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Liver failure --- Therapy

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Background: Pediatric acute liver failure (PALF) is a rare, but devastating disease. We observe a high mortality rate in our study, due to the severe multisystem organ failure. Actually, the transplantation criteria are not well defined. Objectives: This study aimed to review the experience of the pediatric intensive care unit (PICU) of St-Luc University Clinics between March 1989 and December 2016. The clinical and biological variables were collected and studied to compare our results to other studies, but also to identify mortality risk factors and to enrich our view about living liver transplantation (LT).Patients and methods : 1OO children were included, who were 46 boys and 54 girls with a median age of 59,9 months (IC 95 %: 47,2 - 72,5 months). Statistical analysis was performed. Results: 62 children met the transplantation criteria and were considered as candidates for LT; 39 received a primary liver allograft and three of them underwent living liver transplantation. The overall survival is 61%. Some etiologies of PALF are associated with a good prognostic, but although the etiology remains unknown (35%). Statistical analysis identified respiratory distress (OR= 2,9), acute renal dysfunction (OR=2,9) and lactate (OR=1,5) as mortality risk factors and associated !hem in a prognostic model (AUROC =0,83 [0,74 - 0,91] p<0,001).The median waiting time before LT was 2,0 days. If comparing periods 1989-2002 (n=46) to 2003-2016 (n=16), we observe that the children received a primary liver allograft after 2 days as the 11 children died after 6 days. Conclusion: Our results confirm the poor outcome of IHA. Many liver transplantation criteria were proposed in the literature. Based on this study, we can introduce to the criteria we used, respiratory distress, acute renal dysfunction and lactate to help clinicians in their decisions of performing LT. Finally, the living liver graft can offer a better liver quality and faster. The waiting time before LT observed in this study would like to propose living liver graft faster than before. Plenty of ethical questions emerge when the situation is critical for the child. Giving answers is nevertheless essential. Introduction : L'incidence de l'insuffisance hépatique aiguë (IHA) chez l'enfant est faible, mais son pronostic réservé. L'atteinte multi systémique qu'elle entraîne est responsable de la mortalité élevée observée dans cette étude comme par d'autres équipes. Actuellement, les critères de transplantation hépatique (TRH) ne sont pas bien définis. Objectifs : Ce travail de recherche clinique s'intéresse à l'expérience des soins intensifs pédiatriques (SIP) des Cliniques universitaires Saint-Luc (CUSL) dans la prise en charge des IHA chez l'enfant au cours d'une période de 28 ans (1989-2016). La récolte et l'analyse des données cliniques et biologiques des enfants a permis de comparer nos résultats à ceux d'autres équipes et d'identifier des facteurs de risque de mortalité. Enfin, cette étude a permis d'enrichir notre réflexion à propos de la greffe hépatique à partir d'un donneur vivant. Patients et méthodes : Entre mars 1989 et décembre 2016, 100 enfants ont été inclus dans cette étude rétrospective dont 46 garçons et 54 filles avec une moyenne d'âge de 59,9 mois (IC 95 % : 47,2 - 72,5 mois). Une analyse statistique uni-et multivariée a été réalisée. Résultats : 62 enfants présentaient les critères de TRH ; 39 d'entre eux ont bénéficié d'une greffe hépatique, dont seulement trois à partir d'un donneur vivant. La survie globale des enfants est objectivée à 61%. Certaines étiologies influencent nettement le pronostic de l'IHA, mais elle reste tout de même encore indéterminée dans 35 % des cas. L'analyse statistique identifie l'insuffisance respiratoire (OR= 2,9), l'insuffisance rénale aiguë (OR = 2,9) et le lactate (OR =1,5) comme facteurs de risque de mortalité. Leur association est retenu comme modèle pronostic fiable (AUROC =0,83 [0,74 - 0,91] p<0,001).Le délai d'attente moyen en liste d'attente est de 2,0 jours. Si l'on compare les périodes 1989- 2002 (n=46) à 2003-2016 (n=16), nous constatons que les patients greffés ont bénéficié d'un foie après 2 jours, alors que les 11 enfants sont décédés après 6 jours d'attente en liste d'attente. Conclusion: Nos résultats confirment le pronostic réservé de la pathologie par rapport à ceux observés dans la littérature et permettent d'intégrer l'insuffisance respiratoire, l'insuffisance rénale aiguë et le lactate comme facteurs de sévérité supplémentaires lors des prises de décision de mise en liste de TRH. Le délai d'attente de quelques jours observé dans cette étude doit amener à envisager le don vivant plus précocement qu'auparavant, dès le 3ème ou 4ème jour après la mise en liste d'attente.

