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https://www.readbyqxmd.com/read/29667721/diverse-manifestations-of-acute-sickle-cell-hepatopathy-in-pediatric-patients-with-sickle-cell-disease-a-case-series
#1
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
#2
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
#3
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 11, 2018: Revista de Gastroenterología de México
https://www.readbyqxmd.com/read/29645071/daclizumab-in-multiple-sclerosis
#4
REVIEW
F C Perez-Miralles
INTRODUCTION: Daclizumab is a monoclonal antibody directed against the CD25 subunit of the interleukin-2 receptor, investigated as a disease-modifying therapy in relapsing-remitting multiple sclerosis. The present review addresses how the drug was developed, the known mechanism of action of the drug and the up-to-date data of efficacy and safety. DEVELOPMENT: Daclizumab has shown superiority in prevention of relapses against placebo and low-dose interferon beta-1a at a level that puts it on par with the rest of current first-line drugs...
April 16, 2018: Revista de Neurologia
https://www.readbyqxmd.com/read/29642696/periprocedural-and-perioperatory-management-of-patients-with-tricuspid-valve-disease
#5
Fabrizio Monaco, Ambra L DI Prima, Monica DE Luca, Gaia Barucco, Alberto Zangrillo
Tricuspid regurgitation (TR) is a common valvular lesion which may affect morbidity and mortality. It can be related to an intrinsic abnormality of the tricuspid valve leaflets (organic) or secondary to annular dilatation (functional). Often organic and functional TR coexist in the same patient. A long standing TR is associated with ascites, congestive hepatopathy, peripheral edema, renal failure, and abdominal fullness which significantly affect the outcome. In particular, the perioperative course may be complicated due to both the presence of comorbidities and the development of a severe postoperative right ventricle (RV) dysfunction...
April 11, 2018: Minerva Cardioangiologica
https://www.readbyqxmd.com/read/29618761/qil1-dependent-assembly-of-micos-complex-lethal-mutation-in-c19orf70-resulting-in-liver-disease-and-severe-neurological-retardation
#6
J Gödiker, M Grüneberg, I DuChesne, J Reunert, S Rust, C Westermann, Y Wada, G Classen, C D Langhans, K P Schlingmann, R J Rodenburg, R Pohlmann, T Marquardt
Seven subunits of the mitochondrial contact site and cristae junction (CJ) organizing system (MICOS) in humans have been recently described in function and structure. QIL1 (also named MIC13) is a small complex that is crucial for the maintenance and assembling of MICOS. A novel mutation of an essential splice site in the C19orf70 gene encoding QIL1 induces severe mitochondrial encephalopathy, hepatopathy and lactate acidosis consistent with psychomotor retardation. In addition, bilateral kidney stones were observed...
April 4, 2018: Journal of Human Genetics
https://www.readbyqxmd.com/read/29610180/blue-diaper-syndrome-and-pcsk1-mutations
#7
Felix Distelmaier, Diran Herebian, Claudia Atasever, Stefanie Beck-Woedl, Ertan Mayatepek, Tim M Strom, Tobias B Haack
Blue diaper syndrome (BDS) (Online Mendelian Inheritance in Man number 211000) is an extremely rare disorder that was first described in 1964. The characteristic finding is a bluish discoloration of urine spots in the diapers of affected infants. Additional clinical features of the first described patients included diarrhea, inadequate weight gain, hypercalcemia, and nephrocalcinosis. An intestinal defect of tryptophan absorption was postulated as the underlying pathology. However, functional evidence for this theory is lacking...
April 2018: Pediatrics
https://www.readbyqxmd.com/read/29607530/systematic-review-drug-induced-liver-injury-alternative-causes-in-case-series-as-confounding-variables
#8
REVIEW
Rolf Teschke, Gaby Danan
AIMS: Drug-induced liver injury (DILI) is rare as compared to the worldwide frequent acute or chronic liver diseases. Therefore, patients included in series of suspected DILI are at high risk of not having DILI, whereby alternative causes may confound the DILI diagnosis. The aim of this review is to evaluate published case series of DILI for alternative causes. METHODS: Pertinent studies were identified using a computerized search of the Medline database for publications from 1993 through 30 October 2017...
April 1, 2018: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/29551309/mitochondrial-%C3%AE-oxidation-of-saturated-fatty-acids-in-humans
#9
REVIEW
María M Adeva-Andany, Natalia Carneiro-Freire, Mónica Seco-Filgueira, Carlos Fernández-Fernández, David Mouriño-Bayolo
Mitochondrial β-oxidation of fatty acids generates acetyl-coA, NADH and FADH2 . Acyl-coA synthetases catalyze the binding of fatty acids to coenzyme A to form fatty acyl-coA thioesters, the first step in the intracellular metabolism of fatty acids. l-carnitine system facilitates the transport of fatty acyl-coA esters across the mitochondrial membrane. Carnitine palmitoyltransferase-1 transfers acyl groups from coenzyme A to l-carnitine, forming acyl-carnitine esters at the outer mitochondrial membrane. Carnitine acyl-carnitine translocase exchanges acyl-carnitine esters that enter the mitochondria, by free l-carnitine...
