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https://www.readbyqxmd.com/read/28328144/diagnosing-and-preventing-iron-overload
#1
Ganesh Ramanathan, John K Olynyk, Paolo Ferrari
Absolute or functional iron (Fe) deficiency is an important determinant of anemia in hemodialysis patients and parenteral Fe is routinely used to treat this condition in conjunction with erythropoiesis stimulating agents. While restoration of hemoglobin toward the target range is a good outcome of Fe replacement, it is well known that Fe overload and toxicity may be adverse consequences of this therapy. Dialysis clinical practice guidelines recommend tailoring Fe therapy based on transferrin saturation and serum ferritin levels...
March 22, 2017: Hemodialysis International
https://www.readbyqxmd.com/read/28321803/prognosis-of-cirrhotic-patients-admitted-to-intensive-care-unit-a-meta-analysis
#2
Delphine Weil, Eric Levesque, Marc McPhail, Rodrigo Cavallazzi, Eleni Theocharidou, Evangelos Cholongitas, Arnaud Galbois, Heng Chih Pan, Constantine J Karvellas, Bertrand Sauneuf, René Robert, Jérome Fichet, Gaël Piton, Thierry Thevenot, Gilles Capellier, Vincent Di Martino
BACKGROUND: The best predictors of short- and medium-term mortality of cirrhotic patients receiving intensive care support are unknown. METHODS: We conducted meta-analyses from 13 studies (2523 cirrhotics) after selection of original articles and response to a standardized questionnaire by the corresponding authors. End-points were in-ICU, in-hospital, and 6-month mortality in ICU survivors. A total of 301 pooled analyses, including 95 analyses restricted to 6-month mortality among ICU survivors, were conducted considering 249 variables (including reason for admission, organ replacement therapy, and composite prognostic scores)...
December 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/28318950/cholesteryl-ester-storage-disease-an-underdiagnosed-cause-of-cirrhosis-in-adults
#3
REVIEW
Mamta Pant, Kiyoko Oshima
Cholesteryl Ester Storage Disease (CESD), is a rare multisystem autosomal recessive disorder and belongs to the broad family of lysosomal storage disorders. It can present anytime from infancy and childhood to even adulthood. The clinical manifestations are generally severe in infants and with milder forms in adults. One of the prominent sites of involvement is liver. Due to low awareness of this condition among physicians including surgical pathologists, majority of the liver biopsies, especially from the adults are often misdiagnosed as non-alcoholic fatty liver disease/non-alcoholic steatohepatitis or cryptogenic cirrhosis...
February 9, 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28317409/ombitasvir-and-paritaprevir-boosted-with-ritonavir-and-combined-with-dasabuvir-for-chronic-hepatitis-c
#4
Robert Flisiak, Marta Flisiak-Jackiewicz
Hepatitis C is a leading cause of cirrhosis and hepatocellular carcinoma responsible for almost 700,000 deaths worldwide annually. Until 2014, management of HCV infections was based on interferon alfa containing regimens, with efficacy of 40-70% and a high adverse event rate. Interferon-free therapeutic options improved sustained viral response (SVR) rate to >90% and safety profile to placebo-like levels. Areas covered: This article describes all-oral regimen consisting of three direct acting antivirals (DAA) - ombitasvir (OBV), paritaprevir (PTV) and dasabuvir (DSV), which in clinical practice is boosted with ritonavir (r) and sometimes with ribavirin (RBV)...
March 20, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28317394/an-update-on-the-pathophysiology-and-management-of-polycystic-liver-disease
#5
May Yw Wong, Geoffrey W McCaughan, Simone I Strasser
Polycystic liver disease (PLD) is characterized by the presence of multiple cholangiocyte-derived hepatic cysts that progressively replace liver tissue. They are classified as an inherited ciliopathy /cholangiopathy as pathology exists at the level of the primary cilia of cholangiocytes. Aberrant expression of the proteins in primary cilia can impair their structures and functions, thereby promoting cystogenesis. Areas covered: This review begins by looking at the epidemiology of PLD and its natural history...
