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Kernicterus

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https://www.readbyqxmd.com/read/28283555/developmental-genetic-dietary-and-xenobiotic-influences-on-neonatal-hyperbilirubinemia
#1
Mei-Fei Yueh, Shujuan Chen, Nghia Nguyen, Robert H Tukey
Hyperbilirubinemia, caused by the accumulation of unconjugated bilirubin, is one of the most common clinical diagnoses in both premature and term newborns. Owing to the fact that bilirubin is metabolized solely through glucuronidation by UDP-glucuronosyltransferase (UGT) 1A1, it is now known that immaturity of UGT1A1 in combination with overproduction of bilirubin during the developmental stage acts as a bottleneck to bilirubin elimination and predisposes the infant to high TBS levels. While neonatal jaundice is mostly benign, excessively high levels of serum bilirubin in a small percentage of newborns can cause bilirubin-induced neurologic dysfunction (BIND), potentially leading to permanent brain damage, a condition known as kernicterus...
March 10, 2017: Molecular Pharmacology
https://www.readbyqxmd.com/read/28251113/rh-isoimmunized-pregnancy-managed-noninvasively-a-report-of-two-cases
#2
Deepti Jain
Pregnancy with Rh isoimmunization is a grave situation. Two women with indirect Coombs test (ICT) positive were managed conservatively with a favorable outcome. Peak systolic velocity (PSV) of middle cerebral artery (MCA) was measured every 2 weeks, and pregnancy continued till the value remained <1.5 mean of median. In one woman, the pregnancy could be prolonged to 37 weeks when delivery was induced and the neonate did not develop hyperbilirubinemia. In the second woman with bad obstetric history, when a highly positive ICT was reported, intravenous immunoglobulin (IVIG) was given in a single dose of 5 g every 2 weeks starting at 27 weeks, for a total of three doses, along with measurement of PSV of MCA...
January 2017: International Journal of Applied and Basic Medical Research
https://www.readbyqxmd.com/read/28167773/intestinal-ncor1-a-regulator-of-epithelial-cell-maturation-controls-neonatal-hyperbilirubinemia
#3
Shujuan Chen, Wenqi Lu, Mei-Fei Yueh, Eva Rettenmeier, Miao Liu, Johan Auwerx, Ruth T Yu, Ronald M Evans, Kepeng Wang, Michael Karin, Robert H Tukey
Severe neonatal hyperbilirubinemia (SNH) and the onset of bilirubin encephalopathy and kernicterus result in part from delayed expression of UDP-glucuronosyltransferase 1A1 (UGT1A1) and the inability to metabolize bilirubin. Although there is a good understanding of the early events after birth that lead to the rapid increase in serum bilirubin, the events that control delayed expression of UGT1A1 during development remain a mystery. Humanized UGT1 (hUGT1) mice develop SNH spontaneously, which is linked to repression of both liver and intestinal UGT1A1...
February 21, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28155818/fate-of-neural-progenitor-cells-transplanted-into-jaundiced-and-non-jaundiced-rat-brains
#4
Fu-Chen Yang, Sean M Riordan, Michelle Winter, Li Gan, Peter G Smith, Jay L Vivian, Steven M Shapiro, John A Stanford
High levels of bilirubin in infants can cause kernicterus, which includes basal ganglia damage and dystonia. Stem celltransplantation may be an effective treatment for this disease. In this study we transplanted human neural progenitor cells differentiated toward propriospinal interneurons into the striatum of 20-day old spontaneously jaundiced (jj) Gunn rats and nonjaundiced (Nj) littermates. Using immunohistochemical methods, we found that grafted cells survived and grew fibers in jj and Nj brains 3 weeks after transplantation...
