E-Book, Englisch, 990 Seiten
Dancygier Clinical Hepatology
1. Auflage 2009
ISBN: 978-3-642-04519-6
Verlag: Springer
Format: PDF
Kopierschutz: 1 - PDF Watermark
Principles and Practice of Hepatobiliary Diseases: Volume 2
E-Book, Englisch, 990 Seiten
ISBN: 978-3-642-04519-6
Verlag: Springer
Format: PDF
Kopierschutz: 1 - PDF Watermark
Clinical Hepatology – Principles and Practice of Hepatobiliary Diseases provides clear and comprehensive coverage of the etiology, mechanisms of disease, diagnosis, and practical management of the entire spectrum of liver and biliary disorders. It also affords an excellent, evidence-based review of the rapidly expanding field of hepatobiliary diseases.
Autoren/Hrsg.
Weitere Infos & Material
1;Copyright Page;4
2;Dedication;5
3;Foreword;6
4;Preface;7
5;Volume 2;9
6;Contents of Volume 2;14
7;Contributors;19
8;Part IV: Diseases of the Liver;26
8.1;Section X: Developmental Anomalies;27
8.1.1;Chapter 55;28
8.1.1.1;Malformations and Malpositions of the Liver;28
8.1.1.1.1;Riedel’s Lobe;28
8.1.1.1.2;Accessory Liver Lobe(s);28
8.1.1.1.3;Ectopy;28
8.1.1.1.4;Extraabdominal Location of the Liver;29
8.1.1.1.5;Intraabdominal Displacement of the Liver;29
8.1.1.1.6;Agenesis;29
8.1.1.1.7;Atrophy;29
8.1.1.1.8;Zahn’s Grooves;29
8.1.1.1.9;“Corset Liver”;29
8.1.1.1.10;References;30
8.1.2;Chapter 56;31
8.1.2.1;Bile Duct Anomalies;31
8.1.2.1.1;Congenital Liver Fibrosis;31
8.1.2.1.2;Caroli’s Disease and Caroli’s Syndrome;33
8.1.2.1.2.1;Defi nition;33
8.1.2.1.2.2;Pathogenesis;33
8.1.2.1.2.3;Pathology;33
8.1.2.1.2.4;Diagnosis;33
8.1.2.1.2.4.1;Clinical Manifestations;33
8.1.2.1.2.4.2;Technical Examinations;33
8.1.2.1.2.4.3;Differential Diagnosis;33
8.1.2.1.2.4.4;Course and Prognosis;33
8.1.2.1.2.4.5;Therapy;34
8.1.2.1.3;Von Meyenburg Complexes;34
8.1.2.1.4;Biliary Atresia;34
8.1.2.1.4.1;Defi nition;34
8.1.2.1.4.2;Epidemiology;35
8.1.2.1.4.3;Etiology and Pathogenesis;35
8.1.2.1.4.4;Pathology;35
8.1.2.1.4.5;Diagnosis;35
8.1.2.1.4.5.1;Clinical Manifestations;35
8.1.2.1.4.5.2;Technical Examinations;36
8.1.2.1.4.6;Prognosis and Treatment;36
8.1.2.1.4.7;References;36
8.1.3;Chapter 57;37
8.1.3.1;Liver Cysts and Polycystic Liver Disease;37
8.1.3.1.1;Liver Cysts;37
8.1.3.1.1.1;Defi nition;37
8.1.3.1.1.2;Epidemiology;37
8.1.3.1.1.3;Etiology;38
8.1.3.1.1.4;Pathogenesis;38
8.1.3.1.1.5;Diagnosis;38
8.1.3.1.1.5.1;Clinical Manifestations;38
8.1.3.1.1.5.2;Technical Examinations;38
8.1.3.1.1.6;Differential Diagnosis;38
8.1.3.1.1.7;Complications;39
8.1.3.1.1.8;Natural History and Prognosis;39
8.1.3.1.1.9;Therapy;39
8.1.3.1.2;Polycystic Liver Disease;39
8.1.3.1.2.1;Defi nition;39
8.1.3.1.2.2;Epidemiology;39
