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Hepatopathy

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https://www.readbyqxmd.com/read/29916914/somatostatin-receptor-pet-ct-features-of-carcinoid-heart-disease
#1
Saurabh Arora, Averilicia Passah, Nishikant A Damle, Rajiv Narang, Atul Sharma, Madhav P Yadav, Sanjana Ballal, Dinkar Bhasin, Chandrasekhar Bal
We present the case of a 35-year-old woman with metastatic neuroendocrine tumor undergoing treatment with long-acting octreotide and Lu-DOTATATE therapy. We present features of carcinoid heart disease on Ga-DOTANOC PET/CT, which revealed dilated right atrium, pericardial effusion, ascites, and congestive hepatopathy apart from the metastatic lesions in the liver. The scan cardiac findings were confirmed by echocardiography. This highlights the significance of identifying such patients early because additional management is required for carcinoid heart disease per se and a cautious approach is needed in administering amino acid infusion during PRRT that can result in fluid overload...
June 19, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29886410/imaging-findings-of-igg4-related-hepatopathy-a-rare-case-presenting-as-a-hepatic-mass
#2
Youe Ree Kim, Young Hwan Lee, Keum-Ha Choi, Kwon-Ha Yoon
IgG4-related hepatopathy is usually referred to as parenchymal involvement of IgG4-related disease of the liver. As this disease entity was only recently described, little is known of its pathogenesis or imaging findings. We report a rare case of IgG4-related hepatopathy which presented as an ill-defined heterogeneously enhancing hepatic mass with diffusion restriction and a hepatobiliary phase defect in MR and CT images. Pathologic characteristics of the disease and its imaging findings are reviewed.
May 28, 2018: Clinical Imaging
https://www.readbyqxmd.com/read/29882374/the-interactions-between-iron-and-copper-in-genetic-iron-overload-syndromes-and-primary-copper-toxicoses-in-japan
#3
REVIEW
Yasuaki Tatsumi, Ayako Kato, Koichi Kato, Hisao Hayashi
AIM: Iron and copper are trace elements essential for health, and iron metabolism is tightly regulated by cuproproteins. Clarification of the interactions between iron and copper may provide a better understanding of the pathophysiology and treatment strategy for hemochromatosis, Wilson disease, and related disorders. METHODS: The hepcidin/ferroportin system was used to classify genetic iron overload syndromes in Japan, and ceruloplasmin and ATP7B were introduced for subtyping Wilson disease into the severe hepatic and classical forms...
June 7, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29861737/nadph-oxidase-signaling-pathway-mediates-mesenchymal-stem-cell-induced-inhibition-of-hepatic-stellate-cell-activation
#4
Haowen Qiao, Yu Zhou, Xingping Qin, Jing Cheng, Yun He, Yugang Jiang
Background: Bone marrow-derived mesenchymal stem cells (BMSCs) have blossomed into an effective approach with great potential for the treatment of liver fibrosis. The aim of this study was to investigate the underlying antifibrosis mechanisms by which the BMSC inhibit activated hepatic stellate cells (HSCs) in vivo and in vitro. Methods: To study the effect of human bone marrow-derived mesenchymal stem cells (hBM-MSCs) on activated HSCs, we used HSCs and the coculture systems to evaluate the inhibition of activated HSCs from the aspects of the apoptosis of activated HSCs...
2018: Stem Cells International
https://www.readbyqxmd.com/read/29858906/clinical-and-molecular-genetic-characterization-of-two-patients-with-mutations-in-the-phosphoglucomutase-1-pgm1-gene
#5
Yu Ding, Niu Li, Gouying Chang, Juan Li, Ruen Yao, Yiping Shen, Jian Wang, Xiaodong Huang, Xiumin Wang
BACKGROUND: The phosphoglucomutase 1 (PGM1) enzyme plays a central role in glucose homeostasis by catalyzing the inter-conversion of glucose 1-phosphate and glucose 6-phosphate. Recently, PGM1 deficiency has been recognized as a cause of the congenital disorders of glycosylation (CDGs). METHODS: Two Chinese Han pediatric patients with recurrent hypoglycemia, hepatopathy and growth retardation are described in this study. Targeted gene sequencing (TGS) was performed to screen for causal genetic variants in the genome of the patients and their parents to determine the genetic basis of the phenotype...
