Tin-Protoporphyrin in the Management of Children With Crigler-Najjar Disease

The Crigler-Najjar disease is a rare disorder originally described in 1952 and characterized by a severe unconjugated hyperbilirubinemia appearing in the first days of life and persisting throughout life. In 1969, Arias et al proposed to subdivide such patients into two groups. The first group (Crigler-Najjar disease type 1) consisted of the most severely jaundiced infants in whom bilirubin encephalopathy developed resulting in death, usually within the first year of life, and whose plasma bilirubin level did not decrease during treatment with phenobarbital. Their bile was described as virtually colorless and appeared to contain only a trace amount of bilirubin, all in the unconjugated form. In the second group (Crigler-Najjar disease type 2), bilirubin conjugates were detected in normally yellow bile. In these patients, the unconjugated hyperbilirubinemia was less severe and encephalopathy was absent.