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unconjugated hyperbilirubinemia

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https://www.readbyqxmd.com/read/29203650/point-of-care-device-to-diagnose-and-monitor-neonatal-jaundice-in-low-resource-settings
#1
Pelham A Keahey, Mathieu L Simeral, Kristofer J Schroder, Meaghan M Bond, Prince J Mtenthaonnga, Robert H Miros, Queen Dube, Rebecca R Richards-Kortum
Newborns are at increased risk of jaundice, a condition in which excess bilirubin accumulates in blood. Left untreated, jaundice can lead to neurological impairment and death. Jaundice resulting from unconjugated hyperbilirubinemia is easily treated with exposure to blue light, and phototherapy systems have been developed for low-resource settings; however, there are no appropriate solutions to diagnose and monitor jaundice in these settings. To address this need we present BiliSpec, a low-cost reader and disposable lateral flow card designed to measure the concentration of total bilirubin from several drops of blood at the point of care...
December 4, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29200157/an-infant-with-unusually-high-unconjugated-hyperbilirubinemia-due-to-coexistence-of-hereditary-spherocytosis-and-gilbert-syndrome
#2
Ivona Butorac Ahel, Kristina Baraba Dekanic, Goran Palcevski, Jelena Roganovic
Hereditary spherocytosis is the most frequent congenital hemolytic anemia and is characterized with variable degree of anemia, jaundice, and splenomegaly. In the case of severe hyperbilirubinemia out of proportion with hemolysis, other causes of hyperbilirubinemia must be considered. Gilbert syndrome (GS) is an autosomal dominant disorder characterized with intermittent hyperbilirubinemia without any other sign and symptom of liver disease as a result of reduced activity of uridine diphosphate-glucuronyl transferase 1A1...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29176474/hepatic-parenchymal-injury-in-crigler-najjar-type-i
#3
Ellen Mitchell, Sarangarajan Ranganathan, Patrick McKiernan, Robert H Squires, Kevin Strauss, Kyle Soltys, George Mazariegos, James E Squires
BACKGROUND: Crigler-Najjar syndrome type I (CNI) arises from biallelic variants of UGT1A1 that abrogate UGT1A1 activity resulting in unconjugated hyperbilirubinemia. Historically, liver parenchyma in CNI was considered structurally and histologically normal. Recent review of CNI liver explants revealed fibrosis. Our aim was to investigate the association between hepatic histology and disease phenotype in CNI. METHODS: We extracted data from the medical record at the time of liver transplant from 22 patients with CNI at the Children's Hospital of Pittsburgh, and reviewed explant histology...
November 22, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29137095/differences-in-ugt1a1-gene-mutations-and-pathological-liver-changes-between-chinese-patients-with-gilbert-syndrome-and-crigler-najjar-syndrome-type-ii
#4
Lei Sun, Man Li, Liang Zhang, Xiaoying Teng, Xiangmei Chen, Xingang Zhou, Zhiyuan Ma, Liming Qi, Peng Wang
Diagnosis of Crigler-Najjar syndrome type II (CNS-II) and Gilbert syndrome (GS) based on the serum bilirubin concentration is difficult, because this parameter can fluctuate under certain conditions. The aim of this study was to explore differences in UGT1A1 gene mutations, which cause both CNS and GS, and pathological changes between CNS-II and GS.Ninety-five Chinese patients with hereditary unconjugated hyperbilirubinemia were enrolled in this study. Peripheral blood samples obtained from patients were used to evaluate bilirubin levels and for UGT1A1 gene testing...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29132818/bilirubin-albumin-binding-and-unbound-unconjugated-hyperbilirubinemia-in-premature-infants
#5
Sanjiv B Amin, Hongyue Wang
OBJECTIVE: To evaluate the associations between unbound bilirubin (UB) and total serum bilirubin (TSB), bilirubin:albumin molar ratio (BAMR), and bilirubin albumin binding affinity (Ka) as a function of gestational age (GA) in infants born at 24-33 weeks GA. STUDY DESIGN: In a prospective observational study, TSB and UB were measured twice daily at least 8 hours apart during the first postnatal week. Serum albumin was measured to calculate BAMR on each day. The highest UB on each day, corresponding TSB, and serum albumin were used to calculate the Ka on each day...
November 10, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29117957/nocturnal-oxyhemoglobin-desaturation-and-arteriopathy-in-a-pediatric-sickle-cell-disease-cohort
#6
Nomazulu Dlamini, Dawn E Saunders, Michael Bynevelt, Sara Trompeter, Timothy C Cox, Romola S Bucks, Fenella J Kirkham
OBJECTIVE: The purpose of this study of sickle cell disease (SCD) was to determine whether arteriopathy, measurable as intracranial vessel signal loss on magnetic resonance angiography (MRA), was associated with low nocturnal hemoglobin oxygen saturation (SpO2) or hemolytic rate, measurable as reticulocytosis or unconjugated hyperbilirubinemia. METHODS: Ninety-five East London children with SCD without prior stroke had overnight pulse oximetry, of whom 47 (26 boys, 39 hemoglobin SS; mean age 9...
