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chronic bilirubin encephalopathy

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
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...
August 28, 2016: Journal of the Chinese Medical Association: JCMA
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
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
Jin Hee Jeong, In Sung Park, Dong Hoon Kim, Seong Chun Kim, Changwoo Kang, Soo Hoon Lee, Tae Yun Kim, Sang Bong Lee
Hepatic encephalopathy (HE) is a complication associated with worst prognosis in decompensated liver cirrhosis (LC) patients. Previous studies have identified prognostic factors for HE, and recent studies reported an association between systemic inflammatory response syndrome (SIRS) and liver disease. This study aimed to identify prognostic factors for 30-day mortality in alcoholic LC patients with HE who visited the emergency department (ED).This was a retrospective study of alcoholic LC patients with HE from January 1, 2010, to April 30, 2015...
June 2016: Medicine (Baltimore)
Cecilia A Mabogunje, Sarah M Olaifa, Bolajoko O Olusanya
Several clinical guidelines for the management of infants with severe neonatal hyperbilirubinemia recommend immediate exchange transfusion (ET) when the risk or presence of acute bilirubin encephalopathy is established in order to prevent chronic bilirubin encephalopathy or kernicterus. However, the literature is sparse concerning the interval between the time the decision for ET is made and the actual initiation of ET, especially in low- and middle-income countries (LMICs) with significant resource constraints but high rates of ET...
May 8, 2016: World Journal of Clinical Pediatrics
C Li, S Lyu, B Zhu, Z H Wan, W S Liu, L Guo, S L You, S J Xin
OBJECTIVE: To investigate the risk factors for the short-term outcome of patients with hepatitis B virus (HBV)-related acute-on-chronic liver failure (ACLF), and to establish a risk model for predicting the short-term outcome of these patients. METHODS: A total of 338 patients with HBV-related ACLF who were admitted to 30 Lod hospital of PLA hospital from January 2010 to January 2014 were enrolled, and a prospective clinical follow-up was performed for them. Multivariate logistic regression was used to determine the risk factors for short-term (12 weeks) outcome, the predictive model with logistic regression equation was established, and the predictive value of this model was evaluated...
March 20, 2016: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
Qianqian Zhang, Xiaolin Guo, Shixing Zhao, Xiaoli Pang, Yang Wang, Yujiao Zhang, Baorong Chi
The present study aimed to analyze the prognostic factors of acute-on-chronic liver failure (ACLF), with the perspective of an improved selection of optimal therapeutic schemes. A retrospective analysis was used to study 164 patients with ACLF hospitalized between 2010 and 2014 in a single center. Patients were divided into favorable and unfavorable groups, according to the treatment outcomes. General characteristics and clinical manifestations were analyzed to determine whether they would affect the prognosis of the patients with ACLF, with a particular focus on the scoring systems Child-Pugh, model for end-stage liver disease (MELD), MELD with incorporation of sodium (MELD-Na), MELD and serum sodium ratio (MESO) and integrated MELD (iMELD)...
April 2016: Experimental and Therapeutic Medicine
Muhammad Khurram, Wajeeha Qayyum, Muhammad Umar, Zain ul Abedin, Hamama Tul Bushra
BACKGROUND: Model for End Stage Liver Disease (MELD) is scoring system used for prioritization of patients waiting liver transplantation. Patients with decompensated chronic liver disease (DCLD) with higher MELD score and hepatic encephalopathy (HE) have poor outcome. We planned to note association between short-term mortality and various cut-off values of at admission MELD score in HCV related DCLD patients presenting with HE. METHODS: In this descriptive case series, 208 Hepatitis C (HCV) related chronic liver disease (CLD) patients with HE were included...
October 2015: Journal of Ayub Medical College, Abbottabad: JAMC
Seema Alam, Bikrant B Lal, Vikrant Sood, Dinesh Rawat
OBJECTIVES: The aim of the study was to assess the prevalence, profile, outcome, and predictive factors of pediatric acute-on-chronic liver failure (ACLF). METHODS: All children 3 months to 18 years satisfying the Asia Pacific Association for the Study of Liver Diseases definition of ACLF were included. Data were both extracted from records (January 2011 to December 2014) and prospectively collected (January to October 2015). Successful outcome was defined as survival with native liver at 90 days, whereas poor outcome included those who died or received liver transplantation...
October 2016: Journal of Pediatric Gastroenterology and Nutrition
Aisling Barry, Jennifer J Knox, Alice C Wei, Laura A Dawson
The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors' suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice...
February 10, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Wan Yue-Meng, Li-Hong Yang, Jin-Hui Yang, Ying Xu, Jing Yang, Gui-Bo Song
BACKGROUND AND AIMS: Various studies showed that entecavir (ETV) failed to improve the short-term survival in chronic hepatitis B (CHB) patients with severe acute exacerbation (SAE) and hepatic de-compensation or acute-on-chronic liver failure (ACLF). One study concluded that plasma exchange (PE) significantly decreased the short-term mortality of CHB patients with ACLF who were treated with lamivudine (LAM). Our study was designed to examine the effect of PE on CHB patients with ACLF who were treated with ETV...
May 2016: Hepatology International
Gaeun Kim, Young Woo Eom, Soon Koo Baik, Yeonghee Shin, Yoo Li Lim, Moon Young Kim, Sang Ok Kwon, Sei Jin Chang
Based on their ability to differentiate into multiple cell types including hepatocytes, the transplantation of mesenchymal stem cells (MSCs) has been suggested as an effective therapy for chronic liver diseases. The aim of this study was to evaluate the safety, efficacy and therapeutic effects of MSCs in patients with chronic liver disease through a literature-based examination. We performed a systematic review (SR) and meta-analysis (MA) of the literature using the Ovid-MEDLINE, EMBASE and Cochrane Library databases (up to November 2014) to identify clinical studies in which patients with liver diseases were treated with MSC therapy...
