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Transplant hepatology

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https://www.readbyqxmd.com/read/28755196/the-qu%C3%A3-bec-ntbc-study
#1
Fernando Alvarez, Suzanne Atkinson, Manon Bouchard, Catherine Brunel-Guitton, Daniela Buhas, Jean-François Bussières, Josée Dubois, Daphna Fenyves, Paul Goodyer, Martyne Gosselin, Ugur Halac, Patrick Labbé, Rachel Laframboise, Bruno Maranda, Serge Melançon, Aicha Merouani, Grant A Mitchell, John Mitchell, Guy Parizeault, Luc Pelletier, Véronique Phan, Jean-François Turcotte
In this chapter we describe the current Quebec NTBC Study protocol. Quebec's unique characteristics have influenced the development of the protocol, including a high prevalence of hepatorenal tyrosinemia (HT1), universal newborn screening for HT1, availability of treatment with nitisinone (NTBC) and special diet, a large territory, where HT1 treatment is coordinated by a small number of centers. Screened newborns are seen within 3 weeks of birth. Patients with liver dysfunction (prolonged prothrombin time and/or international normalized ratio (INR) provide sensitive, rapidly available indicators) are treated by NTBC and special diet...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28735528/lipids-evergreen-autofluorescent-biomarkers-for-the-liver-functional-profiling
#2
Anna Cleta Croce, Giovanni Bottiroli
Depending on their chemical nature, lipids can be classified in two main categories: hydrophilic, greatly contributing to membrane composition and subcellular organelle compartmentalization, and hydrophobic, mostly triglycerides, greatly enrolled in the storage and production of energy. In both cases, some lipid molecules can be involved as signaling agents in the regulation of metabolism and protective or damaging pathways in responses to harmful stimuli. These events could affect in particular the liver, because of its central role in the maintenance of lipid homeostasis...
June 1, 2017: European Journal of Histochemistry: EJH
https://www.readbyqxmd.com/read/28705567/%C3%A2-%C3%A2-le-service-de-p%C3%A3-diatrie-%C3%A3-tait-notre-deuxi%C3%A3-me-maison%C3%A2-%C3%A2
#3
Emmanuelle Barsky
"THE PAEDIATRICS DEPARTMENT WAS OUR SECOND HOME".: At the age of a few months, Yakub was diagnosed with congenital liver fibrosis then polycystic kidney disease. He has been monitored regularly since then, and has had to be readmitted on several occasions due to complications. Now, aged 15, he is waiting for a liver transplant and is under the care of the adult hepatology department. The transition from paediatrics to the adult services was prepared, in advance, with him and his family. Yakub and his mother's account...
July 2017: Soins. Pédiatrie, Puériculture
https://www.readbyqxmd.com/read/28611264/symptoms-of-daytime-sleepiness-and-sleep-apnea-in-liver-cirrhosis-patients
#4
Abdullah Enezi, Fares Al-Jahdali, Anwar Ahmed, Nahid Shirbini, Abdullah Harbi, Baharoon Salim, Yosra Ali, Aljumah Abdulrahman, Mohd Khan, Abdullah Khaleid, Al-Jahdali Hamdan
Background/propose. Sleep disturbance and excessive daytime sleepiness (EDS) have been reported in patients with hepatic cirrhosis with no hepatic encephalopathy (HE). The objective of this study was to evaluate daytime sleepiness and risk of obstructive sleep apnea (OSA) among liver cirrhosis patients. MATERIAL AND METHODS: A cross-sectional study was conducted at King Abdulaziz Medical City (KAMC)-Riyadh over a period of six months, using a structured questionnaire that investigated: 1) Sleep patterns and daytime sleepiness using the Epworth Sleeping Scale (ESS), and 2) The risk for sleep apnea using the Berlin Questionnaire (BQ)...
