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Hepatopathy

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https://www.readbyqxmd.com/read/29205472/progressive-deafness-dystonia-due-to-serac1-mutations-a-study-of-67-cases
#1
Saskia B Wortmann, Katarzyna Iwanicka-Pronicka, Sema Kalkan Ucar, Bader Alhaddad, Moeenaldeen AlSayed, Mohammed A Al-Owain, Hamad I Al-Zaidan, Shanti Balasubramaniam, Ivo Barić, Dalal Bubshait, Alberto Burlina, John Christodoulou, Wendy K Chung, Roberto Colombo, Niklas Darin, Peter Freisinger, Maria Teresa Garcia Silva, Stephanie Grunewald, Tobias B Haack, Peter M van Hasselt, Omar Hikmat, Friederike Hörster, Pirjo Isohanni, Khushnooda Ramzan, Reka Kovacs-Nagy, Zita Krumina, Elena Martin-Hernandez, Johannes A Mayr, Patricia McClean, Linda De Meirleir, Karin Naess, Lock H Ngu, Magdalena Pajdowska, Shamima Rahman, Gillian Riordan, Lisa Riley, Benjamin Roeben, Frank Rutsch, Rene Santer, Manuel Schiff, Martine Seders, Silvia Sequeira, Wolfgang Sperl, Christian Staufner, Matthis Synofzik, Robert W Taylor, Joanna Trubicka, Konstantinos Tsiakas, Ozlem Unal, Evangeline Wassmer, Yehani Wedatilake, Toni Wolff, Holger Prokisch, Eva Morava, Ewa Pronicka, Ron A Wevers, Arjan P de Brouwer, Roeltje R Maas
OBJECTIVE: 3-MEthylGlutaconic aciduria, Dystonia-Deafness, Hepatopathy, Encephalopathy, Leigh-like syndrome (MEGDHEL) syndrome is caused by biallelic variants in SERAC1. METHODS: Multi centre study concerning the course of disease for each organ system, together with metabolic, neuroradiological and genetic findings. RESULTS: 67 individuals (39 previously unreported) from 59 families were included (age range 5 days - 33.4 years, median age 9 years)...
December 2, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/29197594/fast-and-non-invasive-serum-detection-technology-based-on-surface-enhanced-raman-spectroscopy-and-multivariate-statistical-analysis-for-liver-disease
#2
Liting Shao, Aiying Zhang, Zhen Rong, Chongwen Wang, Xiaofei Jia, Kehan Zhang, Rui Xiao, Shengqi Wang
This study explored a rapid and nondestructive liver disease detection technique based on surface-enhanced Raman spectroscopy (SERS) to realize the early diagnosis, prevention, and treatment of liver disease. SERS signals of serum were obtained from 304 normal individuals, 333 patients with hepatopathy, and 99 patients with esophageal cancer. The Raman spectra of different diseases were compared and diagnostic models of liver disease were established using orthogonal partial least squares discriminant analysis (OPLS-DA)...
November 29, 2017: Nanomedicine: Nanotechnology, Biology, and Medicine
https://www.readbyqxmd.com/read/29196222/volvulus-of-the-sigmoid-colon-is-associated-with-hypotrophy-of-the-left-lateral-segment-of-the-liver-and-the-absence-of-sigmoid-diverticulum
#3
M Barral, L Lassalle, R Dautry, C Eveno, J De Laveaucoupet, S Maitre, A Dohan, M Pocard, P Soyer
PURPOSE: To investigate the possible relationships between sigmoid diverticula, the volume of the left lateral segment of the liver and sigmoid colon volvulus. MATERIAL AND METHODS: The presence of sigmoid diverticula was analyzed in 36 patients (24 men, 12 women; mean age, 70.77±19.86 [SD] years) with sigmoid volvulus (group 1). The volumes of left lateral segment of the liver (i.e., segments 2 and 3 and further referred to as liver 1), liver 2 (i.e., segments 1, 4, 5, 6, 7 and 8), total liver volume and liver volume ratio (LVR) (i...
November 28, 2017: Diagnostic and Interventional Imaging
https://www.readbyqxmd.com/read/29189517/can-early-oral-prolonged-release-oxycodone-with-or-without-naloxone-reduce-the-duration-of-epidural-analgesia-after-cystectomy-a-three-arm-randomized-double-blind-placebo-controlled-trial
#4
Marc P Schneider, Lukas M Löffel, Marc A Furrer, Fiona C Burkhard, Bettina Kleeb, Michele Curatolo, Patrick Y Wuethrich
Thoracic epidural analgesia (TEA) enhances recovery after bowel surgery. Early postoperative prolonged-release oral formulation of oxycodone or oxycodone/naloxone is potentially useful as a second analgesic step to reduce the duration of TEA. We hypothesized that oxycodone would decrease the duration of TEA and combined with naloxone preserve gastrointestinal function. Ninety patients undergoing open cystectomy and urinary diversion were enrolled in this randomized double-blind, three-arm, parallel-group, placebo-controlled single-center trial between September 2015 and February 2017...
