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unconjugated hyperbilirubinemia

Bilal Gondal, Andrew Aronsohn
Jaundice is a clinical manifestation of disorders of underlying bilirubin metabolism, hepatocellular dysfunction, or biliary obstruction. As clinical presentations of yellowing of eyes or skin can be somewhat nonspecific for the underlying etiology of disease, a stepwise approach to evaluation is necessary for accurate diagnosis and effective treatment plan. In this review, we discuss underlying mechanisms of cholestasis and jaundice as well as laboratory and imaging modalities needed to evaluate a patient presenting with hyperbilirubinemia...
December 2016: Seminars in Interventional Radiology
T Malla, B Sathian, K Karmacharya Malla, S Adhikari
Background Urine culture is usually not a part of work-up for neonatal unconjugated hyperbilirubinemia; hence its prevalence remains unknown. Objective This study was done to determine the incidence of urinary tract infection (UTI) in asymptomatic newborns with prolonged unconjugated hyperbilirubinemia and to evaluate which other laboratory parameters are associated with UTIs. Method A prospective observational study where jaundiced newborns otherwise clinically well, were evaluated for UTI. The study was carried out in neonatal intensive care unit of Manipal Teaching Hospital, Pokhara from June 2012 -April 2013...
January 2016: Kathmandu University Medical Journal (KUMJ)
Zsuzsanna Polgar, Yanfeng Li, Xia Li Wang, Chandan Guha, Namita Roy-Chowdhury, Jayanta Roy-Chowdhury
Liver transplantation has been established as a curative therapy for acute and chronic liver failure, as well as liver-based inherited metabolic diseases. Because of the complexity of organ transplantation and the worldwide shortage of donor organs, hepatocyte transplantation is being developed as a bridging therapy until donor organs become available, or for amelioration of inherited liver-based diseases. The Gunn rat is a molecular and metabolic model of Crigler-Najjar syndrome type 1, which is characterized by lifelong unconjugated hyperbilirubinemia due to the lack of uridinediphosphoglucuronate glucuronosyltransferase-1 (UGT1A1)-mediated bilirubin glucuronidation...
2017: Methods in Molecular Biology
Ricardo Zwirtes, Premkumar Narasimhan, Megan M Wind-Rotolo, Dong Xu, Matthew W Hruska, Narendra Kishnani, Elizabeth M Colston, Subasree Srinivasan
The phase 2b EMERGE study compared the efficacy/safety of peginterferon lambda-1a (Lambda) and peginterferon alfa-2a (Alfa), both with ribavirin (RBV), for treatment of chronic hepatitis C virus (HCV) infection. A key safety finding was a higher frequency of hyperbilirubinemia with Lambda/RBV versus Alfa/RBV. To characterize mechanisms of hyperbilirubinemia associated with Lambda/RBV, we conducted a retrospective analysis of safety data from the HCV genotype 1 and genotype 4 cohort of the EMERGE study. Subjects were randomized to once-weekly Lambda (120/180/240 μg) or Alfa (180 μg), with daily RBV, for 48 weeks...
October 6, 2016: Journal of Interferon & Cytokine Research
Di Yan, Christian Domes, Robert Domes, Timea Frosch, Jürgen Popp, Mathias W Pletz, Torsten Frosch
Fiber enhanced resonance Raman spectroscopy (FERS) is introduced for chemically selective and ultrasensitive analysis of the biomolecules hematin, hemoglobin, biliverdin, and bilirubin. The abilities for analyzing whole intact, oxygenated erythrocytes are proven, demonstrating the potential for the diagnosis of red blood cell related diseases, such as different types of anemia and hemolytic disorders. The optical fiber enables an efficient light-guiding within a miniaturized sample volume of only a few micro-liters and provides a tremendously improved analytical sensitivity (LODs of 0...
October 17, 2016: Analyst
Ziba Mosayebi, Maral Rahmani, Shahin Behjati Ardakani, Mahdi Sheikh, Mamak Shariat, Golnaz Rezaeizadeh
BACKGROUND: The existing therapeutic methods for neonatal jaundice are costly, time-consuming and potentially risky. Zinc salts can reduce phototherapy duration by precipitating unconjugated bilirubin in the intestine (bilirubin and zinc can form a complex in physiologic pH); however, zinc toxicity is an issue that must be considered since theoretically bilirubin reduction by phototherapy may increase serum zinc levels, making additional zinc supplementation the potential cause of zinc toxicity...
June 2016: Iranian Journal of Pediatrics
Silvia Gazzin, Libor Vitek, Jon Watchko, Steven M Shapiro, Claudio Tiribelli
Unconjugated bilirubin (UCB) is known to be one of the most potent endogenous antioxidant substances. While hyperbilirubinemia has long been recognized as an ominous sign of liver dysfunction, recent data strongly indicate that mildly elevated bilirubin (BLB) levels can be protective against an array of diseases associated with increased oxidative stress. These clinical observations are supported by new discoveries relating to the role of BLB in immunosuppression and inhibition of protein phosphorylation, resulting in the modulation of intracellular signaling pathways in vascular biology and cancer, among others...
