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Liver disease associated cystic fibrosis

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https://www.readbyqxmd.com/read/27801781/function-and-regulation-of-micrornas-and-their-potential-as-biomarkers-in-paediatric-liver-disease
#1
REVIEW
Diego A Calvopina, Miranda A Coleman, Peter J Lewindon, Grant A Ramm
MicroRNAs (miRNAs) are short non-coding RNAs involved in biological and pathological processes of every cell type, including liver cells. Transcribed from specific genes, miRNA precursors are processed in the cytoplasm into mature miRNAs and as part of the RNA-induced silencing complex (RISC) complex binds to messenger RNA (mRNA) by imperfect complementarity. This leads to the regulation of gene expression at a post-transcriptional level. The function of a number of different miRNAs in fibrogenesis associated with the progression of chronic liver disease has recently been elucidated...
October 27, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27776652/biliary-tract-enhancement-in-gadoxetic-acid-enhanced-mri-correlates-with-liver-function-biomarkers
#2
Yoshifumi Noda, Satoshi Goshima, Kimihiro Kajita, Hiroshi Kawada, Nobuyuki Kawai, Hiromi Koyasu, Masayuki Matsuo, Kyongtae T Bae
PURPOSE: To evaluate the association between gadoxetic-acid-enhanced magnetic resonance (MR) imaging measurements and laboratory and clinical biomarkers of liver function and fibrosis. MATERIALS AND METHODS: One hundred thirty nine consecutive patients with suspected liver disease or liver tumor underwent gadoxetic-acid-enhanced MR imaging. MR imaging measurements during the hepatobiliary phase included biliary tract structure-to-muscle signal intensity ratio (SIR)...
November 2016: European Journal of Radiology
https://www.readbyqxmd.com/read/27555301/prevalence-of-elevated-liver-enzymes-in-children-with-cystic-fibrosis-diagnosed-by-newborn-screen
#3
Samantha A Woodruff, Marci K Sontag, Frank J Accurso, Ronald J Sokol, Michael R Narkewicz
BACKGROUND: Prevalence and risks for elevated liver enzymes have not been studied systematically in children with CF identified by newborn screen. METHODS: 298 CF children identified by newborn screen since 1982. AST, ALT and GGT tested at annual visits. Percent of children with 1 or ≥2 values of elevated AST, ALT and GGT determined. Relationship of liver enzymes to clinical factors or subsequent liver disease was analyzed RESULTS: At least one abnormal value for AST (63%), ALT (93%) and ALT ≥1...
August 20, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27510673/severe-liver-dysfunction-in-an-infant-with-cystic-fibrosis-masquerading-as-metabolic-liver-disease
#4
K P Srikanth, Inusha Panigrahi, Babu Ram Thapa, Kim Vaiphei
We present a rare presentation of cystic fibrosis with neonatal cholestasis. Histological features of mucoviscidosis were present in liver involving the biliary tract, intestinal mucosa, pancreas, and lung. Besides, there was a rare association with autosomal dominant type of polycystic renal disease.
July 2016: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/27445541/relevance-of-3d-cholangiography-and-transient-elastography-to-assess-cystic-fibrosis-associated-liver-disease
#5
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
#6
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/27025865/hypoglycaemia-in-cystic-fibrosis-in-the-absence-of-diabetes-a-systematic-review
#7
REVIEW
N Armaghanian, J C Brand-Miller, T P Markovic, K S Steinbeck
BACKGROUND: Hypoglycaemia in CF in the absence of diabetes or glucose lowering therapies is a phenomenon that is receiving growing attention in the literature. These episodes are sometimes symptomatic and likely have variable aetiologies. Our first aim was to conduct a systematic review of the literature to determine what is known about hypoglycaemia in CF. Our second aim was to assess evidence based guidelines for management strategies. METHODS: A comprehensive search of databases and guideline compiler entities was performed...
May 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/26927601/international-prospective-study-of-distal-intestinal-obstruction-syndrome-in-cystic-fibrosis-associated-factors-and-outcome
#8
Anne Munck, Corinne Alberti, Carla Colombo, Nataliya Kashirskaya, Helmut Ellemunter, Maria Fotoulaki, Roderick Houwen, Eddy Robberecht, Priscilla Boizeau, Michael Wilschanski
BACKGROUND: Distal intestinal obstruction syndrome (DIOS) is a specific complication of cystic fibrosis. METHODS: A study was performed in 10 countries to prospectively evaluate the incidence, associated factors, and treatment modalities in children and adults. RESULTS: 102 patients presented 112 episodes. The incidence of DIOS was similar in children and adults. Medical treatment failed only in cases of complete DIOS (11%). Children with meconium ileus had a higher rate of surgery for DIOS (15% vs...