November 20, 2017: Pain
https://www.readbyqxmd.com/read/29173979/-dose-adaptation-of-the-drugs-used-for-hematopoietic-stem-cell-transplantation-in-patients-with-comorbidity-obesity-chronic-renal-disease-or-hepatopathy-guidelines-from-the-francophone-society-of-bone-marrow-transplantation-and-cellular-therapy-sfgm-tc
#5
Nicolas Simon, Valérie Coiteux, Bénédicte Bruno, Sophie Taque, Amandine Charbonnier, Laetitia Souchet, Laure Vincent, Ibrahim Yakoub-Agha, Yves Chalandon
In September 2016 in Lille, France, the Francophone Society of Bone Marrow Transplantation and Cellular Therapy (SFGM-TC) organized the 7th Allogeneic Stem Cell Transplantation Clinical Practices Harmonization Workshop Series. Our work group focused on chemotherapy drug dose adaptation for hematopoietic stem cell transplantation patients presenting a comorbidity. The purpose of this workshop was to provide recommendations on chemotherapy drug dose adaptation for patient populations receiving hematopoietic stem cell transplantation who also had the following comorbidities: obesity, chronic kidney disease and hepatopathy...
November 22, 2017: Bulletin du Cancer
https://www.readbyqxmd.com/read/29147765/congestive-hepatopathy
#6
Michael L Wells, Sudhakar K Venkatesh
Passive hepatic congestion may result from a variety of distinct cardiovascular conditions. Injury to the liver caused by congestion is often asymptomatic and may not be recognized clinically. Diagnosis of congestive hepatopathy is important as it has the potential to cause complications including hepatic fibrosis and development of benign and malignant liver masses. This review will summarize the pathophysiologic mechanisms of congestive hepatopathy and provide both description and examples of its multimodality imaging findings...
November 17, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/29146975/disturbance-in-plasma-metabolic-profile-in-different-types-of-human-cytomegalovirus-induced-liver-injury-in-infants
#7
Wei-Wei Li, Jin-Jun Shan, Li-Li Lin, Tong Xie, Li-Li He, Yan Yang, Shou-Chuan Wang
Human cytomegalovirus (HCMV) infection in infants is a global problem and the liver is a target organ of HCMV invasion. However, the mechanism by which HCMV causes different types of liver injury is unclear, and there are many difficulties in the differential diagnosis of HCMV infantile cholestatic hepatopathy (ICH) and extrahepatic biliary atresia (EHBA). We established a non-targeted gas chromatography-mass spectrometry metabolomics method in conjunction with orthogonal partial least squares-discriminate analysis based on 127 plasma samples from healthy controls, and patients with HCMV infantile hepatitis, HCMV ICH, and HCMV EHBA to explore the metabolite profile of different types of HCMV-induced liver injury...
November 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29138755/paraneoplastic-hepatopathy-associated-with-gastrointestinal-carcinoid
#8
Dhruv Mehta, Priyanka Chugh, Lavneet Chawla, Daniela Jodorkovsky
Paraneoplastic hepatopathy has been reported with various malignancies, most commonly with renal cell cancer. This non-metastatic hepatic dysfunction in such malignancies is known as Stauffer syndrome. We describe a 61-year-old man who presented with symptoms of bowel obstruction with marked cholestasis and high levels of alkaline phosphatase and bilirubin. Imaging revealed an unremarkable liver and a mass in the ileocecal valve with mesenteric lymphadenopathy. Biopsies were consistent with a carcinoid tumor...
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/29133088/fructose-human-serum-albumin-interaction-undergoes-numerous-biophysical-and-biochemical-changes-before-forming-ages-and-aggregates
#9
Asif Zaman, Zarina Arif, Moinuddin, Khursheed Alam
Fructose is a reducing and highly lipogenic sugar that has unique metabolic effects in the liver. Non-enzymatic fructosylation of proteins generates advanced glycation end products (AGEs). Human serum albumin (HSA) may undergo fructosylation vis-à-vis AGEs formation. High fructose consumption may lead to structurally altered and functionally compromised fructosylated-HSA-AGEs, which can cause damage to hepatocytes resulting in hepatic macro- and microvesicular steatosis. In this study, HSA was incubated with varying concentrations of fructose for 10days and the induced changes were studied...