8.1.3.1.2.3;Etiology and Pathogenesis;39
8.1.3.1.2.4;Pathology;40
8.1.3.1.2.5;Diagnosis;40
8.1.3.1.2.5.1;Clinical Manifestations;40
8.1.3.1.2.5.2;Technical Examinations;40
8.1.3.1.2.6;Natural History and Prognosis;40
8.1.3.1.2.7;Therapy;40
8.1.3.1.2.8;References;41
8.1.4;Chapter 58;42
8.1.4.1;Vascular Anomalies;42
8.1.4.2;References;43
8.2;Section XI: Circulatory and Vascular Disorders;44
8.2.1;Chapter 59;45
8.2.1.1;Hepatic Veins;45
8.2.1.1.1;Acute Hepatic Congestion;45
8.2.1.1.2;Chronic Hepatic Congestion;45
8.2.1.1.3;Budd-Chiari Syndrome;46
8.2.1.1.3.1;Defi nition;46
8.2.1.1.3.2;Epidemiology;46
8.2.1.1.3.3;Etiology and Pathogenesis;46
8.2.1.1.3.4;Pathology;48
8.2.1.1.3.5;Diagnosis;48
8.2.1.1.3.5.1;Clinical Manifestations;48
8.2.1.1.3.5.2;Laboratory Findings;48
8.2.1.1.3.5.3;Imaging Techniques;48
8.2.1.1.3.6;Prognosis and Treatment;49
8.2.1.1.4;Central Hyaline Sclerosis;49
8.2.1.1.5;Prolapse of Hepatocytes into the Central Veins;50
8.2.1.1.6;References;50
8.2.2;Chapter 60;52
8.2.2.1;Sinusoids;52
8.2.2.1.1;Sinusoidal Dilatation;52
8.2.2.1.2;Peliosis Hepatis;53
8.2.2.1.2.1;Defi nition;53
8.2.2.1.2.2;Epidemiology;53
8.2.2.1.2.3;Etiology and Pathogenesis;53
8.2.2.1.2.4;Pathology;53
8.2.2.1.2.5;Diagnosis;53
8.2.2.1.2.5.1;Signs and Symptoms;53
8.2.2.1.2.5.2;Laboratory Parameters;53
8.2.2.1.2.5.3;Imaging Techniques;53
8.2.2.1.2.6;Therapy and Prognosis;54
8.2.2.1.3;Sinusoidal Obstruction Syndrome;54
8.2.2.1.3.1;Defi nition;54
8.2.2.1.3.2;Epidemiology;54
8.2.2.1.3.3;Etiology and Pathogenesis;54
8.2.2.1.3.4;Pathology;55
8.2.2.1.3.5;Diagnosis;55
8.2.2.1.3.5.1;Signs and Symptoms;56
8.2.2.1.3.5.2;Laboratory Parameters;56
8.2.2.1.3.5.3;Imaging Techniques;56
8.2.2.1.3.6;Differential Diagnosis;56
8.2.2.1.3.7;Course and Prognosis;56
8.2.2.1.3.8;Treatment;56
8.2.2.1.4;Changes of Sinusoidal Contents;57
8.2.2.1.5;Perisinusoidal Lesions;57
8.2.2.1.6;References;58
8.2.3;Chapter 61;59
8.2.3.1;Portal Vein;59
8.2.3.1.1;Portal Vein Thrombosis;59
8.2.3.1.1.1;Acute Portal Vein Thrombosis;59
8.2.3.1.1.2;Chronic Portal Vein Thrombosis;60
8.2.4;Chapter 62;65
8.2.4.1;Hepatic Arteries;65
8.2.4.1.1;Congentital Anomalies;65
8.2.4.1.2;Hepatic Artery Occlusion;65
8.2.4.1.3;Hypoxic Hepatitis;66
8.2.4.1.3.1;Defi nition;66
8.2.4.1.3.2;Epidemiology;66
8.2.4.1.3.3;Etiology and Pathogenesis;66
8.2.4.1.3.4;Pathology;66
8.2.4.1.3.5;Diagnosis;67
8.2.4.1.3.5.1;Clinical Manifestations;67
8.2.4.1.3.5.2;Laboratory Examinations;67
8.2.4.1.3.5.3;Imaging Techniques;67
8.2.4.1.3.6;Differential Diagnosis;67
8.2.4.1.3.7;Prognosis and Treatment;67
8.2.4.1.4;Ischemic Cholangiopathy;67
8.2.4.1.5;Aneurysms;67
8.2.4.1.5.1;Etiology;68
