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https://www.readbyqxmd.com/read/28318950/cholesteryl-ester-storage-disease-an-underdiagnosed-cause-of-cirrhosis-in-adults
#1
REVIEW
Mamta Pant, Kiyoko Oshima
Cholesteryl Ester Storage Disease (CESD), is a rare multisystem autosomal recessive disorder and belongs to the broad family of lysosomal storage disorders. It can present anytime from infancy and childhood to even adulthood. The clinical manifestations are generally severe in infants and with milder forms in adults. One of the prominent sites of involvement is liver. Due to low awareness of this condition among physicians including surgical pathologists, majority of the liver biopsies, especially from the adults are often misdiagnosed as non-alcoholic fatty liver disease/non-alcoholic steatohepatitis or cryptogenic cirrhosis...
February 9, 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28318147/effects-of-alfapump-system-on-kidney-and-circulatory-function-in-patients-with-cirrhosis-and-refractory-ascites
#2
Elsa Solà, Santiago Sanchez-Cabús, Ezequiel Rodriguez, Chiara Elia, Raquel Cela, Rebeca Moreira, Elisa Pose, Jordi Sánchez-Delgado, Nuria Cañete, Manuel Morales-Ruiz, Francisco Campos, Jaume Balust, Mónica Guevara, Juan Carlos García-Valdecasas, Pere Ginès
The alfapump system has been proposed as a new treatment for the management of refractory ascites. The system removes ascites from the peritoneal cavity to urinary bladder, producing a continuous low-volume paracentesis. AIM: To investigate the effects of treatment with the alfapump system on kidney and circulatory function in patients with cirrhosis and refractory ascites. METHODS: Prospective study including 10 patients with cirrhosis and refractory ascites. Primary outcomes were changes in glomerular filtration rate (GFR), as assessed by isotopic techniques, and changes in circulatory function assessed by arterial pressure, cardiac output and activity of vasoconstrictor systems...
March 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28317409/ombitasvir-and-paritaprevir-boosted-with-ritonavir-and-combined-with-dasabuvir-for-chronic-hepatitis-c
#3
Robert Flisiak, Marta Flisiak-Jackiewicz
Hepatitis C is a leading cause of cirrhosis and hepatocellular carcinoma responsible for almost 700,000 deaths worldwide annually. Until 2014, management of HCV infections was based on interferon alfa containing regimens, with efficacy of 40-70% and a high adverse event rate. Interferon-free therapeutic options improved sustained viral response (SVR) rate to >90% and safety profile to placebo-like levels. Areas covered: This article describes all-oral regimen consisting of three direct acting antivirals (DAA) - ombitasvir (OBV), paritaprevir (PTV) and dasabuvir (DSV), which in clinical practice is boosted with ritonavir (r) and sometimes with ribavirin (RBV)...
March 20, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28317045/fibrolamellar-variant-of-hepatocellular-carcinoma-presenting-during-pregnancy-management-dilemmas
#4
Nagalapuram Vishnu, Aditya V Kulkarni, Sreenivasan Vidhyalakshmi, Swaminathan Sambandam, Prerna Garg, Venkatakrishnan LeelaKrishnan, Krishnaveni Janarthan, Gursharan Singh, Maninder Kaur, T V Chitra, Biku Joseph John
The Fibrolamellar variant of Hepatocellular Carcinoma (FLHCC) is a rare form of liver cancer that presents in the 3(rd) decade of life, is rarely associated with cirrhosis or chronic Hepatitis B/C virus infection, and usually presents with normal serum alpha-fetoprotein (AFP) levels. FLHCC presenting during pregnancy is extremely rare, with only 4 cases reported. We present a case of FLHCC in pregnancy and discuss the dilemmas in management. A 26 year-old primigravida, 26 weeks of gestation presented with a month's history of obstructive jaundice secondary...
February 2017: Ann Hepatobiliary Pancreat Surg
https://www.readbyqxmd.com/read/28306680/anaesthesia-in-patients-with-liver-disease
#5
Malgorzata H Starczewska, Wint Mon, Peter Shirley
PURPOSE OF REVIEW: The purpose of this review is to summarize the most recent up to date research data and recommendations regarding anaesthetic management of patients with liver disease undergoing surgery. The incidence of chronic liver disease (CLD) continues to rise and perioperative mortality and morbidity remains unacceptably high in this group. Meticulous preoperative assessment and carefully planned anaesthetic management are vital in improving outcomes in patients with liver disease undergoing surgery...
