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cystic fibrosis liver disease

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https://www.readbyqxmd.com/read/28164596/genetic-expression-in-cystic-fibrosis-related-bone-disease-an-observational-transversal-cross-sectional-study
#1
Ioana M Ciuca, Liviu L Pop, Alexandru F Rogobete, Dan I Onet, Bogdan Guta-Almajan, Zoran Popa, Florin G Horhat
BACKGROUND: Cystic fibrosis (CF) is the most frequent monogenic genetic disease with autosomal recessive transmission and characterized by important clinical polymorphism and significant lethal prospective. CF related bone disease occurs frequently in adults with CF. Childhood is the period of bone formation, and therefore, children are more susceptible to low bone density. Several factors like pancreatic insufficiency, hormone imbalance, and physical inactivity contribute to CF bone disease development...
September 1, 2016: Clinical Laboratory
https://www.readbyqxmd.com/read/28158236/pro-resolving-lipid-mediator-resolvin-d1-serves-as-a-marker-of-lung-disease-in-cystic-fibrosis
#2
Olaf Eickmeier, Daniela Fussbroich, Klaus Mueller, Friederike Serve, Christina Smaczny, Stefan Zielen, Ralf Schubert
BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive genetic disorder that affects multiple organs, including the lungs, pancreas, liver and intestine. Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) locus lead to defective proteins and reduced Cl- secretion and Na+ hyperabsorption in the affected organs. In addition, patients suffering from CF display chronic inflammation that contributes to the pathogenesis of CF. Recent work suggests that CF patients have a reduced capacity to biosynthesize specialized pro-resolving lipid mediators (SPMs), which contributes to the development and duration of the unwanted inflammation...
2017: PloS One
https://www.readbyqxmd.com/read/28132082/high-tacrolimus-blood-concentrations-early-after-lung-transplantation-and-the-risk-of-kidney-injury
#3
M A Sikma, C C Hunault, E A van de Graaf, M C Verhaar, J Kesecioglu, D W de Lange, J Meulenbelt
PURPOSE: Lung transplant recipients often develop acute kidney injury (AKI) evolving into chronic kidney disease (CKD). The immunosuppressant tacrolimus might be associated with the emergence of AKI. We analyzed the development and recovery of kidney injury after lung transplantation and related AKI to whole-blood tacrolimus trough concentrations and other factors causing kidney injury. METHODS: We retrospectively studied kidney injury in 186 lung-transplantation patients at the UMC Utrecht between 2001 and 2011...
January 28, 2017: European Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28106773/determinants-of-serum-glycerophospholipid-fatty-acids-in-cystic-fibrosis
#4
Sławomira Drzymała-Czyż, Patrycja Krzyżanowska, Berthold Koletzko, Jan Nowak, Anna Miśkiewicz-Chotnicka, Jerzy A Moczko, Aleksandra Lisowska, Jarosław Walkowiak
The etiology of altered blood fatty acid (FA) composition in cystic fibrosis (CF) is understood only partially. We aimed to investigate the determinants of serum glycerophospholipids' FAs in CF with regard to the highest number of FAs and in the largest cohort to date. The study comprised 172 CF patients and 30 healthy subjects (HS). We assessed Fas' profile (gas chromatography/mass spectrometry), CF transmembrane conductance regulator (CFTR) genotype, spirometry, fecal elastase-1, body height and weight Z-scores, liver disease, diabetes and colonization by Pseudomonas aeruginosa...
January 18, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28094851/blood-transfusion-for-preventing-primary-and-secondary-stroke-in-people-with-sickle-cell-disease
#5
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Winfred C Wang
BACKGROUND: Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting the proportion of sickled cells in the circulation...
January 17, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28012258/functional-defect-of-variants-in-the-adenosine-triphosphate-binding-sites-of-abcb4-and-their-rescue-by-the-cystic-fibrosis-transmembrane-conductance-regulator-potentiator-ivacaftor-vx-770
#6
Jean-Louis Delaunay, Alix Bruneau, Brice Hoffmann, Anne-Marie Durand-Schneider, Véronique Barbu, Emmanuel Jacquemin, Michèle Maurice, Chantal Housset, Isabelle Callebaut, Tounsia Aït-Slimane
: ABCB4 (MDR3) is an adenosine triphosphate (ATP)-binding cassette (ABC) transporter expressed at the canalicular membrane of hepatocytes, where it mediates phosphatidylcholine (PC) secretion. Variations in the ABCB4 gene are responsible for several biliary diseases, including progressive familial intrahepatic cholestasis type 3 (PFIC3), a rare disease that can be lethal in the absence of liver transplantation. In this study, we investigated the effect and potential rescue of ABCB4 missense variations that reside in the highly conserved motifs of ABC transporters, involved in ATP binding...
