A Gunn rat model of preterm hyperbilirubinemia
نویسندگان
چکیده
منابع مشابه
Xenotransplantation of hamster liver into Gunn rats reserves congenital hyperbilirubinemia.
THE Gunn rat is an animal model for Crigler-Najar syndrome. a rare form of congenital unconjugated nonhemolytic hyperbilirubinemia due to the absence of glucoronyl transferase. I.e It leads to deposition of bilirubin in the basal ganglia. culminating in precocious death of the patient. Because the uptake. conjugation. and excretion of bilirubin by hepatocytes involves specific carriers and enzy...
متن کاملHyperbilirubinemia modulates myocardial function, aortic ejection, and ischemic stress resistance in the Gunn rat.
Mildly elevated circulating unconjugated bilirubin (UCB) is associated with protection against hypertension and ischemic heart disease. We assessed whether endogenously elevated bilirubin in Gunn rats modifies cardiovascular function and resistance to ischemic insult. Hearts were assessed ex vivo (Langendorff perfusion) and in vivo (Millar catheterization and echocardiography), and left ventric...
متن کاملOptimizing Exchange Transfusion for Severe Unconjugated Hyperbilirubinemia: Studies in the Gunn Rat
BACKGROUND Severe unconjugated hyperbilirubinemia carries the risk of neurotoxicity. Phototherapy (PT) and exchange transfusion (ET) are cornerstones in the treatment of unconjugated hyperbilirubinemia. Studies to improve ET efficacy have been hampered by the low application of ET in humans and by the lack of an in vivo model. The absence of an appropriate animal model has also prevented to det...
متن کاملTRANSLATIONAL PHYSIOLOGY Hyperbilirubinemia modulates myocardial function, aortic ejection, and ischemic stress resistance in the Gunn rat
Bhavisha Bakrania, Eugene F. Du Toit, Kevin J. Ashton, Can J. Kiessling, Karl-Heinz Wagner, John P. Headrick, and Andrew C. Bulmer Heart Foundation Research Centre, Griffith Health Institute, Griffith University, Gold Coast, Queensland, Australia; Faculty of Health Sciences and Medicine, Bond University, Gold Coast, Queensland, Australia; and Department of Nutritional Science, University of Vie...
متن کاملLifelong elimination of hyperbilirubinemia in the Gunn rat with a single injection of helper-dependent adenoviral vector.
Crigler-Najjar syndrome is a recessively inherited disorder characterized by severe unconjugated hyperbilirubinemia caused by a deficiency of uridine diphospho-glucuronosyl transferase 1A1. Current therapy relies on phototherapy to prevent kernicterus, but liver transplantation presently is the only permanent cure. Gene therapy is a potential alternative, and recent work has shown that helper-d...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 2019
ISSN: 0031-3998,1530-0447
DOI: 10.1038/s41390-019-0599-x