September 2016: Trends in Molecular Medicine
Nilüfer Güzoğlu, Ümit Ayşe Tandircioğlu, Didem Aliefendioğlu
BACKGROUND/AIM: Prematurity is a significant risk factor for developing unconjugated hyperbilirubinemia. This study investigated the current approach to managing hyperbilirubinemia in preterm newborns in Turkey. MATERIALS AND METHODS: A study-specific questionnaire on the management of jaundice in preterm infants was sent to 100 level III neonatal intensive care units in Turkey. RESULTS: Responses were obtained from 84 centers from all regions of Turkey...
2016: Turkish Journal of Medical Sciences
Annanya Soni, Surendra Kumar Kanaujia, Sandeep Kaushik
(1) To study the BERA changes in neonates with unconjugated hyperbilirubinemia. (2) To compare the BERA changes in the neonates with unconjugated hyperbilirubinemia before and after therapy. Thirty consecutive term appropriate for gestational age (AGA) neonates presenting to NICU with total serum bilirubin requiring intervention (using the American Academy of Pediatrics guidelines) were included in the study as cases and thirty normal term AGA neonates with uneventful peri-natal period and a maximum measured serum bilirubin <12 mg/dl in case of term baby were included as controls after obtaining informed consent...
September 2016: Indian Journal of Otolaryngology and Head and Neck Surgery
X H Guo, Y F Sun, M Cui, J B Wang, S Z Han, J Miao
This study was carried out to analyze uridine diphosphate (UDP)-glucuronosyltransferase 1A1 (UGT1A1) gene mutations in neonates with unconjugated hyperbilirubinemia, from two different ethnic groups. Polymerase chain reaction and gene sequencing were used to analyze the differences in genotypes and allele frequencies of different gene mutations among the ethnic groups; this was followed by checking their correlation with the serum bilirubin level and the occurrence of unconjugated hyperbilirubinemia in neonates...
2016: Genetics and Molecular Research: GMR
Rania Hosny Tomerak, Nahed Fahmy Helal, Olfat Gameel Shaker, Mohamed Abdelhamid Yousef
We investigated the association between c-3279T>G and unconjugated neonatal hyperbilirubinemia. In all, 141 neonates were recruited; 63 had hyperbilirubinemia necessitating treatment, and 78 with bilirubin < 7 mg/dl served as the control group. The frequency of occurrence of c-3279T > G allele was significantly higher in the hyperbilirubinemic (49.2%) than in the control group (25.6%). The homozygous (p = 0.001, OR = 17.7 and CI = 3.9-79.3) rather than the heterozygous state (p = 0...
June 17, 2016: Journal of Tropical Pediatrics
Yunqin Wu, Guinan Li, Yong Zhou, Jun Li, Yueyuan Hu
OBJECTIVE: To detect potential mutation of the UGT1A1 gene in a child affected with Crigler-Najjar syndrome type II. METHODS: Blood samples were collected from the patient and his parents for the extraction of genomic DNA. Potential mutation of the UGT1A1 gene was detected with polymerase chain reaction (PCR) and direct sequencing. The child was followed up until the age of 3 years and 6 months. RESULTS: The patient showed persistent unconjugated hyperbilirubinemia...
June 2016: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
Ping Xu, Zhao-Li Ling, Ji Zhang, Ying Li, Nan Shu, Ze-Yu Zhong, Yang Chen, Xin-Yu Di, Zhong-Jian Wang, Li Liu, Xiao-Dong Liu
AIM: Liver failure is associated with dyshomeostasis of efflux transporters at the blood-brain barrier (BBB), which contributes to hepatic encephalopathy. In this study we examined whether breast cancer resistance protein (BCRP), a major efflux transporter at the BBB, was altered during liver failure in rats. METHODS: Rats underwent bile duct ligation (BDL) surgery, and then were sacrificed after intravenous injection of prazosin on d3, d7 and d14. The brains and blood samples were collected...
August 2016: Acta Pharmacologica Sinica
Alisha Nitin Chaubal, Ruchir Patel, Dhaval Choksi, Kaivan Shah, Meghraj Ingle, Prabha Sawant
Crigler Najjar syndrome is associated with indirect hyperbilirubinemia due to a deficiency of enzyme Uridine Di Phospho Glucoronosyl Transferase (UDPGT). Presented here is a case of a female in the first trimester of pregnancy, who was diagnosed to have type 2 Crigler Najjar syndrome. We also discuss the management of this rare disease especially in pregnancy. Unconjugated bilirubin can cross the placental barrier causing neurological damage in the newborn. Patient was carefully monitored during pregnancy and treatment with phenobarbitone in low doses was adjusted such that the serum bilirubin levels were below 10 mg/dL...
