Orthotopic liver transplantation for type I Crigler‐Najjar syndrome

S. S. Kaufman, R. P. Wood, B. W. Shaw, Rodney Smith Markin, P. Rosenthal, B. Gridelli, J. A. Vanderhoof

Research output: Contribution to journalArticle

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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.

Original languageEnglish (US)
Pages (from-to)1259-1262
Number of pages4
JournalHepatology
Volume6
Issue number6
DOIs
StatePublished - Jan 1 1986

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Bilirubin
Liver Transplantation
Phototherapy
Bile
Serum
Uridine Diphosphate
Phenobarbital
Transferases
Gallbladder
Liquid Chromatography
Brain Injuries
Reference Values
Transplantation
Liver

ASJC Scopus subject areas

  • Hepatology

Cite this

Kaufman, S. S., Wood, R. P., Shaw, B. W., Markin, R. S., Rosenthal, P., Gridelli, B., & Vanderhoof, J. A. (1986). Orthotopic liver transplantation for type I Crigler‐Najjar syndrome. Hepatology, 6(6), 1259-1262. https://doi.org/10.1002/hep.1840060606

Orthotopic liver transplantation for type I Crigler‐Najjar syndrome. / Kaufman, S. S.; Wood, R. P.; Shaw, B. W.; Markin, Rodney Smith; Rosenthal, P.; Gridelli, B.; Vanderhoof, J. A.

In: Hepatology, Vol. 6, No. 6, 01.01.1986, p. 1259-1262.

Research output: Contribution to journalArticle

Kaufman, SS, Wood, RP, Shaw, BW, Markin, RS, Rosenthal, P, Gridelli, B & Vanderhoof, JA 1986, 'Orthotopic liver transplantation for type I Crigler‐Najjar syndrome', Hepatology, vol. 6, no. 6, pp. 1259-1262. https://doi.org/10.1002/hep.1840060606
Kaufman SS, Wood RP, Shaw BW, Markin RS, Rosenthal P, Gridelli B et al. Orthotopic liver transplantation for type I Crigler‐Najjar syndrome. Hepatology. 1986 Jan 1;6(6):1259-1262. https://doi.org/10.1002/hep.1840060606
Kaufman, S. S. ; Wood, R. P. ; Shaw, B. W. ; Markin, Rodney Smith ; Rosenthal, P. ; Gridelli, B. ; Vanderhoof, J. A. / Orthotopic liver transplantation for type I Crigler‐Najjar syndrome. In: Hepatology. 1986 ; Vol. 6, No. 6. pp. 1259-1262.
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