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Can I read Recurrent low gamma-glutamyl Transpeptidase cholestasis following liver transplantation for bile salt export pump (BSEP) disease (posttransplant recurrent BSEP disease) on EtoBox?
Recurrent low gamma-glutamyl Transpeptidase cholestasis following liver transplantation for bile salt export pump (BSEP) disease (posttransplant recurrent BSEP disease) by Leah Siebold; Andre A. S. Dick; Richard Thompson; Giuseppe Maggiore; Emanuel Jacquemin; Ronald Jaffe; Sandra Strautnieks; Tassos Grammatikopoulos; Simon Horslen; Peter F. Whitington; Benjamin L. Shneider is a Medicine article available to read on EtoBox.
What is Recurrent low gamma-glutamyl Transpeptidase cholestasis following liver transplantation for bile salt export pump (BSEP) disease (posttransplant recurrent BSEP disease) about?
Bile salt export pump (BSEP) deficiency is a hereditary cholestatic syndrome that results from mutations in the ABCB11 (ATPbinding cassette B11) gene. Severely affected patients develop end-stage liver disease in the first decade of life. Liver transplantation has traditionally been thought of as curative for BSEP disease. We describe the clinical course of 6 patients who developed recurrent low c-glutamyl transpeptidase cholestasis, that mimicks BSEP disease, following transplantation. All had documented genetic defects in ABCB11 that were predicted to lead to a congenital absence of BSEP protein. The time to development of recurrence was variable; 4 underwent repeat liver transplantation for complications of recurrent disease and all 4 again developed recurrent disease after retransplantation. Siblings of these patients who also underwent liver transplantation for BSEP disease have not developed ' 'recurrent'' disease. Three of the patients with ''recurrent'' disease ultimately died, 2 as a direct result of complications of their liver disease.
Who reads Recurrent low gamma-glutamyl Transpeptidase cholestasis following liver transplantation for bile salt export pump (BSEP) disease (posttransplant recurrent BSEP disease)?
It is typically read by researchers, students, and practitioners in Medicine.
- Author
- Leah Siebold; Andre A. S. Dick; Richard Thompson; Giuseppe Maggiore; Emanuel Jacquemin; Ronald Jaffe; Sandra Strautnieks; Tassos Grammatikopoulos; Simon Horslen; Peter F. Whitington; Benjamin L. Shneider
- Publisher
- Ovid Technologies (Wolters Kluwer Health)
- Published
- 2010
- Language
- EN
- Field
- Medicine (Health Sciences)