8.2.4.1.5.2;Diagnosis;68
8.2.4.1.5.2.1;Clinical Manifestations;68
8.2.4.1.5.2.2;Imaging Techniques;68
8.2.4.1.5.3;Prognosis and Therapy;68
8.2.4.1.6;Arterioportal Fistula;68
8.2.4.1.7;Arteritis;69
8.2.4.1.8;Amyloidosis;70
8.2.4.1.9;Vascular Tumors;70
8.2.4.1.10;References;70
8.3;Section XII: Infectious Liver Diseases;71
8.3.1;Chapter 63;72
8.3.1.1;Viral Infections by Hepatotropic Viruses;72
8.3.1.1.1;63.1 Molecular Biology and Immunobiology of Hepatitis Viruses – Approach to Diagnosis and Therapy;73
8.3.1.1.1.1;Molecular Biology of Hepatitis Viruses;73
8.3.1.1.1.1.1;Molecular Biology of Hepatitis A Virus;73
8.3.1.1.1.1.1.1;Morphology, Genome Organization and Replication;73
8.3.1.1.1.1.1.2;Genotypes and Serotypes;74
8.3.1.1.1.1.2;Molecular Biology of Hepatitis E Virus;75
8.3.1.1.1.1.2.1;Morphology and Genome Organization;75
8.3.1.1.1.1.2.2;Genotypes and Serotypes;75
8.3.1.1.1.1.2.3;Replication of the Hepatitis E Virus;76
8.3.1.1.1.1.3;Molecular Biology of Hepatitis B Virus;76
8.3.1.1.1.1.3.1;Genome Organization;76
8.3.1.1.1.1.3.2;HBV Envelope Proteins;77
8.3.1.1.1.1.3.3;HBV Capsid Proteins;78
8.3.1.1.1.1.3.4;HBV Polymerase;80
8.3.1.1.1.1.3.5;HBx-Antigen;80
8.3.1.1.1.1.3.6;Genotypes, Serotypes and HBV-Variants;80
8.3.1.1.1.1.3.7;HBV-Replication Cycle;80
8.3.1.1.1.1.3.8;Hepatitis B Immunology;83
8.3.1.1.1.1.3.9;Immune Response of Acute and Reactivated HBV Infection;83
8.3.1.1.1.1.3.10;Immune Response in Chronic Hepatitis B;84
8.3.1.1.1.1.4;Molecular Biology of Hepatitis D Virus;84
8.3.1.1.1.1.4.1;Genotypes;85
8.3.1.1.1.1.4.2;Hepatitis Delta Antigen;85
8.3.1.1.1.1.4.3;Replication;86
8.3.1.1.1.1.4.4;Immunology;86
8.3.1.1.1.1.4.5;HBV and HDV;87
8.3.1.1.1.1.5;Molecular Biology of Hepatitis C Virus;87
8.3.1.1.1.1.5.1;Genome Organisation;87
8.3.1.1.1.1.5.2;HCV-Quasispecies;88
8.3.1.1.1.1.5.3;Interferon-sensitivity Determining Region in the NS5A Gene;89
8.3.1.1.1.1.5.4;Structural HCV Proteins;89
8.3.1.1.1.1.5.5;Non-structural HCV Proteins;89
8.3.1.1.1.1.5.6;Genotypes and Subtypes;90
8.3.1.1.1.1.5.7;Replication Cycle;90
8.3.1.1.1.2;Diagnostics of Hepatitis Viruses;91
8.3.1.1.1.2.1;Hepatitis A;91
8.3.1.1.1.2.1.1;Anti-HAV-Antibodies and Nucleic Acid Amplification;91
8.3.1.1.1.2.1.2;Conclusion for HAV Diagnosis;92
8.3.1.1.1.2.2;Hepatitis E;92
8.3.1.1.1.2.2.1;Anti-HEV Antibodies and Nucleic Acid Amplification;92
8.3.1.1.1.2.2.2;Conclusion for HEV Diagnosis;93
8.3.1.1.1.2.3;Hepatitis B;93
8.3.1.1.1.2.3.1;Serological Diagnosis of HBV;93
8.3.1.1.1.2.3.1.1;HBsAg and Anti-HBs-Antibodies;95
8.3.1.1.1.2.3.1.2;PreS-Ag and Anti-PreS-Antibodies;95
8.3.1.1.1.2.3.1.3;Anti-HBc-Antibodies;96
