Complications of Cirrhosis

Andrew P. Keaveny • Andrés CárdenasEditors

Complications of Cirrhosis

Evaluation and Management

ISBN 978-3-319-13613-4 ISBN 978-3-319-13614-1 (eBook)DOI 10.1007/978-3-319-13614-1

Library of Congress Control Number: 2015937021

Springer Cham Heidelberg New York Dordrecht London© Springer International Publishing Switzerland 2015This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed.The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use.The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made.

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EditorsAndrew P. KeavenyDepartment of TransplantMayo ClinicJacksonvilleFloridaUSA

Andrés CárdenasInstitut de Malalties Digestives i MetaboliquesUniversity of BarcelonaBarcelonaSpain

v

Preface

The field of hepatology has never been so dynamic as it is today, with advanc-es in the understanding of disease pathogenesis, imaging, and therapeutics all providing an unparalleled opportunity to treat patients who suffer from the complications of cirrhosis. However, such treatments require careful as-sessment for efficacy and value. Moreover, some interventions such as liver transplantation are only available to a select group of patients. Determin-ing disease prognosis, who should be considered for transplant, and how to manage patients that are terminally ill with cirrhosis are challenges faced by practitioners on a daily basis.

Cirrhosis is the twelfth leading cause of mortality in the USA accounting for more than 30,000 deaths per year. In addition, it accounts for 4–5 % of deaths of people between the ages of 45 and 54 years in the USA. These num-bers are driven by the complications patients with cirrhosis suffer. In addi-tion, the economic burden of cirrhosis and its complications are considerable as the estimated national average cost in the USA for treatment ranges from $14 million to $2 billion, depending on disease etiology. Thus a thorough understanding of the diagnosis and management of cirrhosis and its compli-cations are mandatory for the practicing physician.

In this book, we have assembled an outstanding international group of experts that provide insights into the management of the most common com-plications of cirrhosis. We would like to thank them for their exceptional con-tributions that summarize the currently available management options and review increasingly important topics surrounding delivery of care to patients with cirrhosis.

We acknowledge Mr. Andy Kwan and Ms. Portia Wong from Springer, whose support for this project was invaluable to its development and suc-cessful completion.

We would like to thank our families, Gurmeet and Thomas Keaveny, Maria Luisa and Luis Cardenas, for their forbearance during the book’s preparation.

Finally, we remember the countless patients and families who we have cared for over the years—it is our sincere wish that the information so gener-ously provided by this book’s contributors will provide comfort and hope, prolong life, and alleviate suffering to current and future patients.

vii

Contents

Part I Diagnosing and Prediction—Getting It Right Now, Predicting It Right in the Future

1 Pathogenesis and Evolution of Liver Fibrosis: Cirrhosis or Cirrhoses? .................................................................................... 3Francesca Saffioti and Massimo Pinzani

2 Natural History of Cirrhosis ........................................................... 13Guadalupe Garcia-Tsao

3 Prediction of Complications of Cirrhosis: Molecular Biomarkers ..................................................................... 21Mar Coll, Elsa Solà and Pau Sancho-Bru

4 Predictors of Clinical Complications of Cirrhosis ........................ 29Joel P. Wedd, Avash Kalra and Scott W. Biggins

5 Noninvasive Markers of Fibrosis in the Assessment of Cirrhosis ....................................................................................... 39Nikhiel B. Rau and Nezam H. Afdhal

6 Diagnosis of Cirrhosis: Imaging ..................................................... 49Sumit Kumar, Sumeet K. Asrani and Jayant Talwalkar

7 Measurement of Portal Pressure and Transjugular Liver Biopsy ...................................................................................... 57Juan G. Abraldes, Philippe Sarlieve and Puneeta Tandon

Part II Current Management Strategies for the Complications of Cirrhosis

8 Reversal of Cirrhosis ....................................................................... 71Gemma Odena, Eva Stein and Ramon Bataller

9 Esophageal Varices: Primary and Secondary Prophylaxis .......... 83Humberto C. Gonzalez and William Sanchez

viii Contents

10 Acute Variceal Bleeding ................................................................... 93Àngels Escorsell, Virginia Hernández-Gea and Juan C. García-Pagán