October 2015: Journal of Korean Medical Science
E T Contreras, U Giger, J L Malmberg, J M Quimby, P A Schaffer
A 7-month-old female domestic shorthair cat was diagnosed with chronic regenerative hemolytic anemia characterized by increased osmotic fragility of unknown etiology. At 13 months of age, the cat was evaluated for acute collapse. The cat was icteric with severe hyperbilirubinemia but no hematocrit changes. Severe obtundation and lateral recumbency progressed to tetraparesis and loss of proprioception in all 4 limbs, and a cerebellar or brainstem lesion was suspected. Postmortem examination revealed suppurative cholangiohepatitis and acute neuronal necrosis in the nuclei of the brainstem and cerebellum, consistent with bilirubin encephalopathy...
May 2016: Veterinary Pathology
Yu-Bao Zheng, Shi-Bin Xie, Dong-Ying Xie, Liang Peng, Zi-Ying Lei, Hong Deng, Bin-Liang Lin, Chao-Shuang Lin, Zhi-Xin Zhao, Wei-Min Ke, Zhi-Liang Gao
PURPOSE: The present study was done to establish an objective, sensitive prognostic scoring system and to determine the applicability of this model in predicting the 3-month mortality of patients with acute-on-chronic liver failure in hepatitis B (ACLFB). METHODS: We developed a novel prognostic scoring system, calculated from six clinical indices including serum total bilirubin, prothrombin activity, serum creatinine, hepatic encephalopathy, infections, and the depth of ascites from 499 patients with ACLFB...
October 2012: Hepatology International
Ankur Jindal, Ajeet S Bhadoria, Rakhi Maiwall, Shiv K Sarin
BACKGROUND & AIMS: Patients with acute-on-chronic liver failure (ACLF) have high mortality. Cirrhotics with acute kidney injury (AKI) have poor outcome but relevance of AKI and response to terlipressin in ACLF is not known. METHODS: Consecutive ACLF patients with AKI at admission were compared with those without AKI (controls) for mortality at day 7, month 1 and 3, presence of hepatic encephalopathy (HE), spontaneous bacterial peritonitis (SBP) and acute variceal bleed (AVB)...
January 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Deepak Amarapurkar, Mrudul V Dharod, Madhuri Chandnani, Rajiv Baijal, Praveen Kumar, Mayank Jain, Nikhil Patel, Praful Kamani, Sanjeev Issar, Nimish Shah, Sandeep Kulkarni, Sonali Gautam, Apurva Shah, Soham Doshi
BACKGROUND: Acute-on-chronic liver failure (ACLF), defined differently by different associations, lacks consensus on clinical profile, precipitating events and factors predicting mortality. This prospective multicentric study was conducted to determine the relevance of European Association for Study of Liver (EASL) and Asia Pacific Association for Study of Liver (APASL) definitions and to determine prognostic factors predicting the survival. METHODS: Consecutive patients over a 3-month period with any form of acute deterioration were evaluated for presence of ACLF, as defined by APASL or EASL-Chronic Liver Failure (CLIF) criteria...
May 2015: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
Sahabettin Sarı, Alpaslan Yavuz, Aabdussamet Batur, Aydın Bora, Huseyin Caksen
BACKGROUND: The term kernicterus, or bilirubin encephalopathy, is used to describe pathological bilirubin staining of the basal ganglia, brain stem, and cerebellum, and is associated with hyperbilirubinemia. Kernicterus generally occurs in untreated hyperbilirubinemia or cases where treatment is delayed. Magnetic resonance imaging (MRI)-based studies have shown characteristic findings in kernicterus. The objective of our study was to describe the role of (1)H magnetic resonance spectroscopy (MRS) in demonstrating these metabolic changes and to review conventional MRI findings of kernicterus...
2015: Polish Journal of Radiology
Tanushree Sahoo, Anu Thukral, Ramesh Agarwal, Mari Jeeva Sankar
Galactosaemia is a disorder of galactose metabolism in which raised levels of galactose and galactose-1-phosphate damage various organs. Although galactosaemia is a common metabolic liver disease in childhood, it is a rare cause of neonatal hyperbilirubinemia requiring intervention. We report an unusual case of neonatal galactosaemia that at presentation had features of acute bilirubin encephalopathy requiring exchange transfusion and at discharge had features of chronic bilirubin encephalopathy. This case report emphasises the need for timely suspicion and diagnosis of this disease for prevention of chronic morbidity...
2015: BMJ Case Reports
Binhao Zhang, Dafeng Xu, Rui Wang, Peng Zhu, Bin Mei, Gang Wei, Hua Xiao, Bixiang Zhang, Xiaoping Chen
INTRODUCTION: Hepatectomies may exacerbate chronic hepatitis B in patients with high hepatitis B viral (HBV) DNA levels, and could result in hepatic insufficiency. Antiviral treatment is effective for suppressing HBV virus loads. This study investigated whether perioperative antiviral therapy is warranted for resection of hepatocellular carcinoma (HCC) with concurrent HBV infections. METHODS: Patients with HBV-related HCC (n = 112) who underwent major liver resection were retrospectively divided into two groups based on treatment with perioperative antiviral therapy (antiviral group) (n = 72) or absence of antiviral treatment (control group) (n = 40)...
March 2015: International Journal of Surgery
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