August 1, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28507924/improved-outcomes-following-hepatocellular-carcinoma-hcc-diagnosis-in-patients-screened-for-hcc-in-a-large-academic-liver-center-versus-patients-identified-in-the-community
#5
Nneoma Okoronkwo, Yucai Wang, Capecomorin Pitchumoni, Baburao Koneru, Nikolaos Pyrsopoulos
Background and Aims: Hepatocellular carcinoma (HCC) is the sixth most commonly occurring cancer worldwide. Knowledge and adherence to HCC surveillance guidelines has been associated with earlier detection. We sought to evaluate characteristics and outcomes following HCC diagnosis in patients screened for HCC in a large academic liver center versus patients diagnosed and referred from the community. Methods: We reviewed the records of patients diagnosed with HCC in the liver center of an academic institution from January 1999 till December 2013...
March 28, 2017: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/28497759/long-term-challenges-and-perspectives-of-pre-adolescent-liver-disease
#6
REVIEW
Nedim Hadžić, Ulrich Baumann, Pat McKiernan, Valerie McLin, Valerio Nobili
Chronic liver disease is a growing problem that has substantial effects on public health. Many paediatric liver conditions are precursors of adult chronic liver disease, cirrhosis, and hepatocellular carcinoma. Clinical management of Wilson's disease, autoimmune liver disease, and chronic biliary disorders, such as biliary atresia, which remains the most common paediatric chronic liver disease and indication for liver transplantation, is similar in children and adults. In the past 10 or so years, paediatric hepatology has expanded into neighbouring clinical areas, such as metabolic liver diseases and systemic conditions with liver involvement...
June 2017: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28491877/nodular-regenerative-hyperplasia-a-case-of-rare-prognosis
#7
Mindy Lee, Manhal Izzy, Ashwin Akki, Kathryn Tanaka, Harmit Kalia
Introduction: Nodular regenerative hyperplasia (NRH) is a known etiology of noncirrhotic portal hypertension. Cases of biopsy-proven NRH in human immunodeficiency virus (HIV)-positive patients have been described. While these patients often have normal synthetic liver function, several reports described disease progression to liver failure. Case: We here present a 26-year-old woman with history of congenital HIV on antiretroviral therapy complicated by Pneumocystis carinii pneumonia at age 14. CD4 counts have been >300 with undetectable viral load...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28457368/eradication-of-hepatitis-c-after-liver-transplantion-consequences-for-the-organization-of-the-transplant-patient-s-follow-up
#8
G Piai, L Miglioresi, G Valente
The recurrence of the hepatitis C virus (HCV) in the liver graft has been so far the main cause of morbidity and mortality in post-transplantation patients. The treatment has significantly committed the resources of transplant hepatologists despite the poor results obtained with the past standard treatment with the use of interferon plus ribavirin. The new direct-acting antivirals (DAAs) are safe and effective even in the transplant setting, and our purpose was to check whether the eradication of HCV can make predominant other clinical problems that require different skills from those classic to hepatology...
May 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28422408/prevention-of-the-osmotic-demyelination-syndrome-after-liver-transplantation-a-multidisciplinary-perspective
#9
REVIEW
J F Crismale, K A Meliambro, S DeMaria, D B Bronster, S Florman, T D Schiano
The osmotic demyelination syndrome (ODS) is a serious neurologic condition that occurs in the setting of rapid correction of hyponatremia. It presents with protean manifestations, from encephalopathy to the "locked-in" syndrome. ODS can complicate liver transplantation (LT), and its incidence may increase with the inclusion of serum sodium as a factor in the Mayo End-Stage Liver Disease score. A comprehensive understanding of risk factors for the development of ODS in the setting of LT, along with recommendations to mitigate the risk of ODS, are necessary...
April 19, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28405349/is-there-a-standard-for-surgical-therapy-of-hepatocellular-carcinoma-in-healthy-and-cirrhotic-liver-a-comparison-of-eight-guidelines
#10
Giulia Manzini, Doris Henne-Bruns, Franz Porzsolt, Michael Kremer
BACKGROUND AND AIMS: Liver resection (LR) and transplantation are the most reliable treatments for hepatocellular carcinoma (HCC). Aim was to compare different guidelines regarding indication for resection and transplantation because of HCC with and without underlying cirrhosis. METHODS: We compared the following guidelines published after 1 January 2010: American (American Association for the Study of Liver Diseases (AASLD)), Spanish (Sociedad Espanola de Oncologia Medica (SEOM)), European (European Association for the study of liver-European Organization for Research and Treatment of Cancer (EASL-EORTC) and European Society for Medical Oncology-European Society of Digestive Oncology (ESMO-ESDO)), Asian (Asian Pacific Association for the Study of Liver (APASL)), Japanese (Japan Society of Hepatology (JSH)), Italian (Associazione Italiana Oncologia Medica (AIOM)) and German (S3) guidelines...
