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chronic bilirubin encephalopathy

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https://www.readbyqxmd.com/read/28814249/the-neurological-sequelae-of-neonatal-hyperbilirubinemia-definitions-diagnosis-and-treatment-of-the-kernicterus-spectrum-disorders-ksds
#1
Steven Shapiro, Jean Baptiste Le Pichon, Sean M Riordan, Jon Watchkoe
Despite its lengthy history the study of benign jaundice, hyperbilirubinemia and kernicterus suffers from a lack of clarity and consistency in the key terms used to describe both the clinical and pathophysiological nature of these conditions. This has led to multiple terms being used to describe similar conditions (i.e. kernicterus and chronic bilirubin encephalopathy) or the same term being used to describe different concepts (i.e. BIND as a descriptor of the signs of bilirubin neurotoxicity, mild kernicterus, or as a scale to assess bilirubin toxicity)...
August 14, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28663066/successful-management-of-acute-liver-failure-in-italian-children-a-16-year-experience-at-a-referral-centre-for-paediatric-liver-transplantation
#2
A Di Giorgio, A Sonzogni, A Piccichè, G Alessio, E Bonanomi, M Colledan, L D'Antiga
BACKGROUND: Identifying the causes of acute liver failure (ALF) and predictors of death or liver transplantation (LTX) is crucial to decide its management. We aimed to describe features and outcome of ALF in Italian children. METHODS: Retrospective review of cases presenting between 1996-2012. ALF was defined by high transaminases, INR ≥2.0 regardless of hepatic encephalopathy (HE), no evidence of underlying chronic liver disease. RESULTS: 55 children (median age 2...
June 12, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28640024/molecular-adsorbent-recirculating-system-can-reduce-short-term-mortality-among-patients-with-acute-on-chronic-liver-failure-a-retrospective-analysis
#3
Hans U Gerth, Michele Pohlen, Gerold Thölking, Hermann Pavenstädt, Marcus Brand, Anna Hüsing-Kabar, Christian Wilms, Miriam Maschmeier, Iyad Kabar, Josep Torner, Marco Pavesi, Vicente Arroyo, Rafael Banares, Hartmut H J Schmidt
OBJECTIVES: Acute-on-chronic liver failure is associated with numerous consecutive organ failures and a high short-term mortality rate. Molecular adsorbent recirculating system therapy has demonstrated beneficial effects on the distinct symptoms, but the associated mortality data remain controversial. DESIGN: Retrospective analysis of acute-on-chronic liver failure patients receiving either standard medical treatment or standard medical treatment and molecular adsorbent recirculating system...
June 21, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28523565/blood-brain-barrier-permeability-is-exacerbated-in-experimental-model-of-hepatic-encephalopathy-via-mmp-9-activation-and-downregulation-of-tight-junction-proteins
#4
Saurabh Dhanda, Rajat Sandhir
The present study was designed to investigate the mechanisms involved in blood-brain barrier (BBB) permeability in bile duct ligation (BDL) model of chronic hepatic encephalopathy (HE). Four weeks after BDL surgery, a significant increase was observed in serum bilirubin levels. Masson trichrome staining revealed severe hepatic fibrosis in the BDL rats. (99m)Tc-mebrofenin retention was increased in the liver of BDL rats suggesting impaired hepatobiliary transport. An increase in permeability to sodium fluorescein, Evans blue, and fluorescein isothiocyanate (FITC)-dextran along with increase in water and electrolyte content was observed in brain regions of BDL rats suggesting disrupted BBB...
May 18, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28502331/risk-factors-and-outcomes-of-acute-kidney-injury-in-patients-with-hepatitis-b-virus-related-acute-on-chronic-liver-failure
#5
Wei Yuan, Yu-Yi Zhang, Zheng-Guo Zhang, Ying Zou, Hong-Zhou Lu, Zhi-Ping Qian
BACKGROUND: Acute kidney injury (AKI) is common in patients with hepatitis B virus (HBV)-related acute-on-chronic liver failure (ACLF); however, few studies concerning the risk factors and recovery patterns of renal function have been published. MATERIALS AND METHODS: A retrospective analysis of 150 patients with HBV-ACLF was performed. The occurrence, risk factors and functional recovery of AKI among patients with HBV-ACLF were investigated. RESULTS: A total of 90 patients (60%) with HBV-ACLF developed AKI...
