Why is long-term monitoring required in the management 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

Despite medical treatment, patients are at risk for sudden increases in serum bilirubin levels. Parents and physicians should be alert to such bilirubin crises. The child usually presents with altered sensorium, incoordination, slurring of speech, and weakness. Coma may eventually occur.

Treatment of severe episodes of hyperbilirubinemia includes intense phototherapy, exchange transfusion, plasmapheresis, and tin-mesoporphyrin. During periods of illness, kernicterus may occur at a low level of bilirubin.


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