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hyperbilirubinemia adult

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https://www.readbyqxmd.com/read/28892962/crigler-najjar-syndrome-type-2-cns-type-2-an-unwonted-cause-of-jaundice-in-adults
#1
Prabhat Kumar, Gargi Sasmal, Shreya Gupta, Renu Saxena, Sudha Kohli
Crigler Najjar Syndrome (CNS) Type 2 is an uncommon genetic disorder characterised by non-haemolytic unconjugated hyperbilirubinemia. It is caused by mutations in the UGT1A1 gene which codes for the enzyme uridine diphosphate glucoronosyl transferase- 1, required for the conjugation and further excretion of bilirubin from the body. Affected individuals are usually asymptomatic apart from the jaundice and investigations reveal isolated indirect hyperbilirubinemia. It can be conveniently diagnosed by evaluating the response to phenobarbitone in terms of fall in bilirubin levels...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28877352/incidence-risk-factors-and-outcomes-of-hyperbilirubinemia-in-adult-cardiac-patients-supported-by-veno-arterial-ecmo
#2
Lin Lyu, Jingxin Yao, Guodong Gao, Cun Long, Feilong Hei, Bingyang Ji, Jinping Liu, Kun Yu, Qiang Hu, Jinxiao Hu
The aims of this study were to evaluate the incidence, risk factors, and outcomes of hyperbilirubinemia in cardiac patients with veno-arterial (VA) ECMO. Data on 89 adult patients with cardiac diseases who received VA ECMO implantation in our hospital were retrospectively reviewed. All patients were divided into the following three groups: 24 in normal group (N, total bilirubin [TBIL] ≤3 mg/dL), 30 in high bilirubin group (HB, 6 mg/dL ≥ TBIL > 3 mg/dL), and 35 in severe high bilirubin group (SHB, TBIL > 6 mg/dL)...
September 6, 2017: Artificial Organs
https://www.readbyqxmd.com/read/28859538/synergistic-effects-of-polymer-adsorbents-on-the-performance-of-bilirubin-hemoperfusion
#3
Kai-Wang Ma, Da-Hu Yao, Yang Li, Guang-da Li, Hemanth Cherukury, Ai-Hua Jing, Gao-Feng Liang
Neonatal hyperbilirubinemia (jaundice) is a common disease with high incidence. Currently, the clinical inefficiency of adult bilirubin hemoperfusion medical adsorbent is a major technical barrier for the application of hemoperfusion treatment to rescue the severe neonatal jaundice. Based on the well-known principle of synergistic effects, a series of customized bilirubin polymeric compounds, comprised of one or more of the following components (glycidyl methacrylate, sodium acrylate, methacrylic acid isooctyl, hexamethylene diamine, albumin), were designed and fabricated based on molecular design...
September 6, 2017: Journal of Biomaterials Science. Polymer Edition
https://www.readbyqxmd.com/read/28859185/salvage-chemoimmunotherapy-with-inotuzumab-ozogamicin-combined-with-mini-hyper-cvd-for-patients-with-relapsed-or-refractory-philadelphia-chromosome-negative-acute-lymphoblastic-leukemia-a-phase-2-clinical-trial
#4
Elias Jabbour, Farhad Ravandi, Partow Kebriaei, Xuelin Huang, Nicholas J Short, Deborah Thomas, Koji Sasaki, Michael Rytting, Nitin Jain, Marina Konopleva, Guillermo Garcia-Manero, Richard Champlin, David Marin, Tapan Kadia, Jorge Cortes, Zeev Estrov, Koichi Takahashi, Yogin Patel, Maria R Khouri, Jovitta Jacob, Rebecca Garris, Susan O'Brien, Hagop Kantarjian
Importance: The outcome of patients with relapsed or refractory (R/R) acute lymphoblastic leukemia (ALL) is poor. Inotuzumab ozogamicin, a CD22 monoclonal antibody bound to calicheamicin, has single-agent activity in R/R ALL. Objective: To evaluate the efficacy and safety of inotuzumab ozogamicin plus low-intensity chemotherapy in patients with R/R ALL. Design, Setting, and Participants: A single-arm, phase 2 study of adults with R/R B-cell ALL conducted at The University of Texas MD Anderson Cancer Center, Houston...
August 31, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28782473/the-importance-of-hemolysis-and-its-clinical-detection-in-neonates-with-hyperbilirubinemia
#5
Ronald J Wong, Vinod K Bhutani, David K Stevenson
Background: Hyperbilirubinemia is a benign transitional phenomenon that occurs in 60% to 80% of all term infants. The degree of hyperbilirubinemia and hence risk for developing bilirubin-induced neurologic dysfunction or BIND is dependent upon two major processes: (i) bilirubin production and its elimination. Objective: The aim of this review is to address the importance of hemolysis and its clinical detection in neonates with hyperbilirubinemia. Results: In newborns, an increased bilirubin production rate due to hemolysis is often the primary cause of hyperbilirubinemia during the first week of life...