8.3.1.1.1.2.3.1.4;HBeAg and Anti-HBe-Antibodies;96
8.3.1.1.1.2.3.2;Molecular Diagnosis of HBV;96
8.3.1.1.1.2.3.2.1;Qualitative HBV-PCR;96
8.3.1.1.1.2.3.2.2;Quantitative Methods for HBV-DNA Measurement;97
8.3.1.1.1.2.3.2.3;Cobas Amplicor™ HBV-Monitor;97
8.3.1.1.1.2.3.2.4;Branched HBV-DNA (bDNA) Assay;98
8.3.1.1.1.2.3.2.5;Real Time PCR: Cobas Ampliprep/Cobas TaqMan Assay;98
8.3.1.1.1.2.3.2.6;Real Time PCR: Abbott RealTime HBV Assay;99
8.3.1.1.1.2.3.2.7;Real Time PCR: Artus HBV PCR Kit;99
8.3.1.1.1.2.3.3;Conclusion for HBV Diagnosis;99
8.3.1.1.1.2.3.3.1;Acute HBV Infection;99
8.3.1.1.1.2.3.3.2;Chronic HBV Infection;99
8.3.1.1.1.2.4;Hepatitis D;100
8.3.1.1.1.2.4.1;Hepatitis Delta Serology;100
8.3.1.1.1.2.4.2;Molecular Diagnosis of Hepatitis Delta;101
8.3.1.1.1.2.4.3;Morphological HDV Diagnosis;102
8.3.1.1.1.2.4.4;Conclusion for HDV Diagnosis;102
8.3.1.1.1.2.5;Hepatitis C;102
8.3.1.1.1.2.5.1;Serological HCV Diagnostics;103
8.3.1.1.1.2.5.1.1;Enzyme Immunoassays (IgG and IgM);103
8.3.1.1.1.2.5.1.2;Confirmation Tests;103
8.3.1.1.1.2.5.2;Molecular Based HCV Detection Methods;104
8.3.1.1.1.2.5.2.1;Qualitative HCV-RNA Detection;104
8.3.1.1.1.2.5.2.1.1;Reverse Transcription-Polymerase Chain Reaction (RT-PCR);104
8.3.1.1.1.2.5.2.1.2;Qualitative RT-PCR (Amplicor™ HCV 2.0, Roche Diagnostics);105
8.3.1.1.1.2.5.2.1.3;Transcription-Mediated Amplification (TMA, Versant™, Siemens);105
8.3.1.1.1.2.5.2.2;Quantitative HCV-RNA Detection;106
8.3.1.1.1.2.5.2.2.1;Cobas Amplicor™ HCV-Monitor (Roche Diagnostics);106
8.3.1.1.1.2.5.2.2.2;Branched DNA-Hybridising Assay (Versant™ HCV-RNAAssay (bDNA), Siemens);106
8.3.1.1.1.2.5.2.2.3;HCV-SuperQuant™ (National Genetics Institute, NGI);106
8.3.1.1.1.2.5.2.2.4;Real-Time Based HCV-RNA Detection Assays;106
8.3.1.1.1.2.5.2.2.5;COBAS TaqMan (CAP/CTM, Roche Diagnostics);107
8.3.1.1.1.2.5.2.2.6;Abbott RealTime HCV Test (North Chicago, IL, USA);107
8.3.1.1.1.2.5.2.3;HCV Typing Methods;107
8.3.1.1.1.2.5.2.3.1;Genotyping by Sequence Analysis;107
8.3.1.1.1.2.5.2.3.2;Reverse Hybridizing Assay (Versant™ HCV Genotype 2.0 System (LiPA), Siemens);108
8.3.1.1.1.2.5.2.3.3;Trugene (TRUGENE® HCV 5’NC Genotyping Kit, Siemens);108
8.3.1.1.1.2.5.3;Morphological;108
8.3.1.1.1.2.5.4;Conclusion for HCV Diagnosis;108
8.3.1.1.1.3;Immunological and Direct Antiviral Treatment Approaches in Viral Hepatitis;108
8.3.1.1.1.3.1;Chronic Hepatitis B;109
8.3.1.1.1.3.1.1;Nucleos(t)ide Analogs;109
8.3.1.1.1.3.1.1.1;Lamivudine;109
8.3.1.1.1.3.1.1.2;Adefovir Dipivoxil;109
8.3.1.1.1.3.1.1.3;Tenofovir;110
8.3.1.1.1.3.1.1.4;Entecavir;110
8.3.1.1.1.3.1.1.5;Telbivudine;110
8.3.1.1.1.3.1.2;Immunomodulatory Drugs;110