July 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/26882172/supplementation-of-ursodeoxycholic-acid-improves-fat-digestion-and-absorption-in-cystic-fibrosis-patients-with-mild-liver-involvement
#9
Sławomira Drzymała-Czyż, Katarzyna Jończyk-Potoczna, Aleksandra Lisowska, Marek Stajgis, Jarosław Walkowiak
BACKGROUND: Ursodeoxycholic acid (UDCA) supplementation is recommended for cystic fibrosis (CF) patients with associated liver disease. However, its effect on fat digestion and absorption is not known. MATERIALS AND METHODS: In 23 patients with mild liver involvement, a C-mixed triglyceride breath test was performed on UDCA supplementation (with and without pancreatic enzymes - standard and increased dose) and after 1 month of UDCA withdrawal. Cumulative percentage dose recovery [CPDR; median (interquartile range)] has been considered to reflect lipid digestion and absorption...
June 2016: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/26879107/rescue-of-defective-atp8b1-trafficking-by-cftr-correctors-as-a-therapeutic-strategy-for-familial-intrahepatic-cholestasis
#10
Wendy L van der Woerd, Catharina G K Wichers, Anna L Vestergaard, Jens Peter Andersen, Coen C Paulusma, Roderick H J Houwen, Stan F J van de Graaf
BACKGROUND & AIMS: ATP8B1 deficiency is an autosomal recessive liver disease characterized by intrahepatic cholestasis. ATP8B1 mutation p.I661T, the most frequent mutation in European patients, results in protein misfolding and impaired targeting to the plasma membrane. Similarly, mutations in cystic fibrosis transmembrane conductance regulator (CFTR), associated with cystic fibrosis, impair protein folding and trafficking. The aim of this study was to investigate whether compounds that rescue CFTR F508del trafficking are capable of improving p...
June 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/26872817/concomitant-presence-of-aspergillus-fumigatus-and-stenotrophomonas-maltophilia-in-the-respiratory-tract-a-new-risk-for-patients-with-liver-disease
#11
Odile Cabaret, Christine Bonnal, Florence Canoui-Poitrine, Aurélie Emirian, Geoffray Bizouard, Eric Levesque, Bernard Maitre, Vincent Fihman, Jean-Winoc Decousser, Françoise Botterel
Concomitant lung colonization by Aspergillus fumigatus and Stenotrophomonas maltophilia was reported mainly in patients with cystic fibrosis (CF) and immunocompromised patients. The aim of the study was to assess the frequency of co-culture of A. fumigatus and S. maltophilia in respiratory samples of hospitalized patients, and to determine its associated factors. Between 2007 and 2011, all patients who had A. fumigatus in their respiratory samples were retrospectively enrolled in the study. Their clinical and laboratory data, including the presence of S...
May 2016: Journal of Medical Microbiology
https://www.readbyqxmd.com/read/26867851/hepatopulmonary-syndrome-in-patients-with-cystic-fibrosis-and-liver-disease
#12
Oded Breuer, Eyal Shteyer, Michael Wilschanski, Zeev Perles, Malena Cohen-Cymberknoh, Eitan Kerem, David Shoseyov
Hepatopulmonary syndrome (HPS) is a liver-induced lung disorder defined as a triad of liver disease, pulmonary vascular dilatation, and a defect in oxygenation. It can complicate chronic liver disease of any etiology, but is most commonly associated with portal hypertension. Severe liver disease with portal hypertension is present in 2% to 8% of patients with cystic fibrosis (CF), but to date, to our knowledge, only one patient with CF has been reported to suffer from HPS. Here, we describe two patients with CF diagnosed with HPS, one subsequent to unresolved hypoxemia and the other following screening for HPS performed in our center...
