1: Hepatology 1986 Nov-Dec;6(6):1259-62

Orthotopic liver transplantation for type I Crigler-Najjar syndrome

Kaufman SS, Wood RP, Shaw BW Jr, Markin RS, Rosenthal P, Gridelli B, Vanderhoof
JA

Abstract
A neurologically normal 3-year-old girl with Type I Crigler-Najjar syndrome was
successfully treated with orthotopic liver transplantation. Preoperative serum
bilirubin concentrations as high as 31 mg per dl were not diminished with
phenobarbital or phototherapy. Bilirubin fractionation of duodenal bile prior to
transplantation revealed 87.1% unconjugated bilirubin and 12.9% monoconjugates
as determined by alkaline methanolysis-high-performance liquid chromatography.
Postoperatively, the serum bilirubin concentration quickly fell to normal.
Uridine diphosphate glucuronyl transferase activity in the recipient liver was
not detectable. The gallbladder bile bilirubin concentration of 23.9 mg per dl
was less than 15% of previously reported normal values. Since devastating
kernicteric brain injury is the invariable outcome of Type I Crigler-Najjar
syndrome, liver transplantation should be performed when phototherapy cannot
maintain the serum bilirubin concentration at an unequivocally safe level