetes and congenital hypothyroidism was first reported in 2003 and subsequently led to the identification GLIS3 as the mutation responsible for this presentation...
March 2017: Best Practice & Research. Clinical Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28646244/increased-soluble-vcam-1-and-normal-p-selectin-in-cystic-fibrosis-a-cross-sectional-study
#8
Jan K Nowak, Irena Wojsyk-Banaszak, Edyta Mądry, Andrzej Wykrętowicz, Patrycja Krzyżanowska, Sławomira Drzymała-Czyż, Agata Nowicka, Andrzej Pogorzelski, Ewa Sapiejka, Wojciech Skorupa, Mariusz Szczepanik, Aleksandra Lisowska, Jaroslaw Walkowiak
PURPOSE: As life expectancy in cystic fibrosis (CF) increases, questions regarding its potential impact on cardiovascular health arise. Soluble vascular cell adhesion molecule 1 (sVCAM-1), P-selectin (sP-selectin) are proposed as biomarkers of cardiovascular disease. We aimed to: compare their concentrations in clinically stable CF patients and healthy subjects (HS) and verify whether they independently correlate with CF characteristics. METHODS: Serum sVCAM-1 and sP-selectin levels were measured using ELISA...
August 2017: Lung
https://www.readbyqxmd.com/read/28622948/high-level-of-%C3%AE-1-3-d-glucan-antigenaemia-in-cystic-fibrosis-in-the-absence-of-invasive-fungal-disease
#9
Vilma Rautemaa, Heather D Green, Andrew M Jones, Riina Rautemaa-Richardson
β-(1,3)-d-glucan (BDG) is used to rule out invasive fungal disease (IFD) but its usefulness in cystic fibrosis (CF) has not been evaluated. We measured serum BDG in CF patients with no clinical suspicion of IFD. Samples from 46 adult CF patients during a stable period and during pulmonary exacerbation were tested. The association of BDG with clinical variables was analyzed. Three hundred and three non-CF patients with suspected IFD were used as comparators. Both samples were negative in 52% of CF patients, whereas 67% of comparators had only negative results (P=0...
May 18, 2017: Diagnostic Microbiology and Infectious Disease
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
#10
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/28497760/the-intestinal-microbiome-and-paediatric-liver-disease
#11
REVIEW
Daniel H Leung, Dean Yimlamai
The intestinal microbiome has been the intense focus of recent study, but how the microbiota affects connected organs, such as the liver, has not been fully elucidated. The microbiome regulates intestinal permeability and helps to metabolise the human diet into small molecules, thus directly affecting liver health. Several studies have linked intestinal dysbiosis to the severity and progression of liver diseases, such as non-alcoholic fatty liver disease, non-alcoholic steatohepatitis, primary sclerosing cholangitis, total parenteral nutrition-associated liver disease, and cystic fibrosis-associated liver disease...
June 2017: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28472055/abdominal-symptoms-in-cystic-fibrosis-and-their-relation-to-genotype-history-clinical-and-laboratory-findings
#12
Harold Tabori, Christin Arnold, Anke Jaudszus, Hans-Joachim Mentzel, Diane M Renz, Steffen Reinsch, Michael Lorenz, Ruth Michl, Andrea Gerber, Thomas Lehmann, Jochen G Mainz
BACKGROUND & AIMS: Abdominal symptoms (AS) are a hallmark of the multiorgan-disease cystic fibrosis (CF). However, the abdominal involvement in CF is insufficiently understood and, compared to the pulmonary manifestation, still receives little scientific attention. Aims were to assess and quantify AS and to relate them to laboratory parameters, clinical findings, and medical history. METHODS: A total of 131 patients with CF of all ages were assessed with a new CF-specific questionnaire (JenAbdomen-CF score 1...
2017: PloS One
https://www.readbyqxmd.com/read/28422310/adult-onset-cystic-fibrosis-liver-disease-diagnosis-and-characterization-of-an-underappreciated-entity
#13
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/28416225/b-type-natriuretic-peptide-overexpression-ameliorates-hepatorenal-fibrocystic-disease-in%C3%A2-a%C3%A2-rat%C3%A2-model-of-polycystic-kidney-disease
#14
Sara J Holditch, Claire A Schreiber, Peter C Harris, Nicholas F LaRusso, Marina Ramirez-Alvarado, Alessandro Cataliotti, Vicente E Torres, Yasuhiro Ikeda
Polycystic kidney disease (PKD) involves progressive hepatorenal cyst expansion and fibrosis, frequently leading to end-stage renal disease. Increased vasopressin and cAMP signaling, dysregulated calcium homeostasis, and hypertension play major roles in PKD progression. The guanylyl cyclase A agonist, B-type natriuretic peptide (BNP), stimulates cGMP and shows anti-fibrotic, anti-hypertensive, and vasopressin-suppressive effects, potentially counteracting PKD pathogenesis. Here, we assessed the impacts of guanylyl cyclase A activation on PKD progression in a rat model of PKD...
