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Autoimmune, Cholestatic and Biliary Disease
Version of Record online: 28 JAN 2010
Copyright © 2010 American Association for the Study of Liver Diseases
Volume 51, Issue 5, pages 1645–1655, May 2010
How to Cite
Davit-Spraul, A., Fabre, M., Branchereau, S., Baussan, C., Gonzales, E., Stieger, B., Bernard, O. and Jacquemin, E. (2010), ATP8B1 and ABCB11 analysis in 62 children with normal gamma-glutamyl transferase progressive familial intrahepatic cholestasis (PFIC): Phenotypic differences between PFIC1 and PFIC2 and natural history. Hepatology, 51: 1645–1655. doi: 10.1002/hep.23539
Supported by Assistance Publique, Hôpitaux de Paris and Ministère de la Santé (Paris, France); grants: Innovations Technologiques 2002/2003, Progrès Médical 2004/2005 and DHOS 2005/2006.
Potential conflict of interest: Nothing to report. Additional Supporting Information may be found in the online version of this article.
- Issue online: 22 APR 2010
- Version of Record online: 28 JAN 2010
- Accepted manuscript online: 28 JAN 2010 12:00AM EST
- Manuscript Accepted: 21 DEC 2009
- Manuscript Received: 29 AUG 2009
|HEP_23539_SupportingFigure1A-H.tif||1615K||Supporting Figure 1. Supporting Figure 1. Immunohistochemical detection of BSEP in the liver of patients with PFIC1, PFIC2 or PFIC?. A. normal human liver; B. Patient PFIC1 n ° 10 (homozygous missense and splice mutations); C. Patient PFIC2 n° 2 (homozygous nonsense mutation); D. Patient PFIC2 n° 12 (homozygous missense mutation); E. Patient PFIC2 n° 30 (heterozygous nonsense mutation); F. Patient PFIC2 n ° 29 (heterozygous missense mutation) ; G. Patient PFIC2 n° 32 )heterozygous missense mutation(; (H) PFIC? n° 9. The livers display normal canalicular staining (A, B), lack of canalicular staining (C, D, E, F and H) and focal positive and negative canalicular staining (G). PFIC?, normal-GGT PFIC of unknown cause. HE staining, original magnification: A-F: 125 ×; G and H: 250 ×.|
|HEP_23539_SupportingTable1A-C.doc||139K||Supporting Table 1|
|HEP_23539_SupportingTable2.doc||45K||Supporting Table 2|
|HEP_23539_SupportingTable3.doc||67K||Supporting Table 3|
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