In a patient with Crigler-Najjar syndrome who develops kernicterus, what treatment should be considered?

In the context of Crigler-Najjar syndrome, which is characterized by a deficiency in the enzyme UDP-glucuronosyltransferase, leading to an accumulation of unconjugated bilirubin, kernicterus arises when this elevated bilirubin level causes neurotoxicity. Plasma exchange is an appropriate treatment option for patients experiencing severe hyperbilirubinemia, particularly when there is a risk of kernicterus. This procedure helps to rapidly reduce the bilirubin levels in the bloodstream by removing bilirubin-containing plasma and replacing it with donor plasma or a suitable alternative.

While phototherapy is often used as a first-line treatment to manage bilirubin levels in neonates with jaundice, in cases of Crigler-Najjar syndrome with manifest kernicterus, the effectiveness of phototherapy is limited. Volume resuscitation and Rho(D) immunoglobulin do not directly address the hyperbilirubinemia associated with this condition, as they are more relevant to other types of jaundice or hemolytic conditions. Thus, for a patient who has developed kernicterus as a result of Crigler-Najjar syndrome, plasma exchange would be a critical intervention to mitigate the potentially devastating neurologic effects of high