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Treatment of the Crigler–Najjar Syndrome Type I with Hepatocyte Transplantation
Open accessAbstract
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. . . .
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Cites
Artificial liver: A forthcoming attraction
Sussman, N. L., & Kelly, J. H. (1993) · Hepatology · doi:10.1002/hep.1840170632 · Cited by 46
Cited by
Uygun, B. E., Soto-Gutierrez, A., Yagi, H., Izamis, M.-L., Guzzardi, M. A., Shulman, C., Milwid, J., Kobayashi, N., Tilles, A., Berthiaume, F., et al. (2010) · Nature Medicine · doi:10.1038/nm.2170 · Cited by 1,370
Emerging issues in AAV-mediated in vivo gene therapy
Colella, P., Ronzitti, G., & Mingozzi, F. (2018) · Molecular Therapy - Methods & Clinical Development · doi:10.1016/j.omtm.2017.11.007 · Cited by 881
Differentiation of human embryonic stem cells into hepatocytes in 2D and 3D culture systems in vitro
Baharvand, H., Hashemi, S. M., Kazemi Ashtiani, S., & Farrokhi, A. (2006) · The International Journal of Developmental Biology · doi:10.1387/ijdb.052072hb · Cited by 429
Decellularized human liver as a natural 3D-scaffold for liver bioengineering and transplantation
Mazza, G., Rombouts, K., Rennie Hall, A., Urbani, L., Vinh Luong, T., Al-Akkad, W., Longato, L., Brown, D., Maghsoudlou, P., Dhillon, A. P., et al. (2015) · Scientific Reports · doi:10.1038/srep13079 · Cited by 415
Human hepatocyte transplantation: current experience and future challenges
Dhawan, A., Puppi, J., Hughes, R. D., & Mitry, R. R. (2010) · Nature Reviews Gastroenterology & Hepatology · doi:10.1038/nrgastro.2010.44 · Cited by 362
Metadata
- Authors
- Fox, I. J., Chowdhury, J. R., Kaufman, S. S., Goertzen, T. C., Chowdhury, N. R., Warkentin, P. I., Dorko, K., Sauter, B. V., & Strom, S. C.
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1998-05-14
- Volume
- 338
- Issue
- 20
- Pages
- 1422-1427
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W1979686921
- Cited by
- 1,023
- References
- 1
- FWCI
- 43.21
- Type
- journal article
- Language
- en
- Open access
- bronze
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- CoChat. (2026). Citation check: Treatment of the Crigler–Najjar Syndrome Type I with Hepatocyte Transplantation. https://app.cochat.ai/papers/10.1056/nejm199805143382004
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- Fox, I. J., Chowdhury, J. R., Kaufman, S. S., Goertzen, T. C., Chowdhury, N. R., Warkentin, P. I., Dorko, K., Sauter, B. V., & Strom, S. C. (1998). Treatment of the Crigler–Najjar Syndrome Type I with Hepatocyte Transplantation. New England Journal of Medicine. https://doi.org/10.1056/nejm199805143382004
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