March 16, 2017: Current Opinion in Anaesthesiology
https://www.readbyqxmd.com/read/28293379/clinical-features-histology-and-histogenesis-of-combined-hepatocellular-cholangiocarcinoma
#6
REVIEW
Shweta Gera, Mark Ettel, Gabriel Acosta-Gonzalez, Ruliang Xu
Combined hepatocellular-cholangiocarcinoma (CHC) is a rare tumor with poor prognosis, with incidence ranging from 1.0%-4.7% of all primary hepatic tumors. This entity will be soon renamed as hepato-cholangiocarcinoma. The known risk factors for hepatocellular carcinoma (HCC) have been implicated for CHC including viral hepatitis and cirrhosis. It is difficult to diagnose this tumor pre-operatively. The predominant histologic component within the tumor largely determines the predominant radiographic features making it a difficult distinction...
February 28, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28293378/hepatorenal-syndrome-update-on-diagnosis-and-therapy
#7
EDITORIAL
Juan G Acevedo, Matthew E Cramp
Hepatorenal syndrome (HRS) is a manifestation of extreme circulatory dysfunction and entails high morbidity and mortality. A new definition has been recently recommended by the International Club of Ascites, according to which HRS diagnosis relies in serum creatinine changes instead that on a fixed high value. Moreover, new data on urinary biomarkers has been recently published. In this sense, the use of urinary neutrophil gelatinase-associated lipocalin seems useful to identify patients with acute tubular necrosis and should be employed in the diagnostic algorithm...
February 28, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28288507/anticoagulation-in-cirrhosis-a-new-paradigm
#8
Filippo Leonardi, Nicola De Maria, Erica Villa
The liver plays a crucial role in coagulation cascade. Global hemostatic process is profoundly influenced by the presence of liver disease and its complications. Patients with cirrhosis have impaired synthesis of most of the factors involved in coagulation and fibrinolysis process due to a reduced liver function and altered platelet count secondary to portal hypertension. Altered routine tests and thrombocytopenia were considered in the past as associated with increased risk of bleeding. These concepts explain both the routine use of plasma and/or platelets transfusion in patients with liver cirrhosis, especially before invasive procedures, and why these patients were considered "auto-anticoagulated"...
March 14, 2017: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/28286557/covered-versus-bare-stents-for-transjugular-intrahepatic-portosystemic-shunt-an-updated-meta-analysis-of-randomized-controlled-trials
#9
Xingshun Qi, Yulong Tian, Wei Zhang, Zhiping Yang, Xiaozhong Guo
BACKGROUND: Transjugular intrahepatic portosystemic shunt (TIPS) is a standard treatment option for the management of portal hypertension in liver cirrhosis. Since the introduction of covered stents, shunt patency has been greatly improved. However, it remains uncertain about whether covered stents could improve survival. A meta-analysis of randomized controlled trials has been performed to compare the outcomes of covered versus bare stents for TIPS. METHODS: PubMed, EMBASE, and Cochrane Library databases were searched to identify the relevant randomized controlled trials...
January 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/28285495/pharmacological-interventions-for-acute-hepatitis-c-infection-an-attempted-network-meta-analysis
#10
REVIEW
Maria Kalafateli, Elena Buzzetti, Douglas Thorburn, Brian R Davidson, Emmanuel Tsochatzis, Kurinchi Selvan Gurusamy
BACKGROUND: Hepatitis C virus (HCV) is a single-stranded RNA (ribonucleic acid) virus that has the potential to cause inflammation of the liver. The traditional definition of acute HCV infection is the first six months following infection with the virus. Another commonly used definition of acute HCV infection is the absence of HCV antibody and subsequent seroconversion (presence of HCV antibody in a person who was previously negative for HCV antibody). Approximately 40% to 95% of people with acute HCV infection develop chronic HCV infection, that is, have persistent HCV RNA in their blood...