February 2016: Chest
https://www.readbyqxmd.com/read/26782282/-specific-infections-in-organ-transplantation
#13
M Cornberg, B Schlevogt, J Rademacher, A Schwarz, M Sandherr, G Maschmeyer
This article is concerned with the important topic of infections associated with organ transplantation and includes a discussion on four subtopics. The first section describes the current options in the prevention and therapy of viral hepatitis in association with liver transplantation. Infections with hepatitis B, C, D (delta) and E are discussed with special emphasis on the interferon-free treatment of hepatitis C with the new antiviral drugs.The second section deals with Pseudomonas aeruginosa (PA) infections following lung transplantation (LuTx), which is one of the most frequently detected pathogens in the airway after LuTx...
January 2016: Der Internist
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
#14
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/26480348/increased-transcript-complexity-in-genes-associated-with-chronic-obstructive-pulmonary-disease
#15
Lela Lackey, Evonne McArthur, Alain Laederach
Genome-wide association studies aim to correlate genotype with phenotype. Many common diseases including Type II diabetes, Alzheimer's, Parkinson's and Chronic Obstructive Pulmonary Disease (COPD) are complex genetic traits with hundreds of different loci that are associated with varied disease risk. Identifying common features in the genes associated with each disease remains a challenge. Furthermore, the role of post-transcriptional regulation, and in particular alternative splicing, is still poorly understood in most multigenic diseases...
2015: PloS One
https://www.readbyqxmd.com/read/26436368/isolated-liver-disease-in-a-patient-with-a-cftr-genotype-f508del-12tg-5t-and-470mv-a-new-face-of-an-old-disease
#16
Andrea D Praticò, Elena R Praticò, Novella Rotolo, Stefania Salafia, Chiara Franzonello, Salvatore Leonardi
Today the knowledge of genotype-phenotype correlation in cystic fibrosis is enriched by the growing discoveries of new mutations of the CFTR gene. Although the combination of two severe mutations usually leads to the classic disease (pulmonary and pancreatic insufficiency, sterility, nasal polyposis), the presence of a complex genotype characterized by severe and milder mutations or polymorphism can cause a hidden disease, which is often asymptomatic at early ages. We report on a case of a 15 years old boy, in whom the only clinical signs of CF were chronic hypertransaminasemia and hyperbilirubinemia, and in whom it was demonstrated the presence of the mutations F508del associated with TG11-9T-470M in one allele and TG12-5T-470V in the other allele...
November 2015: Annals of Hepatology
https://www.readbyqxmd.com/read/26370551/lack-of-association-between-haptoglobin-phenotype-and-cystic-fibrosis-outcomes
#17
Michal Shteinberg, Joseph Rivlin, Michal Gur, Muriel Konopnicki, Nili Stein, Michael M Tunney, J Stuart Elborn, Damian G Downey, Elinor Johnston, Hadar Shalom, Andrew Levy
Haptoglobin (Hp), a heme-Iron chelator, has different isoforms which are associated with variable tendency toward infections: Hp 1-1, Hp 2-1, and Hp 2-2. Cystic fibrosis (CF) outcomes are variable and influenced by genetic and environmental factors. The aim of this study was to determine whether Hp phenotype influenced disease severity in CF. One hundred forty-two CF patients from two centers were analyzed for Haptoglobin phenotype using gel electrophoresis of hemoglobin enriched serum. Clinical and microbiological data including bacterial colonization status, lung function, presence of CF-related diabetes and liver disease, rate of exacerbation, and mortality were compared between Hp phenotype groups...
December 2015: Lung
https://www.readbyqxmd.com/read/26254836/baseline-ultrasound-and-clinical-correlates-in-children-with-cystic-fibrosis
#18
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
#19
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/26199136/stimulation-of-nuclear-receptor-peroxisome-proliferator-activated-receptor-%C3%AE-limits-nf-%C3%AE%C2%BAb-dependent-inflammation-in-mouse-cystic-fibrosis-biliary-epithelium
#20
Roberto Scirpo, Romina Fiorotto, Ambra Villani, Mariangela Amenduni, Carlo Spirli, Mario Strazzabosco
UNLABELLED: Cystic fibrosis-associated liver disease is a chronic cholangiopathy that negatively affects the quality of life of cystic fibrosis patients. In addition to reducing biliary chloride and bicarbonate secretion, up-regulation of toll-like receptor 4/nuclear factor kappa light-chain-enhancer of activated B cells (NF-κB)-dependent immune mechanisms plays a major role in the pathogenesis of cystic fibrosis-associated liver disease and may represent a therapeutic target. Nuclear receptors are transcription factors that regulate several intracellular functions...
November 2015: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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