E80: Disorders of porphyrin and bilirubin metabolism

E80.5: Crigler-Najjar syndrome

When old blood cells are broken down, bile pigments come into being. These bilirubins are modified by a protein in the liver.

You do not have this protein, or you only have a small amount of it. This condition is congenital, which means you were born with it. If the protein is not working properly, bile pigments will accumulate in the body. The less you have of this protein, the more bile pigments accumulate. That is especially the case when the liver has to do a lot of work. The liver has to work a lot when you drink alcohol or when you have contact with pathogens. As a result, your skin usually looks yellow. Sometimes you may have problems with speaking or hearing.