August 7, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28715820/therapeutic-plasma-exchange-in-neonates-and-infants-successful-use-of-a-miniaturized-machine
#6
Enrico Vidal, Francesco Garzotto, Mattia Parolin, Chiara Manenti, Anna Zanin, Massimo Bellettato, Giuseppe Remuzzi, Stuart L Goldstein, Luisa Murer, Claudio Ronco
Therapeutic plasma exchange (TPE) in neonates and small infants is a treatment method at the forefront that may become a potentially life-saving procedure in a wide array of severe conditions. Indications for TPE in the pediatric population have been mainly derived from adult literature, with neonatal hyperbilirubinemia being the most notable exception. The only alternative to TPE in small pediatric patients is manual blood exchange transfusion, which, however, bears an unacceptably high risk of severe complications...
2017: Blood Purification
https://www.readbyqxmd.com/read/28708931/a-randomized-phase-2-study-of-idarubicin-and-cytarabine-with-clofarabine-or-fludarabine-in-patients-with-newly-diagnosed-acute-myeloid-leukemia
#7
Elias Jabbour, Nicholas J Short, Farhad Ravandi, Xuelin Huang, Lianchun Xiao, Guillermo Garcia-Manero, William Plunkett, Varsha Gandhi, Koji Sasaki, Naveen Pemmaraju, Naval G Daver, Gautam Borthakur, Nitin Jain, Marina Konopleva, Zeev Estrov, Tapan M Kadia, William G Wierda, Courtney D DiNardo, Mark Brandt, Susan M O'Brien, Jorge E Cortes, Hagop Kantarjian
BACKGROUND: Fludarabine and clofarabine are purine nucleoside analogues with established clinical activity in patients with acute myeloid leukemia (AML). METHODS: Herein, the authors evaluated the efficacy and safety of idarubicin and cytarabine with either clofarabine (CIA) or fludarabine (FIA) in adults with newly diagnosed AML. Adults with newly diagnosed AML who were deemed suitable for intensive chemotherapy were randomized using a Bayesian adaptive design to receive CIA (106 patients) or FIA (76 patients)...
July 14, 2017: Cancer
https://www.readbyqxmd.com/read/28617443/bilirubin-neurotoxicity-is-associated-with-proteasome-inhibition
#8
Hongbiao Huang, Mingxing Guo, Ningning Liu, Chong Zhao, Haoyu Chen, Xiaoli Wang, Siyan Liao, Ping Zhou, Yuning Liao, Xin Chen, Xiaoying Lan, Jinghong Chen, Dacai Xu, Xiaofen Li, Xianping Shi, Li Yu, Yuqiang Nie, Xuejun Wang, Chang-E Zhang, Jinbao Liu
The molecular mechanism underlying bilirubin neurotoxicity remains obscure. Ubiquitin-proteasome system-mediated proteolysis is pivotal to virtually all cellular processes and cell survival. Here we report for the first time that bilirubin at a clinically relevant elevated level impairs proteasomal function via inhibiting both the 19S proteasome-associated deubiquitinases (USP14 and UCHL5) and the chymotrypsin-like (CT-like) peptidase activity of 20S proteasomes, thereby contributing to bilirubin neurotoxicity...
June 15, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28538440/neurologic-injury-in-adults-supported-with-veno-venous-extracorporeal-membrane-oxygenation-for-respiratory-failure-findings-from-the-extracorporeal-life-support-organization-database
#9
Roberto Lorusso, Sandro Gelsomino, Orlando Parise, Michele Di Mauro, Fabio Barili, Gijs Geskes, Enrico Vizzardi, Peter T Rycus, Raf Muellenbach, Thomas Mueller, Antonio Pesenti, Alain Combes, Giles Peek, Bjorn Frenckner, Matteo Di Nardo, Justyna Swol, Jos Maessen, Ravi R Thiagarajan
OBJECTIVES: To assess in-hospital neurologic (CNS) complications in adult patients undergoing veno-venous extracorporeal membrane oxygenation for respiratory failure. DESIGN: Retrospective analysis of the Extracorporeal Life Support Organization's data registry. SETTING: Data reported to Extracorporeal Life Support Organization from 350 international extracorporeal membrane oxygenation centers during 1992-2015. PATIENTS: Adults (≥ 18 yr old) supported with veno-venous extracorporeal membrane oxygenation for respiratory failure...