March 13, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28281514/-ultrasound-elastography-of-the-liver-for-assessing-the-risk-of-complications-of-its-cirrhosis-of-different-etiologies
#11
N Yu Stukova, Yu A Kucheryavyi, E A Maevskaya, I V Maev
AIM: To study the results of dynamic liver ultrasound elastography (LUE) in assessing the risk of complications of liver cirrhosis (LC) of different etiologies and to elaborate a monitoring program for estimation of the predictive value of elastography in patients with LC. SUBJECTS AND METHODS: A total of 194 patients with LC of different etiologies, including 56 patients with alcoholic cirrhosis, 48 with LC and an outcome of nonalcoholic fatty liver disease, 53 with LC and an outcome of chronic hepatitis C, 23 with LC and an outcome of chronic hepatitis B, and 14 with an outcome of coinfection with hepatitis B and D viruses, were examined...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28281295/management-of-people-with-intermediate-stage-hepatocellular-carcinoma-an-attempted-network-meta-analysis
#12
REVIEW
Davide Roccarina, Avik Majumdar, Douglas Thorburn, Brian R Davidson, Emmanuel Tsochatzis, Kurinchi Selvan Gurusamy
BACKGROUND: There is significant uncertainty in the treatment of intermediate-stage hepatocellular carcinoma which is defined by the Barcelona Clinic Liver Cancer (BCLC) as hepatocellular carcinoma stage B with large, multi-nodular, Child-Pugh status A to B, performance status 0 to 2, and without vascular occlusion or extrahepatic disease. OBJECTIVES: To assess the comparative benefits and harms of different interventions used in the treatment of intermediate-stage hepatocellular carcinoma (BCLC stage B) through a network meta-analysis and to generate rankings of the available interventions according to their safety and efficacy...
March 10, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28277807/magnetic-resonance-imaging-and-transient-elastography-in-the-management-of-nonalcoholic-fatty-liver-disease-nafld
#13
Ma Ai Thanda Han, Rola Saouaf, Walid Ayoub, Tsuyoshi Todo, Edward Mena, Mazen Noureddin
Nonalcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease and cirrhosis worldwide and the second most common cause of liver transplantation in major medical centers. Because liver steatosis and fibrosis severity are related to disease morbidity and mortality, the extent of disease, and disease progression, they need to be assessed and monitored. In addition, innovation with new drug developments requires disease staging and monitoring in both phase 2 and 3 clinical trials. Currently, disease assessment in both clinical practice and research is mostly performed by liver biopsy, an invasive, procedure with risks...
March 9, 2017: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/28274107/pathophysiology-and-management-of-alcoholic-liver-disease-update-2016
#14
REVIEW
Felix Stickel, Christian Datz, Jochen Hampe, Ramon Bataller
Alcoholic liver disease (ALD) is a leading cause of cirrhosis, liver cancer, and acute and chronic liver failure and as such causes significant morbidity and mortality. While alcohol consumption is slightly decreasing in several European countries, it is rising in others and remains high in many countries around the world. The pathophysiology of ALD is still incompletely understood but relates largely to the direct toxic effects of alcohol and its main intermediate, acetaldehyde. Recently, novel putative mechanisms have been identified in systematic scans covering the entire human genome and raise new hypotheses on previously unknown pathways...
March 15, 2017: Gut and Liver
https://www.readbyqxmd.com/read/28273410/cost-effectiveness-analysis-of-ledipasvir-sofosbuvir-in-patients-with-chronic-hepatitis-c-treatment-of-patients-with-absence-or-mild-fibrosis-compared-to-patients-with-advanced-fibrosis
#15
M Buti, R Domínguez-Hernández, I Oyagüez, M A Casado, R Esteban
OBJECTIVE: To evaluate the cost-effectiveness of Ledipasvir/Sofosbuvir (LDV/SOF) in treatment-naïve patients with chronic hepatitis C (CHC) genotype 1 (GT1) in the absence or mild fibrosis (F0-F1) versus advanced fibrosis (F2-F4), from the perspective of the Spanish Health System. METHODS: A Markov model was developed to simulate disease progression, estimating costs and outcomes [life years gained, (LYG), and quality adjusted life years, (QALY)] derived from starting with LDV/SOF in patients with F0-F1 compared with F2-F4...