June 2, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29850300/young-man-with-hepatomegaly-a-case-of-glycogenic-hepatopathy
#6
Walid Abboud, Saif Abdulla, Mohammed Al Zaabi, Ramzi Moufarrej
Glycogenic hepatopathy is a rare but potentially reversible condition characterized by hepatomegaly and elevated liver enzymes occurring in poorly controlled type 1 diabetes mellitus patients and often requires a liver biopsy to confirm the diagnosis. We present the case of a young man who was admitted with diabetic ketoacidosis in the setting of poorly controlled diabetes mellitus type 1 and was noted to have significantly elevated transaminases that continued to worsen despite appropriate treatment of the diabetic ketoacidosis...
2018: Case Reports in Hepatology
https://www.readbyqxmd.com/read/29844855/isolated-hepatic-non-obstructive-sinusoidal-dilatation-20-year-single-center-experience
#7
Dharma Budi Sunjaya, Guilherme Piovezani Ramos, Manuel Bonfim Braga Neto, Ryan Lennon, Taofic Mounajjed, Vijay Shah, Patrick Sequeira Kamath, Douglas Alano Simonetto
AIM: To characterize isolated non-obstructive sinusoidal dilatation (SD) by identifying associated conditions, laboratory findings, and histological patterns. METHODS: Retrospectively reviewed 491 patients with SD between 1995 and 2015. Patients with obstruction at the level of the small/large hepatic veins, portal veins, or right-sided heart failure were excluded along with history of cirrhosis, hepatic malignancy, liver transplant, or absence of electrocardiogram/cardiac echocardiogram...
May 27, 2018: World Journal of Hepatology
https://www.readbyqxmd.com/read/29792719/-sinusoidal-obstruction-syndrome-induced-by-monocrotaline-in-a-large-animal-experiment-a-pilot-study
#8
R Pálek, V Liška, V Třeška, J Rosendorf, M Emingr, V Tégl, A Králíčková, K Bajcurová, M Jiřík, Z Tonar
INTRODUCTION: Sinusoidal obstruction syndrome (SOS) is a disease which is caused by toxic injury to hepatic sinusoids. This syndrome is most frequently caused by myeloablative radiochemotherapy in patients before hematopoietic stem cells transplantation and also by oxaliplatin mainly in patients with colorectal liver metastases. The aim of this study was to establish a large animal model of SOS, which would enable further study of this disease and facilitate translation of experimental outcomes into human medicine...
2018: Rozhledy V Chirurgii: Měsíčník Československé Chirurgické Společnosti
https://www.readbyqxmd.com/read/29783990/severe-hepatopathy-and-neurological-deterioration-after-start-of-valproate-treatment-in-a-6-year-old-child-with-mitochondrial-tryptophanyl-trna-synthetase-deficiency
#9
Elise Vantroys, Joél Smet, Arnaud V Vanlander, Sarah Vergult, Ruth De Bruyne, Frank Roels, Hedwig Stepman, Herbert Roeyers, Björn Menten, Rudy Van Coster
BACKGROUND: The first subjects with deficiency of mitochondrial tryptophanyl-tRNA synthetase (WARS2) were reported in 2017. Their clinical characteristics can be subdivided into three phenotypes (neonatal phenotype, severe infantile onset phenotype, Parkinson-like phenotype). RESULTS: Here, we report on a subject who presented with early developmental delay, motor weakness and intellectual disability and who was considered during several years as having a non-progressive encephalopathy...
May 21, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29782205/nad-repletion-produces-no-therapeutic-effect-in-mice-with-respiratory-chain-complex-iii-deficiency-and-chronic-energy-deprivation
#10
Janne Purhonen, Jayasimman Rajendran, Saara Tegelberg, Olli-Pekka Smolander, Eija Pirinen, Jukka Kallijärvi, Vineta Fellman
Biosynthetic precursors of NAD+ can replenish a decreased cellular NAD+ pool and, supposedly via sirtuin (SIRT) deacetylases, improve mitochondrial function. We found decreased hepatic NAD+ concentration and downregulated biosynthesis in Bcs1lp.S78G knock-in mice with respiratory chain complex III deficiency and mitochondrial hepatopathy. Aiming at ameliorating disease progression via NAD+ repletion and improved mitochondrial function, we fed these mice nicotinamide riboside (NR), a NAD+ precursor. A targeted metabolomics verified successful administration and suggested enhanced NAD+ biosynthesis in the treated mice, although hepatic NAD+ concentration was unchanged at the end point...