August 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28507111/real-life-assessment-of-the-safety-and-effectiveness-of-the-new-tablet-and-intravenous-formulations-of-posaconazole-in-the-prophylaxis-of-invasive-fungal-infections-via-analysis-of-343-courses
#10
Frank P Tverdek, Sang Taek Heo, Samuel L Aitken, Bruno Granwehr, Dimitrios P Kontoyiannis
Posaconazole is the preferred mold-active azole for prophylaxis against invasive fungal infections (IFIs) in patients with hematological malignancy. Delayed-release tablet and intravenous formulations of posaconazole have recently become available, but clinical data are limited. We sought to examine the real-world pharmacokinetics and prophylactic effectiveness of the new formulations of posaconazole given as prophylaxis for patients with hematological malignancy. A retrospective cohort of all consecutive adult inpatients with hematological malignancy who received ≥3 days of tablet or intravenous posaconazole therapy for primary IFI prophylaxis at the M...
August 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28333690/diagnosis-and-evaluation-of-hyperbilirubinemia
#11
Jeremy I Sullivan, Don C Rockey
PURPOSE OF REVIEW: Jaundice, the physical finding associated with hyperbilirubinemia, results when the liver is unable to properly metabolize or excrete bilirubin. The purpose of this review is to examine some of the most common causes of jaundice in adults, provide insight into the diagnostic evaluation of jaundice, and review information on the outcomes of patients with jaundice. RECENT FINDINGS: An elevated level of bilirubin almost always indicates the presence of an underlying disease state...
May 2017: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/28314169/increased-mortality-in-critically-ill-patients-with-mild-or-moderate-hyperbilirubinemia
#12
Charalampos Pierrakos, Dimitrios Velissaris, Peter Felleiter, Massimo Antonelli, Philippe Vanhems, Yasser Sakr, Jean-Louis Vincent
PURPOSE: Increased bilirubin concentrations may be related to outcome, but this has not been well studied. We evaluated the relationship between total serum bilirubin levels and outcome in critically ill patients. MATERIALS AND METHODS: Data were analyzed from adult critically ill patients included in a 1-day international prevalence study after excluding those with an obvious underlying cause of hyperbilirubinemia. RESULTS: Serum bilirubin concentrations were >1 mg/dL in 2803 (31%) of 8973 patients, and these patients had higher mortality rates than patients with lower bilirubin concentrations (30% vs 21%, P<...
February 13, 2017: Journal of Critical Care
https://www.readbyqxmd.com/read/28303655/cholangiohepatitis-in-dairy-cattle-13-cases
#13
D E Gomez, E Doré, D Francoz, A Desrochers, H Pierre, G Fecteau
BACKGROUND: The signalment, clinicopathologic, bacteriological, histopathological, ultrasonographic characteristics, and the treatment and outcomes of adult cattle with cholangiohepatitis are poorly defined. ANIMALS: Thirteen Holstein cows with cholangiohepatitis. METHODS: Retrospective study of medical records of cattle admitted to the CHUV and the AVC between 1992 and 2012 and 2000 and 2012, respectively, for cattle older than 3 months of age with a histopathological diagnosis of cholangiohepatitis...
May 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28217609/acute-pancreatitis-due-to-malaria-a-case-report-of-five-patients-and-review-of-literature
#14
Kundavaram Paul Prabhakar Abhilash, Atif Shaikh Iqbal Ahmed, Sowmya Sathyendra, Ooriapadickal Cherian Abraham
Malaria is endemic in large parts of India and can cause multiorgan failure and death. Acute pancreatitis as a complication is rare and is potentially fatal. This case series describes five adult patients between 2005 and 2010 who presented with a short duration febrile illness and diagnosed to have malaria with acute pancreatitis. The mean age of the five patients with acute pancreatitis was 40.4 years and four of them were males. None of them were alcohol consumers and did not have any other risk factor for acute pancreatitis...
July 2016: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/28145671/evaluation-of-jaundice-in-adults
#15
REVIEW
Matthew V Fargo, Scott P Grogan, Aaron Saguil
Jaundice in adults can be an indicator of significant underlying disease. It is caused by elevated serum bilirubin levels in the unconjugated or conjugated form. The evaluation of jaundice relies on the history and physical examination. The initial laboratory evaluation should include fractionated bilirubin, a complete blood count, alanine transaminase, aspartate transaminase, alkaline phosphatase, ?-glutamyltransferase, prothrombin time and/or international normalized ratio, albumin, and protein. Imaging with ultrasonography or computed tomography can differentiate between extrahepatic obstructive and intrahepatic parenchymal disorders...