March 18, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28303605/performance-of-scoring-systems-to-predict-mortality-of-patients-with-acute-on-chronic-liver-failure-a-systematic-review-and-meta-analysis
#6
Yi-Xiang Zheng, Xiao Zhong, Ya-Jun Li, Xue-Gong Fan
BACKGROUND: Acute-on-chronic liver failure (ACLF) has characteristic feature of multisystem organ failure, rapid progression and low early transplant-free survival. AIM: We performed a meta-analysis to determine the accuracy of five scoring systems in predicting mortality of ACLF patients. METHODS: A systematic database search was performed and retrieved articles were graded according to methodological quality. Collated data was meta-analyzed by hierarchical summarized receiver operating characteristic (HSROC) model and bivariate model to evaluate the diagnostic accuracy of scoring systems RESULT: Of 4223 studies identified, 26 studies involving 4732 ACLF patients were included...
March 16, 2017: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28302006/transplantation-for-large-liver-tumors-in-the-setting-of-abernethy-malformation
#7
İlgin Özden, Ayşen Yavru, Mine Güllüoğlu, Aydin Alper, Orhan Bilge, Serdar Cantez, Özlem Durmaz
In this study, we report our experiences on the role of transplantation in 2 patients with large liver tumors in the setting of Abernethy malformation. Patient 1 was a 17-year-old boy who was referred for hepatic masses and recurrent hepatic encephalopathy episodes. Computed tomography and magnetic resonance imaging showed 2 large tumors (4 and 8 cm) in the liver. The portal vein drained directly into the vena cava. Core biopsy of the larger mass revealed fibrosis and regenerative hyperplasia. There were hyperintense signals in the T1-weighted images in the globus pallidus...
March 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28300583/synthesis-of-new-c-25-and-c-26-steroidal-acids-as-potential-ligands-of-the-nuclear-receptors-daf-12-lxr-and-gr
#8
María V Dansey, María C Del Fueyo, Adriana S Veleiro, Pablo H Di Chenna
A new methodology to obtain C-25 and C-26 steroidal acids starting from pregnenolone is described. Construction of the side chain was achieved by applying the Mukaiyama aldol reaction with a non-hydrolytic work-up to isolate the trapped silyl enol ether with higher yields. Using this methodology we synthesized three new steroidal acids as potential ligands of DAF-12, Liver X and Glucocorticoid nuclear receptors and studied their activity in reporter gene assays. Our results show that replacement of the 21-CH3 by a 20-keto group in the side chains of the cholestane scaffold of DAF-12 or Liver X receptors ligands causes the loss of the activity...
March 11, 2017: Steroids
https://www.readbyqxmd.com/read/28299189/molecular-signature-of-anastasis-for-reversal-of-apoptosis
#9
Ho Man Tang, C Conover Talbot, Ming Chiu Fung, Ho Lam Tang
Apoptosis is a type of programmed cell death that is essential for normal organismal development and homeostasis of multicellular organisms by eliminating unwanted, injured, or dangerous cells. This cell suicide process is generally assumed to be irreversible. However, accumulating studies suggest that dying cells can recover from the brink of cell death. We recently discovered an unexpected reversibility of the execution-stage of apoptosis in vitro and in vivo, and proposed the term anastasis (Greek for "rising to life") to describe this cell recovery phenomenon...
2017: F1000Research
https://www.readbyqxmd.com/read/28296808/waitlisted-candidates-with-polycystic-liver-disease-are-more-likely-to-be-transplanted-than-those-with-chronic-liver-failure
#10
Sahil D Doshi, Therese Bittermann, Thomas D Schiano, David Seth Goldberg
BACKGROUND: Polycystic liver disease (PCLD) is characterized by cystic replacement of the hepatic parenchyma, leading to hepatic dysfunction, portal hypertension, and hepatomegaly. Patients with liver dysfunction and/or symptomatic disease are eligible for liver transplantation. However, little is known about these patients' waitlist outcomes relative to others with chronic liver disease. METHODS: We used OPTN/UNOS data from February 27, 2002 to December 31, 2015 to compare waitlist outcomes of adult patients with PCLD to those with chronic liver failure (CLF) and hepatocellular carcinoma (HCC)...
