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Orthotopic liver transplantation for type I Crigler-Najjar syndrome

✍ Scribed by Stuart S. Kaufman; R. Patrick Wood; Byers W. Shaw Jr.; Rodney S. Markin; Philip Rosenthal; Bruno Gridelli; Jon A. Vanderhoof

Book ID
102237456
Publisher
John Wiley and Sons
Year
1986
Tongue
English
Weight
461 KB
Volume
6
Category
Article
ISSN
0270-9139

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✦ Synopsis

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 16% 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.

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