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...

Crigler-Najjar syndrome type II is a rare familial disorder of bilirubin conjugation with consecutive life-long unconjugated hyperbilirubinemia. In the presence of severe hyperbilirubinemia, a fetus or an adult is at risk for neurological defects in this syndrome. This paper is the first report emphasizing details about this disorder in two patients from Turkey. The diagnosis was made on the ba...

Hereditary or familial hiperbilirubinemia comprises a group of syndromes (Dubin-Johnson’s, Rotor’s, hepatic storage disease) in which hyperbilirubinemia, predominantly unconjugated or conjugated, occurs as an isolated biochemical abnormality without evidence of either hepatocellular necrosis or cholestasis. We present a patient with Dubin-Johnson syndrome, one of the familial disorders associat...

In the newborn period, unconjugated hyperbilirubinemia (UHB) is common, multifactoral, and associated with a variety of physiologic and pathologic conditions. The most commonly identified pathologic cause leading to hyperbilirubinemia is hemolytic disease of the newborn. We report a five-days-old female infant with neonatal jaundice secondary to splenic hematoma.

I have read with great interest the article by Bilgen et al., which appeared in the last issue [2006; 48 (1)] of the journal. The relationship (reason or result) between neonatal, especially prolonged hyperbilirubinemia and urinary tract infection is highly questionable, and investigation and debate on the topic are ongoing1,2. However, there are some methodological and propositional question m...

BACKGROUND AND OBJECTIVE Crigler-Najjar syndromes type I and II and Gilbert's syndrome are familial unconjugated hyperbilirubinemias caused by genetic lesions involving a single complex locus encoding for bilirubin-UDP-glucuronosyltransferase which is involved in the detoxification of bilirubin by conjugation with glucuronic acid. Over the last few years a number of different mutations affectin...