February 3, 2017: Cell Transplantation
https://www.readbyqxmd.com/read/28117009/update-on-predicting-severe-hyperbilirubinemia-and-bilirubin-neurotoxicity-risks-in-neonates
#5
Pearl W Chang, Thomas B Newman, M Jeffrey Maisels
Extreme hyperbilirubinemia and kernicterus, though rare, continue to occur despite the adoption of universal screening. Unless they are known to have glucose-6-phosphate dehydrogenase deficiency, infants who currently develop kernicterus in high resource countries are often otherwise healthy newborns discharged from the well-baby nursery. In this review, we highlight risk factors that increase the risk of a newborn ≥35 weeks gestational age developing severe hyperbilirubinemia, as well as the risk factors that increase the hyperbilirubinemic infant's risk of kernicterus...
January 23, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28099134/carboxyhemoglobin-the-forgotten-parameter-of-neonatal-hyperbilirubinemia
#6
Douggl G N Bailey, Hans Fuchs, Roland Hentschel
BACKGROUND: Neonatal hyperbilirubinemia is influenced by a wide variety of factors, one of which is hemolysis. Serious hyperbilirubinemia may lead to a kernicterus with detrimental neurologic sequelae. Patients suffering from hemolytic disease have a higher risk of developing kernicterus. Carbon monoxide (CO), a byproduct of hemolysis or heme degradation, was described by Sjöstrand in the 1960s. It is transported as carboxyhemoglobin (COHb) and exhaled through the lungs. We were interested in a potential correlation between COHb and total serum bilirubin (TSB) and the time course of both parameters...
January 18, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28094087/-management-of-jaundice-in-the-newborn%C3%A2-35%C3%A2-gw-from-screening-to-follow-up-after-discharge-guidelines-for-clinical-practice
#7
A Cortey, L Renesme, J Raignoux, A Bedu, C Casper, P Tourneux, P Truffert
Jaundice due to unconjugated bilirubin is an everyday condition in the neonatal period because it results from the adaptation of bilirubin metabolism at this time of life. Hyperbilirubinemia has a potential neurotoxicity and although it most often resolves spontaneously, it can lead to acute and sometimes chronic encephalopathy. The latter condition is called kernicterus and induces severe and irreversible neurological sequelae. This rare complication is still reported in all countries throughout the world even if severe hyperbilirubinemia can be prevented and critical points points of failure in jaundice management are identified...
February 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28071585/newborn-bilirubin-screening-for-preventing-severe-hyperbilirubinemia-and-bilirubin-encephalopathy-a-rapid-review
#8
Kalpana Bhardwaj, Tiffany Locke, Anne Biringer, Allyson Booth, Elizabeth Kathleen Darling, Shelley Dougan, Jane Harrison, Stephen Hill, Ana Johnson, Susan Makin, Beth Potter, Thierry Lacaze-Masmonteil, Julian Little
hyperbilirubinemia [2], every newborn should be assessed for the risk of developing severe hyperbilirubinemia with the help of predischarge total serum bilirubin or transcutaneous bilirubin measurements and/or assessments of clinical risk factors. The aim of this rapid review is 1) to review the evidence for 1) predicting and preventing severe hyperbilirubinemia and bilirubin encephalopathy, 2) determining the efficacy of home/community treatments (home phototherapy) in the prevention of severe hyperbilirubinemia, and 3) non-invasive/transcutaneous methods for estimating serum bilirubin level...
January 10, 2017: Current Pediatric Reviews
https://www.readbyqxmd.com/read/28057969/chronic-kernicterus-magnetic-resonance-imaging-findings
#9
Bruno Niemeyer de Freitas Ribeiro, Gabriela de Almeida Lima, Nina Ventura, Emerson Leandro Gasparetto, Edson Marchiori
No abstract text is available yet for this article.