8.3.1.1.1.3.1.2.1;Protein Vaccination;110
8.3.1.1.1.3.1.2.2;DNA Vaccination (Genetic Immunization, Genetic Vaccination);111
8.3.1.1.1.3.1.2.3;Monoclonal Antibodies;111
8.3.1.1.1.3.1.2.4;Adoptive T Cell Transfer;111
8.3.1.1.1.3.1.3;Drugs Specific Against Viral RNA/DNA;111
8.3.1.1.1.3.1.3.1;Antisense-Oligonucleotides;111
8.3.1.1.1.3.1.3.2;Ribozymes;112
8.3.1.1.1.3.1.3.3;Dominant-Negative Mutants;112
8.3.1.1.1.3.2;Chronic Hepatitis C;113
8.3.1.1.1.3.2.1;Inhibitors of the Inosine Monophosphate Dehydrogenase;113
8.3.1.1.1.3.2.1.1;VX-497;113
8.3.1.1.1.3.2.1.2;Ribavirin;113
8.3.1.1.1.3.2.2;CpG-Oligo(deoxy)nucleotides;113
8.3.1.1.1.3.2.3;Vaccinations;114
8.3.1.1.1.3.2.3.1;DNA Vaccines;114
8.3.1.1.1.3.2.3.2;Protein Vaccines;114
8.3.1.1.1.3.2.3.3;Epitope Vaccine;114
8.3.1.1.1.3.2.4;Amantadine and Rimantadine;115
8.3.1.1.1.3.2.5;Specific Enzyme Inhibitors;115
8.3.1.1.1.3.2.5.1;Helicase Inhibitors;116
8.3.1.1.1.3.2.5.2;Protease Inhibitors;116
8.3.1.1.1.3.2.5.3;Polymerase Inhibitors;116
8.3.1.1.1.3.2.6;Drugs Specifically Directed Against Viral RNA;117
8.3.1.1.1.3.2.6.1;Inhibitors of the Internal Ribosome Entry Site;117
8.3.1.1.1.3.2.6.2;Antisense-Oligodeoxynucleotides;117
8.3.1.1.1.3.2.6.3;Ribozymes;118
8.3.1.1.1.3.2.7;References;118
8.3.1.1.2;63.2 Pathology of Acute and Chronic Viral Hepatitis;121
8.3.1.1.2.1;General Features of Acute Viral Hepatitis;121
8.3.1.1.2.2;General Features of Chronic Viral Hepatitis;123
8.3.1.1.2.3;Pathology of Specific Hepatitis Viruses;125
8.3.1.1.2.3.1;Hepatitis A Virus (HAV);125
8.3.1.1.2.3.2;Hepatitis B Virus (HBV);126
8.3.1.1.2.3.3;Hepatitis C Virus (HCV);127
8.3.1.1.2.3.4;Hepatitis D Virus (HDV);130
8.3.1.1.2.3.5;Hepatitis E Virus (HEV);130
8.3.1.1.2.4;Grading and Staging of Chronic Hepatitis;130
8.3.1.1.2.4.1;References;132
8.3.1.1.3;63.3 Hepatitis A to E. Epidemiology, Clinical Manifestations, Prevention and Therapy;134
8.3.1.1.3.1;Hepatitis A;135
8.3.1.1.3.1.1;Epidemiology;135
8.3.1.1.3.1.2;Pathogenesis;137
8.3.1.1.3.1.3;Clinical Presentation;137
8.3.1.1.3.1.3.1;Symptoms and Signs;137
8.3.1.1.3.1.3.2;Laboratory Findings;138
8.3.1.1.3.1.4;Diagnosis;138
8.3.1.1.3.1.5;Differential Diagnosis;139
8.3.1.1.3.1.6;Natural Course and Prognosis;140
8.3.1.1.3.1.6.1;Cholestatic Hepatitis;140
8.3.1.1.3.1.6.2;Relapsing Hepatitis;140
8.3.1.1.3.1.6.3;Fulminant Hepatitis and Acute Liver Failure;140
8.3.1.1.3.1.7;Prevention;141
8.3.1.1.3.1.7.1;Passive Immunization;141
8.3.1.1.3.1.7.2;Active Immunization;142
8.3.1.1.3.1.8;Therapy;143
8.3.1.1.3.1.9;References;143
8.3.1.1.3.2;Hepatitis B;144
8.3.1.1.3.2.1;Definition;144
8.3.1.1.3.2.2;Epidemiology;144
8.3.1.1.3.2.3;Pathogenesis;146