May 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28395968/-aetiology-outcomes-and-prognostic-indicators-of-paediatric-acute-liver-failure
#6
Juan José Gilbert Pérez, Belén Jordano Moreno, Mónica Rodríguez Salas
INTRODUCTION: Acute liver failure (ALF) is a multisystem disease with severe impairment of liver function of acute onset. The Paediatric End-stage Liver Disease (PELD) score is used as a predictor of mortality in chronic liver disease, however experience is limited in ALF. OBJECTIVES: To evaluate the aetiology and outcomes of children with ALF in a Children's Liver Transplant Centre, and to investigate the validity of PELD as a prognostic indicator. PATIENTS AND METHODS: A retrospective study was conducted on patients diagnosed with ALF in our hospital from 2000 to 2013 using the criteria of the Paediatric ALF Study Group...
April 7, 2017: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/28340835/can-patients-who-develop-cerebral-death-in-fulminant-liver-failure-despite-liver-transplantation-be-previously-forseen
#7
K B Sarici, S Karakas, E Otan, V Ince, C Koc, S Koc, H Bayraktar, C Aydin, C Kayaalp, S Gungor, Y Kablan, S Yilmaz
BACKGROUND: The outcome of medical treatment is worse in fulminant liver failure (FLF) developing on acute or chronic ground. Recently, liver transplantations with the use of living and cadaveric donors have been performed in these diseases and good results obtained. In this study, we aimed to present the factors affecting the recovery of cerebral functions after liver transplantation in hepatic encephalopathy (HE) developing in FLF, to identify irreversible patient groups and to prevent unnecessary liver transplantation...
April 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28296808/waitlisted-candidates-with-polycystic-liver-disease-are-more-likely-to-be-transplanted-than-those-with-chronic-liver-failure
#8
Sahil D Doshi, Therese Bittermann, Thomas D Schiano, David Seth Goldberg
BACKGROUND: Polycystic liver disease (PCLD) is characterized by cystic replacement of the hepatic parenchyma, leading to hepatic dysfunction, portal hypertension, and hepatomegaly. Patients with liver dysfunction and/or symptomatic disease are eligible for liver transplantation. However, little is known about these patients' waitlist outcomes relative to others with chronic liver disease. METHODS: We used Organ Procurement and Transplantation Network/United Network for Organ Sharing data from February 27, 2002 to December 31, 2015 to compare waitlist outcomes of adult patients with PCLD to those with chronic liver failure (CLF) and hepatocellular carcinoma...
August 2017: Transplantation
https://www.readbyqxmd.com/read/28195876/development-and-validation-of-a-prognostic-model-for-acute-on-chronic-hepatitis-b-liver-failure
#9
Fangyuan Gao, Le Sun, Xieqiong Ye, Yao Liu, Huimin Liu, Mingfan Geng, Xiaoshu Li, Xue Yang, Yuxin Li, Rui Wang, Jialiang Chen, Gang Wan, Yuyong Jiang, Xianbo Wang
AIM: The CANONIC study proposed the Chronic Liver Failure Consortium acute-on-chronic liver failure (CLIF-C ACLF) prognostic model at the European Association for the Study of the Liver-CLIF diagnosis. This study aimed to develop and validate a prognostic model for predicting the short-term mortality of hepatitis B virus (HBV) ACLF as defined by the Asia-Pacific Association for the Study of the Liver. PATIENTS AND METHODS: A retrospective cohort of 381 HBV ACLF patients and a prospective cohort of 192 patients were included in this study...
June 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28094087/-management-of-jaundice-in-the-newborn%C3%A2-35%C3%A2-gw-from-screening-to-follow-up-after-discharge-guidelines-for-clinical-practice
#10
A Cortey, L Renesme, J Raignoux, A Bedu, C Casper, P Tourneux, P Truffert
Jaundice due to unconjugated bilirubin is an everyday condition in the neonatal period because it results from the adaptation of bilirubin metabolism at this time of life. Hyperbilirubinemia has a potential neurotoxicity and although it most often resolves spontaneously, it can lead to acute and sometimes chronic encephalopathy. The latter condition is called kernicterus and induces severe and irreversible neurological sequelae. This rare complication is still reported in all countries throughout the world even if severe hyperbilirubinemia can be prevented and critical points points of failure in jaundice management are identified...
