What is the role of plasma exchange transfusion in the treatment of Crigler-Najjar syndrome type 1?

Updated: May 21, 2019
  • Author: Hisham Nazer, MBBCh, FRCP, DTM&H; Chief Editor: BS Anand, MD  more...
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Answer

As previously mentioned, emergent management of bilirubin encephalopathy involves plasma exchange transfusion, which acts by removing bilirubin-saturated albumin and providing free protein, which draws bilirubin from the tissues. Plasma exchange should be accompanied by long-term phototherapy. Treatment with exchange transfusions and phototherapy should be initiated early.


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