The Mount Sinai Journal of Medicine

 

Volume 70 Number 4
September 2003
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Grand Rounds
Primary Biliary Cirrhosis: A Mount Sinai Perspective
242-250

Nancy Bach, M.D.1, and Joseph A. Odin, M.D., Ph.D.2

1Assistant Clinical Professor and 2Assistant Professor, Division of Liver Diseases, Department of Medicine, Mount Sinai School of Medicine, New York, NY.

Address all correspondence to Joseph A. Odin, M.D., Ph.D., Division of Liver Diseases, Department of Medicine, Box 1123, Mount Sinai School of Medicine, One East 100th Street, New York, NY 10029.

Supported by the Artzt Family PBC Foundation, a Schering/AASLD Advanced Hepatology Fellowship Award, and an
NIH K08 Award (NIDDK).

Adapted from a Grand Rounds presentation to the Department of Medicine, Mount Sinai School of Medicine, New York,
NY, on November 14, 2000, and updated as of December 2002.

ABSTRACT

Individuals afflicted with primary biliary cirrhosis (PBC) first undergo chronic, nonsuppurative destruction of their intrahepatic bile ducts, eventually leading to cirrhosis. Over nearly 50 years, many faculty members at the Mount Sinai School of Medicine, including Dr. Hans Popper and Dr. Fenton Schaffner, have made important contributions to our understanding of the natural history and histopathologic evolution of PBC. And today, many patients with PBC continue to be cared for at Mount Sinai. In the absence of a cure for the disease, these patients continue to be enrolled in clinical
trials and, when necessary, in the Mount Sinai liver transplant program. The establishment of the Center for the Study of Primary Biliary Cirrhosis at Mount Sinai, supported by the Artzt Family Foundation Trust, has enabled the faculty to expand both clinical and basic science initiatives related to primary biliary cirrhosis. Several of these new initiatives are described below and placed in the context of our current understanding of the immunopathogenesis of PBC.

KEY WORD

Primary biliary cirrhosis, methotrexate, celiac disease, apoptosis, cholangiocytes, autoantibody, protein oxidation, pyruvate dehydrogenase, genetics.


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