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hyperbilirubinemia -neonate

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https://www.readbyqxmd.com/read/28612667/all-that-glitters-yellow-is-not-gold-presentation-and-pathophysiology-of-bile-cast-nephropathy
#1
Mitchell Pitlick, Prerna Rastogi
BACKGROUND: Acute kidney injury (AKI) often manifests in patients with liver disease because of a prerenal cause and presents as acute tubular necrosis or hepatorenal syndrome. Distinguishing between these entities is important for prognosis and treatment. Some patients may develop AKI related to their underlying liver disease: for example, membranoproliferative glomerulonephritis or IgA nephropathy. Bile cast nephropathy is an often ignored differential diagnosis of AKI in the setting of obstructive jaundice...
June 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28609589/hyperbilirubinemia-the-common-denominator-of-yellow-urticaria
#2
A Combalia, X Fustà, A Guilabert, J M Mascaró, T Estrach
Rosso et al recently enlarged to ten the list of patients diagnosed with yellow urticaria (YU). These cases of YU remind clinicians that the skin, besides forming a biological barrier to protect internal organs and systems from the external environment, frequently reflects underlying internal diseases. YU has been described secondary to infective hepatitis1, liver cirrhosis (regardless of the cause) (2, 3, 4, 5, 6) , acute liver failure(7) , and metastatic disease of the liver(8) . This article is protected by copyright...
June 13, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28605161/the-use-of-therapeutic-plasma-exchange-to-reduce-serum-bilirubin-in-a-dog-with-kernicterus
#3
Tricia Tovar, Sarah Deitschel, Christine Guenther
OBJECTIVE: To describe the use of a manual method of therapeutic plasma exchange to reduce total serum bilirubin, manage kernicterus, and halt progression of neurological dysfunction in a dog with immune-mediated hemolytic anemia (IMHA). CASE SUMMARY: A 5-year-old male neutered Lhasa Apso diagnosed with IMHA developed acute onset neurologic signs consistent with kernicterus. Manual therapeutic plasma exchange was performed in an attempt to reduce total serum bilirubin...
June 12, 2017: Journal of Veterinary Emergency and Critical Care
https://www.readbyqxmd.com/read/28603997/inhibition-of-human-ugt1a1-mediated-bilirubin-glucuronidation-by-polyphenolic-acids-impact-safety-of-popular-salvianolic-acid-a-b-containing-drugs-and-herbal-products
#4
Guo Ma, Ying Zhang, Wenyan Chen, Zhifang Tang, Xiaoming Xin, Ping Yang, Xiaoqin Liu, Weimin Cai, Ming Hu
Bilirubin-related adverse reactions (ADR, e.g., jaundice and hyperbilirubinemia) induced by herbs rich in certain polyphenolic acids are widely reported. However, the causes and the mechanisms underlying these ADR are not well understood. The purpose of this article is to determine the mechanism by which certain polyphenolic acids inhibit UGT1A1-mediated bilirubin glucuronidation, leading to jaundice or hyperbilirubinemia. We investigated in vitro inhibitory effects on bilirubin glucuronidation of salvianolic acid A (SAA), salvianolic acid B (SAB), danshensu (DSS), protocatechuic aldehyde (PA), and rosmarinic acid (RA), as well as two Salvia miltiorrhiza injections (DSI and CDI) rich in polyphenolic acids...
June 22, 2017: Molecular Pharmaceutics
https://www.readbyqxmd.com/read/28601886/auna2-a-novel-type-of-non-syndromic-slowly-progressive-auditory-synaptopathy-auditory-neuropathy-with-autosomal-dominant-inheritance
#5
Ruth Lang-Roth, Eva Fischer-Krall, Cornelia Kornblum, Gudrun Nürnberg, Dieter Meschede, Ingrid Goebel, Peter Nürnberg, Dirk Beutner, Christian Kubisch, Martin Walger, Alexander E Volk
BACKGROUND: Auditory synaptopathy/neuropathy (AS/AN) is a heterogeneous disorder, which may be caused by environmental factors like postnatal hyperbilirubinemia or by genetic factors. The genetic forms are subdivided into syndromic and non-syndromic types, and show different inheritance patterns with a strong preponderance of autosomal-recessive forms. To date, only a single locus for non-syndromic autosomal-dominant AS/AN (AUNA1) has been reported in a single family, in which a non-coding DIAPH3 mutation was subsequently described as causative...
