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Hepatopathy

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https://www.readbyqxmd.com/read/28813731/a-case-of-fulminant-hepatic-failure-secondary-to-congestive-heart-failure-without-evidence-of-acute-cardiac-decompensation
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
BACKGROUND AND AIMS: 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. METHODS: 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...
May 31, 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
#10
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
#11
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
https://www.readbyqxmd.com/read/28529811/glycogenic-hepatopathy-resolution-with-minimal-glucose-control
#12
Abhimanyu Chandel, Brittany Scarpato, Jeanette Camacho, Miles McFarland, Shaffer Mok
We describe a presentation of glycogenic hepatopathy in a poorly controlled type I diabetic patient. As patients with glycogenic hepatopathy often have nonspecific complaints, diagnosis tends to be delayed and laboratory and imaging data are often indistinguishable from nonalcoholic fatty liver disease. Our patient's diagnosis of glycogenic hepatopathy required a liver biopsy, which demonstrated the characteristic pathology. Her symptoms resolved with minimal alteration to her insulin regimen and only slightly improved glucose control...
2017: Case Reports in Hepatology
https://www.readbyqxmd.com/read/28526384/stereology-shows-that-damaged-liver-recovers-after-protein-refeeding
#13
Silvio Pires Gomes, Andréa Almeida Pinto da Silva, Amanda Rabello Crisma, Primavera Borelli, Francisco Javier Hernandez-Blazquez, Mariana P de Melo, Barbara Bacci, Andrzej Loesch, A Augusto Coppi
OBJECTIVE: The aim of the present study was to investigate the putative effects of a low-protein diet on the three-dimensional structure of hepatocytes and determine whether this scenario could be reversed by restoring the adequate levels of protein to the diet. METHODS: Using design-based stereology, the total number and volume of hepatocytes were estimated in the liver of mice in healthy and altered (by protein malnutrition) conditions and after protein renutrition...
June 2017: Nutrition
https://www.readbyqxmd.com/read/28523311/study-concerning-the-histopathological-changes-in-chronic-hepatopathies
#14
Cristian Marius Daina, Lucia Georgeta Daina, Marinela Bonta, Daniela Rahotă, Gabriela MuŢiu
Chronic liver diseases represent a public health problem both at global level and in our country. Their significance is due not only to the large number of cases but also to their severe complications and associated diseases, which increase the gravity of prognosis. Hepatopathies generally develop by formation and accumulation of fibrous tissue, which leads to architectural distortions in the structure of the liver. Hepatic fibrosis is the result of chronic injury and plays a direct role in the pathogenesis of the hepatocellular dysfunction and portal hypertension...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/28506373/heart-disease-and-the-liver-pathologic-evaluation
#15
REVIEW
Anne Knoll Koehne de Gonzalez, Jay H Lefkowitch
Liver injury due to acute and chronic heart failure has long been recognized. This article discusses the concepts of acute cardiogenic liver injury (ACLI) and cardiac or congestive hepatopathy (CH) along with their clinical manifestations and sequelae. Histologically, ACLI manifests as centrilobular hepatocellular necrosis, whereas CH is associated with centrilobular hepatocyte atrophy, dilated sinusoids, and perisinusoidal fibrosis, progressing to bridging fibrosis and ultimately cirrhosis. ACLI is associated with marked increases in aminotransferase levels, whereas CH is associated with a cholestatic pattern of laboratory tests...
June 2017: Gastroenterology Clinics of North America
https://www.readbyqxmd.com/read/28499323/-cmv-associated-acute-liver-failure-in-a-patient-receiving-tocilizumab-for-systemic-lupus-erythematosus
#16
Sophie Fromhold-Treu, Andreas Erbersdobler, Manuela Turan, Gunther Neeck, Georg Lamprecht
A 41-year-old female patient was admitted because of febrile jaundice and acute liver failure. The quick and the bilirubin were 21 % and 258 µmol/l, and there was hepatic encephalopathy I°. AST and AP had a maximum of 612 and 215 U/l. Despite a strong left shift in the differential, the CRP had a maximum of 15 mg/l. Because of an atypically presenting systemic lupus erythematosus, she had been treated with Azathioprine, steroids and Tocilizumab until 12 days before admission. The diagnostic workup revealed CMV hepatitis and necrotizing hepatopathy, which was interpreted as toxic hepatitis...
May 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/28493820/deoxyguanosine-kinase-deficiency-a-report-of-four-patients
#17
Özlem Ünal, Burcu Hişmi, Mustafa Kılıç, Hayriye Hızarcıoğlu Gülşen, Turgay Coşkun, Serap Hatice Sivri, Ali Dursun, Aysel Yüce, Ayşegül Tokatlı
BACKGROUND: Hepatic involvement is a common feature in childhood mitochondrial disorders. Deoxyguanosine kinase (DGUOK) deficiency is one of the mitochondrial DNA depletion syndromes associated with hepatocerebral syndrome. Hepatic disease and neurologic dysfunction occurs within weeks after birth. Low birth weight is one of the common features. This study aims to describe the clinical and laboratory features of four infants carrying four different pathogenic variants in the DGUOK gene...
May 24, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28491317/marathoning-with-myotonic-dystrophy-type-2-proximal-myotonic-myopathy-and-leukopenia
#18
Josef Finsterer, Georg Safoschnik, Martina Witsch-Baumgartner
OBJECTIVES: A mild, slowly progressive course of proximal myotonic myopathy, also known as myotonic dystrophy type 2, over years allowing the patient to continue with extreme sport activity, has been only rarely reported. METHODS: Case report. RESULTS: The patient is a 54-year-old female sport teacher who developed myotonia of the distal upper limbs at the age of 32 years. Over the following 22 years, myotonia spreaded to the entire musculature...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28459846/gene-expression-patterns-in-the-progression-of-canine-copper-associated-chronic-hepatitis
#19
Karen Dirksen, Bart Spee, Louis C Penning, Ted S G A M van den Ingh, Iwan A Burgener, Adrian L Watson, Marian Groot Koerkamp, Jan Rothuizen, Frank G van Steenbeek, Hille Fieten
Copper is an essential trace element, but can become toxic when present in abundance. The severe effects of copper-metabolism imbalance are illustrated by the inherited disorders Wilson disease and Menkes disease. The Labrador retriever dog breed is a novel non-rodent model for copper-storage disorders carrying mutations in genes known to be involved in copper transport. Besides disease initiation and progression of copper accumulation, the molecular mechanisms and pathways involved in progression towards copper-associated chronic hepatitis still remain unclear...
2017: PloS One
https://www.readbyqxmd.com/read/28458594/hepatic-lesions-that-mimic-metastasis-on-radiological-imaging-during-chemotherapy-for-gastrointestinal-malignancy-recent-updates
#20
REVIEW
Sung-Hye You, Beom Jin Park, Yeul Hong Kim
During chemotherapy in patients with gastrointestinal malignancy, the hepatic lesions may occur as chemotherapy-induced lesions or tumor-associated lesions, with exceptions for infectious conditions and other incidentalomas. Focal hepatic lesions arising from chemotherapy-induced hepatopathies (such as chemotherapy-induced sinusoidal injury and steatosis) and tumor-associated eosinophilic abscess should be considered a mimicker of metastasis in patients with gastrointestinal malignancy. Accumulating evidence suggests that chemotherapy for gastrointestinal malignancy in the liver has roles in both the therapeutic effects for hepatic metastasis and injury to the non-tumor bearing hepatic parenchyma...
May 2017: Korean Journal of Radiology: Official Journal of the Korean Radiological Society
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