Budd-Chiari Syndrome

253 Pages·2020·7.127 MB·English

by  Xingshun Qi

Budd-Chiari
Syndrome
Xingshun Qi
Editor
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Budd-Chiari Syndrome
Xingshun Qi
Editor
Budd-Chiari Syndrome
Editor
Xingshun Qi
Department of Gastroenterology
General Hospital of Northern Theater Command
Shenyang
Liaoning
China
ISBN 978-981-32-9231-4 ISBN 978-981-32-9232-1 (eBook)
https://doi.org/10.1007/978-981-32-9232-1
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Foreword

The reader is here proposed a book comprehensively covering the description and
management of Budd–Chiari syndrome, the disease resulting from the obstruction
of the hepatic venous outflow tract. This is a complex disorder where the concur-
rence of several of a dozen different conditions is needed. That this concurrence is
uncommon explains the extreme rarity of the disease. This disorder is also complex
in as much as we do not really know why its clinical expression varies so widely
from patient to patient. Natural compensatory mechanisms that are or are not set in
motion are probably involved, but we do not clearly understand how to mobilize
them in the sickest patients and to protect them in others. Such a complexity makes
the scope of knowledge to be considered, and the strength of the decisions to be
made, particularly challenging.
As an outstanding achievement, this book provides a full coverage of all signifi-
cant topics authored by the best possible investigators. As all advancing topics,
those of Budd–Chiari syndrome have long been habited by controversies and debate.
These controversies are also accounted for in this book, which will ultimately help
the reader in navigating within the complexity rather than providing a simple but
erroneous single fitting management for all patients.
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vi Foreword
The coordinator is to be strongly congratulated for his most successful efforts for
the immense benefit to the patients.
Dominique Valla
Service d’Hépatologie, DHU Unity,
Pôle des Maladies de l’Appareil Digestif,
Hôpital Beaujon, Assistance Publique-Hôpitaux de Paris,
Clichy, France
Centre de Référence des Maladies Vasculaires du Foie,
French Network for Rare Liver Diseases,
European Reference Network Rare-Liver,
Clichy, France
Preface
Budd–Chiari syndrome is a vascular disorder of the liver which can cause fulminant
liver injury and lethal portal hypertension-related complications. My extreme inter-
est at this disorder is enlightened by my doctoral program under the mentorship of
Prof. Daiming Fan and Prof. Guohong Han and is further aggravated by numerous
excellent publications by Prof. Dominique Charles Valla and his Clichy team.
The first work that I have done in this field, in retrospect, is to systematically
review the worldwide literature regarding the prevalence of JAK2 V617F mutation
in Budd–Chiari syndrome. At that time, no relevant work had been performed in
Chinese population, despite there are a relatively large number of patients with
Budd–Chiari syndrome in China. Thus, my second work focused on the data from
Chinese patients with Budd–Chiari syndrome. Notably, we found a huge difference
in the prevalence of JAK2 V617F mutation between West and China. Afterward,
with the support of Prof. Guohong Han, 169 Chinese patients with Budd–Chiari
syndrome, who were treated by his team, were invited to examine nearly all throm-
botic risk factors recommended by the major Western practice guidelines. The work
further suggested the discrepancy in the etiological distribution of Budd–Chiari
syndrome between West and China. Subsequently, the interventional treatment for
Budd–Chiari syndrome, including percutaneous recanalization and transjugular
intrahepatic portosystemic shunt, is also involved in my doctoral program.
Reading new papers about Budd–Chiari syndrome via the PubMed database has
become a part of my daily routine. Meanwhile, I am enthusiastic on systematically
reviewing the scientific publications about this disorder in the contemporary era,
which can clarify what have been done in the past and indicate what should be done
in the future. Besides, the systematic review of literature suggests that “Valla DC”
is the most frequent author. As known, Prof. Valla should have the largest number of
high-impact publications in this field, which inspire more investigators, including
me, to do further in-depth work. In addition, Prof. Valla, a European hepatologist, is
not only an important contributor of both European Association for the Study of the
Liver Practice Guideline and Baveno VI consensus regarding Budd–Chiari syn-
drome, which are representative in Europe, but also the only one un-American con-
tributor for American Association for the Study of Liver Diseases Practice Guideline
regarding Budd–Chiari syndrome, which is representative in the United States. To
the best of my knowledge, he is held in high esteem by peers, and especially some
experts propose that “Budd–Chiari syndrome,” a term originating from the family
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viii Preface
names of two doctors who recognized this disorder at the earliest, should be modi-
fied as “Budd–Chiari–Valla syndrome.” More importantly, he is very kind and
warmhearted to give insightful comments and improve my work since the beginning
of my research career.
On the basis of the disease severity and my interest and experience, I actively
launch this book project in the Springer, which is the leading publisher in this world,
and invite international specialists to summarize the most updated research findings
and further promote the physician’s perception and judgment of Budd–Chiari
syndrome.
Shenyang, China Xingshun Qi
February 6, 2019
Acknowledgment
First of all, as for the success of this book project “Budd–Chiari syndrome,” I am
particularly grateful to the great contributions by all chapter authors who are very
skilled at the management of Budd–Chiari syndrome and have published lots of
high-impact papers in this field. I deeply understand that they are often very busy in
their academic commission and personal business. What deserves my deepest admi-
ration and respect is that some chapter authors still insist on finishing the chapters
in spite of their own illness or their parent’s passing.
I should also express my sincere thanks for Miss Xuewen Li and Joanne Jiao, the
in-house editors of the Springer, and Miss Kripa Guruprasad, the coordinator of the
book project, who made all-out efforts to publish this book more smoothly and
efficiently.
Finally, I am very indebted to my wife, Jun Liu, for her continuous support for
my work.
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Contents
1 History of Budd–Chiari Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
Monica Pellone, Alberto Zanetto, and Marco Senzolo
2 Epidemiology of Budd–Chiari Syndrome . . . . . . . . . . . . . . . . . . . . . . . . 15
Nicoletta Riva and Walter Ageno
3 Pathology of Budd–Chiari Syndrome and Hepatic
Vein Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27
Ian R. Wanless
4 Imaging of Budd–Chiari Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
Morgane Van Wettere, Onorina Bruno, Valérie Vilgrain,
and Maxime Ronot
5 Thrombophilia and Primary Budd–Chiari Syndrome . . . . . . . . . . . . . 57
Massimo Primignani
6 Budd–Chiari Syndrome and Myeloproliferative Neoplasms . . . . . . . . 73
Valerio De Stefano and Elena Rossi
7 Budd–Chiari Syndrome and Paroxysmal Nocturnal
Hemoglobinuria. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89
Andrés Lázaro Brodsky and Gregorio Raúl Cordini
8 Budd–Chiari Syndrome in Patients with Antiphospholipid
Antibodies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107
Sciascia Savino and Radin Massimo
9 Hepatocellular Carcinoma in Budd–Chiari Syndrome . . . . . . . . . . . . . 113
Nawel Afredj and Nabil Debzi
10 Anticoagulation for Budd–Chiari Syndrome . . . . . . . . . . . . . . . . . . . . . 131
Audrey Payancé and Aurélie Plessier
11 Percutaneous Recanalization for Budd–Chiari Syndrome . . . . . . . . . . 147
Amar Mukund and Arpit Taunk
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