8.3.1.1.3.2.3.1;Hepatitis B Stages;147
8.3.1.1.3.2.4;Clinical Manifestations;148
8.3.1.1.3.2.4.1;Acute Hepatitis B;148
8.3.1.1.3.2.4.2;Chronic Hepatitis B;148
8.3.1.1.3.2.4.3;Extrahepatic Manifestations;149
8.3.1.1.3.2.4.3.1;HBV-associated Glomerulonephritis;149
8.3.1.1.3.2.4.3.2;Polyarteritis Nodosa;150
8.3.1.1.3.2.5;Diagnosis;150
8.3.1.1.3.2.6;Differential Diagnosis;150
8.3.1.1.3.2.7;Major Patterns of Chronic Hepatitis B;151
8.3.1.1.3.2.7.1;HBeAg-Positive Chronic Hepatitis B;151
8.3.1.1.3.2.7.2;HBeAg-negative Chronic Hepatitis B;152
8.3.1.1.3.2.7.3;Inactive HBsAg Carrier State;153
8.3.1.1.3.2.7.4;Occult Hepatitis B Virus infection;153
8.3.1.1.3.2.8;Natural Course and Prognosis;154
8.3.1.1.3.2.8.1;Acute Hepatitis B;154
8.3.1.1.3.2.8.2;Fulminant Hepatitis B;155
8.3.1.1.3.2.8.3;Chronic Hepatitis B;155
8.3.1.1.3.2.8.3.1;Age;155
8.3.1.1.3.2.8.3.2;Immunosuppression;156
8.3.1.1.3.2.8.3.3;HBV Genotype;156
8.3.1.1.3.2.8.3.4;Viral Load;156
8.3.1.1.3.2.8.3.5;Coinfections;157
8.3.1.1.3.2.8.4;Spontaneous Resolution;158
8.3.1.1.3.2.8.4.1;HBeAg Seroconversion;158
8.3.1.1.3.2.8.4.2;HBsAg Seroconversion;158
8.3.1.1.3.2.8.5;Hepatitis Flares;158
8.3.1.1.3.2.8.6;Liver Fibrosis and Cirrhosis;159
8.3.1.1.3.2.8.7;Hepatocellular Carcinoma;159
8.3.1.1.3.2.9;Prevention;160
8.3.1.1.3.2.9.1;Passive Immunization;160
8.3.1.1.3.2.9.2;Active Immunization (Vaccination);161
8.3.1.1.3.2.9.3;Suppression of HBV-Reactivation;162
8.3.1.1.3.2.10;Treatment;162
8.3.1.1.3.2.10.1;Treatment of Acute Hepatitis B;162
8.3.1.1.3.2.10.2;Treatment of Chronic Hepatitis B;162
8.3.1.1.3.2.10.2.1;Goals of Therapy;163
8.3.1.1.3.2.10.2.2;Indications;164
8.3.1.1.3.2.10.3;Drugs Used in the Treatment of Hepatitis B;164
8.3.1.1.3.2.10.4;Interferons;165
8.3.1.1.3.2.10.4.1;Standard Interferon;165
8.3.1.1.3.2.10.4.2;Pegylated Interferon;166
8.3.1.1.3.2.10.5;Nucleosides and Nucleotides;169
8.3.1.1.3.2.10.5.1;Lamivudine;169
8.3.1.1.3.2.10.5.2;Adefovir Dipivoxil;171
8.3.1.1.3.2.10.5.3;Entecavir;173
8.3.1.1.3.2.10.5.4;Telbivudine;174
8.3.1.1.3.2.10.5.5;Tenofovir, Emtricitabine, Clevudine;174
8.3.1.1.3.2.10.6;Combination Therapy;175
8.3.1.1.3.2.10.7;Special Therapeutic Problems;176
8.3.1.1.3.2.10.7.1;Liver Cirrhosis;176
8.3.1.1.3.2.10.7.2;Coinfection with Other Viruses;176
8.3.1.1.3.2.10.7.3;HBV-Induced Glomerulonephritis;177
8.3.1.1.3.2.10.7.4;Children;177
8.3.1.1.3.2.10.7.5;Asian Patients;177
8.3.1.1.3.2.10.7.6;Liver Transplantation;177
8.3.1.1.3.2.10.7.7;Nonresponders and Drug Resistance;178
8.3.1.1.3.2.11;References;181
8.3.1.1.3.3;Hepatitis C;189
8.3.1.1.3.3.1;Epidemiology;189
8.3.1.1.3.3.2;Transmission;190
8.3.1.1.3.3.3;Pathogenesis;190
