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https://www.readbyqxmd.com/read/28919271/-autoimmune-hepatitis-immunological-diagnosis
#1
Imane Brahim, Ikram Brahim, Raja Hazime, Brahim Admou
Autoimmune hepatopathies (AIHT) including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune cholangitis (AIC), represent an impressive entities in clinical practice. Their pathogenesis is not perfectly elucidated. Several factors are involved in the initiation of hepatic autoimmune and inflammatory phenomena such as genetic predisposition, molecular mimicry and/or abnormalities of T-regulatory lymphocytes. AIHT have a wide spectrum of presentation, ranging from asymptomatic forms to severe acute liver failure...
September 14, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28888897/hepatobiliary-disease-in-children-and-adolescents-with-cystic-fibrosis
#2
Fernanda de S Nascimento, Nelson A Sena, Tatiane da A Ferreira, Cibele D F Marques, Luciana R Silva, Edna Lúcia S de Souza
OBJECTIVES: The aims of the study were to determine the frequency of hepatobiliary disease in patients with CF and to describe the sociodemographic, clinical, and laboratory profile of these patients. METHODS: This was a retrospective, descriptive, and analytical study of 55 patients diagnosed with CF fibrosis, aged between 3 months and 21 years, followed-up from January 2008 to June 2016 in a referral center. Medical records were consulted, including sociodemographic, clinical and laboratory data, including hepatobiliary alterations, imaging studies, genetic studies, liver biopsies, and upper digestive endoscopies...
September 7, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/28888439/preparation-characterization-and-alcoholic-liver-injury-protective-effects-of-algal-oligosaccharides-from-gracilaria-lemaneiformis
#3
Min Jin, Haifeng Liu, Yanping Hou, Zhuhua Chan, Wenjie Di, Li Li, Runying Zeng
Oligosaccharides derived from seaweeds possess diverse biological functions. However, little is known about their effects on liver damage. In this study, algal oligosaccharides (AOS) were prepared from Gracilaria lemaneiformis by biodegradation approaches. HPLC analysis showed AOS were composed of agarooligosaccharides and neoagarooligosaccharides. In vivo animal studies showed AOS significantly attenuated alcohol-induced hepatopathy in mice to some extent, as revealed by the normalization of several serum liver-damage markers...
October 2017: Food Research International
https://www.readbyqxmd.com/read/28877511/severe-neurotoxicity-requiring-mechanical-ventilation-in-a-dog-envenomed-by-a-red-bellied-black-snake-pseudechis-porphyriacus-and-successful-treatment-with-an-experimental-bivalent-whole-equine-igg-antivenom
#4
A M Padula, E M Leister
Snakebite in dogs from Pseudechis porphyriacus (red-bellied black snake; RBBS) is a common envenomation treated by veterinarians in Australia where this snake occurs. This case report describes the successful treatment of a clinically severe RBBS envenomation in a dog with an experimental bivalent equine whole IgG antivenom and mechanical ventilation, following its presentation in a cyanotic state. The cause of the cyanosis and respiratory distress was considered due to paralysis from neurotoxins in RBBS venom...
September 4, 2017: Toxicon: Official Journal of the International Society on Toxinology
https://www.readbyqxmd.com/read/28875438/non-invasive-evaluation-of-nafld-with-indocyanine-green-clearance-test-a-preliminary-study-in-morbidly-obese-patients-undergoing-bariatric-surgery
#5
Pierre Eric Danin, Rodolphe Anty, Stephanie Patouraux, Marc Raucoules-Aimé, Jean Gugenheim, Albert Tran, Philippe Gual, Antonio Iannelli
BACKGROUND: Overweight and obesity dramatically increased in the last years. Hepatic complication of obesity, integrated in the term of non-alcoholic fatty liver disease (NAFLD), is a spectrum of abnormality ranging from steatosis to non-alcoholic steatohepatitis (NASH), potentially leading to cirrhosis. Liver biopsy remains the gold standard to evaluate the stage of NAFLD; however, the procedure is invasive. The indocyanine green (ICG) clearance test is performed since years to assess hepatic function before partial hepatectomy, or after liver transplantation...