November 2016: Radiologia Brasileira
https://www.readbyqxmd.com/read/28025333/modulation-of-bilirubin-neurotoxicity-by-the-abcb1-transporter-in-the-ugt1-lethal-mouse-model-of-neonatal-hyperbilirubinemia
#10
Luka Bočkor, Giulia Bortolussi, Simone Vodret, Alessandra Iaconcig, Jana Jašprová, Jaroslav Zelenka, Libor Vitek, Claudio Tiribelli, Andrés F Muro
Moderate neonatal jaundice is the most common clinical condition during newborn life. However, a combination of factors may result in acute hyperbilirubinemia, placing infants at risk of developing bilirubin encephalopathy and death by kernicterus. While most risk factors are known, the mechanisms acting to reduce susceptibility to bilirubin neurotoxicity remain unclear. The presence of modifier genes modulating the risk of developing bilirubin-induced brain damage is increasingly being recognised. The Abcb1 and Abcc1 members of the ABC family of transporters have been suggested to have an active role in exporting unconjugated bilirubin from the central nervous system into plasma...
December 25, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/27863965/robustness-of-the-reichert-unistat-bilirubinometer-for-analysis-of-hemolyzed-samples-from-neonates
#11
Christopher R McCudden, Kathy Fleming, Michelle Warr
BACKGROUND AND OBJECTIVES: Bilirubin is routinely measured in neonates to avoid the irreversible effects of kernicterus. Grossly hemolyzed samples are routinely rejected under the assumption that bilirubin cannot be accurately measured in hemolyzed samples. The objective of this study was to determine the effect of hemolysis on bilirubin measurement in neonates. METHODS: Data included 2-years of paired bilirubin results (n=70) where hemolyzed and non-hemolyzed samples were collected and measured within 6h of each other...
March 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/27746217/a-new-rat-model-of-neonatal-bilirubin-encephalopathy-kernicterus
#12
Naser Amini, Nasim Vousooghi, Mansoureh Soleimani, Ali Samadikuchaksaraei, Mehdi Akbari, Hosein Safakheil, Pezhman Atafimanesh, Ali Shahbazi, Peiman Brouki Milan, Sara Ramezani, Masoud Mozafari, Mohammad Taghi Joghataei
INTRODUCTION: Hemolytic kernicterus, an indirect bilirubin-induced brain dysfunction, is associated with hyper-bilirubinemia in mammalian neonates. In this study, a new model of kernicterus has been developed using intra-peritoneal injections of phenyl hydrazine and subcutaneous injections of sulfisoxazole. These drugs can potentially induce kernicterus in neonatal through changes in hemolysis and hypo-albumin. METHODS: For this purpose, 7-day-old male Wistar rats (n=72; mean weight 11±1g) were used...
October 13, 2016: Journal of Pharmacological and Toxicological Methods
https://www.readbyqxmd.com/read/27591005/bilirubin-induced-neurological-dysfunction-a-clinico-radiological-neurophysiological-correlation-in-30-consecutive-children
#13
Ronald van Toorn, Philip Brink, Johan Smith, Christelle Ackermann, Regan Solomons
The clinical expression of bilirubin-induced neurological dysfunction varies according to severity and location of the disease. Definitions have been proposed to describe different bilirubin-induced neurological dysfunction subtypes. Our objective was to describe the severity and clinico-radiological-neurophysiological correlation in 30 consecutive children with bilirubin-induced neurological dysfunction seen over a period of 5 years. Thirty children exposed to acute neonatal bilirubin encephalopathy were included in the study...
September 2, 2016: Journal of Child Neurology
https://www.readbyqxmd.com/read/27587993/a-hypothesis-for-using-pathway-genetic-load-analysis-for-understanding-complex-outcomes-in-bilirubin-encephalopathy
#14
Sean M Riordan, Douglas C Bittel, Jean-Baptiste Le Pichon, Silvia Gazzin, Claudio Tiribelli, Jon F Watchko, Richard P Wennberg, Steven M Shapiro
Genetic-based susceptibility to bilirubin neurotoxicity and chronic bilirubin encephalopathy (kernicterus) is still poorly understood. Neonatal jaundice affects 60-80% of newborns, and considerable effort goes into preventing this relatively benign condition from escalating into the development of kernicterus making the incidence of this potentially devastating condition very rare in more developed countries. The current understanding of the genetic background of kernicterus is largely comprised of mutations related to alterations of bilirubin production, elimination, or both...