2017: BMJ Open Gastroenterology
https://www.readbyqxmd.com/read/28321910/the-coming-of-age-of-a-young-subspecialty-paediatric-hepatology
#11
REVIEW
Antal Németh
Paediatric hepatology dates from the 1970s and it is the youngest of the organ-specific subspecialties. As then there have been impressive achievements in the fields of anatomical, metabolic, immunological and neoplastic diseases, and the advent of modern molecular biology has resulted in a marked increase in exact diagnoses. Liver transplants provided enormous stimulus for the discipline. Due to changing morbidity patterns, the discipline faces new challenges, such as environment- and lifestyle-induced liver diseases, but different forms of chronic viral hepatitis are diminishing...
March 21, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28295581/pharmacokinetics-of-prolonged-release-tacrolimus-versus-immediate-release-tacrolimus-in-de-novo-liver-transplantation-a-randomized-phase-iii-substudy
#12
Bo-Göran Ericzon, Evaristo Varo, Pavel Trunečka, Lutz Fischer, Michele Colledan, Bruno Gridelli, Andrés Valdivieso, John O'Grady, James Dickinson, Nasrullah Undre
BACKGROUND: With the same dose of tacrolimus, lower systemic exposure on the first day of dosing has been reported for prolonged-release tacrolimus compared with immediate-release tacrolimus, prompting investigation of differing initial doses. METHODS: This substudy of a double-blind, randomized, phase III trial in de novo liver transplant recipients compared the pharmacokinetics of once-daily prolonged-release tacrolimus (initial dose: 0.2 mg/kg/day) versus twice-daily immediate-release tacrolimus (initial dose: 0...
March 12, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28176349/early-detection-and-curative-treatment-of-hepatocellular-carcinoma-a-cost-effectiveness-analysis-in-france-and-in-the-united-states
#13
COMPARATIVE STUDY
Benjamin Cadier, Julie Bulsei, Pierre Nahon, Olivier Seror, Alexis Laurent, Isabelle Rosa, Richard Layese, Charlotte Costentin, Carole Cagnot, Isabelle Durand-Zaleski, Karine Chevreul
Hepatocellular carcinoma (HCC) is the leading cause of death in patients with cirrhosis. Patients outside clinical trials seldom benefit from evidence-based monitoring. The objective of this study was to estimate the cost-effectiveness of complying with HCC screening guidelines. The economic evaluation compared surveillance of patients with cirrhosis as recommended by the guidelines ("gold-standard monitoring") to "real-life monitoring" from the health care system perspective. A Markov model described the history of the disease and treatment course including current first-line curative treatment: liver resection, radiofrequency ablation (RFA), and liver transplantation...
April 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28147426/-spontaneous-bacterial-peritonitis
#14
Ľubomír Skladaný, Silvia Kasová, Anna Purgelová, Natália Bystrianska, Svetlana Adamcová-Selčanová
AIM OF STUDY: Spontaneous bacterial peritonitis (SBP) is the most frequent infectious complication of liver cirrhosis with serious consequences. Initially, SBP is always treated with empirical, not targeted, antibiotic therapy. Since a retrospective study performed in our department showed suboptimal effectiveness (only 40 %) of empirical antibiotic therapy in accordance with the EASL guidelines, a decision was made to change the protocol. The aims of this prospective study were to determine: (1) the incidence and characteristics of SBP in real clinical practice - in a liver unit of a tertiary hospital, (2) the effectiveness of new antibiotic therapy selected based on analysis of the spectrum of pathogens and their resistance to antibiotics as identified in a retrospective cohort study on SBP carried out in our department, (3) mortality, and to compare these findings with the literature data...
