Clinical hepatology / 2.

1537 Pages·2010·39.6 MB·English

by  Henryk Dancygier

Clinical Hepatology
Henryk Dancygier
Clinical Hepatology
Principles and Practice
of Hepatobiliary Diseases
Volume 2
With Contributions by
H.-D. Allescher · U. Beuers · H. Blum · R. S. Brown, Jr · E. Cay · H. Dancygier
S. Dasarathy · C. F. Dietrich · M. Doss · A. Dragan · S. M. Erturk · S. A. Fink
L. S. Friedman · G. Gerken · D. Häussinger · P. Hilgard · M. A. Kern · A. Koch
J. H. Lefkowitch · U. Leuschner · T. Longerich · A. J. McCullough · U. Merle
J. Mössner · S. Mueller · A. Niedenthal · C. Niederau · B. Riemann · J. N. Rogart
P. Ros · C. Sarrazin · P. Schirmacher · O. Schober · H. K. Seitz · S. H. Sigal
F. Stenschke · U. Stölzel · C. P. Strassburg · W. Stremmel · S. Susser · C. Trautwein
M. Tröltzsch · I. S. Weisberg · M. Wiedman · C. Wittekind · H. Witzigmann
P. S. Yachimski · S. Zeuzem
Foreword by Scott L. Friedman
Henryk Dancygier, MD
Professor of Medicine
Chair, Department of Medicine II
Klinikum Offenbach, Goethe University Frankfurt/Main
Starkenburgring 66
63069 Offenbach, Germany
email: [email protected]
Adjunct Professor of Medicine
Department of Medicine, Division of Liver Diseases
Mount Sinai School of Medicine
New York, NY, USA
email: [email protected]
ISBN: 978-3-642-04509-7 e-ISBN: 978-3-642-04519-6
DOI: 10.1007/978-3-642-04519-6
Springer Heidelberg Dordrecht London New York
Library of Congress Control Number: 2009929701
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Dedication
Dedicated to Dr. Herbert Falk (1924–2008).
A humanist of great generosity, supporter of scientifi c advancement
and the always visible, but never dominant magnanimous patron of
global medical education.
v
Foreword
Modern hepatology would be unrecognizable to those clinicians and pathologists,
who founded the fi eld only a generation ago. From a discipline that was largely obser-
vational – with few diagnostic tests and even fewer therapies – has emerged an excit-
ing area that is among the most rapidly changing in all of medicine, and which now
offers remarkable new tools and treatments. This textbook edited by Professor
Dancygier beautifully captures this dynamism of hepatology, and in doing so provides
a remarkably complete opus. The work is beautifully laid out exactly as a clinician
would think, weaving in the science underlying clinical hepatology with precision and
clarity. Standardized and gorgeous drawings, comprehensive tables, and a very consis-
tent style are among its most valuable assets – quite simply, this book is fun to read!
While thoroughly modern, this work still proudly draws upon the roots of our
specialty. A strong emphasis on pathology, patterns of injury, clinical presentations of
disease, and approaches to clinical problems harken back to hepatology’s earliest
treatises. The book is particularly reminiscent of early editions of Dame Sheila
Sherlock’s classic single-author textbook, the fi fth edition of which I read cover-to-
cover while spending 3 months at the Royal Free Hospital as a medical student in
1977; a signed copy sits proudly on my shelf to this day, and I suspect that many will
come to value Professor Dancygier’s book for many of its similar virtues. The highly
personal stamp of Professor Dancygier infuses this book with cohesion, and conveys
the wonders of clinical hepatology. Underscoring another enduring feature of our
specialty is the book’s transatlantic fl avor, with authors from throughout Germany
and the USA. This connection between our two countries is also personal – Professor
Dancygier’s invitation for me to speak at a conference in Munich in 1989 was my fi rst
international meeting, and I am proud that our professional association and friendship
continue to this day as a result of that fi rst meeting 20 years ago.
I am delighted to be associated with Clinical Hepatology. Thus, it is a great per-
sonal privilege to introduce this unique and valuable textbook, which is sure to appeal
to practitioners of today and ignite a spark of enthusiasm among the hepatologists of
tomorrow.
Scott L. Friedman, M.D.
President, American Association for the Study of Liver Diseases
Fishberg Professor and Chief, Division of Liver Diseases
The Mount Sinai School of Medicine
vii
Preface
Clinical hepatology is thriving. Hepatology has evolved from a pure diagnostic art to
a science in which new treatment options are emerging at a quick pace. At this excit-
ing time, based on the gratifying success of the German Edition of 2003, Springer
Publishers have asked me to prepare a US-American/International edition. Gravely
miscalculating the amount of labor, I gladly accepted this challenge.
The present book is not merely an updated version of the German edition but a
completely new work with new US-American authors, a transatlantic endeavor. In
common with its German predecessor, however, it not only aims to provide know-