May 21, 2018: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29741464/immunohistochemical-characterisation-of-the-hepatic-stem-cell-niche-in-feline-hepatic-lipidosis-a-preliminary-morphological-study
#11
Chiara Valtolina, Joris H Robben, Robert P Favier, Jan Rothuizen, Guy Cm Grinwis, Baukje A Schotanus, Louis C Penning
Objectives The aim of this study was to describe the cellular and stromal components of the hepatic progenitor cell niche in feline hepatic lipidosis (FHL). Methods Immunohistochemical staining for the progenitor/bile duct marker (K19), activated Kupffer cells (MAC387), myofibroblasts (alpha-smooth muscle actin [α-SMA]) and the extracellular matrix component laminin were used on seven liver biopsies of cats with FHL and three healthy cats. Double immunofluorescence stainings were performed to investigate co-localisation of different cell types in the hepatic progenitor cell (HPC) niche...
May 1, 2018: Journal of Feline Medicine and Surgery
https://www.readbyqxmd.com/read/29739065/the-activity-of-alcohol-dehydrogenase-isoenzymes-and-aldehyde-dehydrogenase-in-the-sera-of-patients-with-autoimmune-hepatitis
#12
Wojciech Jelski, Blanka Wolszczak-Biedrzycka, Elzbieta Zasimowicz-Majewska, Karolina Orywal, Joanna Piechota, Tadeusz W Lapinski, Marzena Bednarczyk, Maciej Szmitkowski
BACKGROUND: Autoimmune hepatitis (AIH) is a progressive inflammatory hepatopathy and an important cause of end-stage liver. The liver cells' destruction is reflected by increased activity of different enzymes in the serum. These enzymes include alcohol dehydrogenase (ADH) and aldehyde dehydrogenase (ALDH), which play a significant role in the metabolism of many biological substances and exist mainly in the liver. In this study we investigated the activity of alcohol dehydrogenase and its isoenzymes and the total activity of ALDH in the sera of patients with autoimmune hepatitis...
April 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29721513/an-unusual-presentation-of-glycogenic-hepatopathy-with-bridging-fibrosis
#13
Jagannath M Sherigar, Yoness Darouichi, Debra Guss, Smruti R Mohanty
Glycogenic hepatopathy is a rare and under-recognized complication of poorly controlled diabetes mellitus. We report a patient who presented with predominant elevation in alkaline phosphatase and liver biopsy showing bridging fibrosis, which is an unusual presentation of glycogenic hepatopathy. This case emphasizes the fact that glycogenic hepatopathy can also present with a cholestatic pattern of liver abnormality and with liver fibrosis, which warrants further study because severe fibrosis can progress to cirrhosis...
2018: ACG Case Reports Journal
https://www.readbyqxmd.com/read/29713145/inhibition-of-ghrelin-o-acyltransferase-attenuated-lipotoxicity-by-inducing-autophagy-via-ampk-mtor-pathway
#14
Shaoren Zhang, Yuqing Mao, Xiaoming Fan
Background: Nonalcoholic fatty liver disease (NAFLD) has been considered the most commonly occurring chronic hepatopathy in the world. Ghrelin o -acyltransferase (GOAT) is an acylation enzyme which has an acylated position 3 serine on ghrelin. Recent investigation revealed that activated autophagy could attenuate liver steatosis. The aim of this study was to explore therapeutic roles that inhibit GOAT exerted in NAFLD, and its potential association with autophagy. Materials and methods: Human LO2 cells were pretreated with siRNA-GOAT to induce liver steatosis using free fatty acids (FFAs)...
2018: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/29709217/glycogenic-hepatopathy
#15
Johad Khoury, Yaniv Zohar, Naim Shehadeh, Tarek Saadi
BACKGROUND: Glycogenic hepatopathy (GH) is a disorder associated with uncontrolled diabetes mellitus, most commonly type 1, expressed as right upper quadrant abdominal pain, hepatomegaly and increased liver enzymes. The diagnosis may be difficult, because laboratory and imaging tests are not pathognomonic. Although GH may be suggested based on clinical presentation and imaging studies, the gold standard for diagnosis is a liver biopsy, showing a significant accumulation of glycogen within the hepatocytes...
