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unconjugated hyperbilirubinemia

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https://www.readbyqxmd.com/read/29390925/diagnostic-criteria-and-contributors-to-gilbert-s-syndrome
#1
Karl-Heinz Wagner, Ryan G Shiels, Claudia Anna Lang, Nazlisadat Seyed Khoei, Andrew C Bulmer
Hyperbilirubinemia is a well-known condition in the clinical setting; however, the causes of elevated serum bilirubin are diverse, as are the clinical ramifications of this condition. For example, diagnoses of individuals vary depending on whether they exhibit an unconjugated or conjugated hyperbilirubinemia. Diagnoses can include conditions of disordered bilirubin metabolism (Gilbert's, Crigler-Najjar, Rotor, or Dubin-Johnson syndromes) or an acquired disease, including alcoholic/non-alcoholic fatty liver disease, hepatotropic hepatitis, cirrhosis, or hepato-biliary malignancy...
February 1, 2018: Critical Reviews in Clinical Laboratory Sciences
https://www.readbyqxmd.com/read/29386646/-gilbert-s-like-syndrome-as-part-of-a-spectrum-of-persistent-unconjugated-hyperbilirubinemia-in-post-chronic-hepatitis-patients
#2
Jin Ye, Lianlian Cui, Yingqiao Zhou, Ying Huang, Omar Banafa, Xiaohua Hou, Zhen Ding, Rong Lin
Gilbert's syndrome (GS) patients present with remittent unconjugated hyperbilirubinemia. In this study, we investigated the correlation between polymorphisms in the gene encoding UDP-glucuronosyltransferase, UGT1A1, and the development of unconjugated hyperbilirubinemia in clinical GS and post-hepatitis hyperbilirubinemia. Blood samples were collected from 285 patients, including 85 patients who were clinically diagnosed with GS, 70 patients who had indirect hyperbilirubinemia during the recovery period of chronic liver diseases, 109 patients with normal hepatic function and 21 chronic active hepatitis patients...
January 31, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29357878/unconjugated-bilirubin-induces-pyroptosis-in-cultured-rat-cortical-astrocytes
#3
Jie Feng, Mengwen Li, Qian Wei, Shengjun Li, Sijie Song, Ziyu Hua
BACKGROUND: Bilirubin-induced neurological dysfunction (BIND), a severe complication of extreme neonatal hyperbilirubinemia, could develop into permanent neurodevelopmental impairments. Several studies have demonstrated that inflammation and nerve cell death play important roles in bilirubin-induced neurotoxicity; however, the underlying mechanism remains unidentified. METHODS: The present study was intended to investigate whether pyroptosis, a highly inflammatory form of programmed cell death, participated in the bilirubin-mediated toxicity on cultured rat cortical astrocytes...
January 22, 2018: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29295636/caffeine-prevents-bilirubin-induced-cytotoxicity-in-cultured-newborn-rat-astrocytes
#4
Mehmet Deliktaş, Hacer Ergin, Aydın Demiray, Hakan Akça, Özmert M A Özdemir, Mehmet Bülent Özdemir
OBJECTIVE: Unconjugated bilirubin (UCB) may cause neurotoxicity in preterm neonates due to immaturity of UGT1A1 leading to bilirubin accumulation in the brain. Caffeine used in the treatment of apnea of prematurity was reported to decrease mechanical ventilation requirement, the frequencies of bronchopulmonary dysplasia, patent ductus arteriosus, cerebral palsy and neurodevelopmental disorders in very low birth weight infants. However, the effect of caffeine on hyperbilirubinemia was not yet clarified...
January 2, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29203650/point-of-care-device-to-diagnose-and-monitor-neonatal-jaundice-in-low-resource-settings
#5
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 19, 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
#6
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
#7
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
#8
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
#9
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...
January 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29117957/nocturnal-oxyhemoglobin-desaturation-and-arteriopathy-in-a-pediatric-sickle-cell-disease-cohort
#10
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...
December 12, 2017: Neurology
https://www.readbyqxmd.com/read/29079291/mechanisms-of-redox-interactions-of-bilirubin-with-copper-and-the-effects-of-penicillamine
#11
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
#12
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
#13
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
#14
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...
November 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28815739/kernicterus-in-a-boy-with-ornithine-transcarbamylase-deficiency-a-case-report
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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