2016: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/27546055/the-choroid-plexus-in-health-and-in-disease-dialogues-into-and-out-of-the-brain
#15
Fernanda Marques, João Carlos Sousa, Alexandra Brito, Jens Pahnke, Cecilia Santos, Margarida Correia-Neves, Joana Almeida Palha
This article brings the choroid plexus into the context of health and disease. It is remarkable that the choroid plexus, composed by the monolayer of epithelial cells that lie in a highly vascularized stroma, floating within the brain ventricles, gets so little attention in major physiology and medicine text books and in the scientific literature in general. Consider that it is responsible for producing most of the about 150mL of cerebrospinal fluid that fills the brain ventricles and the subarachnoid space and surrounds the spinal cord in the adult human brain, which is renewed approximately 2-3 times daily...
August 18, 2016: Neurobiology of Disease
https://www.readbyqxmd.com/read/27511502/management-of-hyperbilirubinemia-in-preterm-infants-in-turkey
#16
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
https://www.readbyqxmd.com/read/27382636/exchange-transfusion-for-neonatal-hyperbilirubinemia-in-johannesburg-south-africa-from-2006-to-2011
#17
Daynia E Ballot, Gilbert Rugamba
Background. Severe hyperbilirubinaemia requiring exchange transfusion has become less common in recent years; however, kernicterus still occurs. The aim of this study was to review babies undergoing exchange transfusion for severe hyperbilirubinaemia in a Johannesburg hospital. Methodology. This was a retrospective review of babies who required exchange transfusion in both the neonatal and the paediatric wards from June 1, 2006, to December 31, 2011. Results. There were 64 patients who underwent 67 exchange transfusions...
2016: International Scholarly Research Notices
https://www.readbyqxmd.com/read/27235212/glucose-6-phosphate-dehydrogenase-deficiency-and-the-need-for-a-novel-treatment-to-prevent-kernicterus
#18
REVIEW
Anna D Cunningham, Sunhee Hwang, Daria Mochly-Rosen
Hyperbilirubinemia occurs frequently in newborns, and in severe cases can progress to kernicterus and permanent developmental disorders. Glucose-6-phosphate dehydrogenase (G6PD) deficiency, one of the most common human enzymopathies, is a major risk factor for hyperbilirubinemia and greatly increases the risk of kernicterus even in the developed world. Therefore, a novel treatment for kernicterus is needed, especially for G6PD-deficient newborns. Oxidative stress is a hallmark of bilirubin toxicity in the brain...
June 2016: Clinics in Perinatology
https://www.readbyqxmd.com/read/27235209/bilirubin-induced-neurotoxicity-in-the-preterm-neonate
#19
REVIEW
Jon F Watchko
Bilirubin-induced neurotoxicity in preterm neonates remains a clinical concern. Multiple cellular and molecular cascades likely underlie bilirubin-induced neuronal injury, including plasma membrane perturbations, excitotoxicity, neuroinflammation, oxidative stress, and cell cycle arrest. Preterm newborns are particularly vulnerable secondary to central nervous system immaturity and concurrent adverse clinical conditions that may potentiate bilirubin toxicity. Acute bilirubin encephalopathy in preterm neonates may be subtle and manifest primarily as recurrent symptomatic apneic events...
June 2016: Clinics in Perinatology
https://www.readbyqxmd.com/read/27214680/the-clinical-outcomes-of-deep-gray-matter-injury-in-children-with-cerebral-palsy-in-relation-with-brain-magnetic-resonance-imaging
#20
Ja Young Choi, Yoon Seong Choi, Dong-Wook Rha, Eun Sook Park
UNLABELLED: In the present study we investigated the nature and extent of clinical outcomes using various classifications and analyzed the relationship between brain magnetic resonance imaging (MRI) findings and the extent of clinical outcomes in children with cerebral palsy (CP) with deep gray matter injury. The deep gray matter injuries of 69 children were classified into hypoxic ischemic encephalopathy (HIE) and kernicterus patterns. HIE patterns were divided into four groups (I-IV) based on severity...
August 2016: Research in Developmental Disabilities
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