Liver Failure, Acute --- Child --- Intensive Care Units, Pediatric

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Chronic hepatitis represents a big problem for public health with more than 400 million persons infected around the world, and this, despite the existence of a safe and effective. Chronic evolution of the disease affects especially children with rates reaching 90% during the perinatal period, while for adults, infectious episodes are generally acute (only 5% of cases lead to chronicity). Moreover, children are commonly asymptomatic during this progressive disease until adult age, leading to installation of worse complications such as cirrhosis and hepatocellular carcinoma. Treatment for children as early as possible seems to be therefore necessary. The first approved treatment for pediatric chronic hepatitis B is interferon alpha by subcutaneous injection 3 times a week and less frequent for the pegylated form. This treatment is effective for about one quarter of patients with a negativity of viral DNA and loss of Hbe antigen. Furthermore, 10% of responding patients show a loss of Hbs antigen, sign of the beginning of healing phase. Unfortunately, interferon is effective for only a little proportion of patients and induces many adverse effects included fever, flu-like symptoms of growth impairment by children. As second choice for hepatitis B therapy, guidelines recommend the use of nucleoside. However, this molecule is often compromised by emergence of resistance. A third molecule, Adefovir, could then be proposed as alternative therapy. Anyway, all treatments currently available for chronic hepatitis B remain very limited, particularly for patients described negative for Hbe antigen (for whom chances of success are reduced). Use of new promising molecules or combination therapy to reduce risks of resistance gives more hope for the next few years. However, to hope a significant and durable reduction of the infectious incidence, and perhaps finally eradication of hepatitis B, only huge prevention programs (universal vaccination of children and persons considered as at risk) could be effective L’hépatite B chronique représente un véritable fléau pour la santé publique avec plus de 400 millions de personnes touchées dans le monde et ce, malgré l’existence d’un vaccin sûre et efficace. L’évolution chronique de l’infection concerne surtout l’enfant avec des taux atteignant 90% en période périnatale alors que pour l’adulte sain, l’épisode infectieux est la plupart du temps aigu (seulement 5% évoluent vers la chronicité).De plus, les enfants présentent le plus souvent la pathologie sous sa forme asymptomatique et laissent de ce fait progresser les lésions hépatiques jusqu’à l’âge adultes, à long terme, des complications graves telles que la cirrhose ou l’hépatocarcinome peuvent faire leur apparition.Un traitement chez l’enfant dès le plus jeune âge parait ainsi justifié.Le traitement de première ligne est l’interféron alpha donné par injection sous-cutanée à raison de 3 fois par semaine ou sa forme pégylée nécessitant une fréquence d’injection moins importante. Ce traitement est efficace dans un quart des cas (patients Ag Hbe positifs) se traduisant par une négativation de l’ADN viral et perte de l’antigène Hbe. En outre, 10% des patients répondant montrent aussi une perte de l’antigène Hbs signe du début de la phase de guérison. Malheureusement, l’interféron n’est efficace que chez une faible proportion des patients et comporte de nombreux effets secondaires comme de la fièvre, un syndrome grippal ou encore une perturbation de croissance chez l’enfant. Comme traitement de deuxième intention, on recommande le recours à un analogue nucléosidique, la lamivudine. Cependant celle-ci est souvent sujette à l’émergence de résistance rendant inefficace la thérapie. Une troisième molécule, l’Adéfovir peut alors être envisagée comme alternative. Malgré tout, les traitements actuellement disponibles pour l’enfant restent encore fort limités, surtout pour les patients négatifs pour l’Ag Hbe où les chances de succès thérapeutiques sont encore plus faibles. Le recours à de nouvelles molécules prometteuses ou à des combinaisons thérapeutiques pour diminuer les risques de résistance donne néanmoins plus d’espoir pour les années à venir. Néanmoins, pour espérer une diminution significative et durable de l’incidence de l’infection, en vue d’une éventuelle éradication de la maladie seuls de vastes programmes de prévention (vaccination universelle du nourrisson, des enfants et personnes considérées comme à risque) pourraient s’avérer efficaces

Hepatitis B virus --- Hepatitis B --- Hepatitis B, Chronic --- Liver Failure, Acute --- Child

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Chronic liver failure is a frequent condition in clinical practice that encompasses all manifestations of patients with end-stage liver diseases. Chronic liver failure is a multiorgan syndrome that affects the liver, kidneys, brain, heart, lungs, adrenal glands, and vascular, coagulation, and immune systems. Chronic Liver Failure: Mechanisms and Management covers for the first time all aspects of chronic liver failure in a single book, from pathogenesis to current management. Each chapter is written by a worldwide known expert in their area and all provide the latest state-of-the-art knowledge. This volume is specifically designed to provide answers to clinical questions to all doctors dealing with patients with liver diseases, not only clinical gastroenterologists and hepatologists, but also to internists, nephrologists, intensive care physicians, and transplant surgeons.

