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Previous Volume 338:1422-1427 May 14, 1998 Number 20 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

	Full Text PDF Editorial by Lake, J. R. Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited PubMed Citation

Crigler–Najjar syndrome type I is a recessively inherited disorder characterized by severe unconjugated hyperbilirubinemia beginning at birth. The syndrome results from an absence of hepatic uridine diphosphoglucuronate (UDP) glucuronosyltransferase activity, which is essential for the conjugation and excretion of bilirubin. Because of the accumulation of unconjugated bilirubin in plasma, patients are at risk for kernicterus.¹ Although phototherapy successfully reduces serum bilirubin levels, patients are again at risk for kernicterus around the time of puberty, when phototherapy becomes less effective.² The necessary daily duration of phototherapy often approaches 14 to 16 hours. At present, liver transplantation is the only definitive treatment.^3,4

. . . [Full Text of this Article]

Case Report

Methods

The Hepatocyte Donor

Isolation and Processing of Hepatocytes

Viability and Function of Hepatocytes

Transplantation of Hepatocytes

Measurement of Bilirubin-UDP-Glucuronosyltransferase Activity and Bilirubin Conjugates

Results

Hemodynamic and Biochemical Response to the Intraportal Infusion of Hepatocytes

Changes in the Serum Bilirubin Level and Requirement for Phototherapy

Analysis of Bile

Liver Biopsy and Bilirubin-UDP-Glucuronosyltransferase Activity

Discussion

Source Information

From the Departments of Surgery (I.J.F.), Pediatrics (S.S.K.), Radiology (T.C.G.), and Pathology and Laboratory Medicine (P.I.W.), University of Nebraska Medical Center, Omaha; the Departments of Medicine and Molecular Genetics and the Marion Bessin Liver Research Center, Albert Einstein College of Medicine, New York (J.R.C., N.R.C., B.V.S.); and the Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh (K.D., S.C.S.). Presented in part at the 48th annual meeting of the American Association for the Study of Liver Diseases, Chicago, November 7–11, 1997.

Address reprint requests to Dr. Fox at the Department of Surgery, University of Nebraska Medical Center, 600 S. 42nd St., Omaha, NE 68198-3285.

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