November 8, 2017: Neurology
https://www.readbyqxmd.com/read/29079291/mechanisms-of-redox-interactions-of-bilirubin-with-copper-and-the-effects-of-penicillamine
#7
Bojana Božić, Jelena Korać, Dalibor M Stanković, Marina Stanić, Ana Popović-Bijelić, Jelena Bogdanović Pristov, Ivan Spasojević, Milica Bajčetić
Toxic effects of unconjugated bilirubin (BR) in neonatal hyperbilirubinemia have been related to redox and/or coordinate interactions with Cu(2+). However, the development and mechanisms of such interactions at physiological pH have not been resolved. This study shows that BR reduces Cu(2+) to Cu(1+) in 1:1 stoichiometry. Apparently, BR undergoes degradation, i.e. BR and Cu(2+) do not form stable complexes. The binding of Cu(2+) to inorganic phosphates, liposomal phosphate groups, or to chelating drug penicillamine, impedes redox interactions with BR...
October 26, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/28954873/chronic-auditory-toxicity-in-late-preterm-and-term-infants-with-significant-hyperbilirubinemia
#8
MULTICENTER STUDY
Sanjiv B Amin, Satish Saluja, Arvind Saili, Mark Orlando, Hongyue Wang, Nirupama Laroia, Asha Agarwal
BACKGROUND AND OBJECTIVES: Significant hyperbilirubinemia (SHB) may cause chronic auditory toxicity (auditory neuropathy spectrum disorder and/or sensorineural hearing loss); however, total serum bilirubin (TSB) does not discriminate neonates at risk for auditory toxicity. Our objective was to compare TSB, bilirubin albumin molar ratio (BAMR), and unbound bilirubin (UB) for their association with chronic auditory toxicity in neonates with SHB (TSB ≥20 mg/dL or TSB that met criteria for exchange transfusion)...
October 2017: Pediatrics
https://www.readbyqxmd.com/read/28892962/crigler-najjar-syndrome-type-2-cns-type-2-an-unwonted-cause-of-jaundice-in-adults
#9
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/28888563/bilirubin-uridine-diphosphate-glucuronosyltransferase-polymorphism-as-a-risk-factor-for-prolonged-hyperbilirubinemia-in-japanese-preterm-infants
#10
Takahide Yanagi, Sayuri Nakahara, Yoshihiro Maruo
OBJECTIVE: To determine whether a variant of the bilirubin uridine diphosphate-glucuronosyltransferase gene (UGT1A1*6) is a risk factor for prolonged hyperbilirubinemia in preterm infants. STUDY DESIGN: UGT1A1 genotypes in 46 Japanese preterm infants (<37 weeks of gestation) were compared with UGT1A1 genotypes in 38 control infants, using polymerase chain reaction-direct sequencing. Prolonged unconjugated hyperbilirubinemia was defined as serum total bilirubin concentration of >150 µmol/L (8...
September 6, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28815739/kernicterus-in-a-boy-with-ornithine-transcarbamylase-deficiency-a-case-report
#11
Eduardo López-Corella, Isabel Ibarra-González, Cynthia Fernández-Lainez, Miguel Á Rodríguez-Weber, Sara Guillén-Lopez, Leticia Belmont-Martínez, David Agüero-Linares, Marcela Vela-Amieva
Ornithine transcarbamylase deficiency (OTCD) is an X-linked urea cycle defect associated with severe and usually fatal hyperammonemia. This study describes a patient with early onset lethal OTCD due to a known pathogenic variant (c.298+1G>A), as well as the novel autopsy finding of kernicterus with relatively low blood concentration of unconjugated bilirubin (UCB) (11.55 mg/dL). The patient was a full-term male with a family history of two previous male siblings who died as newborns after acute neurologic deterioration...
August 16, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28782473/the-importance-of-hemolysis-and-its-clinical-detection-in-neonates-with-hyperbilirubinemia
#12
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/28751579/promoterless-gene-targeting-without-nucleases-rescues-lethality-of-a-crigler-najjar-syndrome-mouse-model
#13
Fabiola Porro, Giulia Bortolussi, Adi Barzel, Alessia De Caneva, Alessandra Iaconcig, Simone Vodret, Lorena Zentilin, Mark A Kay, Andrés F Muro
Crigler-Najjar syndrome type I (CNSI) is a rare monogenic disease characterized by severe neonatal unconjugated hyperbilirubinemia with a lifelong risk of neurological damage and death. Liver transplantation is the only curative option, which has several limitations and risks. We applied an in vivo gene targeting approach based on the insertion, without the use of nucleases, of a promoterless therapeutic cDNA into the albumin locus of a mouse model reproducing all major features of CNSI Neonatal transduction with the donor vector resulted in the complete rescue from neonatal lethality, with a therapeutic reduction in plasma bilirubin lasting for at least 12 months, the latest time point analyzed...
