Arias syndrome

Crigler-Najjar syndrome type II (Arias syndrome) is milder, usually benign, and caused by partial deficiency of bilirubin UDP-glucuronyltransferase. Jaundice may not appear until the second or third decade of life. The monoglucuronide is the predominant pigment in bile. Phenobarbital induces the enzyme. Dominant and recessive inheritance patterns have been described. An accurate diagnosis of type 1, as opposed to type 2 Crigler-Najjar syndrome, is essential since orthotopic liver transplantation is an important therapy for type 1 patients. 

H., Shorey, J., Combes, B. Viral hepatitis complicating the Dubin-Johnson syndrome. Gastroenterolcw 1972 63 331-339 Wolkoff, A.W., Wolpert, E., Pascasio, F.N., Arias, I.M. Rotor s syndrome. A distinct inheritable pathophysiologic entity. Amer. J. Med. 1976 60 173-179... 

Bilbao, J.L., Pueyo, J.C., Longo, JJM., Arias, M., Herrero, JJ., Benito, A., Barettino, M.D., Perotti, J.R, Pardo, F. Interventional therapeutic techniques in Budd-Chiari syndrome. Cardiovasc. Intervent. Radiol. 1997 20 112-119... 

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