8.3.1.1.3.3.4;Clinical Manifestations and Diagnosis;192
8.3.1.1.3.3.4.1;Acute Hepatitis C;193
8.3.1.1.3.3.4.2;Chronic Hepatitis C;193
8.3.1.1.3.3.4.3;Extrahepatic Manifestations of Chronic Hepatitis C;194
8.3.1.1.3.3.4.3.1;Mixed Cryoglobulinemia;194
8.3.1.1.3.3.4.3.2;Glomerulonephritis;195
8.3.1.1.3.3.4.3.3;Polyarteritis Nodosa;195
8.3.1.1.3.3.4.3.4;Porphyria Cutanea Tarda;196
8.3.1.1.3.3.4.3.5;Sjögren’s Syndrome;196
8.3.1.1.3.3.4.3.6;Sarcoidosis;196
8.3.1.1.3.3.4.3.7;Thyroid Disorders;196
8.3.1.1.3.3.4.3.8;Dermatologic Disorders;196
8.3.1.1.3.3.4.3.9;Metabolic Manifestations;197
8.3.1.1.3.3.4.3.10;Hematologic-Oncologic Disorders;197
8.3.1.1.3.3.4.3.11;Nervous System and Psychiatric Disorders;198
8.3.1.1.3.3.4.3.12;Others;198
8.3.1.1.3.3.5;Differential Diagnosis;198
8.3.1.1.3.3.6;Natural Course and Prognosis;198
8.3.1.1.3.3.6.1;Chronic Hepatitis C with Persistently Normal Aminotransferases;200
8.3.1.1.3.3.6.2;Hepatitis C and Pregnancy;200
8.3.1.1.3.3.6.3;Hepatitis C in Children;200
8.3.1.1.3.3.6.4;Coinfection with HBV;200
8.3.1.1.3.3.6.5;Coinfection with GBV-C/HGV;200
8.3.1.1.3.3.6.6;Coinfection with HIV;201
8.3.1.1.3.3.6.7;a1-Antitrypsin Deficiency;201
8.3.1.1.3.3.6.8;Porphyria Cutanea Tarda;201
8.3.1.1.3.3.6.9;Diabetes Mellitus;201
8.3.1.1.3.3.7;Prevention;201
8.3.1.1.3.3.7.1;Approach to Accidental Exposure (Needle Stick Injury);201
8.3.1.1.3.3.8;Therapy;202
8.3.1.1.3.3.8.1;Therapy of Acute Hepatitis C;202
8.3.1.1.3.3.8.2;Therapy of Chronic Hepatitis C;202
8.3.1.1.3.3.8.2.1;Definitions of Response to Treatment;202
8.3.1.1.3.3.8.2.2;Drugs used in the Treatment of Chronic Hepatitis C;202
8.3.1.1.3.3.8.2.3;Combination Treatment;203
8.3.1.1.3.3.8.2.4;Results of Treatment;205
8.3.1.1.3.3.8.2.5;Individualizing Treatment;205
8.3.1.1.3.3.8.2.6;New Approaches to Treatment;206
8.3.1.1.3.3.8.3;Therapeutic Problems in Special Patient Populations;206
8.3.1.1.3.3.8.3.1;Patients with Persistently Normal Aminotransferase Levels (PNALT);206
8.3.1.1.3.3.8.3.2;Nonresponders and Relapsers;206
8.3.1.1.3.3.8.3.3;Liver Cirrhosis;208
8.3.1.1.3.3.8.3.4;Recurrent Disease after Liver Transplantation;208
8.3.1.1.3.3.8.3.5;End-Stage Renal Disease;208
8.3.1.1.3.3.8.3.6;Children;208
8.3.1.1.3.3.8.3.7;Ethnic Groups;209
8.3.1.1.3.3.8.3.8;Intravenous Drug Users;209
8.3.1.1.3.3.9;References;209
8.3.1.1.3.4;Hepatitis D;217
8.3.1.1.3.4.1;Epidemiology;217
8.3.1.1.3.4.2;Pathogenesis;217
8.3.1.1.3.4.3;Clinical Manifestations;218
8.3.1.1.3.4.4;Diagnosis;218
8.3.1.1.3.4.5;Natural Course and Prognosis;218
8.3.1.1.3.4.6;Prevention;219
8.3.1.1.3.4.7;Therapy;219
8.3.1.1.3.4.8;References;219
8.3.1.1.3.5;Hepatitis E;220