April 18, 2016: World Journal of Hepatology
Akitaka Nomura, Yoshihiro Maruo, Takashi Taga, Yoshihiro Takeuchi
BACKGROUND: Chemotherapy for malignant neoplasms sometimes induces unconjugated hyperbilirubinemia, resulting in the early cessation of treatment. We evaluated the role of variations in the bilirubin uridine-5-diphosphate (UDP)-glucuronosyltransferase gene (UGT1A1) in unconjugated hyperbilirubinemia development during chemotherapy in pediatric patients with leukemia. METHODS: UGT1A1 allelic variations were evaluated in 25 Japanese pediatric leukemia patients with hyperbilirubinemia (peak serum bilirubin concentration 3...
August 2016: Pediatric Research
Nikolaos Papadopoulos, Melanie Deutsch, Athanasios Georgalas, Helias Poulakidas, Lazaros Karnesis
Hyperbilirubinemia is an adverse reaction of simeprevir (SMV). The majority of these patients were taking concurrent ribavirin presenting elevated unconjugated hyperbilirubinemia due to hemolysis. However, cases of hepatic failure with elevated bilirubin level have also been reported in patients with decompensated cirrhosis. We describe a 51-year-old female patient with HbS beta 0-thalassemia and recently diagnosed compensated cirrhosis due to chronic hepatitis C infection. Laboratory evaluation revealed total bilirubin: 2...
2016: Case Reports in Hematology
Toshio Shimizu, Kazuya Fukuoka, Masayuki Takeda, Tutomu Iwasa, Takeshi Yoshida, Joanna Horobin, Mitchell Keegan, Lou Vaickus, Ajit Chavan, Mahesh Padval, Kazuhiko Nakagawa
PURPOSE: VS-6063 (also known as defactinib or PF-04554878) is a second-generation inhibitor of focal adhesion kinase and proline-rich tyrosine kinase-2. This phase 1 study evaluated the safety and tolerability, pharmacokinetics, and clinical activity of VS-6063 in Japanese subjects with advanced solid tumor malignancies in a first-in-Asian study setting. METHODS: VS-6063 was administered orally twice daily (b.i.d.) in 21-day cycles to cohorts of three subjects each with a standard 3 + 3 dose-escalation design until disease progression or unacceptable toxicity...
May 2016: Cancer Chemotherapy and Pharmacology
Rajendra G Kulkarni, K B Lakshmidevi, Vidya Ronghe, U S Dinesh
SETTINGS: This study was done in a tertiary care hospital having bed strength of more than 700 beds at SDM Medical College of Medical Sciences and Hospital, Dharwad, located in Northern Karnataka. AIM: The study was done to ascertain prevalence of Gilbert's syndrome in healthy blood donors and review the literature about feasibility of utilizing blood components from Gilbert's syndrome donors. MATERIALS AND METHODS: The study was done for 18 months and 7030 whole blood units were collected and all the units were subjected to mandatory transfusion-transmitted screening and all the plasma bags which were icteric on visual inspection were subjected to hematological and biochemical investigations to rule out other causes of hyperbilirubinemia...
January 2016: Asian Journal of Transfusion Science
Samuel Raimundo Fernandes, Carlos Miguel Moura, Beatriz Rodrigues, Luís Araújo Correia, Helena Cortez-Pinto, José Velosa
BACKGROUND: Crigler-Najjar syndrome (CN) is a very rare genetic disorder characterized by an inability to conjugate bilirubin. Contrary to CN type I, patients with CN II exhibit residual capacity to conjugate bilirubin and may present a normal life expectancy. CASE PRESENTATION: We report an unusual late diagnosis of CN type II in an 80-year-old female admitted with severe acute cholangitis. While the patient present typical clinical and radiologic signs of bile duct obstruction and cholangitis, her blood analysis showed severe unconjugated hyperbilirubinemia...
March 11, 2016: BMC Gastroenterology
Dirk R de Waart, Jyoti Naik, Karina S Utsunomiya, Suzanne Duijst, Kam Ho-Mok, A Ruth Bolier, Johan Hiralall, Laura N Bull, Piter J Bosma, Ronald P J Oude Elferink, Coen C Paulusma
UNLABELLED: ATP11C is a homolog of ATP8B1, both of which catalyze the transport of phospholipids in biological membranes. Mutations in ATP8B1 cause progressive familial intrahepatic cholestasis type1 in humans, which is characterized by a canalicular cholestasis. Mice deficient in ATP11C are characterized by a conjugated hyperbilirubinemia and an unconjugated hypercholanemia. Here, we have studied the hypothesis that ATP11C deficiency interferes with basolateral uptake of unconjugated bile salts, a process mediated by organic anion-transporting polypeptide (OATP) 1B2...
July 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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