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Gilbert Syndrome

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Gilbert syndrome is a fairly common genetic disorder that affects how the liver processes bilirubin. Bilirubin is the yellow pigment left over when the body breaks down old red blood cells.

The liver makes it excretable through glucuronidation, a chemical step that prepares it to leave the body, and an enzyme called UGT1A1 handles that step. In Gilbert syndrome that enzyme retains only 30% to 50% of its normal activity. So some of the bilirubin goes unprocessed, in its unconjugated form, and builds up. The recycling plant works half shifts: on a quiet day it keeps up, on a heavy day the load piles up.

That is why fasting, a passing illness, menstruation or dehydration bring on episodes of jaundice, that yellow tint of the skin and the eyes.

In brief

Gilbert syndrome is a common genetic disorder of bilirubin metabolism in the liver.

The UGT1A1 enzyme retains only 30% to 50% of its activity, so glucuronidation drops and unconjugated bilirubin builds up.

The recycling plant works half shifts: on a quiet day it keeps up, on a heavy day the load piles up.

That is why fasting, an intercurrent illness, menstruation or dehydration trigger episodes of jaundice.

Sources (1)
  1. National Center for Biotechnology Information, StatPearls PublishingGilbert Syndrome, StatPearlsPublished

This page explains the topic in general terms; it does not tell anyone what treatment to follow.

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