ledge in hepatology but also to promote an understanding of liver diseases, and to
create joy in dealing with clinical hepatological problems. It is intended for every-
body caring for adult patients with hepatobiliary problems, particularly gastroente-
rologists/hepatologists, internists and clinical pathologists.
My aim was not to provide an encyclopedic behemoth. Instead, I intended to cre-
ate a comprehensive, up-to-date (references until early 2009 are included), practical
and readable book that outlines the current standards of diagnosis and treatment in
hepatology and its associated biliary disorders. By elaborating on concepts in hepa-
tology, disease mechanisms, common clinical problems and rare diseases alike it tries
to serve the needs not only of the novice in hepatology, but also of the experienced
practicing clinician. Ultimately, the success of the book will be determined by its
ability to provide answers to clinically relevant questions and to guide clinical
decisions.
The competent clinical hepatologist, like hardly any other clinician, has to inte-
grate histopathological, biochemical, immunological, instrumental and clinical skills.
The organization of the text follows this principle. It is divided into 3 main parts with
30 sections covering Basic Principles, Clinical Methods, and Hepatobiliary Diseases.
Starting from basic concepts the fi eld of clinical hepatology gradually unfolds. Unlike
other Hepatology Textbooks I preferred not to include a “stand alone” chapter on
liver pathology. Instead hepatopathology has been integrated throughout the entire
text as it represents an integral part of clinical hepatology. The fi rst fi ve sections inte-
grate structure and function of the liver and basically provide a general pathology of
the liver. The clinical chapters are stringently organized and uniformly structured to
enable rapid retrieval of the desired information. In order to enhance readability I
have accepted some redundancy, especially in chapters dealing with hepatocellular
transporters.
I am indebted to my coauthors, all renowned experts in hepatology. Without their
help the creation of a textbook of this volume would not have been possible. My
ix
x Preface
special thanks go to a young gastroenterologist from Yale, Jason Rogart. He not only
served as a proof reader for the contributions by authors whose native language is not
English, but also as an author of several chapters and as an editorial assistant. My
thanks go also to Ms. Annette Hinze, Meike Stoeck, and Mr. Claus-Dieter Bachem
from Springer Publishers who skillfully supported the development of the Textbook.
Last but not least I thank my wife Hellena for her endurance and unwavering support.
After immerging into the project and resurfacing after fi nishing the last chapter she
was still there.
Offenbach, May 2009 Henryk Dancygier
Volume 1
A. Basic Principles
Part I Structure and Function of the Liver
Part II Pathophysiology and Morphology of Liver Injury
B. Clinical Methods
Part III Evaluation of the Patient with Hepatobiliary Disease
Volume 2
C. Hepatobiliary Diseases
Part IV Diseases of the Liver
Part V Diseases of the Gallbladder and Extrahepatic Bile Ducts
Contents of Volume 1
A. Basic Principles
Part I Structure and Function of the Liver . . . . . . . . . . . . . . . . . . 3
Section I Embryology, Anatomy, and Histology. . . . . . . . . . . . . . . . . . . . . . 5
1 Embryonic Development. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
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2 Gross Anatomy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
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3 Microscopic Anatomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15
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Section II Fundamentals of Hepatic Physiology and Biochemistry. . . . . . 53
4 Hepatic Circulation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55
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5 Hepatocellular Transport . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61
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6 Hepatic Metabolism. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75
Henryk Dancygier, Uta Merle, Wolfgang Stremmel, and Claus Niederau
7 Formation and Secretion of Bile and Bilirubin Metabolism . . . . . . . . 103
Ulrich Leuschner
8 Hepatic Biotransformation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127
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9 Functional Heterogeneity and Metabolic Zonation. . . . . . . . . . . . . . . . 131
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10 Liver Cell Hydration and Cell Function . . . . . . . . . . . . . . . . . . . . . . . . 137
Dieter Häussinger
11 The Liver as an Immune Organ. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141
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