December 2016: Klinická Mikrobiologie a Infekc̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28144156/update-on-the-clinical-management-of-wilson-s-disease
#15
REVIEW
Peter Hedera
Wilson's disease (WD), albeit relatively rare, is an important genetic metabolic disease because of highly effective therapies that can be lifesaving. It is a great imitator and requires a high index of suspicion for correct and timely diagnosis. Neurologic, psychiatric and hepatologic problems in WD are very nonspecific, and we discuss the most common clinical phenotypes. The diagnosis remains laboratory based, and here we review the most important challenges and pitfalls in laboratory evaluation of WD, including the emerging role of genetic testing in WD diagnosis...
2017: Application of Clinical Genetics
https://www.readbyqxmd.com/read/28073407/-the-practice-guideline-on-prophylaxis-and-treatment-of-hepatitis-b-for-liver-transplantation-in-china-2016-edition
#16
(no author information available yet)
No abstract text is available yet for this article.
December 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/28062872/hepatotoxicity-by-herbs-a-practical-review-of-a-neglected-disease
#17
REVIEW
Jean A Donet, Kay Sornmayura, Matthew Gulau, Eugene Schiff
Herbs are commonly used worldwide for the treatment of various diseases, constituting a multi-billion dollar market. Unfortunately, hepatotoxicity induced by herbs is also common. The true incidence and prevalence are not known. There is need for more strict regulations andexperimental and pre-clinical studies regarding its efficacy and safety. There is no gold standard for the diagnosis of herbs-induced liver injury (HILI) and it constitutes a diagnostic challenge for the clinician, whereestablishing causality could be cumbersome...
October 2016: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/28025746/gender-specific-differences-in-baseline-peak-and-delta-serum-creatinine-the-nacseld-experience
#18
MULTICENTER STUDY
Jacqueline G O'Leary, Florence Wong, K Rajender Reddy, Guadalupe Garcia-Tsao, Patrick S Kamath, Scott W Biggins, Michael B Fallon, Ram M Subramanian, B Maliakkal, Leroy Thacker, Jasmohan S Bajaj
BACKGROUND: Women have lower serum creatinine values than men for similar renal function. AIMS: We aimed to determine the differential effect of baseline, peak, and delta creatinine between genders on outcomes in infected hospitalized cirrhotic patients. METHODS: North American Consortium for the Study of End-Stage Liver Disease is a 15-center consortium of tertiary care hepatology centers prospectively enrolling infected cirrhotic inpatients...
March 2017: Digestive Diseases and Sciences
https://www.readbyqxmd.com/read/28012259/reduction-in-liver-transplant-wait-listing-in-the-era-of-direct-acting-antiviral-therapy
#19
MULTICENTER STUDY
Jennifer A Flemming, W Ray Kim, Carol L Brosgart, Norah A Terrault
Direct-acting antiviral (DAA) therapy, recently approved for patients with decompensated cirrhosis (DC) secondary to hepatitis C virus (HCV), is associated with improved hepatic function. We analyzed trends in liver transplant (LT) wait-listing (WL) to explore potential impact of effective medical therapy on WL registration. This is a cohort study using the Scientific Registry of Transplant Recipients database from 2003 to 2015. A total of 47,591 adults wait-listed for LT from HCV, hepatitis B virus (HBV), and nonalcoholic steatohepatitis (NASH) were identified...
March 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28012258/functional-defect-of-variants-in-the-adenosine-triphosphate-binding-sites-of-abcb4-and-their-rescue-by-the-cystic-fibrosis-transmembrane-conductance-regulator-potentiator-ivacaftor-vx-770
#20
Jean-Louis Delaunay, Alix Bruneau, Brice Hoffmann, Anne-Marie Durand-Schneider, Véronique Barbu, Emmanuel Jacquemin, Michèle Maurice, Chantal Housset, Isabelle Callebaut, Tounsia Aït-Slimane
ABCB4 (MDR3) is an adenosine triphosphate (ATP)-binding cassette (ABC) transporter expressed at the canalicular membrane of hepatocytes, where it mediates phosphatidylcholine (PC) secretion. Variations in the ABCB4 gene are responsible for several biliary diseases, including progressive familial intrahepatic cholestasis type 3 (PFIC3), a rare disease that can be lethal in the absence of liver transplantation. In this study, we investigated the effect and potential rescue of ABCB4 missense variations that reside in the highly conserved motifs of ABC transporters, involved in ATP binding...
February 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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