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Crigler-Najjar Syndrome

Crigler-Najjar Syndrome Types 1 and 2 are genetic disorders of bilirubin metabolism. Found in bile, bilirubin supports fat breakdown and helps to prepare it for absorption. The body produces roughly 250 mg of water-insoluable unconjugated bilirubin daily. It travels to the liver, where it becomes water-soluble conjugated bilirubin and is excreted into the bile.

In Type 1 Crigler-Najjar syndrome, unconjugated bilirubin does not convert in the liver, resulting in a concentration of serum bilirubin. Type 1 usually develops in the first three days of life and is marked by pale stools. Left untreated, toxic concentrations (Kernicterus) can build in the first month. Phototherapy, exchange infusions and limiting bilirubin production are among treatment options. Liver transplant is successful in a small number of patients. Type 2 is a less severe disorder, generally presenting in neonates. Bilirubin concentrations may be elevated to and after three weeks of life, and is marked by jaundice and normal stool color. In the bile, bilirubin levels are almost normal. Medication to reduce serum bilirubin levels is effective.