February 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28091405/evaluation-of-helicobacter-pylori-infection-in-patients-with-chronic-hepatic-disease
#11
Ju Huang, Jun Cui
BACKGROUND: The 13C urea breath test (13C-UBT) is the gold standard for detecting Helicobacter pylori infection. H. pylori pathogenesis in patients with hepatitis B virus (HBV) and related diseases remains obscure. We used 13C-UBT to detect H. pylori infection in patients with chronic HBV infection, HBV-related cirrhosis, HBV-related hepatic carcinoma, and other chronic hepatic diseases. METHODS: A total of 131 patients with chronic hepatitis B (HB), 179 with HBV-related cirrhosis, 103 with HBV-related hepatic carcinoma, 45 with HBV-negative hepatic carcinoma, and 150 controls were tested for H...
January 20, 2017: Chinese Medical Journal
https://www.readbyqxmd.com/read/28081589/preemptive-antiviral-therapy-with-entecavir-can-reduce-acute-deterioration-of-hepatic-function-following-transarterial-chemoembolization
#12
Sun Hong Yoo, Jeong Won Jang, Jung Hyun Kwon, Seung Min Jung, Bohyun Jang, Jong Young Choi
BACKGROUND/AIMS: Hepatic damage during transarterial chemoembolization (TACE) is a critical complication in patients with hepatitis B virus (HBV)-related hepatocellular carcinoma (HCC). Apart from its role in preventing HBV reactivation, there is some evidence for the benefits of preemptive antiviral therapy in TACE. This study evaluated the effect of preemptive antiviral therapy on acute hepatic deterioration following TACE. METHODS: This retrospective observational study included a prospectively collected cohort of 108 patients with HBV-related HCC who underwent TACE between January 2007 and January 2013...
December 2016: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/28071585/newborn-bilirubin-screening-for-preventing-severe-hyperbilirubinemia-and-bilirubin-encephalopathy-a-rapid-review
#13
Kalpana Bhardwaj, Tiffany Locke, Anne Biringer, Allyson Booth, Elizabeth Kathleen Darling, Shelley Dougan, Jane Harrison, Stephen Hill, Ana Johnson, Susan Makin, Beth Potter, Thierry Lacaze-Masmonteil, Julian Little
hyperbilirubinemia [2], every newborn should be assessed for the risk of developing severe hyperbilirubinemia with the help of predischarge total serum bilirubin or transcutaneous bilirubin measurements and/or assessments of clinical risk factors. The aim of this rapid review is 1) to review the evidence for 1) predicting and preventing severe hyperbilirubinemia and bilirubin encephalopathy, 2) determining the efficacy of home/community treatments (home phototherapy) in the prevention of severe hyperbilirubinemia, and 3) non-invasive/transcutaneous methods for estimating serum bilirubin level...
January 10, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/27984174/emergency-tips-in-a-child-pugh-b-patient-when-does-the-window-of-opportunity-open-and-close
#14
REVIEW
Jonel Trebicka
Transjugular intrahepatic portosystemic shunt (TIPS) is used to treat complications of cirrhosis such as variceal bleeding and refractory ascites, but it also bears the risk of liver failure, overt hepatic encephalopathy (HE) and cardiac decompensation. Variceal bleeding may be controlled using endoscopic and medical treatment in patients with compensated cirrhosis; in decompensated patients, however, TIPS improves survival. Therefore, an early TIPS (within 72h or if later, still early after bleeding) might improve the survival of patients by preventing an inflammatory response and bacterial translocation...
October 29, 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/27784430/-prognostic-value-of-a-logistic-regression-model-based-on-keratin-18-in-patients-with-hbv-related-liver-failure
#15
Q R Gan, X Y Jiang, X Zhang, M S Chen, C Pan
Objective: To investigate the association between serum keratin 18 (K18) level and prognosis in patients with HBV-related acute-on-chronic liver failure (ACLF). Methods: A total of 120 patients who visited Department of Hepatology in Fuzhou Infectious Disease Hospital and were diagnosed with HBV-related ACLF from December 2012 to March 2014 were enrolled and followed up for 3 months. The patients were divided into death group and survival group. The serum levels of K18 fragments (M30 and M65) were measured and related laboratory data were collected to analyze the differences in M30, M65, M30/M65, and other laboratory markers...