February 1, 2017: American Family Physician
https://www.readbyqxmd.com/read/28093842/gilbert-syndrome-in-patients-with-type-1-diabetes-prevalence-glycemic-control-and-microalbuminuria
#16
Sigal Singer, Nurit Pilpel, Orit Pinhas-Hamiel
BACKGROUND: Gilbert syndrome (GS) is a common hereditary condition, characterized by intermittent unconjugated hyperbilirubinemia. In adults with type 2 diabetes and GS, a markedly lower prevalence of nephropathy was documented, suggesting a beneficial effect of hyperbilirubinemia. We investigated the prevalence of GS among individuals with type 1 diabetes mellitus (T1DM), and the prevalence of microalbuminuria. METHODS: The prevalence of GS was assessed in 401 (204 female) patients with T1DM, median age 21...
January 17, 2017: Pediatric Diabetes
https://www.readbyqxmd.com/read/28049113/update-on-pediatric-overuse
#17
REVIEW
Eric R Coon, Paul C Young, Ricardo A Quinonez, Daniel J Morgan, Sanket S Dhruva, Alan R Schroeder
As concerns over health care-related harms and costs continue to mount, efforts to identify and combat medical overuse are needed. Although much of the recent attention has focused on health care for adults, children are also harmed by overuse. Using a structured PubMed search and manual tables of contents review, we identified important articles on pediatric overuse published in 2015. These articles were evaluated according to the quality of the methods, the magnitude of clinical effect, and the number of patients potentially affected and were categorized into overdiagnosis, overtreatment, and overutilization...
February 2017: Pediatrics
https://www.readbyqxmd.com/read/27980881/obstetric-obesity-is-associated-with-neonatal-hyperbilirubinemia-with-high-prevalence-in-native-hawaiians-and-pacific-island-women
#18
Luc Ra Rougée, Shogo J Miyagi, Abby C Collier
Obesity and pregnancy both place the liver under metabolic stress, but interactions between obstetric obesity and bilirubin metabolism have not been studied. We determined associations between obesity, maternal/neonatal bilirubin levels, and uridine 5'diphosphate-glucuronosyltransferase 1A1 (UGT1A1) enzyme that eliminates bilirubin. Adult livers were analyzed for UGT1A1 expression, activity, and bilirubin clearance by pharmacokinetic modeling. Then, matched maternal and neonatal sera (N = 450) were assayed for total and unconjugated bilirubin...
December 2016: Hawai'i Journal of Medicine & Public Health: a Journal of Asia Pacific Medicine & Public Health
https://www.readbyqxmd.com/read/27943244/genetic-variations-in-bilirubin-metabolism-genes-and-their-association-with-unconjugated-hyperbilirubinemia-in-adults
#19
Ashish S Chiddarwar, Selma Z D'Silva, Roshan B Colah, Kanjaksha Ghosh, Malay B Mukherjee
OBJECTIVE: The present study was undertaken to investigate the genotype and allele frequencies of the variants in the four bilirubin metabolism genes (UGT1A1, OATP2, HMOX1, and BLVRA) and their association with hyperbilirubinemia. MATERIAL AND METHODS: Genotyping of 17 genetic variants was performed in 115 adults with hyperbilirubinemia and 150 controls by PCR-RFLP, GeneScan analysis, and direct DNA sequencing. RESULTS: Genetic polymorphisms of the UGT1A1 promoter, specifically the T-3279G phenobarbital-responsive enhancer module and (TA)7 dinucleotide repeat, as well as the intron and coding region variants of the OATP2, HMOX1, and BLVRA genes, were significantly higher among the cases than the controls...
January 2017: Annals of Human Genetics
https://www.readbyqxmd.com/read/27704169/hepatic-expression-of-transcription-factors-affecting-developmental-regulation-of-ugt1a1-in-the-han-chinese-population
#20
Ya-Li Nie, Hang He, Jiang-Feng Li, Xiang-Guang Meng, Liang Yan, Pei Wang, Shu-Jie Wang, Hong-Zheng Bi, Li-Rong Zhang, Quan-Cheng Kan
PURPOSE: Complete or partial inactivity of UGT1A1, the unique enzyme responsible for bilirubin glucuronidation, is commonly associated with hyperbilirubinemia. We investigated the dynamic expression of UGT1A1, and that of the transcription factors (TFs) involved in its developmental regulation, during human hepatic growth in Han Chinese individuals. METHODS: Eighty-eight prenatal, pediatric, and adult liver samples were obtained from Han Chinese individuals. Quantitative real-time polymerase chain reaction was used to evaluate mRNA expression of UGT1A1 and TFs including PXR, CAR, HNF1A, HNF4A, PPARA, etc...
January 2017: European Journal of Clinical Pharmacology
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