11 Gastric and Ectopic Varices ............................................................ 103Daniel Schmidt and P. Aiden McCormick

12 Portal Hypertensive Gastropathy and Gastric Antral Vascular Ectasia ............................................................................... 111Mary Drinane and Vijay H. Shah

13 Ascites and Refractory Ascites ........................................................ 121Angelo Paredes and Arun J. Sanyal

14 Hyponatremia in Cirrhosis ............................................................. 131Elliot B. Tapper and Andrés Cárdenas

15 Hepatorenal Syndrome .................................................................... 141Elsa Solà and Pere Ginès

16 Hepatic Encephalopathy ................................................................. 149Ankit V. Patel and Jasmohan S. Bajaj

17 Cirrhotic Cardiomyopathy: Pathogenic Mechanisms and Management Strategies ............................................................ 159Hongqun Liu and Samuel S. Lee

18 Hepatopulmonary Syndrome .......................................................... 169David S. Goldberg and Michael B. Fallon

19 Portopulmonary Hypertension ....................................................... 177Rodrigo Cartin-Ceba and Michael J. Krowka

20 Portal Vein Thrombosis ................................................................... 187Dominique-Charles Valla

21 Coagulation Disorders in Patients with Cirrhosis ........................ 195Patrick G. Northup, Nicolas M. Intagliata and Neeral L. Shah

22 Hepatocellular Carcinoma .............................................................. 207Bubu A. Banini and Lewis R. Roberts

23 Cholangiocarcinoma ........................................................................ 219William C. Palmer and Denise M. Harnois

24 Bacterial Infections in Patients with Cirrhosis ............................. 229Thierry Gustot and Richard Moreau

ixContents

25 Acute-on-Chronic Liver Failure ..................................................... 243Vicente Arroyo and Javier Fernandez

26 Liver Support Systems .................................................................... 251Mansoor Bangash, Banwari Agarwal and Rajiv Jalan

27 Liver Transplantation ...................................................................... 259Nizar A. Mukhtar and Oren K. Fix

28 Assessment and Management of the Patient with Cirrhosis Undergoing Surgery ......................................................................... 271Surakit Pungpapong and David P. Nunes

29 Promoting a Healthy Lifestyle, Managing Hyperlipidemia, Diabetes Mellitus, Hypertension, and Immunizations in Patients with Cirrhosis .................................................................... 281David P. Nunes and Surakit Pungpapong

30 Malnutrition in Patients with Cirrhosis ......................................... 289Angela Vizzini and Jaime Aranda-Michel

31 Bone Disease in Patients with Cirrhosis......................................... 295Albert Parés and Núria Guañabens

Part III Cost, Quality and End-of-Life Care in Cirrhosis

32 The Economic Cost of Cirrhosis ..................................................... 309Fasiha Kanwal and Michael L. Volk

33 Delivering Quality Care to Patients with Cirrhosis: A Practical Guide for Clinicians ......................................................... 317Michael L. Volk and Fasiha Kanwal

34 Quality of Life Issues for Patients with Cirrhosis ......................... 323Zobair M. Younossi and Linda Henry

35 Palliative Care in Cirrhosis ............................................................. 337Maria L. Yataco, Robert Shannon and Andrew P. Keaveny

36 Ethical Issues in End-Stage Liver Disease: A Framework for Assessment and the Challenges ................................................. 347Stephen Chris Pappas

Index ........................................................................................................ 355

xi

Contributors

Juan G. Abraldes Cirrhosis Care Clinic, Liver Unit, Division of Gastroen-terology, University of Alberta, Edmonton, AB, Canada

Nezam H. Afdhal Division of Gastroenterology/Liver Center, Beth Israel Deaconess Medical Center, Boston, MA, USA

Banwari Agarwal Critical Care Medicine, University College London, London, UK

Jaime Aranda-Michel Department of Hepatology and Liver Transplanta-tion, Swedish Health Systems, Swedish Medical Center/First Hill Campus, Seattle, WA, USA