March 15, 2017: Transplantation
https://www.readbyqxmd.com/read/28295601/idiopathic-hyperammonemia-after-solid-organ-transplantation-primarily-a-lung-problem-a-single-center-experience-and-systematic-review
#11
Dustin Krutsinger, Alejandro Pezzulo, Amy E Blevins, Robert M Reed, Michael Voigt, Michael Eberlein
BACKGROUND: Idiopathic hyperammonemia syndrome (IHS) is an uncommon, often deadly complication of solid organ transplantation. IHS cases in solid organ transplantation seem to occur predominantly in lung transplant (LTx) recipients. However to the best of our knowledge the occurrence of IHS has not been systematically evaluated. We set out to identify all reported cases of IHS following non-liver solid organ transplantations. METHODS: Retrospective review of our institutional experience and systematic review of the literature...
March 10, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28295152/causally-treatable-hereditary-neuropathies-in-fabry-s-disease-transthyretin-related-familial-amyloidosis-and-pompe-s-disease
#12
REVIEW
J Finsterer, J Wanschitz, S Quasthoff, S Iglseder, W Löscher, W Grisold
OBJECTIVES: Most acquired neuropathies are treatable, whereas genetic neuropathies respond to treatment in Fabry's disease (FD), transthyretin-related familial amyloidosis (TTR-FA), and Pompe's disease (PD). This review summarizes and discusses recent findings and future perspectives concerning etiology, pathophysiology, clinical presentation, diagnosis, treatment, and outcome of neuropathy in FD, TTR-FA, and PD. METHODS: Literature review. RESULTS: Neuropathy in FD concerns particularly small, unmyelinated, or myelinated sensory fibers (small fiber neuropathy [SFN]) and autonomic fibers, manifesting as acroparesthesias, Fabry's crises, or autonomous disturbances...
March 12, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/28293634/secs-sinusoidal-endothelial-cells-liver-microenvironment-and-fibrosis
#13
REVIEW
Vaishaali Natarajan, Edward N Harris, Srivatsan Kidambi
Liver fibrosis is a wound-healing response to chronic liver injury such as alcoholic/nonalcoholic fatty liver disease and viral hepatitis with no FDA-approved treatments. Liver fibrosis results in a continual accumulation of extracellular matrix (ECM) proteins and paves the way for replacement of parenchyma with nonfunctional scar tissue. The fibrotic condition results in drastic changes in the local mechanical, chemical, and biological microenvironment of the tissue. Liver parenchyma is supported by an efficient network of vasculature lined by liver sinusoidal endothelial cells (LSECs)...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28293378/hepatorenal-syndrome-update-on-diagnosis-and-therapy
#14
EDITORIAL
Juan G Acevedo, Matthew E Cramp
Hepatorenal syndrome (HRS) is a manifestation of extreme circulatory dysfunction and entails high morbidity and mortality. A new definition has been recently recommended by the International Club of Ascites, according to which HRS diagnosis relies in serum creatinine changes instead that on a fixed high value. Moreover, new data on urinary biomarkers has been recently published. In this sense, the use of urinary neutrophil gelatinase-associated lipocalin seems useful to identify patients with acute tubular necrosis and should be employed in the diagnostic algorithm...
February 28, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28292877/the-effects-of-dual-ppar%C3%AE-%C3%AE-agonism-compared-with-ace-inhibition-in-the-btbrob-ob-mouse-model-of-diabetes-and-diabetic-nephropathy
#15
Anette Ericsson, Pernilla Tonelius, Mark Lal, Alan Sabirsh, Gerhard Böttcher, Lena William-Olsson, Maria Strömstedt, Camilla Johansson, Gina Hyberg, Sofia Tapani, Ann-Cathrine Jönsson-Rylander, Robert Unwin
The leptin-deficient BTBRob/ob mouse develops progressive albuminuria and morphological lesions similar to human diabetic nephropathy (DN), although whether glomerular hyperfiltration, a recognized feature of early DN that may contribute to renal injury, also occurs in this model is not known. Leptin replacement has been shown to reverse the signs of renal injury in this model, but in contrast, the expected renoprotection by angiotensin-converting enzyme (ACE) inhibition in BTBRob/ob mice seems to be limited...