March 8, 2017: Journal of Viral Hepatitis
https://www.readbyqxmd.com/read/28267072/an-attempt-to-determine-the-prevalence-of-two-inborn-errors-of-primary-bile-acid-synthesis-results-of-a-european-survey
#16
Jörg Jahnel, Evelyn Zöhrer, Björn Fischler, Lorenzo D'Antiga, Dominique Debray, Antal Dezsofi, Dorothea Haas, Nedim Hadzic, Emmanuel Jacquemin, Thierry Lamireau, Giuseppe Maggiore, Pat J McKiernan, Pier Luigi Calvo, Henkjan J Verkade, Loreto Hierro, Valerie McLin, Ulrich Baumann, Emmanuel Gonzales
OBJECTIVE: Inborn errors of primary bile acid (BA) synthesis are genetic cholestatic disorders leading to accumulation of atypical BA with deficiency of normal BA. Unless treated with primary BA, chronic liver disease usually progresses to cirrhosis and liver failure before adulthood. We sought to determine the prevalence of two common disorders, 3β-hydroxy-Δ-C27-steroid dehydrogenase (3β-HSD) and Δ-3-oxosteroid-5β-reductase (Δ-3-oxoR) deficiencies and to describe current diagnostic and treatment strategies among different European paediatric hepatology centres...
March 6, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28255371/non-invasive-imaging-techniques-in-assessing-non-alcoholic-fatty-liver-disease-a-current-status-of-available-methods
#17
REVIEW
A M Lăpădat, I R Jianu, B S Ungureanu, L M Florescu, D I Gheonea, S Sovaila, I A Gheonea
Non-alcoholic fatty liver disease (NAFLD) is an ailment affecting and increasing a number of people worldwide diagnosed via non-invasive imaging techniques, at a time when a minimum harm caused by medical procedures is rightfully emphasized, more sought after, than ever before. Liver steatosis should not be taken lightly even if its evolution is largely benign as it has the potential to develop into non-alcoholic steatohepatitis (NASH) or even more concerning, hepatic cirrhosis, and hepatocellular carcinoma (HCC)...
January 2017: Journal of Medicine and Life
https://www.readbyqxmd.com/read/28251119/fibrosis-assessment-in-patients-with-chronic-hepatitis-b-virus-hbv-infection
#18
REVIEW
Pathik Parikh, John D Ryan, Emmanuel A Tsochatzis
Chronic hepatitis B virus (HBV) infection is a major cause of liver morbidity and mortality worldwide. While a proportion of the 250 million individuals chronically infected with HBV will not come to significant harm or require therapy, many others risk developing complications of the end-stage liver disease such as decompensated cirrhosis and hepatocellular carcinoma (HCC), without intervention. Due to the complex natural history of HBV infection, patients require an expert assessment to interpret biochemistry, viral serology and appropriately stage the disease, and to initiate monitoring and/or therapy where indicated...
February 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28251116/pediatric-hepatitis-b-treatment
#19
REVIEW
Haruki Komatsu, Ayano Inui, Tomoo Fujisawa
Although the introduction of hepatitis B vaccine has been contributing to the reduction in the prevalence of hepatitis B virus (HBV) carriers worldwide, the treatment of children with chronic HBV infection is a challenge to be addressed. HBeAg seroconversion, which induces low replication of HBV, is widely accepted as the first goal of antiviral treatment in children with chronic hepatitis B. However, spontaneous HBeAg seroconversion is highly expected in children with chronic HBV infection. Therefore, the identification of children who need antiviral treatment to induce HBeAg seroconversion is essential in the management of chronic HBV infection...
February 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28251010/neonatal-liver-failure-and-congenital-cirrhosis-due-to-gestational-alloimmune-liver-disease-a-case-report-and-literature-review
#20
Carolina Roos Mariano da Rocha, Renata Rostirola Guedes, Carlos Oscar Kieling, Marina Rossato Adami, Carlos Thadeu Schmidt Cerski, Sandra Maria Gonçalves Vieira
Neonatal liver failure (NLF) is a major cause of neonatal morbidity and mortality, presenting as acute liver failure and/or congenital cirrhosis. Many affected patients show antenatal signs of fetal injury. There are several causes of NLF and early diagnosis is mandatory to elucidate the etiology and determine a specific treatment or the best management strategy. Gestational alloimmune liver disease associated with neonatal hemochromatosis (GALD-NH) is a rare but potentially treatable cause of NLF. It should be considered in any neonate with fetal signs of disease and postnatal signs of liver failure with no other identifiable causes...
2017: Case Reports in Pediatrics
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