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Transplant hepatology

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https://www.readbyqxmd.com/read/29112085/diagnosis-and-management-of-paediatric-autoimmune-liver-disease-espghan-hepatology-committee-position-statement
#1
Giorgina Mieli-Vergani, Diego Vergani, Ulrich Baumann, Piotr Czubkowski, Dominique Debray, Antal Dezsofi, Björn Fischler, Girish Gupte, Loreto Hierro, Giuseppe Indolfi, Jörg Jahnel, Françoise Smets, Henkjan J Verkade, Nedim Hadzic
Paediatric autoimmune liver disease is characterised by inflammatory liver histology, circulating autoantibodies and increased levels of IgG, in the absence of a known etiology. Three conditions have a likely autoimmune pathogenesis: autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC), and de novo AIH after liver transplantation. Two types of paediatric AIH are recognized according to seropositivity for smooth muscle and/or antinuclear antibody (SMA/ANA, AIH-1) or liver kidney microsomal type 1 and/or anti-liver cytosol type 1 antibodies (anti-LKM-1/anti-LC-1; AIH-2)...
November 3, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29098318/-wilson-s-disease-what-has-been-confirmed-in-diagnostic-and-therapy
#2
REVIEW
E-D Pfister
Wilson's disease (WD) is a rare autosomal recessive disorder characterized by abnormal copper accumulation. Presenting a broad variety of phenotypes and, thus, being a chameleon within the group of metabolic diseases, the manifold clinical symptoms of WD can include hepatologic, neurologic, and psychiatric manifestations. Early onset presentations in infancy and late-onset manifestations in adults older than 70 years of age have been described. If the typical laboratory blood test values are missing, the diagnosis of WD may be difficult and often involves a combination of different parameters...
November 2, 2017: Der Internist
https://www.readbyqxmd.com/read/29080241/next-generation-sequencing-in-pediatric-hepatology-and-liver-transplantation
#3
REVIEW
Emanuele Nicastro, Lorenzo D'Antiga
Next Generation Sequencing (NGS) has revolutionized the analysis of human genetic variations, offering a highly cost-effective way to diagnose monogenic diseases (MDs). Since nearly half of children with chronic liver disorders have a genetic cause and approximately 20% of pediatric liver transplants are performed in children with MDs, NGS offers the opportunity to significantly improve the diagnostic yield in this field. Among the NGS strategies, the use of targeted gene panels has proven useful to rapidly and reliably confirm a clinical suspicion, whereas the whole exome sequencing (WES) with variants filtering has been adopted to assist the diagnostic work up in unclear clinical scenarios...
October 28, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28983682/biliary-atresia-unity-in-diversity
#4
REVIEW
Claus Petersen
Biliary atresia (BA) is a rare disease of unknown origin and unsatisfying outcome. Single, multicenter and national evaluations of epidemiological and outcome data on BA have been periodically published over the course of decades. However, the diversity of the registered parameters and outcome measures impede comparability and cumulative analysis of these very worthwhile studies. Taking into account the fact that BA is a good example of translational research and transition of patients from pediatric surgery and hepatology to transplant surgery and hepatology in general, the interdisciplinary community should make every effort to develop a common platform upon which further activities are conducted...
December 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28977113/primary-sclerosing-cholangitis-in-children-and-adolescents
#5
Eleonora Druve Tavares Fagundes, Alexandre Rodrigues Ferreira, Caroline Caldeira Hosken, Thaís Costa Nascentes Queiroz
BACKGROUND: Primary sclerosing cholangitis is a rare disease, but its prevalence has been underestimated in children and adolescents due to broad variation in clinical presentation as well as diagnostic challenges in this life period. OBJECTIVE: To evaluate children and adolescents with primary sclerosing cholangitis and to describe their clinical, laboratorial, histopathological, and cholangiography conditions. METHODS: This is an observational descriptive research that took place from 2005 to 2016 and included all the patients seen in the Outpatient Unit for Pediatric Hepatology of Hospital das Clinicas of UFMG who had been diagnosed with primary sclerosing cholangitis before the age of 18...
October 2, 2017: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/28837507/intestinal-rehabilitation-programs-in-the-management-of-pediatric-intestinal-failure-and-short-bowel-syndrome
#6
Russell J Merritt, Valeria Cohran, Bram P Raphael, Timothy Sentongo, Diana Volpert, Brad W Warner, Praveen S Goday
Intestinal failure is a rare, debilitating condition that presents both acute and chronic medical management challenges. The condition is incompatible with life in the absence of the safe application of specialized and individualized medical therapy that includes surgery, medical equipment, nutritional products, and standard nursing care. Intestinal rehabilitation programs are best suited to provide such complex care with the goal of achieving enteral autonomy and oral feeding with or without intestinal transplantation...
November 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28755196/the-qu%C3%A3-bec-ntbc-study
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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...
October 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
#16
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
#17
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...
November 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
#18
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
#19
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
#20
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
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