March 2017: Physiological Reports
https://www.readbyqxmd.com/read/28289805/comparison-of-lindane-and-carbaryl-pesticide-bioaccumulation-in-the-common-sole-solea-solea
#16
Frapiccini Emanuela, Scarcella Giuseppe, Guicciardi Stefano, Betti Mattia, Marini Mauro
Organochlorines and carbamates are common pesticides predominantly employed in agriculture. Large amounts of pesticides make their way into rivers and marine habitats. They accumulate in aquatic organisms through different exposure routes and gradually move up the food chain. Since contaminant bioaccumulation in animals is affected by several factors, this work harnessed several different approaches to explore the persistence of lindane, a long banned organochlorine pesticide, and carbaryl, a newer generation pesticide, in common sole (Solea solea), a major commercial species in Adriatic fisheries...
March 13, 2017: Bulletin of Environmental Contamination and Toxicology
https://www.readbyqxmd.com/read/28285817/update-on-lysosomal-acid-lipase-deficiency-diagnosis-treatment-and-patient-management
#17
Carmen Camarena, Luis J Aldamiz-Echevarria, Begoña Polo, Miguel A Barba Romero, Inmaculada García, Jorge J Cebolla, Emilio Ros
Lysosomal acid lipase deficiency (LALD) is an ultra-rare disease caused by a congenital disorder of the lipid metabolism, characterized by the deposition of cholesterol esters and triglycerides in the organism. In patients with no enzyme function, the disease develops during the perinatal period and is invariably associated with death during the first year of life. In all other cases, the phenotype is heterogeneous, although most patients develop chronic liver diseases and may also develop an early cardiovascular disease...
March 9, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28284629/fermented-rapeseed-meal-is-effective-in-controlling-salmonella-enterica-serovar-typhimurium-infection-and-improving-growth-performance-in-broiler-chicks
#18
Amin Ashayerizadeh, Behrouz Dastar, Mahmoud Shams Shargh, Alireza Sadeghi Mahoonak, Saeed Zerehdaran
The aim of present experiment was to assess the effects of fermented rapeseed meal (FRSM) on Salmonella enterica serovar Typhimurium (S. Typhimurium) colonization and growth performance in broiler chicks. Two hundred forty day-old male Cobb 500 broiler chicks were divided into six experimental treatments with four replicates and 10 birds per each. The treatments were including two positive and negative controls which birds received a basal corn-soybean diet as well as four others which birds received the diets that rapeseed meal (RSM) or FRSM was replaced with soybean meal at 50 and 100% levels...
March 2017: Veterinary Microbiology
https://www.readbyqxmd.com/read/28283691/-modulation-of-the-intestinal-microbiota-by-nutritional-interventions
#19
S Derer, H Lehnert, C Sina, A E Wagner
Humans live in symbiosis with billions of commensal bacteria. The so-called microbiota live on different biological interfaces such as the skin, the urogenital tract and the gastrointestinal tract. Commensal bacteria replace potentially pathogenic microbes, synthesize vitamins and ferment dietary fibre. An imbalance in the bacterial composition of the intestinal microbiota has been associated with various diseases including gut-associated disorders such as inflammatory bowel diseases, colorectal cancer and nonalcoholic fatty liver disease...
March 10, 2017: Der Internist
https://www.readbyqxmd.com/read/28282685/-extracorporeal-liver-replacement-therapy-already-standard-procedure
#20
Hans U Gerth, Michele Pohlen, Hartmut H J Schmidt
No abstract text is available yet for this article.
March 2017: Deutsche Medizinische Wochenschrift
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