|1.||Bosma, P J: 1 article (02/2014)|
|2.||Aubert, D: 1 article (02/2014)|
|3.||Montenegro-Miranda, P S: 1 article (02/2014)|
|4.||Ten Bloemendaal, L: 1 article (02/2014)|
|5.||de Waart, D R: 1 article (02/2014)|
|6.||Ferry, N: 1 article (02/2014)|
|7.||Pichard, V: 1 article (02/2014)|
|8.||Duijst, S: 1 article (02/2014)|
|9.||Lee, Brian R: 1 article (01/2014)|
|10.||Soto-Gutierrez, Alejandro: 1 article (01/2014)|
02/01/2014 - "Adenoviral (AdV) and Adenovirus-associated viral (AAV) vectors both are used for in vivo gene therapy of inherited liver disorders, such as Crigler-Najjar syndrome type 1. In a relevant animal model, the Gunn rat, both vectors efficiently correct the severe hyperbilirubinemia characteristic of this liver disorder. "
03/06/2006 - "Patients with Crigler-Najjar syndrome type 1 and Gunn rats, mutant strain of the Wistar rats, bear an autosomal recessive disorder resulting in hyperbilirubinemia. "
07/01/2005 - "Crigler-Najjar syndrome type 1 (CNS1) is characterized by a severe unconjugated hyperbilirubinemia from birth. "
05/01/2008 - "Treatment of Crigler-Najjar Syndrome type 1 by hepatic progenitor cell transplantation: a simple procedure for management of hyperbilirubinemia."
03/01/1998 - "Crigler-Najjar syndrome type 1 (CN-1) is a recessively inherited, potentially lethal disorder characterized by severe unconjugated hyperbilirubinemia resulting from deficiency of the hepatic enzyme bilirubin-UDP-glucuronosyltransferase. "
03/01/1998 - "Crigler-Najjar syndrome type 1 (CN type 1) is an autosomal recessive disorder characterized by nonhemolytic jaundice resulting from mutations to the gene encoding bilirubin-UDP-glucuronosyltransferase (UDPGT). "
09/01/1993 - "Crigler-Najjar syndrome type 1 (CN-1) is a familial disorder characterized by severe unconjugated hyperbilirubinemia and jaundice and leads to kernicterus, neurological damage, and eventual death unless treated with liver transplantation. "
|3.||Inborn Urea Cycle Disorders
|4.||Infantile Refsum Disease (Infantile Phytanic Acid Storage Disease)
04/01/2006 - "There was a range of aetiologies of liver disease: familial hypercholesterolaemia, Crigler-Najjar syndrome type 1, urea cycle defects, infantile Refsum disease, glycogen storage disease type Ia, inherited factor VII deficiency and progressive familial intrahepatic cholestasis type 2. Clinical improvement and partial correction of the metabolic abnormality was observed in most cases. "
|5.||Acute Liver Failure (Fulminant Hepatic Failure)
|1.||Uridine Diphosphate (UDP)
|2.||Glucuronosyltransferase (UDP Glucuronosyltransferase)
|4.||bilirubin glucuronoside glucuronosyltransferase
|7.||Hepatorenal form of glycogen storage disease
|8.||progressive familial intrahepatic 1 Cholestasis
|9.||tranilast (N 5')
|2.||Transplantation (Transplant Recipients)