February 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28009031/contemporary-national-trends-of-cystic-fibrosis-hospitalizations-and-co-morbidities-in-the-united-states
#7
Kshitij Chatterjee, Abhinav Goyal, Nishi Shah, Krishna Kakkera, Rajani Jagana, Paula Anderson
INTRODUCTION: Cystic fibrosis (CF) is a life-limiting multisystemic genetic disease. Patients with CF have a high rate of hospitalization. We attempt to ascertain national trends of inpatient stays, prevalence of various co-morbidities during hospitalizations, outcomes and discharge disposition among CF patients. MATERIAL AND METHODS: Data from the National Inpatient Sample (NIS) was used to identify all hospitalizations of patients with CF and their demographic characteristics from 2003 to 2013...
2016: Adv Respir Med
https://www.readbyqxmd.com/read/27833495/postoperative-pain-management-of-liver-transplantation-in-cystic-fibrosis-is-it-time-to-start-us-guided-neuraxial-blocks
#8
M Piazza, G Martucci, A Arcadipane
Cystic fibrosis (CF) is the most common life-limiting genetic disease in Caucasians. Declining lung function is the principal cause of death, but liver involvement can lead to the need for liver transplantation. General anesthesia has detrimental effects on pulmonary function, increasing perioperative morbidity and mortality in CF patients. Regional anesthetic techniques improve outcomes by reducing anesthetic drugs and administration of opioids, and hastening extubation, awakening, and restarting respiratory of physiotherapy...
October 2016: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/27801781/function-and-regulation-of-micrornas-and-their-potential-as-biomarkers-in-paediatric-liver-disease
#9
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/27801752/survey-on-clinical-practice-of-primary-prophylaxis-in-portal-hypertension-in-children
#10
Odile Jeanniard-Malet, Mathieu Duché, Alexandre Fabre
Primary prophylaxis in portal hypertension in children is controversial, as there are few studies documenting its efficacy on the risk of bleeding.28 centres out of the 38 we contacted returned a completed questionnaire about their clinical practices. More than 75% of the centres use endoscopy to screen patients diagnosed with portal cavernoma, biliary atresia, cystic fibrosis and other fibrotic chronic liver diseases with suspected portal hypertension.In cases of grade 2 varices with red marks and grade 3 varices more than 90% of centres perform sclerotherapy or endoscopic variceal ligation (EVL)...
October 31, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27789099/1-carbon-cycle-metabolites-methylate-their-way-to-fatty-liver
#11
REVIEW
Amy Karol Walker
Fatty liver is a complex disease often accompanying metabolic syndrome and Type 2 diabetes mellitus (T2DM). Hepatosteatosis may have roots in multiple metabolic abnormalities. However, metabolic dysfunction in the 1-carbon cycle (1CC), which produces the methyl donor S-adenosylmethionine (SAM) and phosphatidylcholine (PC), induces hepatic lipogenesis in model systems. Human diseases where 1CC or PC synthesis is disrupted, such as alcoholism, congenital lipodystrophy, or cystic fibrosis, often present with fatty liver...
January 2017: Trends in Endocrinology and Metabolism: TEM
https://www.readbyqxmd.com/read/27782957/correlation-of-transient-elastography-with-severity-of-cystic-fibrosis-related-liver-disease
#12
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
https://www.readbyqxmd.com/read/27776652/biliary-tract-enhancement-in-gadoxetic-acid-enhanced-mri-correlates-with-liver-function-biomarkers
#13
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/27709245/cystic-fibrosis-a-clinical-view
#14
REVIEW
Carlo Castellani, Baroukh M Assael
Cystic fibrosis (CF), a monogenic disease caused by mutations in the CFTR gene on chromosome 7, is complex and greatly variable in clinical expression. Airways, pancreas, male genital system, intestine, liver, bone, and kidney are involved. The lack of CFTR or its impaired function causes fat malabsorption and chronic pulmonary infections leading to bronchiectasis and progressive lung damage. Previously considered lethal in infancy and childhood, CF has now attained median survivals of 50 years of age, mainly thanks to the early diagnosis through neonatal screening, recognition of mild forms, and an aggressive therapeutic attitude...