Vicente Arroyo Liver Unit, Institute of Digestive Diseases and Metabolism, University of Barcelona, Barcelona, Spain

Sumeet K. Asrani Baylor University Medical Center, Dallas, TX, USA

Jasmohan S. Bajaj Division of Gastroenterology, Hepatology, and Nutri-tion, Virginia Commonwealth University and McGuire VA Medical Center, Richmond, VA, USA

Mansoor Bangash Intensive Care Medicine, University College London, London, UK

Bubu A. Banini Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA

Ramon Bataller Departments of Medicine and Nutrition, University of North Carolina, Chapel Hill, NC, USA

Scott W. Biggins Division of Gastroenterology and Hepatology, University of Colorado Denver Aurora, CO, USA

Andrés Cárdenas Institut de Malalties Digestives i Metaboliques, Univer-sity of Barcelona, Hospital Clinic, Barcelona, Spain

xii Contributors

Rodrigo Cartin-Ceba Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA

Mar Coll Institut d’Investigacions Biomèdiques August Pi iSunyer (IDIBAPS), Centre Esther Koplowitz, Barcelona, Spain

Mary Drinane Gastroenterology Research Unit, Mayo Clinic, Rochester, MN, USA

Àngels Escorsell ICU-Liver Unit, Centro de Investigación Biomédica en Red de Enfermedades Hepáticas as (CIBERehd), Barcelona, Spain

Michael B. Fallon Division of Gastroenterology, Hepatology, and Nutrition, Department of Internal Medicine, The University of Texas Health Science Center at Houston, Houston, TX, USA

Javier Fernandez Liver Unit, Hospital Clinic, University of Barcelona, Barcelona, Spain

Oren K. Fix Swedish Medical Center/First Hill Campus, Seattle, WA, USA

Juan C. García-Pagán Barcelona Hepatic Hemodynamic Lab-Liver Unit, Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Hospital Clínic, Barcelona, Spain

Guadalupe Garcia-Tsao Digestive Diseases Section, Yale University School of Medicine and VA-CT Healthcare System, New Haven, CT, USA

Pere Ginès Liver Unit, Hospital Clínic, University of Barcelona School of Medicine, Barcelona, Catalunya, Spain

David S. Goldberg Division of Gastroenterology, Department of Medicine, University of Pennsylvania, Philadelphia, PA, USA

Humberto C. Gonzalez William J von Liebig Center for Transplantation and Clinical Regeneration, Mayo Clinic, Rochester, MN, USA

Núria Guañabens Metabolic Bone Diseases Unit, Department of Rheu-matology, Hospital Clínic, CIBERhed, IDIBAPS, University of Barcelona, Barcelona, Spain

Thierry Gustot Department of Gastroenterology and Hepato-Pancreatol-ogy, Erasme Hospital, Brussels, Belgium

Denise M. Harnois Mayo Clinic, Department of Transplant, Jacksonville, FL, USA

xiiiContributors

Linda Henry Betty and Guy Beatty Center for Integrated Research, Inova Health System, Falls Church, VA, USA

Virginia Hernández-Gea Barcelona Hepatic Hemodynamic Lab-Liver Unit, Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Hospital Clínic, Barcelona, Spain

Nicolas M. Intagliata Division of Gastroenterology and Hepatology, Uni-versity of Virginia, Charlottesville, VA, USA

Rajiv Jalan Hepatology, Institute of Liver and Digestive Health, Liver Fail-ure Group, University College London Medical School, London, UK

Avash Kalra University of Colorado Denver Department of Medicine, Aurora, CO, USA

Fasiha Kanwal Division of Gastroenterology and Hepatology, Baylor Col-lege of Medicine, Houston, TX 77030, USA

Andrew P. Keaveny Department of Transplant, Mayo Clinic, Jacksonville, FL, USA

Michael J. Krowka Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA

Sumit Kumar Abington Memorial Hospital, Abington, PA, USA

Samuel S. Lee Liver Unit, Cumming School of Medicine, University of Calgary, Calgary, AB