March 15, 2018: Mitochondrion
https://www.readbyqxmd.com/read/29538616/anti-fibrotic-role-and-mechanism-of-periplaneta-americana-extracts-in-ccl4-induced-hepatic-fibrosis-in-rats
#10
Dingchun Li, Wu Li, Yihui Chen, Lihui Liu, Dehong Ma, Hongtu Wang, Lu Zhang, Shenjun Zhao, Qin Peng
Hepatic fibrosis is resulted from sustained wound-healing responses to various harmful stimuli, including viral infection, drug toxicity, alcohol, and autoimmune hepatopathy, and it has recently attracted the attention of an increasing number of researchers and clinical workers. The aims of this study were to examine the anti-fibrotic effects of extracts of Periplaneta americana (EPA) on CCl4-induced hepatic fibrosis in rats, to preliminary determine the anti-fibrotic efficacy of EPA, and to identify a potential and effective therapeutic agent to attenuate hepatic fibrosis...
March 12, 2018: Acta Biochimica et Biophysica Sinica
https://www.readbyqxmd.com/read/29536017/metabolic-abnormalities-detected-in-phase-ii-evaluation-of-doxycycline-in-dogs-with-multicentric-b-cell-lymphoma
#11
Kelly R Hume, Skylar R Sylvester, Lucia Borlle, Cheryl E Balkman, Angela L McCleary-Wheeler, Mary Pulvino, Carla Casulo, Jiyong Zhao
Doxycycline has antiproliferative effects in human lymphoma cells and in murine xenografts. We hypothesized that doxycycline would decrease canine lymphoma cell viability and prospectively evaluated its clinical tolerability in client-owned dogs with spontaneous, nodal, multicentric, substage a, B-cell lymphoma, not previously treated with chemotherapy. Treatment duration ranged from 1 to 8 weeks (median and mean, 3 weeks). Dogs were treated with either 10 ( n  = 6) or 7.5 ( n  = 7) mg/kg by mouth twice daily...
2018: Frontiers in Veterinary Science
https://www.readbyqxmd.com/read/29527255/glycogenic-hepatopathy-a-narrative-review
#12
REVIEW
Jagannath M Sherigar, Joline De Castro, Yong Mei Yin, Debra Guss, Smruti R Mohanty
Glycogenic hepatopathy (GH) is a rare complication of the poorly controlled diabetes mellitus characterized by the transient liver dysfunction with elevated liver enzymes and associated hepatomegaly caused by the reversible accumulation of excess glycogen in the hepatocytes. It is predominantly seen in patients with longstanding type 1 diabetes mellitus and rarely reported in association with type 2 diabetes mellitus. Although it was first observed in the pediatric population, since then, it has been reported in adolescents and adults with or without ketoacidosis...
February 27, 2018: World Journal of Hepatology
https://www.readbyqxmd.com/read/29506377/-italian-cystic-fibrosis-registry-report-2011-2014
#13
Barbara Giordani, Annalisa Amato, Fabio Majo, Gianluca Ferrari, Serena Quattrucci, Laura Minicucci, Rita Padoan, Giovanna Floridia, Gianna Puppo Fornaro, Domenica Taruscio, Marco Salvatore
INTRODUCTION: The Italian Cystic Fibrosis Registry (ICFR) is based on a new agreement about the data flow towards the Registry signed on October, 4th 2016 by the Centre for Rare Diseases of the Italian National Institute of Health (NIH), the clinicians of the Italian National Referral and Support Centres for Cystic Fibrosis, the Paediatric Hospital "Bambino Gesù" (Rome), the Italian Cystic Fibrosis Society, and the Italian League for Cystic Fibrosis. OBJECTIVES: The aim of the present Report is to improve the knowledge on cystic fibrosis (CF) through the epidemiological description of Italian patients...
January 2018: Epidemiologia e Prevenzione
https://www.readbyqxmd.com/read/29506132/beyond-aesthetic-consequences-congestive-hepatopathy-caused-by-pectus-excavatum
#14
Robin Baudouin, Eric Levesque, René Jancovici
Pectus excavatum can lead to right ventricle compression. Although extremely rare, congestive hepatopathy should be considered when patients with severe pectus excavatum present with cardiovascular involvement.