July 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/27782896/variceal-bleeding-in-cirrhotic-patients-what-is-the-best-prognostic-score
#16
Asmaa N Mohammad, Khairy H Morsy, Moustafa A Ali
BACKGROUND/AIMS: To find the most accurate, suitable, and applicable scoring system for the prediction of outcome in cirrhotic patients with bleeding varices. MATERIALS AND METHODS: A prospective study was conducted comprising 120 cirrhotic patients with acute variceal bleeding who were admitted to Tropical Medicine and Gastroenterology Department in Sohag University Hospital, over a 1-year period (1/2015 to 1/2016). The clinical, laboratory, and endoscopic parameters were studied...
September 2016: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/27587993/a-hypothesis-for-using-pathway-genetic-load-analysis-for-understanding-complex-outcomes-in-bilirubin-encephalopathy
#17
Sean M Riordan, Douglas C Bittel, Jean-Baptiste Le Pichon, Silvia Gazzin, Claudio Tiribelli, Jon F Watchko, Richard P Wennberg, Steven M Shapiro
Genetic-based susceptibility to bilirubin neurotoxicity and chronic bilirubin encephalopathy (kernicterus) is still poorly understood. Neonatal jaundice affects 60-80% of newborns, and considerable effort goes into preventing this relatively benign condition from escalating into the development of kernicterus making the incidence of this potentially devastating condition very rare in more developed countries. The current understanding of the genetic background of kernicterus is largely comprised of mutations related to alterations of bilirubin production, elimination, or both...
2016: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/27580512/role-of-cyclooxygenase-isoforms-in-encephalopathy-of-cirrhotic-rats
#18
Hsin-Ling Ho, Shao-Jung Hsu, Fa-Yauh Lee, Hui-Chun Huang, I-Fang Hsin, Ming-Chih Hou, Shou-Dong Lee
BACKGROUND: Hepatic encephalopathy (HE) is a complex neuropsychiatric syndrome secondary to acute or chronic liver failure. However, its pathophysiology remains obscure. Recently, we found that the inhibition of cyclooxygenase by indomethacin aggravated HE in rats with thioacetamide-induced acute hepatic failure, suggesting a pivotal role of cyclooxygenase in HE. This study was aimed at surveying the roles of cyclooxygenase isoforms responsible for prostaglandins synthesis, cyclooxygenase-1 (COX1) and COX2, in cirrhotic rats with HE...
November 2016: Journal of the Chinese Medical Association: JCMA
https://www.readbyqxmd.com/read/27470622/-prognostic-analysis-of-acute-on-chronic-liver-failure-after-withdrawal-of-nucleos-t-ide-analogues-for-antiviral-treatment-of-chronic-hepatitis-b
#19
S H Ou, Y P Chen, R L Jiang, J Peng
OBJECTIVE: To investigate the prognostic factors for acute-on-chronic liver failure (ACLF) after the withdrawal of nucleos(t)ide analogues (NAs) for the antiviral treatment of chronic hepatitis B (CHB). METHODS: The clinical data of 67 hospitalized patients with ACLF after withdrawal of NAs for the antiviral treatment of CHB were analyzed retrospectively. RESULTS: The HBeAg status before initial treatment and after recurrence, course of the antiviral treatment, duration from the withdrawal of NAs to recurrence, and type of NAs before and after withdrawal were not associated with the prognosis of ACLF...
April 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/27464283/-an-unexpected-cause-of-pulmonary-hypertension
#20
Lisa Baumann, Susanne Haen, Christoph Berg, Ferruh Artunc, Reimer Riessen, Werner Spengler, Falko Fend, Michael Haap
HISTORY AND ADMISSION FINDINGS: We report on a 48-year-old man presenting with progressive hepatopathy and encephalopathy for two weeks based on a chronic hepatitis C. He takes ledipasvir and sofosbuvir (Harvoni) and ribavirin for almost 24 weeks. After admission to hospital his state deteriorated rapidly. He is directly transferred to the medical intensive care unit, where he died on day 3. INVESTIGATIONS: During the physical examination, a pronounced jaundice and significant peripheral edema were found...
July 2016: Deutsche Medizinische Wochenschrift
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