April 2018: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/29704975/1000-consecutive-liver-transplants-descriptive-analysis-and-evolution-of-a-single-center
#16
José Tinoco González, Jose María Álamo Martínez, Carmen Bernal Bellido, Gonzalo Suárez Artacho, Luis Miguel Marín Gómez, Lydia Barrera-Pulido, Javier Padillo Ruíz, Miguel Ángel Gómez Bravo
Between 1991 and 2013, 1,000 liver transplantations were performed at Virgen del Rocio Hospital (Seville, Spain). A retrospective study was conducted, analyzing the characteristics of recipients and donors, indications, surgical technique, complications and survival in 2 different stages (1991-2002 vs. 2003-2013) coinciding with the implementation of the MELD scale as a prioritization model. The most frequent indication were of hepatopathy of hepatocellular origin in 48.8%. There was a significant increase in the indications for hepatocarcinoma (8...
May 2018: Cirugía Española
https://www.readbyqxmd.com/read/29672883/assessment-of-advanced-liver-fibrosis-and-the-risk-for-hepatic-decompensation-in-patients-with-congestive-hepatopathy
#17
REVIEW
Alexander Lemmer, Lisa B VanWagner, Daniel Ganger
Congestive hepatopathy (CH) arises from chronically elevated right sided heart pressures transmitted to the liver by passive venous congestion. Over time CH can lead to hepatic bridging fibrosis, decompensated cirrhosis, and hepatocellular carcinoma. Currently there are no evidence-based guidelines to direct appropriate screening or management of patients with CH partly because of the inability of current clinical tools (serum tests, imaging studies, liver stiffness measurements, and liver biopsy) to accurately estimate hepatic fibrosis or the risk for hepatic decompensation...
April 19, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29667721/diverse-manifestations-of-acute-sickle-cell-hepatopathy-in-pediatric-patients-with-sickle-cell-disease-a-case-series
#18
Lydia H Pecker, Nidhi Patel, Susan Creary, Anil Darbari, Emily Riehm Meier, Deepika S Darbari, Ross M Fasano
The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non-SCD cause of hepatopathy in a patient with viral hepatitis...
April 18, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29661458/ombitasvir-paritaprevir-ritonavir-therapy-in-a-kidney-transplant-recipient-with-chronic-hepatitis-c-virus-genotype-1-infection-a-case-report-on-the-importance-of-considering-drug-drug-interactions-and-monitoring-cyclosporine-levels
#19
S Takeuchi, M Takamura, T Yoshida, K Takahashi, K Hayashi, S Hashimoto, S Yamagiwa, M Tasaki, Y Nakagawa, K Saito, Y Tanabe, Y Tomita, S Terai
A 74-year-old Japanese man with a history of chronic hepatitis C and kidney transplant (KT) was administered pegylated-interferon plus ribavirin therapy. However, this therapy was ineffective. The patient was then hospitalized to receive ombitasvir (OBV) plus paritaprevir (PTV) plus ritonavir (r) antiviral combination therapy. He tested negative for the virus after 4 weeks, and completed 12 weeks of treatment. The patient ultimately achieved a sustained virological response after the 12 weeks of treatment. Cyclosporine (CyA) trough levels, during the OBV-PTV-r therapy, reached a peak within 5 days of initiating therapy, and increases in serum creatinine and total bilirubin were also observed...
April 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29655574/current-treatment-for-non-alcoholic-fatty-liver-disease
#20
C Moctezuma-Velázquez
Non-alcoholic fatty liver disease is the most prevalent hepatopathy, estimated at 30% in the general population. In the coming years, it will likely be the most common indication for liver transplantation and the most frequent cause of hepatocellular carcinoma. Current treatment for non-alcoholic fatty liver disease is based on dietary and exercise interventions that have been shown to be efficacious, even for reverting fibrosis. Unfortunately, compliance with general measures involving lifestyle modifications is very poor, making pharmacologic strategies a necessary option...
April 2018: Revista de Gastroenterología de México
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