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cystic fibrosis liver disease

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https://www.readbyqxmd.com/read/27833495/postoperative-pain-management-of-liver-transplantation-in-cystic-fibrosis-is-it-time-to-start-us-guided-neuraxial-blocks
#1
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
#2
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
#3
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
#4
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...
October 24, 2016: Trends in Endocrinology and Metabolism: TEM
https://www.readbyqxmd.com/read/27782957/correlation-of-transient-elastography-with-severity-of-cystic-fibrosis-related-liver-disease
#5
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
#6
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
#7
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...
October 5, 2016: 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
#8
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
#9
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
#10
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...
September 21, 2016: Kidney International
https://www.readbyqxmd.com/read/27598492/-risk-behaviors-among-adolescents-with-complex-diseases
#11
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
#12
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
#13
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
https://www.readbyqxmd.com/read/27510673/severe-liver-dysfunction-in-an-infant-with-cystic-fibrosis-masquerading-as-metabolic-liver-disease
#14
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/27469184/transplantation
#15
REVIEW
Albert Faro, Alexander Weymann
Despite improvement in median life expectancy and overall health, some children with cystic fibrosis (CF) progress to end-stage lung or liver disease and become candidates for transplant. Transplants for children with CF hold the promise to extend and improve the quality of life, but barriers to successful long-term outcomes include shortage of suitable donor organs; potential complications from the surgical procedure and immunosuppressants; risk of rejection and infection; and the need for lifelong, strict adherence to a complex medical regimen...
August 2016: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27445541/relevance-of-3d-cholangiography-and-transient-elastography-to-assess-cystic-fibrosis-associated-liver-disease
#16
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/27423176/cystic-fibrosis-liver-disease-and-ursodeoxycholic-acid-one-small-step-forward-miles-to-go
#17
EDITORIAL
Michael R Narkewicz
No abstract text is available yet for this article.
October 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27405894/liver-and-lung-transplantation-in-cystic-fibrosis-an-adult-cystic-fibrosis-centre-s-experience
#18
S Sivam, Y Al-Hindawi, J Di Michiel, C Moriarty, P Spratt, P Jansz, M Malouf, M Plit, H Pleass, A Havryk, D Bowen, P Haber, A R Glanville, P T P Bye
Liver disease develops in one-third of patients with cystic fibrosis (CF). It is rare for liver disease to have its onset after 20 years of age. Lung disease, however, is usually more severe in adulthood. A retrospective analysis was performed on nine patients. Three patients required lung transplantation approximately a decade after liver transplant, and another underwent combined liver and lung transplants. Four additional patients with liver transplants are awaiting assessment for lung transplants. One patient is awaiting combined liver and lung transplants...
July 2016: Internal Medicine Journal
https://www.readbyqxmd.com/read/27382190/epithelial-anion-transport-as-modulator-of-chemokine-signaling
#19
REVIEW
Andrea Schnúr, Péter Hegyi, Simon Rousseau, Gergely L Lukacs, Guido Veit
The pivotal role of epithelial cells is to secrete and absorb ions and water in order to allow the formation of a luminal fluid compartment that is fundamental for the epithelial function as a barrier against environmental factors. Importantly, epithelial cells also take part in the innate immune system. As a first line of defense they detect pathogens and react by secreting and responding to chemokines and cytokines, thus aggravating immune responses or resolving inflammatory states. Loss of epithelial anion transport is well documented in a variety of diseases including cystic fibrosis, chronic obstructive pulmonary disease, asthma, pancreatitis, and cholestatic liver disease...
2016: Mediators of Inflammation
https://www.readbyqxmd.com/read/27334896/rare-case-of-hepatic-gaucheroma-in-a-child-on-enzyme-replacement-therapy
#20
Sophy Korula, Penny Owens, Amanda Charlton, Kaustuv Bhattacharya
BACKGROUND: We present a 6 year old boy with type I Gaucher treated from 16 months with ERT, developing focal Gaucheroma in the liver at 3.5 years. CASE: The subject presented at 13 months of age with anaemia, thrombocytopenia and hepatosplenomegaly. Gaucher disease was confirmed by leucocyte enzyme assay. A homozygous change: c.1193G>A (p.Arg398Gln) in the GBA gene was identified. He had normal neurology with normal saccades. Imiglucerase was administered at 60 IU/kg/fortnight from 15 months as per Australian regulations with good clinical response...
June 23, 2016: JIMD Reports
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