November 10, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/29115798/acute-alcoholic-hepatitis-a-literature-review-and-proposal-of-treatment
#10
Gianni Testino, Silvia Leone
Severe acute alcoholic hepatitis (AAH) can lead to a clinical picture with a sixmonth mortality rate in more than 70% of cases. This clinical picture is characterized by: jaundice with a duration of less than three months, jaundice at the first failure event, serum bilirubin greater than 5 mg / dL, ratio AST / ALT> 2: 1, AST less than 500 IU / L, ALT <300 IU / L, neutrophil leukocytosis and a GGT increase. In addition, encephalopathy, fever, asthenia and coagulopathy may be present. Its onset may also be characterized by portalhypertensionrelated complications, particularly bleeding and hepatorenal syndrome...
November 7, 2017: Minerva Medica
https://www.readbyqxmd.com/read/29112118/nutritional-therapies-in-congenital-disorders-of-glycosylation-cdg
#11
REVIEW
Peter Witters, David Cassiman, Eva Morava
Congenital disorders of glycosylation (CDG) are a group of more than 130 inborn errors of metabolism affecting N-linked, O-linked protein and lipid-linked glycosylation. The phenotype in CDG patients includes frequent liver involvement, especially the disorders belonging to the N-linked protein glycosylation group. There are only a few treatable CDG. Mannose-Phosphate Isomerase (MPI)-CDG was the first treatable CDG by high dose mannose supplements. Recently, with the successful use of d-galactose in Phosphoglucomutase 1 (PGM1)-CDG, other CDG types have been trialed on galactose and with an increasing number of potential nutritional therapies...
November 7, 2017: Nutrients
https://www.readbyqxmd.com/read/29100896/liver-disease-and-heart-failure-back-and-forth
#12
REVIEW
Michele Correale, Nicola Tarantino, Rossella Petrucci, Lucia Tricarico, Irma Laonigro, Matteo Di Biase, Natale Daniele Brunetti
In their clinical practice, physicians can face heart diseases (chronic or acute heart failure) affecting the liver and liver diseases affecting the heart. Systemic diseases can also affect both heart and liver. Therefore, it is crucial in clinical practice to identify complex interactions between heart and liver, in order to provide the best treatment for both. In this review, we sought to summarize principal evidence explaining the mechanisms and supporting the existence of this complicate cross-talk between heart and liver...
October 31, 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/29046779/anesthetic-management-during-the-first-combined-heart-liver-transplant-performed-in-korea-a-case-report
#13
Hyejin Park, Jungchan Park, Jonghwan Lee, Gaabsoo Kim
Herein, we describe the anesthetic management during the first combined heart-liver transplant (CHLT) performed in Korea. Though CHLT is a rare procedure, accumulating evidence suggests that it is a feasible option for patients with coexisting heart and liver failure. A 45-year-old female patient presented with severe cardiac dysfunction requiring extracorporeal membrane oxygenation (ECMO) support and secondary congestive hepatopathy. The patient underwent consecutive heart and liver transplantation using extracorporeal circulatory devices-heart transplant with cardiopulmonary bypass, and liver transplant with peripheral ECMO...
October 2017: Korean Journal of Anesthesiology
https://www.readbyqxmd.com/read/29021975/effect-of-melatonin-versus-vitamin-d-as-antioxidant-and-hepatoprotective-agents-in-stz-induced-diabetic-rats
#14
Abdulmonim A Alqasim, Essam Eldin M Noureldin, Sami H Hammadi, Ghada E Esheba
BACKGROUND: Diabetes mellitus (DM) is a serious chronic disease, with multiple complications including hepatopathy associated with imbalance of the oxidative status. The purpose of this study is to observe possible protective effects of vitamin-D and melatonin on glucose profile, antioxidant-oxidant status, lipid peroxidation, and histopathological protection of the liver in streptozotocin-induced diabetic rats. METHODS: Eighty three male albino rats were divided into nine groups as follows: G1 (n = 10) Normal control rats; G2 (n = 8) were normal rats treated with melatonin only; G3 (n = 10) were normal rats treated with vitamin D only; G4 (n = 9) were diabetic rats, which received no medications; G5 (n = 8) were diabetic rat treated with insulin only; G6 (n = 10) were diabetic rats treated with melatonin only; G7 (n = 9) were diabetic rats treated with melatonin and insulin; G8 (n = 9) were diabetic rats treated with vitamin D only; G9 (n = 10) were diabetic rats treated with vitamin D and insulin...