September 5, 2017: Obesity Surgery
https://www.readbyqxmd.com/read/28868358/glycogenic-hepatopathy-a-complication-of-uncontrolled-diabetes
#6
Medha Satyarengga, Yelena Zubatov, Sylvaine Frances, Gopal Narayanswami, Rodolfo J Galindo
OBJECTIVE: To describe a case of hepatomegaly and elevated transaminases in a patient with glycogenic hepatopathy (GH) as a complication of uncontrolled diabetes. METHODS: Clinical, laboratory, and pathological information are described. RESULTS: An 18-year-old male with uncontrolled type 1 diabetes and recurrent diabetic ketoacidosis (DKA) presented with abdominal distention and severe hyperglycemia. Physical examination revealed massive hepatomegaly...
2017: AACE Clinical Case Reports
https://www.readbyqxmd.com/read/28868180/acute-and-chronic-hepatobiliary-manifestations-of-sickle-cell-disease-a-review
#7
REVIEW
Rushikesh Shah, Cesar Taborda, Saurabh Chawla
Sickle cell disease (SCD) is a common hemoglobinopathy which can affect multiple organ systems in the body. Within the digestive tract, the hepatobiliary system is most commonly affected in SCD. The manifestations range from benign hyperbilirubinemia to overt liver failure, with the spectrum of acute clinical presentations often referred to as "sickle cell hepatopathy". This is an umbrella term referring to liver dysfunction and hyperbilirubinemia due to intrahepatic sickling process during SCD crisis leading to ischemia, sequestration and cholestasis...
August 15, 2017: World Journal of Gastrointestinal Pathophysiology
https://www.readbyqxmd.com/read/28856167/expressions-of-orphan-nuclear-receptor-tr3-nur77-in-chronic-hepatopathy-and-its-clinical-significance
#8
Yingling Zeng, Xiaoguang Ye, Degui Liao, Shizhang Huang, Huinan Mao, Dezheng Zhao, Huiyan Zeng
OBJECTIVE: Although great success has been achieved in cancer treatment, current cancer therapies, including anti-tumorigenesis and anti-angiogenesis, still face the problems of insufficient efficacy, resistance and intrinsic refractoriness, in addition to their toxic side effects. There is a demand to identify additional targets that can be blocked to turn off the downstream effects of most, if not all, pathways. Our studies suggest that orphan nuclear receptor TR3 (human)/Nur77 (mouse) is such a target...
August 2017: Journal of Clinical & Experimental Oncology
https://www.readbyqxmd.com/read/28835821/slow-ventricular-tachycardia-presenting-with-acute-liver-failure
#9
Andreas Wannhoff, Christian Nusshag, Wolfgang Stremmel, Uta Merle
OBJECTIVES: Cardiac hepatopathy is an important differential diagnosis of acute liver failure. Slow ventricular tachycardia (slow VT) is a ventricular tachycardia (VT), in which heart rate is below the typical frequency of VT. We here report a case of acute liver failure in a patient with slow VT. METHODS: The 64-year old male patient with history of cardiac pacemaker implantation for complete atrioventricular block was referred to our intensive care unit because of acute liver failure...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28813731/a-case-of-fulminant-hepatic-failure-secondary-to-congestive-heart-failure-without-evidence-of-acute-cardiac-decompensation
#10
Kalyan Wagle, Oluwaseun A Akinseye, Prakash Shrestha, Madhuri Chandnani, Raiko Munankarmi, Vivian Ripin, Jimmy Yee
There are so far only a few reported cases of acute fulminant hepatic failure resulting from acute cardiomyopathy. This is a rare occurrence, especially in patients that do not exhibit any signs and symptoms of acute cardiac decompensation. We report a case of fulminant liver failure with nondiagnostic work up for the common causes of liver failure. This patient had concurrent history of congestive heart failure, but did not have acute decompensation. Right upper quadrant sonogram revealed hepatomegaly of 15 cm, trace amount of perihepatic ascites, pericholecystic fluid, and also thickened edematous gallbladder wall with no stones, no common bile duct stones, and no portal vein thrombosis...
