CRIGLER NAJJAR SYNDROME TYPE I, A RARE BUT SEVERE CAUSE OF UNCONJUGATED HYPERBILIRUBINEMIA IN CHILDREN
Abstract
Crigler Najjar Syndrome Type I is a rare and severe autosomal recessive disorder of bilirubin metabolism. It has been associated with consanguinity in some patients. Infants without any evidence of hemolysis, develop severe, permanent, unconjugated hyperbilirubinemia within the first few days of life resulting in chronic kernicterus.
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References
Hong Y.S, Jin J.Y, Ryoung Lee W.R. A case of Gilbert’s syndrome in a neonate with severe hyperbilirubinemia. J Korean Soc Neonatol 2010; 17:266-9c Ne
Canu G, Minucci A, Zuppi C, Capoluongo E: Gilbert and Crigler Najjar syndromes: an update of the UDP-glucuronosyltransferase 1A1 (UGT1A1) gene mutation database. Blood Cells Mol Dis 2013, 50(4):273-280. Strassburg CP: Hyperbilirubinemia syndromes (Gilbert-Meulengracht, Crigler-Najjar, Dubin- Johnson, and Rotor syndrome). Best Pract Res Clin Gastroenterol 2010, 24(5):555-71. Center for Arab Genomic Studies A division of Sheikh Hamadan Award for Medical Sciences. The catalogue for Transmission Genetics in Arabs CTGA Database Aloulou H, Thabet A.B, Khanfir S, Mansour L.B, Chabchoub I, Labrune P, et. al. La Maladie De Crigler Najjar De Type I En Tunisie : Etude De 30 observations. La tunisie Medicale - 2010 ; 88:10 : 707-09
Canu G, Minucci A, Zuppi C, Capoluongo E: Gilbert and Crigler Najjar syndromes: an update of the UDP-glucuronosyltransferase 1A1 (UGT1A1) gene mutation database. Blood Cells Mol Dis 2013, 50(4):273-280. Strassburg CP: Hyperbilirubinemia syndromes (Gilbert-Meulengracht, Crigler-Najjar, Dubin- Johnson, and Rotor syndrome). Best Pract Res Clin Gastroenterol 2010, 24(5):555-71. Center for Arab Genomic Studies A division of Sheikh Hamadan Award for Medical Sciences. The catalogue for Transmission Genetics in Arabs CTGA Database Aloulou H, Thabet A.B, Khanfir S, Mansour L.B, Chabchoub I, Labrune P, et. al. La Maladie De Crigler Najjar De Type I En Tunisie : Etude De 30 observations. La tunisie Medicale - 2010 ; 88:10 : 707-09
Published
2016-01-01
Section
Articles