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Underexcretion disorders refers to intrinsic enzyme defects in bilirubin metabolism that can lead to elevations in either conjugated (Dubin-Johnson and Rotor syndromes) or unconjugated (Crigler-Najjar and Gilbert syndromes) bilirubin.
Of the genes involved in bilirubin metabolism, UGT1A1 has been the most widely studied because of its essential role in hepatic bilirubin glucuronosylation.
Bilirubin entry into the brain is dependent on the integrity of the blood-brain barrier, and its persistence in the brain is determined by a variety of transporters (13) as well as by bilirubin metabolism.
The biochemistry of bilirubin metabolism is well described, although the accompanying diagram is rather poor and perhaps there is a little too much emphasis on biliary atresia as a rare cause of neonatal jaundice.