April 2017: South Dakota Medicine: the Journal of the South Dakota State Medical Association
https://www.readbyqxmd.com/read/28799459/comparative-study-with-propensity-score-matching-analysis-of-two-different-methods-of-transection-during-hemi-right-hepatectomy-ultracision-harmonic-scalpel-versus-cavitron-ultrasonic-surgical-aspirator
#11
François Appéré, Tullio Piardi, Ricardo Memeo, Sophie Lardière-Deguelte, Mikael Chetboun, Daniele Sommacale, Patrick Pessaux, Reza Kianmanesh
BACKGROUND: Several devices are available for liver parenchyma transection (LPT). The aim of this study was to compare the Ultracision Harmonic scalpel (UHS) with the Cavitron Ultrasonic Surgical Aspirator (CUSA) among patients who underwent hemi-right hepatectomies (RH) to homogenize transection areas. METHODS: From September 2012 to June 2015, 24 patients who underwent the UHS surgery approach were matched with 24 patients who underwent the CUSA transection procedure for RH using propensity score matching...
August 1, 2017: Surgical Innovation
https://www.readbyqxmd.com/read/28794993/limitations-of-galactose-therapy-in-phosphoglucomutase-1-deficiency
#12
Kristine Nolting, Julien H Park, Laura C Tegtmeyer, Andrea Zühlsdorf, Marianne Grüneberg, Stephan Rust, Janine Reunert, Ingrid Du Chesne, Volker Debus, Eric Schulze-Bahr, Robert C Baxter, Yoshinao Wada, Christian Thiel, Emile van Schaftingen, Ralph Fingerhut, Thorsten Marquardt
INTRODUCTION: Phosphoglucomutase 1 deficiency (PGM1 deficiency) has been identified as both, glycogenosis and congenital disorder of glycosylation (CDG). The phenotype includes hepatopathy, myopathy, oropharyngeal malformations, heart disease and growth retardation. Oral galactose supplementation at a dosage of 1 g per kg body weight per day is regarded as the therapy of choice. RESULTS: We report on a patient with a novel disease causing mutation, who was treated for 1...
December 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28776642/diagnosis-of-monogenic-liver-diseases-in-childhood-by-next-generation-sequencing
#13
Amelie Stalke, Britta Skawran, Bernd Auber, Thomas Illig, Brigitte Schlegelberger, Norman Junge, Imeke Goldschmidt, Christoph Leiskau, Nils von Neuhoff, Ulrich Baumann, Eva-Doreen Pfister
Next-Generation Sequencing (NGS) has opened up novel diagnostic opportunities for children with unidentified, but suspected inherited diseases. We describe our single-center experience with NGS diagnostics in standard clinical scenarios in pediatric hepatology. We investigated 135 children with suspected inherited hepatopathies, where initially no causative pathogenic variant had been identified, with an amplicon-based NGS panel of 21 genes associated with acute and chronic hepatopathies. In 23 of these patients we detected pathogenic or likely pathogenic variants in ten different genes...
August 4, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28757868/-13-c-methacetin-breath-test-for-assessment-of-microsomal-liver-function-methodology-and-clinical-application
#14
REVIEW
Katarzyna Gorowska-Kowolik, Agata Chobot, Jaroslaw Kwiecien
Assessment of the liver function, and the need of constant monitoring of the organ's capacity, concerns not only patients with primary liver diseases, but also those at risk of hepatopathies secondary to other chronic diseases. Most commonly, the diagnostics is based on measurements of static biochemical parameters, which allow us to draw conclusions only indirectly about the function and the degree of damage of the organ. On the other hand, liver biopsy is an invasive procedure and therefore it is associated with a considerable risk of complications...
2017: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/28725306/histology-and-glutamine-synthetase-immunoreactivity-in-liver-biopsies-from-patients-with-congestive-heart-failure
#15
Bela Horvath, Lei Zhu, Daniela Allende, Hao Xie, John Guirguis, Michael Cruise, Deepa T Patil, Robert O'Shea, John Rivas, Reyna Yordanka, Nan Lan, Xiuli Liu
BACKGROUND: Long-standing congestive heart failure can induce a constellation of histopathology changes in the liver that can range from mild sinusoidal dilation to advanced fibrosis and loss of normal perivenular expression of glutamine synthetase (GS). Liver biopsies might be performed to assess the perioperative risk of these patients or to determine the need of synchronous liver transplant. We aimed to assess interobserver agreement in recognizing these liver histologic features in patients undergoing evaluation for heart transplantation and to examine whether immunohistochemistry of GS will aid the diagnosis of cardiac hepatopathy (CH)...