June 10, 2017: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/28597405/hyperbilirubinemia-hypertension-and-ckd-the-links
#6
REVIEW
Ibrahim Mortada
PURPOSE OF REVIEW: This review aims to highlight recent advances on the role of hyperbilirubinemia in hypertension and chronic kidney disease, with a focus on the pathophysiological mechanisms explaining the protective effects of bilirubin. An overview of pharmacologic induction of hyperbilirubinemia will also be discussed. RECENT FINDINGS: The findings depict a protective role of bilirubin in the development of hypertension and cardiovascular diseases. Hyperbilirubinemia is also negatively correlated with the development and progression of chronic kidney disease...
July 2017: Current Hypertension Reports
https://www.readbyqxmd.com/read/28592168/homozygous-mutation-on-the-%C3%AE-globin-polyadenylation-signal-in-a-tunisian-patient-with-%C3%AE-thalassemia-intermedia-and-coinheritance-of-gilbert-s-syndrome
#7
Faten Haddad, Nawel Trabelsi, Leila Chaouch, Imen Darragi, Meriem Oueslati, Imen Boudriga, Dorra Chaouachi, Wijdene El-Borgi, Raouf Hafsia, Salem Abbes, Houyem Ouragini
We report here the clinical, hematological and molecular data in a 50-year-old patient with β-thalassemia intermedia (β-TI) caused by a homozygous β(+) mutation on the β-globin gene polyadenylation (polyA) signal (AATAAA>AAAAAA). β Haplotype analysis was accomplished by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Haplotype and framework analysis showed that this mutation is associated with the [- - - - + + +] β haplotype and framework 1 (CCGCT) (FW1). This mutation was previously reported in the heterozygous state in association with the codon 9 (+TA) mutation in a β-TI patient originating from Tunisia...
June 8, 2017: Hemoglobin
https://www.readbyqxmd.com/read/28590786/liver-fibrosis-associated-with-crigler-najjar-syndrome-in-a-compound-heterozygote
#8
Cynthia R Fata, Lynette A Gillis, M Cristina Pacheco
Crigler-Najjar syndrome is a hereditary unconjugated hyperbilirubinemia. Two forms of the disease are recognized. Type I is more severe and results in kernicterus if left untreated, and Type II is less severe and responds to phenobarbital. While Crigler-Najjar syndrome is thought by many to have normal liver histology, few reports of the liver pathology exist. Herein, we present a 19-year-old patient with Crigler-Najjar who underwent liver transplantation. The liver showed marked canalicular cholestasis with portal and variable, delicate, bridging fibrosis...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28588020/enasidenib-in-mutant-idh2-relapsed-or-refractory-acute-myeloid-leukemia
#9
Eytan M Stein, Courtney D DiNardo, Daniel A Pollyea, Amir T Fathi, Gail J Roboz, Jessica K Altman, Richard M Stone, Daniel J DeAngelo, Ross L Levine, Ian W Flinn, Hagop M Kantarjian, Robert Collins, Manish R Patel, Arthur E Frankel, Anthony Stein, Mikkael A Sekeres, Ronan T Swords, Bruno C Medeiros, Christophe Willekens, Paresh Vyas, Alessandra Tosolini, Qiang Xu, Robert D Knight, Katharine E Yen, Sam Agresta, Stéphane de Botton, Martin S Tallman
Recurrent mutations in isocitrate dehydrogenase 2 (IDH2) occur in ~12% of patients with acute myeloid leukemia (AML). Mutated IDH2 proteins neomorphically synthesize 2-hydroxyglutarate resulting in DNA and histone hypermethylation, leading to blocked cellular differentiation. Enasidenib (AG-221/CC-90007) is a first-in-class, oral, selective inhibitor of mutant-IDH2 enzymes. This first-in-human, phase 1/2 study assessed the maximum tolerated dose (MTD), pharmacokinetic and pharmacodynamic profiles, safety, and clinical activity of enasidenib in patients with mutant-IDH2 advanced myeloid malignancies...
June 6, 2017: Blood
https://www.readbyqxmd.com/read/28584457/analysis-of-the-clinical-profile-in-patients-with-plasmodium-falciparum-malaria-and-its-association-with-parasite-density
#10
Praveen Mangal, Shilpa Mittal, Kamal Kachhawa, Divya Agrawal, Bhabagrahi Rath, Sanjay Kumar
BACKGROUND: Malaria remains a major health hazard in the modern world, particularly in developing countries. In Plasmodium falciparum malaria, there is a direct correlation between asexual erythrocytic stage parasite density and disease severity. Accordingly, the correlations between parasite density and various clinical presentations, severity, and outcome were examined in falciparum malaria in India. MATERIALS AND METHODS: The study was conducted in a tertiary health-care center in North India...
