Which medications are indicated in the treatment of unconjugated hyperbilirubinemia?

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

In Crigler-Najjar syndrome type 2, patients with symptomatic jaundice are occasionally treated to improve their quality of life.

In patients with Crigler-Najjar syndrome type 1, phenobarbital, ursodeoxycholic acid, calcium (infusions), metalloporphyrins, cholestyramine, chlorpromazine, and clofibrate (no longer on the US market), as well as alkalinization of urine, have all been considered as potential therapies. Problems associated with the use of cholestyramine include taste and concern about bile salt depletion and fat malabsorption. The exact roles and adverse effects of many of these drugs are not yet defined.

Metalloporphyrins have been used as a synthetic analogue of heme to inhibit the heme oxygenase enzyme, the rate-limiting step in heme catabolism to bilirubin. Tin mesoporphyrin (SnMp) is the drug of choice (DOC) for clinical use because of its increased potency, stability, and photophysical properties.

For Gilbert syndrome, no medical therapy is needed. As with Crigler-Najjar syndrome type 2, phenobarbital has been shown to decrease bilirubin production.


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