January 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/27685428/update-of-literature-from-cystic-fibrosis-registries-2012-2015-part-6-epidemiology-nutrition-and-complications
#15
REVIEW
Donatello Salvatore, Roberto Buzzetti, Gianni Mastella
Patient registries provide useful information to afford more knowledge on rare diseases like Cystic Fibrosis (CF). Twenty-two studies originating from national CF registries, focusing on demographics, survival, genetics, nutritional status, and non-pulmonary complications, were published between December 2011 and March 2015. The purpose of this review article is to examine these reports, aiming attention to the clinical characteristics of CF patients included in the registries, current, and estimated future epidemiological data, the role of gender gap, the increasing survival in different countries...
September 29, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27679730/is-a-fatty-pancreas-a-banal-lesion
#16
REVIEW
Andrzej Smereczyński, Katarzyna Kołaczyk
So far, a fatty pancreas has been related to obesity and the ageing processes in the body. The current list of pathogenetic factors of the condition is clearly extended with genetically conditioned diseases (cystic fibrosis, Shwachman-Diamond syndrome and Johanson-Blizzard syndrome), pancreatitis, especially hereditary and obstructive, metabolic and hormonal disorders (hypertriglyceridemia, hypercholesterolemia, hyperinsulinemia and hypercortisolemia), alcohol overuse, taking some medicines (especially adrenal cortex hormones), disease of the liver and visceral adiposis...
September 2016: Journal of Ultrasonography
https://www.readbyqxmd.com/read/27665116/ultrasound-based-imaging-methods-of-the-kidney-recent-developments
#17
REVIEW
Jean-Michel Correas, Dany Anglicheau, Dominique Joly, Jean-Luc Gennisson, Mickael Tanter, Olivier Hélénon
In recent years, several novel ultrasound (US)-based techniques have emerged for kidney diagnostic imaging, including tissue stiffness assessment with elastography, Ultrasensitive Doppler techniques, and contrast-enhanced ultrasonography to assess renal microvascularization. Renal elastography has become available with the development of noninvasive quantitative techniques, following the rapidly growing field of liver fibrosis diagnosis. With the increased incidence of chronic kidney disease, noninvasive diagnosis of renal fibrosis can be of critical value...
December 2016: Kidney International
https://www.readbyqxmd.com/read/27598492/-risk-behaviors-among-adolescents-with-complex-diseases
#18
Francisco Funes Díaz, Verónica Gaete Pinto
UNLABELLED: Adolescents with complex diseases may have a higher frequency of risk behaviors than their healthy peers. AIM: To characterize risk behaviors in adolescents with complex chronic diseases. PATIENTS AND METHODS: Risk behaviors were assessed by means of a self-administered questionnaire designed for this purpose, in adolescents aged 10 to 19 years, who attended a pediatrics specialties clinic due to cystic fibrosis, congenital craniofacial anomalies, liver transplantation, kidney transplantation and spinal dysraphism...
June 2016: Revista Médica de Chile
https://www.readbyqxmd.com/read/27555301/prevalence-of-elevated-liver-enzymes-in-children-with-cystic-fibrosis-diagnosed-by-newborn-screen
#19
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/27542808/pathophysiologic-evaluation-of-the-transgenic-cftr-gut-corrected-porcine-model-of-cystic-fibrosis
#20
Stephen T Ballard, Jessica W Evans, Holly S Drag, Michele Schuler
This study evaluated the pulmonary pathophysiology of the transgenic CFTR "gut-corrected" cystic fibrosis (CF) pigs. Four sows produced 18 piglets of which 11 were stillborn with only 2 animals surviving beyond 2 weeks. Failure to survive beyond the neonatal period by 5 piglets was judged to result from metabolic dysfunction related to genetic manipulation for CFTR gut expression or due to cloning artifact. Plasma analysis showed very low plasma proteins, highly elevated liver enzymes, and severe acidosis. All surviving offspring received furosemide for systemic edema...
August 19, 2016: American Journal of Physiology. Lung Cellular and Molecular Physiology
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