April 2017: Journal of Global Infectious Diseases
https://www.readbyqxmd.com/read/28583043/intracranial-stenting-for-nilotinib-treatment-associated-cerebrovascular-stenosis-in-chronic-myeloid-leukemia
#11
Tomohiko Ozaki, Hajime Nakamura, Nobuyuki Izutsu, Hiroaki Masaie, Jun Ishikawa, Manabu Kinoshita
One of the second-generation tyrosine kinase inhibitors (TKIs), nilotinib, is increasingly used for imatinib-resistant or intolerant chronic myeloid leukemia (CML). Nilotinib is considered well tolerated with few side effects including hyperglycemia, hyperbilirubinemia and elevated levels of pancreatic enzymes. However, there is growing evidence that nilotinib accelerates atherosclerosis and causes peripheral arterial occlusive disease such as stroke, transient ischemic attack (TIA) and cardiovascular diseases...
January 1, 2017: Interventional Neuroradiology
https://www.readbyqxmd.com/read/28567595/do-alpha-thalassemia-fetal-hemoglobin-and-the-ugt1a1-polymorphism-have-an-influence-on-serum-bilirubin-levels-and-cholelithiasis-in-patients-with-sickle-cell-disease
#12
Laura Alencastro de Azevedo, Joyce Bonazzoni, Sandrine Comparsi Wagner, Mariela Granero Farias, Christina M Bittar, Liane Daudt, Simone Martins de Castro
BACKGROUND: Increased destruction of erythrocytes in patients with sickle cell disease results in chronic hyperbilirubinemia and leads to the formation of gallstones. OBJECTIVES: The objective of this study was to determine the combined influence of alpha thalassemia, fetal hemoglobin, and the UGT1A1 polymorphism on serum bilirubin levels and cholelithiasis in patients with sickle cell disease. METHODS: We analyzed 72 patients treated in the outpatient hematology unit of the Clinical Hospital of Porto Alegre...
May 31, 2017: Molecular Diagnosis & Therapy
https://www.readbyqxmd.com/read/28554119/effectiveness-and-safety-of-ledipasvir-sofosbuvir%C3%A2-ribavirin-in-the-treatment-of-hcv-infection-the-real-world-harvest-study
#13
Robert Flisiak, Mariusz Łucejko, Włodzimierz Mazur, Ewa Janczewska, Hanna Berak, Krzysztof Tomasiewicz, Iwona Mozer-Lisewska, Dorota Kozielewicz, Andrzej Gietka, Katarzyna Sikorska, Marta Wawrzynowicz-Syczewska, Krzysztof Nowak, Dorota Zarębska-Michaluk, Joanna Musialik, Krzysztof Simon, Aleksander Garlicki, Robert Pleśniak, Barbara Baka-Ćwierz, Iwona Olszok, Krystyna Augustyniak, Wojciech Stolarz, Jolanta Białkowska, Anna Badurek, Anna Piekarska
BACKGROUND: To evaluate the effectiveness and safety of ledipasvir/sofosbuvir (LDV/SOF)±ribavirin (RBV) regimen in a real-world setting. METHODS: Patients received a fixed-dose combination tablet containing LDV and SOF with or without RBV, for 8, 12 or 24 weeks. Patients were assessed at baseline, end of treatment, and 12 weeks after the end of treatment. The primary effectiveness endpoint was sustained virologic response 12 weeks after the end of treatment (SVR12)...
May 26, 2017: Advances in Medical Sciences
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
#14
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...