2017: Journal of Diabetes and Metabolic Disorders
https://www.readbyqxmd.com/read/28994151/negative-hepatic-computed-tomographic-attenuation-pattern-in-a-dog-with-vacuolar-hepatopathy-and-hepatic-fat-accumulation-secondary-to-cushing-s-syndrome
#15
Andrea Carloni, Michaela Paninarova, Damiano Cavina, Mariarita Romanucci, Leonardo Della Salda, Simone Teodori, Massimo Vignoli
This report describes an unusual computed tomographic (CT) hepatic pattern, characterized by negative attenuation values (from -19.59 to -28.85 Hounsfield Units, HU) in a canine patient with severe Cushing's syndrome. Attenuation values of the splenic parenchyma (63.26 HU) and abdominal fat (-118.34 HU) were within normal limits. The negative hepatic attenuation values allowed a CT diagnosis of severe hepatic fatty infiltration that was subsequently confirmed by tissue-core biopsy and histopathological examination...
October 9, 2017: Veterinary Radiology & Ultrasound
https://www.readbyqxmd.com/read/28979694/polyene-phosphatidylcholine-inhibited-the-inflammatory-response-in-lps-stimulated-macrophages-and-ameliorated-the-adjuvant-induced-rat-arthritis
#16
Wei Pan, Wen-Ting Hao, Hui-Wen Xu, Su-Ping Qin, Xiang-Yang Li, Xiao-Mei Liu, Fen-Fen Sun, Hui Li, Ren-Xian Tang, Kui-Yang Zheng
This study sought to investigate the anti-inflammatory effect of Polyene Phosphatidylcholine (PPC), a clinical drug that is used to treat hepatopathy, on lipopolysaccharide (LPS)-stimulated macrophages and on bovine collagen II-induced arthritis (CIA) rats. In stimulated primary and Raw264.7 macrophages by LPS, PPC significantly down-regulated the relative expression of mRNA such as IL-6, TNF-α, TLR-2, TLR-4, MyD88, and NF-κB while up-regulated IL-10 and TGF-β expression. Moreover, the concentration of IL-6, TNF-α, IL-10, and TGF-β in the cultured supernatants showed the similar tendency with their mRNA alterations...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/28966228/sensory-axonal-polyneuropathy-due-to-2-4-dinitrophenol
#17
Shintaro Izumoto, Akitoshi Taniguchi, Hitoshi Mochizuki, Kazutaka Shiomi, Masamitsu Nakazato
A 24-year-old man developed subacute onset of numbness and pain in the upper and lower limbs. Physical examination demonstrated decreased pinprick sensation, but was otherwise normal. Blood and cerebrospinal fluid parameters were normal except for mild hepatic dysfunction. No data were suggestive of connective tissue disease. Nerve conduction studies demonstrated sensory neuropathy. A detailed medical interview revealed that the patient had been taking self-imported 2,4-dinitrophenol (DNP) for 2 months to decrease body weight...
October 27, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28957471/malarial-hepatopathy-a-6-year-retrospective-observational-study-from-uttarakhand-north-india
#18
Nidhi Kaeley, Sohaib Ahmad, Nadia Shirazi, Rohan Bhatia, Nowneet K Bhat, Saurabh Srivastava, Minakshi Dhar, Manoj Kumar
Background: Jaundice in malaria is multifactorial. Plasmodium falciparum causes malarial hepatopathy in around 2.5% of cases. The spectrum of hepatic dysfunction in patients with malaria varies from mild clinical and biochemical abnormalities to fulminant hepatic failure, although hepatic encephalopathy almost never occurs. We undertook this study in order to estimate the magnitude of hepatopathy in malaria, and study the associated clinical features, complications and outcome of patients...
May 1, 2017: Transactions of the Royal Society of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/28919271/-autoimmune-hepatitis-immunological-diagnosis
#19
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
#20
Fernanda de S Nascimento, Nelson A Sena, Tatiane da A Ferreira, Cibele D F Marques, Luciana R Silva, Edna Lúcia Souza
OBJECTIVES: The aims of the study were to determine the frequency of hepatobiliary disease in patients with cystic fibrosis 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 cystic fibrosis, aged between 3 months and 21 years, followed-up from January 2008 to June 2016 in a referral center. Medical records were consulted and sociodemographic, clinical and laboratory data, including hepatobiliary alterations, imaging studies, genetic studies, liver biopsies, and upper digestive endoscopies were registered...
September 7, 2017: Jornal de Pediatria
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