March 1, 2018: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/29502917/inborn-errors-of-metabolism-with-hepatopathy-metabolism-defects-of-galactose-fructose-and-tyrosine
#15
REVIEW
Didem Demirbas, William J Brucker, Gerard T Berry
The liver is one of the most essential organs in metabolism and is responsible for metabolizing a wide variety of molecules from amino acids to sugars. Although it is responsible for many essential metabolic processes, it is one of the most severely affected by metabolic disease because, in many cases, it is the first to be exposed to the toxic intermediates. The metabolism of galactose, fructose, and tyrosine involve the liver and although there are systemic findings in metabolic disease involved with these substrates, severe hepatopathy is a common presenting aspect of galactosemia, hereditary fructose intolerance, and tyrosinemia type I...
April 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29500649/using-t1-mapping-in-cardiovascular-magnetic-resonance-to-assess-congestive-hepatopathy
#16
Isabel Kazour, Suraj D Serai, Stavra A Xanthakos, Robert J Fleck
The goal of this study was to assess the ability of quantitative T1 cardiovascular magnetic resonance (CMR) imaging to calculate liver extracellular volume (ECV) in patients with varying degrees of congestive hepatopathy (CH). T1 measurements and ECV calculations were performed retrospectively in three cohorts of patients: normal cardiac function, tetralogy of fallot (TOF) repair and Fontan palliation. All CMR studies included modified look-locker inversion recovery (MOLLI) T1 mapping scans performed pre- and post-injection of a gadolinium-based contrast agent (GBCA)...
March 3, 2018: Abdominal Radiology
https://www.readbyqxmd.com/read/29492237/senescence-and-cell-death-in-chronic-liver-injury-roles-and-mechanisms-underlying-hepatocarcinogenesis
#17
REVIEW
Mengchao Xiao, Wenjian Chen, Chao Wang, Yingfu Wu, Shiwei Zhu, Chuyang Zeng, Yongchao Cai, Changcheng Liu, Zhiying He
Chronic liver injury (CLI) is a complex pathological process typically characterized by progressive destruction and regeneration of liver parenchymal cells due to diverse risk factors such as alcohol abuse, drug toxicity, viral infection, and genetic metabolic disorders. When the damage to hepatocytes is mild, the liver can regenerate itself and restore to the normal state; when the damage is irreparable, hepatocytes would undergo senescence or various forms of death including apoptosis, necrosis and necroptosis...
February 2, 2018: Oncotarget
https://www.readbyqxmd.com/read/29485210/evaluation-of-potential-serum-biomarkers-of-hepatic-fibrosis-and-necroinflammatory-activity-in-dogs-with-liver-disease
#18
Chantel Raghu, Joanne Ekena, John M Cullen, Craig B Webb, Lauren A Trepanier
BACKGROUND: Serum interleukin 6 (IL-6), chemokine ligand 2 (CCL2), C-reactive protein (CRP), and the ratio of aspartate transaminase to alanine transaminase (AST:ALT) have been correlated with fibrosis and necroinflammatory activity in humans with various hepatopathies. HYPOTHESIS/OBJECTIVES: To determine whether increases in serum IL-6, CCL2, CRP, or AST:ALT were associated with moderate to severe fibrosis or necroinflammatory activity in dogs with various hepatopathies...
February 27, 2018: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/29469373/-imaging-findings-and-progress-of-schistosomal-hepatopathy
#19
Li Hang, Lu Zhi-Yan
Schistosomal hepatopathy is widespread in China, and there were more than 30 000 schistosomiasis patients with serious liver fibrosis in 2015. This article reviews the pathological manifestations, clinical presentations, imaging findings as well as imaging diagnosis value of schistosomal hepatopathy, and mainly reviews the progress of the typical imaging manifestations of schistosomal hepatopathy and new imaging methods for evaluating the liver fibrosis.
July 27, 2017: Zhongguo Xue Xi Chong Bing Fang Zhi za Zhi, Chinese Journal of Schistosomiasis Control
https://www.readbyqxmd.com/read/29441475/robot-assisted-distal-gastrectomy-with-lymph-node-dissection-for-gastric-cancer-in-a-patient-with-situs-inversus-partialis-a-case-report-with-video-file
#20
Yuki Aisu, Yoshio Kadokawa, Shigeru Kato, Daiki Yasukawa, Yusuke Kimura, Tomohide Hori
BACKGROUND: Situs inversus is a rare congenital condition that is currently classified into two types: complete situs inversus (situs inversus totalis, SIT) and partial situs inversus (situs inversus partialis, SIP). In SIP patients, some organs are inverted and others are in their expected position, and individual patient variation in organ position increases surgical difficulty. Several surgeons have performed laparoscopic or robotic surgeries in situs inversus patients, but almost all were SIT patients...
February 13, 2018: Surgical Case Reports
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