BookXingshun Qi, editor.
Summary: This book offers a systematic introduction to Budd-Chiari syndrome, a rare but life-threatening vascular disorder of the liver, which refers to obstructions at any site from the hepatic veins to the suprahepatic inferior vena cava. The book mainly covers the history, epidemiology, etiology and risk factors, pathology, diagnosis, pharmacological and interventional aspects, surgical treatment, etiological treatment, and prognostic assessment of Budd-Chiari syndrome. Notably, it also presents cutting-edge research findings in this field. The early diagnosis of Budd-Chiari syndrome is vital, as it allows clinicians to pursue more targeted and effective treatment strategies, thus improving patient outcomes. However, much information, including the latest advances, has not yet been fully disseminated in current clinical practice. This book addresses that gap, helping physicians to improve diagnosis and establish standard treatment strategies, helping researchers conduct clinical and experimental studies, and even helping patients and their relatives to recognize this disease.
Contents:
Intro; Foreword; Preface; Acknowledgment; Contents;
1: History of Budd-Chiari Syndrome; 1.1 The Obstruction of the Hepatic Veins: A Historic Journey from the First Reports to the Modern Concept of Budd-Chiari Syndrome; 1.2 History of Treatment and Current Management in BCS; References;
2: Epidemiology of Budd-Chiari Syndrome; 2.1 Incidence of Budd-Chiari Syndrome; 2.2 Prevalence of Budd-Chiari Syndrome in the General Population; 2.3 Sex Distribution of Patients with Budd-Chiari Syndrome; 2.4 Age Distribution of Patients with Budd-Chiari Syndrome 2.5 Prevalence of Budd-Chiari Syndrome in Particular Categories of Patients2.6 Conclusion; References;
3: Pathology of Budd-Chiari Syndrome and Hepatic Vein Obstruction; 3.1 Introduction; 3.2 Pathology; 3.2.1 Distribution of Hepatic Outflow Obstruction; 3.2.2 Vena Cava Lesions; 3.2.3 Intrahepatic Hepatic Vein Obstruction; 3.2.4 Portal Vein Involvement; 3.2.5 Patterns of Parenchymal Extinction, Atrophy, and Regeneration; 3.2.6 Small Duct Changes Including Regeneration (Progenitor Reaction); 3.2.7 Large Regenerative Nodules and Adenoma; 3.2.8 Hepatocellular Adenoma and Carcinoma 3.3 Pathogenesis of Budd-Chiari Syndrome3.4 Clinical Use of Biopsy; 3.4.1 Differential Diagnosis; 3.4.2 Assessment of Duration and Severity of Disease; References;
4: Imaging of Budd-Chiari Syndrome; 4.1 Direct Signs: Depicting the Vascular Anomalies; 4.2 Indirect Signs: Depicting the Consequences of Venous Anomalies; 4.3 Diagnostic Imaging; 4.3.1 Ultrasound Examination with Doppler Analysis; 4.3.2 Computed Tomography; 4.3.3 Magnetic Resonance Imaging; 4.3.4 Venography; 4.4 Treatment Planning; 4.5 Focal Liver Lesions and Budd-Chiari Syndrome; 4.6 Conclusion; References
5: Thrombophilia and Primary Budd-Chiari Syndrome5.1 Introduction; 5.2 Thrombophilia and Hypercoagulability; 5.3 Budd-Chiari Syndrome As a Multifactorial Disease; 5.4 Inherited Thrombophilia in Budd-Chiari Syndrome; 5.4.1 Thrombophilia Due to Loss of Anticoagulation Function; 5.4.2 Inherited Thrombophilia Due to Gain of Procoagulant Function; 5.4.2.1 Factor V Leiden; 5.4.2.2 Prothrombin Gene Mutation; 5.4.2.3 Hyperhomocysteinemia; 5.4.2.4 Factor VIII; 5.4.2.5 Other Risk Factors; 5.5 Acquired Thrombophilia in Budd-Chiari Syndrome 5.5.1 Philadelphia-Chromosome Negative Chronic Myeloproliferative Neoplasms (MPN)5.5.2 Antiphospholipid Syndrome; 5.5.3 Paroxysmal Nocturnal Haemoglobinuria (PNH); 5.5.4 Behçet's Disease and Other Acquired Disorders; 5.6 Thrombophilia Screening; 5.7 Concluding Remarks; References;
6: Budd-Chiari Syndrome and Myeloproliferative Neoplasms; 6.1 Introduction; 6.2 Budd-Chiari Syndrome and Myeloproliferative Neoplasms; 6.3 Molecular Diagnosis of MPN-Related Budd-Chiari Syndrome; 6.3.1 JAK2 V617F Mutation; 6.3.2 CALR Mutations; 6.3.3 MPL Exon 10 and JAK2 Exon 12 Mutations