September 2017: Kidney International
https://www.readbyqxmd.com/read/28339466/in-silico-search-for-modifier-genes-associated-with-pancreatic-and-liver-disease-in-cystic-fibrosis
#15
Pascal Trouvé, Emmanuelle Génin, Claude Férec
Cystic Fibrosis is the most common lethal autosomal recessive disorder in the white population, affecting among other organs, the lung, the pancreas and the liver. Whereas Cystic Fibrosis is a monogenic disease, many studies reveal a very complex relationship between genotype and clinical phenotype. Indeed, the broad phenotypic spectrum observed in Cystic Fibrosis is far from being explained by obvious genotype-phenotype correlations and it is admitted that Cystic Fibrosis disease is the result of multiple factors, including effects of the environment as well as modifier genes...
2017: PloS One
https://www.readbyqxmd.com/read/28260452/unusual-indications-for-a-liver-transplant-a-single-center-experience
#16
Aydincan Akdur, Mahir Kirnap, Ebru H Ayvazoglu Soy, Figen Ozcay, Gokhan Moray, Gulnaz Arslan, Mehmet Haberal
OBJECTIVES: This study sought to evaluate the efficacy of liver transplant for unusual liver diseases. MATERIALS AND METHODS: The results of 476 patients who underwent liver transplant from 1988 to January 2015 were retrospectively analyzed. Two hundred forty-five of them were adult patients and 231 of them were pediatric. Thirty-one patients had unusual liver disease. RESULTS: Of the 31 patients with unusual liver disease, 9 (29%) were adult and 22 (71%) were pediatric patients...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28250993/imaging-the-abdominal-manifestations-of-cystic-fibrosis
#17
REVIEW
C D Gillespie, M K O'Reilly, G N Allen, S McDermott, V O Chan, C A Ridge
Cystic fibrosis (CF) is a multisystem disease with a range of abdominal manifestations including those involving the liver, pancreas, and kidneys. Recent advances in management of the respiratory complications of the disease has led to a greater life expectancy in patients with CF. Subsequently, there is increasing focus on the impact of abdominal disease on quality of life and survival. Liver cirrhosis is the most important extrapulmonary cause of death in CF, yet significant challenges remain in the diagnosis of CF related liver disease...
2017: International Journal of Hepatology
https://www.readbyqxmd.com/read/28250901/over-the-scope-clip-closure-of-long-lasting-gastrocutaneous-fistula-after-percutaneous-endoscopic-gastrostomy-tube-removal-in-immunocompromised-patients-a-single-center-case-series
#18
Henriette Heinrich, Christoph Gubler, Piero V Valli
Over-the-scope-clips (OTSC(®)) have been shown to be an effective and safe endoscopic treatment option for the closure of gastrointestinal perforations, leakages and fistulae. Indications for endoscopic OTSC(®) treatment have grown in number and also include gastro cutaneous fistula (GCF) after percutaneous endoscopic gastrostomy (PEG) tube removal. Non-healing GCF is a rare complication after removal of PEG tubes and may especially develop in immunosuppressed patients with multiple comorbidities. There is growing evidence in the literature that OTSC(®) closure of GCF after PEG tube removal is emerging as an effective, simple and safe endoscopic treatment option...
February 16, 2017: World Journal of Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/28249265/role-of-the-g-protein-coupled-bile-acid-receptor-tgr5-in-liver-damage
#19
REVIEW
Maria Reich, Caroline Klindt, Kathleen Deutschmann, Lina Spomer, Dieter Häussinger, Verena Keitel
BACKGROUND: TGR5 (G protein-coupled bile acid receptor 1, M-Bar) is a G protein-coupled cell surface receptor responsive to bile acids (BA) and different steroid hormones. TGR5 mRNA is detected almost ubiquitious in human and rodent tissues with a very high expression in gallbladder, liver and intestine. In liver, TGR5 is found in sinusoidal endothelial cells, Kupffer cells and cholangiocytes. Activation of TGR5 triggers an elevation of intracellular cyclic AMP and further downstream signalling...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28239354/matrix-metalloproteinases-7-and-kidney-fibrosis
#20
REVIEW
Ben Ke, Chuqiao Fan, Liping Yang, Xiangdong Fang
Matrix metalloproteinase-7 (MMP-7) is a secreted zinc- and calcium-dependent endopeptidase that degrades a broad range of extracellular matrix substrates and additional substrates. MMP-7 playsa crucial role in a diverse array of cellular processes and appears to be a key regulator of fibrosis in several diseases, including pulmonary fibrosis, liver fibrosis, and cystic fibrosis. In particular, the relationship between MMP-7 and kidney fibrosis has attracted significant attention in recent years. Growing evidence indicates that MMP-7 plays an important role in the pathogenesis of kidney fibrosis...
2017: Frontiers in Physiology
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