Removal of protein-bound and unbound unconjugated bilirubin by perfusion of plasma through an anion-exchange resin in a case of Crigler-Najjar syndrome type I

Ann Clin Biochem 2003;40:528-533
doi:10.1258/000456303322326443
© 2003 Association for Clinical Biochemistry

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Original Articles


Hiroshi Ihara,
Yoshio Shino,
Naotaka Hashizume,
Norikazu Shimizu,
Tsugutoshi Aoki and
Mitsutaka Yoshida


Department of Laboratory Medicine, Toho University School of Medicine, 2-17-6 Ohashi, Meguro, Tokyo 1538515, Japan;
Department of Laboratory Medicine, Toho University School of Medicine, 2-17-6 Ohashi, Meguro, Tokyo 1538515, Japan;
Department of Laboratory Medicine, Toho University School of Medicine, 2-17-6 Ohashi, Meguro, Tokyo 1538515, Japan;
Department of Pediatrics, Toho University School of Medicine, 2-17-6 Ohashi, Meguro, Tokyo 1538515, Japan;
Department of Pediatrics, Toho University School of Medicine, 2-17-6 Ohashi, Meguro, Tokyo 1538515, Japan;
Department of Applied Physics and Chemistry, Fukui University of Technology, 3-6-1 Gakuen, Fukui 9108505, Japan


Background: Patients with Crigler-Najjar syndrome, type I (CNS-I)have an inherited absence of hepatocellular bilirubin uridinediphosphate-glucuronosyltransferase activity, which resultsin severe unconjugated hyperbilirubinaemia, often causing kernicterusand death in infancy or childhood.

Methods: Our patient is a 19-year-old Japanese man with CNS-Idiagnosed by the complete absence of the hepatocellular enzymein a liver biopsy and genotyping. The efficacies of the removalof protein-bound (PBB) and unbound (UB) unconjugated bilirubinby phototherapy, plasma perfusion and liver transplantationwere compared in the patient.

Results: At the age of 5 years, phototherapy treatment reducedthe patient’s PBB by 21% and UB by 34%, and 98% of the bilirubinproduced daily was removed. At the age of 16 years, plasma perfusioncombined with nightly phototherapy completely removed the dailyproduction of bilirubin; however, by 24 h post-treatment, thePBB and UB were again increased. Apparently, these treatmentswere effective in reducing PBB and UB, but the effect was onlytemporary. Following liver transplantation, PBB and UB decreasedto normal concentrations.

Conclusions: Liver transplantation as a potential cure shouldbe performed at a younger age, particularly in confirmed CNS-Icases for which reliable effects of phototherapy cannot be guaranteed.


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