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