End Stage Liver Disease. --- Liver -- Failure. --- Liver Failure. --- Liver. --- Liver Failure --- Hepatic Insufficiency --- End Stage Liver Disease --- Liver Diseases --- Digestive System Diseases --- Diseases --- Medicine --- Health & Biological Sciences --- Gastroenterology --- Liver --- Failure. --- Failure of the liver --- Hepatic failure --- Hepatic insufficiency --- Liver failure --- Medicine. --- Gastroenterology. --- Hepatology. --- Surgical transplantation. --- Medicine & Public Health. --- Transplant Surgery. --- Clinical medicine. --- Transplantation of organs, tissu. --- Medicine, Clinical --- Internal medicine --- Digestive organs --- Gastroenterology . --- Medical transplantation --- Organ transplantation --- Organ transplants --- Organs (Anatomy) --- Surgical transplantation --- Tissue transplantation --- Tissues --- Transplant surgery --- Transplantation surgery --- Transplants, Organ --- Surgery --- Preservation of organs, tissues, etc. --- Procurement of organs, tissues, etc. --- Transplantation

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In this Special Issue of the journal, advancements in the treatment of liver diseases are illustrated by international experts in the field. New treatment options for primary biliary cirrhosis and, hopefully, primary sclerosing cholangitis are discussed. Up-to-date pharmacological therapy for preventing liver cirrhosis decompensation and treating acute-on-chronic liver failure is highlighted. Furthermore, new treatments for cholangiocarcinoma, based on biological and tissue markers, will be available in the near future, aiming to surpass the current unsatisfactory results of traditional therapies. Immunotherapy has been applied to hepatocellular carcinoma (HCC). The new first-line treatment, combining atezolizumab plus bevacizumab for HCC in the intermediate and advanced stages, will allow for an increase in patient survival in the near future. Liver transplantation (LT) remains the preferred treatment for many patients with end-stage liver diseases and HCC. The selection criteria for LT in patients with HCC moved from morphological to dynamic criteria, such as those derived from the assessment of tumor responses to locoregional and/or systemic treatments before transplantation. This allowed many patients who would have been excluded from a transplantation with the old selection criteria to access one. Finally, a very interesting issue regarding new indications for liver transplantation is illustrated.

Public health & preventive medicine --- tolvaptan --- cirrhotic ascites --- survival rate --- furosemide --- primary biliary cholangitis --- autoantibodies --- ursodeoxycholic acid --- treatment response --- second line therapy --- primary biliary cholangitis (PBC) --- primary sclerosing cholangitis (PSC) --- clinical trials --- ursodeoxycholic acid (UDCA) --- Farnesoid X Receptor (FXR) agonist --- Pan-Peroxisome Proliferator-Activated Receptor (PPAR) agonists --- liver cancer --- systemic treatment --- immunotherapy --- real-world --- unresectable hepatocellular carcinoma --- cirrhosis --- decompensation --- bleeding --- varices --- survival --- infection --- alcoholic hepatitis --- acute-on-chronic liver failure --- cholangiocarcinoma --- colorectal cancer metastases --- hepatocellular carcinoma --- liver transplantation --- Milan criteria --- alpha-fetoprotein --- solid organ transplantation --- liver injury --- immunosuppressant --- SARS-CoV-2 --- humoral response --- vaccination --- portal-systemic shunt --- ammonia --- vigilance --- HBV --- HDV --- antivirals --- functional cure --- pharmacology --- acute-on-chronic liver failure (ACLF) --- liver transplantation (LT) --- decompensated cirrhosis --- portal hypertension --- ascites --- non-selective beta-blockers --- TIPS --- rifaximin --- human albumin --- statins --- targeted therapy --- effective hypovolemia --- anti-mineralocorticoids --- loop diuretics --- vaptans --- n/a