May 22, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28531870/an-unusual-case-of-jaundice-biliary-tumor-thrombus-in-fibrolamellar-hepatocellular-carcinoma
#15
Jairo A Espinosa, Alex Merlo, Mohamed-Omar Arafeh, Gitonga Munene
BACKGROUND: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare and unique variant of hepatocellular carcinoma (HCC) whose presentation remains inadequately described. We present a resectable case of FL-HCC which involved tumor thrombus of the common bile duct. PRESENTATION: A 27 year-old male presenting with jaundice, abdominal pain, vomiting, hepatic dysfunction and hyperbilirubinemia was found to have a large liver mass and lymphadenopathy on preoperative imaging...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28523596/pharmacokinetics-and-safety-of-vismodegib-in-patients-with-advanced-solid-malignancies-and-hepatic-impairment
#16
Ghassan K Abou-Alfa, Lionel D Lewis, Patricia LoRusso, Michael Maitland, Priya Chandra, Sravanthi Cheeti, Dawn Colburn, Sarah Williams, Brian Simmons, Richard A Graham
PURPOSE: Vismodegib is a Hedgehog pathway inhibitor approved for the treatment of advanced basal cell carcinoma. Currently, the pharmacokinetics (PK) and safety of vismodegib in patients with hepatic dysfunction are unknown and are the objective of this study. METHODS: Patients with advanced solid malignancies and hepatic impairment were enrolled into one of four cohorts: normal [bilirubin (bili) < upper limit of normal (ULN)], mild (ULN < bili ≤ 1...
May 18, 2017: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/28515509/anti-nuclear-cytoplasmic-antibody-associated-vasculitis-a-probable-adverse-effect-of-sofosbuvir-treatment-in-chronic-hepatitis-c-patients
#17
Youssef K Ahmad, Salwa Tawfeek, Mohamed Sharaf-Eldin, Hassan E Elbatea, Abdelrahman Kobtan, Ferial El-Kalla, Rehab Badawi, Sherief Abd-Elsalam
Background: Egypt has the largest hepatitis C virus (HCV) epidemic worldwide. Sofosbuvir is an antiviral drug acting by inhibition of the HCV NS5B polymerase. It has shown high efficacy in combination with several other drugs and has a low reported rate of side effects. Objective: The aim of this prospective cohort study was to assess the safety of sofosbuvir-based treatment regimens used to treat chronic hepatitis C infections and to detect any side effects of sofosbuvir not previously reported. Methods: We studied treatment side effects in 3,000 patients with chronic HCV infection treated with sofosbuvir and ribavirin for 24 weeks or treated by pegylated interferon, sofosbuvir, and ribavirin triple therapy for 12 weeks...
April 2017: Hospital Pharmacy
https://www.readbyqxmd.com/read/28512537/decreased-dentin-tubules-density-and-reduced-thickness-of-peritubular-dentin-in-hyperbilirubinemia-related-green-teeth
#18
Rodrigo Neves-Silva, Fabio-Abreu Alves, Alberto Antunes, Mario-Fernando Goes, Marcelo Giannini, Maria-Dânia Tenório, José-Lécio Machado, Adriana-Franco Paes-Leme, Marcio-Ajudarte Lopes, Alan-Roger Santos-Silva
BACKGROUND: It is stated anecdotally that patients with liver diseases in childhood who develop green teeth have increased risk for rampant caries, which may be secondary to changes in dental structure. The aim of this study was to test the hypothesis that hyperbilirubinemia affects the dentin morphology of green teeth. MATERIAL AND METHODS: Sixteen primary teeth were prepared and divided into two groups (green teeth, n = 8 and control, n = 8), which were transversely fractured across the cervical third of the dental crowns; dentin was prepared and sputter-coated with gold, and examined under a scanning electron microscope...
May 2017: Journal of Clinical and Experimental Dentistry
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-analysis-of-343-courses
#19
Frank P Tverdek, Sang Taek Heo, Samuel L Aitken, Bruno Granwehr, Dimitrios P Kontoyiannis
BACKGROUND: Posaconazole is the preferred mold-active azole for prophylaxis against invasive fungal infection (IFI) in patients with hematologic malignancy (HM). 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 in patients with HM. METHODS: A retrospective cohort of all consecutive adult inpatients with HM who received ≥3 days of posaconazole as tablet or intravenous from 12/1/2013-12/31/2015 for primary IFI prophylaxis at MD Anderson Cancer center...
May 15, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28494109/oncology-drug-dosing-in-gilbert-s-syndrome-associated-with-ugt1a1-a-summary-of-the-literature
#20
Vincent H Ha, Jennifer Jupp, Roger Y Tsang
Gilbert's Syndrome (GS) is a hereditary condition that affects approximately 10% of the population. It is characterized by intermittent, unconjugated hyperbilirubinemia in the absence of hepatocellular damage and hemolysis. Although GS is often described as a benign laboratory finding, it may alter drug metabolism by decreasing the ability to conjugate drugs. Genetic polymorphisms, specifically the UGT1A1*28 allele, may reduce glucuronidation by 30%, which severely impacts the ability to metabolize certain medications...
May 11, 2017: Pharmacotherapy
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