Distinguishing Gilbert's Syndrome (and Crigler-Najjar Disease Type II) from Crigler-Najjar Disease Type I

Gilbert's syndrome and Crigler-Najjar Disease (CND) Type II can be distinguished from Crigler-Najjar Disease Type I by a number of tests.

Parameters:

(1) amount of bilirubin monoglucuronide in bile

(2) effect of phenobarbital on serum unconjugated and total bilirubin

(3) hepatic UDP-glucuronosyl transferase activity

Parameters	Gilbert's or CND Type II	CND Type I
bilirubin monoglucuronide in bile	present	absent or very low
effect of phenobarbital	decrease in unconjugated and total bilirubin	no change
hepatic UDP-glucuronosyl transferase activity	below normal but present	absent

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