Results 21 to 30 of about 4,105 (205)

Liver Transplantation Outcomes in Crigler-Najjar Syndrome in Iran: A Single-Center Retrospective Cohort Study Over 20 Years. [PDF]

Health Sci Rep
ABSTRACT Background and Aims Crigler‐Najjar Syndrome (CNS) is a rare autosomal recessive disorder caused by uridine diphosphate glucuronosyltransferase (UGT1A1) deficiency, leading to unconjugated hyperbilirubinemia. Without treatment, patients are at high risk of kernicterus and irreversible neurological damage.
Teimoury S, Beyzaei Z, Shamsaeefar A, Kazemi K, Nikeghbalian S, Malekhosseini SA, Geramizadeh B.   +6 more
europepmc   +2 more sources

Developmental outcome of severe neonatal indirect hyperbilirubinemia [PDF]

Journal of Research in Clinical Medicine, 2021
Background: Indirect hyperbilirubinemia is one of the most common causes of hospitalization in the neonatal period and its potential association with brain damage is well established.
Manizheh Mostafa Gharehbaghi, Seifolah Heidatabady, Masoomeh Ghasempour, Mahsa Alizade   +3 more
doaj   +1 more source

Free Bilirubin Induces Neuro-Inflammation in an Induced Pluripotent Stem Cell-Derived Cortical Organoid Model of Crigler-Najjar Syndrome

Cells, 2023
Bilirubin-induced neurological damage (BIND), which might progress to kernicterus, occurs as a consequence of defects in the bilirubin conjugation machinery, thus enabling albumin-unbound free bilirubin (BF) to cross the blood–brain barrier and ...
Abida Islam Pranty, Wasco Wruck, James Adjaye   +2 more
doaj   +1 more source

Bilirubin-Induced Transcriptomic Imprinting in Neonatal Hyperbilirubinemia

Biology, 2023
Recent findings indicated aberrant epigenetic control of the central nervous system (CNS) development in hyperbilirubinemic Gunn rats as an additional cause of cerebellar hypoplasia, the landmark of bilirubin neurotoxicity in rodents.
John Paul Llido, Emanuela Fioriti, Devis Pascut, Mauro Giuffrè, Cristina Bottin, Fabrizio Zanconati, Claudio Tiribelli, Silvia Gazzin   +7 more
doaj   +1 more source

Genetic Association of UGT1A1 Promoter Variants (c.-3279T>G and c.-3156G>A) with Neonatal Hyperbili-rubinemia in an Iranian Population [PDF]

Iranian Journal of Neonatology, 2021
Background: Several studies have reported that two promoter variants (c.-3279T>G and c.-3156G>A) in UDP-glucuronosyltransferase (UGT1A1) gene may contribute to neonatal hyperbilirubinemia.
Nasim Pouralizadeh, Gholamali Mamouri, Abbas Boskabadi, Hassan Boskabadi, Houshang Rafatpanah, Ali Moradi, Hassan Mehrad-Majd   +6 more
doaj   +1 more source

MGE-Like Neural Progenitor Cell Survival and Expression of Parvalbumin and Proenkephalin in a Jaundiced Rat Model of Kernicterus

Cell Transplantation, 2022
Kernicterus is a permanent condition caused by brain damage from bilirubin toxicity. Dystonia is one of the most debilitating symptoms of kernicterus and results from damage to the globus pallidus (GP).
Fu-Chen Yang, Jay L. Vivian, Catherine Traxler, Steven M. Shapiro, John A. Stanford   +4 more
doaj   +1 more source

Demystifying non-invasive approaches for screening jaundice in low resource settings: a review

Frontiers in Pediatrics, 2023
All national and international pediatric guidelines universally prescribe meticulous bilirubin screening for neonates as a critical measure to mitigate the incidence of acute bilirubin encephalopathy (ABE) and Kernicterus.
Umme Abiha, Umme Abiha, Dip Sankar Banerjee, Saptarshi Mandal   +3 more
doaj   +1 more source

The Causes and Risk Factors in Patients with Kernicterus Referred to the Clinic of Pediatric Neurology during the Years 2011 to 2016

مجله دانشکده پزشکی اصفهان, 2018
Background: Neonatal jaundice has potentially severe side effects such as kernicterus. Prevention of kernicterus and hyperbilirubinemia is based on finding neonates with risk factors and starting treatment as soon as possible. Therefore, this study aimed
Jafar Nasiri, Mohammadreza Ghazavi, Mohammadali Pourmirzaei, Abdorahim Pak   +3 more
doaj   +1 more source

Bilirubin-Induced Neurological Damage: Current and Emerging iPSC-Derived Brain Organoid Models

Cells, 2022
Bilirubin-induced neurological damage (BIND) has been a subject of studies for decades, yet the molecular mechanisms at the core of this damage remain largely unknown.
Abida Islam Pranty, Sara Shumka, James Adjaye   +2 more
doaj   +1 more source

MRI in Kernicterus

Pediatric Neurology Briefs, 1995
The magnetic resonance images (MRI) of three children with athetotic cerebral palsy and severe neonatal jaundice were examined in the Department of Pediatric Neurology, Ohzora-no-iye Hospital and Seirei-Mikatahara General Hospital, Shizuoka, Japan.
J Gordon Millichap
doaj   +1 more source