June 2017: Gastroenterology Research
https://www.readbyqxmd.com/read/28674631/liver-stiffness-assessed-by-fibrosis-4-index-predicts-mortality-in-patients-with-heart-failure
#16
Yu Sato, Akiomi Yoshihisa, Yuki Kanno, Shunsuke Watanabe, Tetsuro Yokokawa, Satoshi Abe, Tomofumi Misaka, Takamasa Sato, Satoshi Suzuki, Masayoshi Oikawa, Atsushi Kobayashi, Takayoshi Yamaki, Hiroyuki Kunii, Kazuhiko Nakazato, Shu-Ichi Saitoh, Yasuchika Takeishi
OBJECTIVE: Liver dysfunction due to heart failure (HF) is known as congestive hepatopathy. It has recently been reported that liver stiffness assessed by transient elastography reflects increased central venous pressure. The Fibrosis-4 (FIB4) index (age (years) × aspartate aminotransferase (IU/L)/platelet count (10(9)/L) × square root of alanine aminotransferase (IU/L)) is expected to be useful for evaluating liver stiffness in patients with non-alcoholic fatty liver disease. We aimed to investigate the impact of the FIB4 index on HF prognosis, with consideration for liver fibrosis markers and underlying cardiac function...
2017: Open Heart
https://www.readbyqxmd.com/read/28580825/notable-imaging-features-of-the-liver-in-a-chinese-patient-with-recurrent-immunoglobulin-g4-hepatopathy
#17
H Li, F Peng, F Tian
No abstract text is available yet for this article.
June 5, 2017: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/28575497/loss-of-hepatic-lrpprc-alters-mitochondrial-bioenergetics-regulation-of-permeability-transition-and-trans-membrane-ros-diffusion
#18
Alexanne Cuillerier, Shamisa Honarmand, Virgilio J J Cadete, Matthieu Ruiz, Anik Forest, Sonia Deschênes, Claudine Beauchamp, Guy Charron, John D Rioux, Christine Des Rosiers, Eric A Shoubridge, Yan Burelle
The French-Canadian variant of Leigh Syndrome (LSFC) is an autosomal recessive oxidative phosphorylation (OXPHOS) disorder caused by a mutation in LRPPRC, coding for a protein involved in the stability of mitochondrially-encoded mRNAs. Low levels of LRPPRC are present in all patient tissues, but result in a disproportionately severe OXPHOS defect in the brain and liver, leading to unpredictable subacute metabolic crises. To investigate the impact of the OXPHOS defect in the liver, we analyzed the mitochondrial phenotype in mice harboring an hepatocyte-specific inactivation of Lrpprc...
August 15, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28559828/multisystem-disease-including-eosinophilia-and-progressive-hyper-creatine-kinase-emia-over-10-years-suggests-mitochondrial-disorder
#19
Josef Finsterer, Johannes Huber
BACKGROUND: Eosinophilia has not been reported as a manifestation of a mitochondrial disorder (MID). Here, we report a patient with clinical features suggesting a MID and permanent eosinophilia, multisystem disease, and progressive hyper-creatine-kinase (CK)-emia for at least 10 years. MATERIALS AND METHODS: Methods applied included a clinical exam, blood chemical investigations, electrophysiological investigations, imaging, and invasive cardiological investigations...
January 2017: Case Reports in Neurology
https://www.readbyqxmd.com/read/28533680/preparation-characterization-and-in-vitro-vivo-studies-of-oleanolic-acid-loaded-lactoferrin-nanoparticles
#20
Xiaojing Xia, Haowei Liu, Huixia Lv, Jing Zhang, Jianping Zhou, Zhiying Zhao
Oleanolic acid (OA), a pentacyclic triterpene, is used to safely and economically treat hepatopathy. However, OA, a Biopharmaceutics Classification System IV category drug, has low bioavailability owing to low solubility (<1 μg/mL) and biomembrane permeability. We developed a novel OA nanoparticle (OA-NP)-loaded lactoferrin (Lf) nanodelivery system with enhanced in vitro OA dissolution and improved oral absorption and bioavailability. The OA-NPs were prepared using NP albumin-bound technology and characterized using dynamic light scattering, scanning electron microscopy, X-ray powder diffraction, differential scanning calorimetry, and in vitro dissolution test...
2017: Drug Design, Development and Therapy
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