October 2017: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/28707558/effect-of-unconjugated-hyperbilirubinemia-on-neonatal-autonomic-functions-evaluation-by-heart-rate-variability
#14
Rahmi Özdemir, Özgür Olukman, Cem Karadeniz, Kıymet Çelik, Nagehan Katipoğlu, Murat Muhtar Yılmazer, Şebnem Çalkavur, Timur Meşe, Sertaç Arslanoğlu
BACKGROUND: Serum bilirubin levels beyond the physiological limits, may lead to alterations in autonomic regulation in a newborn infant. Heart rate variability (HRV), is a noninvasive and quantitative marker of the activity of the autonomic nervous system (ANS). To date, few studies have demonstrated the undesirable effects of severe unconjugated hyperbilirubinemia (UHB) on autonomic functions, and only one study has used HRV as a marker of the autonomic activity. However, the relationship between altered cardiac autonomic functions and UHB by using the HRV derived from 24-hour Holter electrocardiography (ECG) recording has not been investigated previously...
July 25, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28590786/liver-fibrosis-associated-with-crigler-najjar-syndrome-in-a-compound-heterozygote
#15
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/28563973/phototherapy-for-neonatal-hyperbilirubinemia
#16
REVIEW
Susumu Itoh, Hitoshi Okada, Toru Kuboi, Takashi Kusaka
Approximately 60 years ago in England, phototherapy for neonatal hyperbilirubinemia was used in clinical practice. It was introduced in Japan approximately 50 years ago. At that time, the mechanism underlying the serum bilirubin concentration decrease by phototherapy was still unknown. The mechanism was identified by chemists, biochemists, and pediatricians. Clarification started with the report that unconjugated bilirubin was excreted into bile after photoirradiation in Gunn rats. After confirmation of the molecular structure of bilirubin on X-ray analysis, the mechanism for bile excretion of unconjugated bilirubin was verified based on geometric configurational photoisomers in the Gunn rat...
September 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28512725/clofibrate-as-an-adjunct-to-phototherapy-for-unconjugated-hyperbilirubinemia-in-term-neonates
#17
Prasad Kumar, B Adhisivam, B Vishnu Bhat
OBJECTIVE: To evaluate the efficacy of oral clofibrate as an adjunct to phototherapy for unconjugated hyperbilirubinemia in term neonates. METHODS: This randomized controlled trial was done in the level III neonatal intensive care unit (NICU) of a tertiary care hospital. Ninety term neonates with unconjugated hyperbilirubinemia with serum bilirubin 15-25 mg/dl were randomized to either intervention group (single dose of clofibrate in a dose of 50 mg/kg prior to starting phototherapy) or standard care group (only phototherapy)...
October 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28502442/neonatal-cholestasis
#18
REVIEW
Erin Lane, Karen F Murray
Neonatal jaundice is common and usually not concerning when it is secondary to unconjugated hyperbilirubinemia, below the neurotoxic level, and resolves early. Primary care providers should be vigilant, however, about evaluating infants in whom jaundice presents early, is prolonged beyond 2 weeks of life, or presents at high levels. Even in well-appearing infants, fractionated (direct and indirect) bilirubin levels should be obtained in these clinical scenarios to evaluate for potential cholestasis. This review presents an approach to the evaluation of a jaundiced infant and discusses diagnosis and management of several causes of neonatal cholestasis...
June 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/28494109/oncology-drug-dosing-in-gilbert-syndrome-associated-with-ugt1a1-a-summary-of-the-literature
#19
REVIEW
Vincent H Ha, Jennifer Jupp, Roger Y Tsang
Gilbert syndrome (GS) is a hereditary condition that affects ~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% that severely impacts the ability to metabolize certain medications. Antineoplastic agents used in oncologic settings have toxic side effects, and alterations in metabolism may result in severe or even life-threatening toxicities...
August 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28490767/biliverdin-reductase-inhibitors-did-not-improve-severe-unconjugated-hyperbilirubinemia-in-vivo
#20
Remco van Dijk, Sem J Aronson, Dirk R de Waart, Stan F van de Graaf, Suzanne Duijst, Jurgen Seppen, Ronald Oude Elferink, Ulrich Beuers, Piter J Bosma
We aimed to identify potent biliverdin reductase (BVRA) inhibitors as a novel concept for the treatment of severe unconjugated hyperbilirubinemia. 1280 FDA-approved compounds were screened in vitro for their ability to inhibit human and rat BVRA activity and 26 compounds were identified as BVRA inhibitors. Montelukast and Disulfiram were selected as potentially clinically applicable drugs and tested to reduce serum unconjugated bilirubin (UCB) levels in the Ugt1a1-deficient rat, a model for chronic unconjugated hyperbilirubinemia...
May 10, 2017: Scientific Reports
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