Hongqun Liu Liver Unit, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada

P. Aiden McCormick Liver Unit, St Vincent’s University Hospital and Uni-versity College Dublin, Dublin, Ireland

Richard Moreau Département Hospitalo-Univeristaire (DHU) UNITY, Service d’Hépatologie, Hôpital Beaujon, Paris, France

Nizar A. Mukhtar Division of Gastroenterology, Department of Medicine, University of California, San Francisco, CA, USA

Patrick G. Northup Division of Gastroenterology and Hepatology, Univer-sity of Virginia, Charlottesville, VA, USA

xiv Contributors

David P. Nunes Section of Gastroenterology, Boston Medical Center, Boston, MA, USA

Gemma Odena Division of Gastroenterology and Hepatology, Departments of Medicine and Nutrition, University of North Carolina, Chapel Hill, NC, USA

William C. Palmer Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, FL, USA

Stephen Chris Pappas Department of Medicine, Baylor College of Medi-cine, Houston, TX, USA

Angelo Paredes VCU Medical Center, MCV, Richmond, VA, USA

Albert Parés Liver Unit, Hospital Clínic, CIBERhed, IDIBAPS, University of Barcelona, Barcelona, Spain

Ankit V. Patel Division of Gastroenterology, Hepatology, and Nutrition, Virginia Commonwealth University and McGuire VA Medical Center, Rich-mond, VA, USA

Massimo Pinzani Institute for Liver and Digestive Health, Sheila Sherlock Liver Centre, University College London, Royal Free Hospital, London, UK

Surakit Pungpapong Department of Transplant, Mayo Clinic, Jacksonville, FL, USA

Nikhiel B. Rau Division of Gastroenterology/Liver Center, Beth Israel Dea-coness Medical Center, Boston, MA, USA

Lewis R. Roberts Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA

Francesca Saffioti Institute for Liver and Digestive Health, Sheila Sherlock Liver Centre, University College London, Royal Free Hospital, London, UK

William Sanchez William J von Liebig Center for Transplantation and Clin-ical Regeneration, Mayo Clinic, Rochester, MN, USA

Pau Sancho-Bru Institut d’Investigacions Biomèdiques August Pi iSunyer (IDIBAPS), Centre Esther Koplowitz, Barcelona, Spain

Arun J. Sanyal VCU Medical Center, MCV, Richmond, VA, USA

Philippe Sarlieve Department of Radiology, University of Alberta, Edmon-ton, AB, Canada

xvContributors

Daniel Schmidt Liver Unit, St Vincent’s University Hospital and University College Dublin, Dublin, Ireland

Vijay H. Shah Gastroenterology Research Unit, Mayo Clinic, Rochester, MN, USA

Neeral L. Shah Division of Gastroenterology and Hepatology, University of Virginia, Charlottesville, VA, USA

Robert Shannon Department of Family Medicine, Mayo Clinic, Jackson-ville, FL, USA

Elsa Solà Liver Unit, Hospital Clínic, University of Barcelona School of Medicine, Barcelona, Spain

Eva Stein Division of Gastroenterology and Hepatology, Departments of Medicine and Nutrition, University of North Carolina, Chapel Hill, NC, USA

Jayant Talwalkar Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA

Puneeta Tandon Cirrhosis Care Clinic, Liver Unit, Division of Gastroenter-ology, University of Alberta, Edmonton, AB, Canada

Elliot B. Tapper Division of Gastroenterology and Hepatology, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, USA

Dominique-Charles Valla DHU UNITY, Service d’Hépatologie, Hôpital Beaujon, Clichy, France

Angela Vizzini Department of Nutrition, Mayo Clinic, Jacksonville FL, USA

Michael L. Volk Division of Gastroenterology and Hepatology, University of Michigan Health System, Ann Arbor, MI, USA

Joel P. Wedd Division of Digestive Diseases, Emory University Hepatology and Liver Transplantation, Atlanta, GA, USA

Maria L. Yataco Department of Transplant, Mayo Clinic, Jacksonville, FL, USA

Zobair M. Younossi Center for Liver Diseases, Department of Medicine, Inova Fairfax Hospital, Falls Church, VA, USA