8.3.1.1.3.5.1;Epidemiology;220
8.3.1.1.3.5.2;Pathogenesis;221
8.3.1.1.3.5.3;Clinical Presentation;221
8.3.1.1.3.5.4;Diagnosis;221
8.3.1.1.3.5.5;Differential Diagnosis;221
8.3.1.1.3.5.6;Natural Course and Prognosis;221
8.3.1.1.3.5.7;Prevention;221
8.3.1.1.3.5.8;Therapy;222
8.3.1.1.3.5.9;References;222
8.3.2;Chapter 64;223
8.3.2.1;Viral Infections by Nonhepatotropic Viruses;223
8.3.2.1.1;Herpes Viruses;223
8.3.2.1.1.1;Herpes Simplex Virus 1 and 2;223
8.3.2.1.1.2;Human Herpesvirus 6 and 7;224
8.3.2.1.1.3;Human Herpesvirus 8;225
8.3.2.1.1.4;Varicella-Zoster Virus;225
8.3.2.1.1.5;Cytomegalovirus;225
8.3.2.1.1.6;Epstein-Barr Virus;226
8.3.2.1.2;Adenoviruses;227
8.3.2.1.3;Enteroviruses;227
8.3.2.1.4;Paramyxoviruses;227
8.3.2.1.5;Togaviruses;227
8.3.2.1.6;Arboviruses;228
8.3.2.1.6.1;Yellow Fever Virus;228
8.3.2.1.6.2;Dengue Virus;228
8.3.2.1.6.3;Hantavirus;228
8.3.2.1.7;Arenaviruses;228
8.3.2.1.7.1;Lassavirus;228
8.3.2.1.7.2;Junin- and Machupovirus;229
8.3.2.1.8;Filoviruses;229
8.3.2.1.8.1;Marburg Virus;229
8.3.2.1.8.2;Ebola Virus;229
8.3.2.1.9;Parvoviruses;229
8.3.2.1.10;Coronaviruses;229
8.3.2.1.11;References;229
8.3.3;Chapter 65;231
8.3.3.1;Bacterial Liver Abscess and Other Bacterial Infections;231
8.3.3.1.1;Bacterial Liver Abscess;232
8.3.3.1.1.1;Definition;232
8.3.3.1.1.2;Epidemiology;232
8.3.3.1.1.3;Etiology and Pathogenesis;232
8.3.3.1.1.4;Diagnosis;233
8.3.3.1.1.4.1;Clinical Manifestations;233
8.3.3.1.1.4.2;Technical Investigations;233
8.3.3.1.1.5;Differential Diagnosis;234
8.3.3.1.1.6;Course and Prognosis;234
8.3.3.1.1.7;Therapy;234
8.3.3.1.2;Actinomycetaceae;235
8.3.3.1.3;Bartonellae;235
8.3.3.1.4;Borreliae;235
8.3.3.1.5;Brucellae;236
8.3.3.1.6;Burkholderia pseudomallei;236
8.3.3.1.7;Campylobacter Species;236
8.3.3.1.8;Chlamydiae;236
8.3.3.1.9;Clostridium perfringens;236
8.3.3.1.10;Ehrlichiae;237
8.3.3.1.11;Francisella tularensis;237
8.3.3.1.12;Gonococci;237
8.3.3.1.13;Legionella pneumophila;237
8.3.3.1.14;Leptospira;237
8.3.3.1.15;Listeria monocytogenes;238
8.3.3.1.16;Mycobacteria;238
8.3.3.1.17;Mycoplasma pneumoniae;239
8.3.3.1.18;Pneumococci;239
8.3.3.1.19;Rickettsiae;239
8.3.3.1.20;Salmonellae;239
8.3.3.1.21;Shigellae;240
8.3.3.1.22;Staphylococcus aureus;240
8.3.3.1.23;Treponema pallidum;240
8.3.3.1.24;Tropheryma whipplei;241
8.3.3.1.25;Yersiniae;241
8.3.3.1.26;References;241
8.3.4;Chapter 66;243
8.3.4.1;Amebic Liver Abscess and Other Protozoal Diseases;243
8.3.4.1.1;Amebiasis;243
8.3.4.1.1.1;Definition;243
8.3.4.1.1.2;Epidemiology;243
8.3.4.1.1.3;Etiology, Pathogenesis and Pathology;244
8.3.4.1.1.4;Diagnosis;244
8.3.4.1.1.4.1;Clinical Manifestations;244
8.3.4.1.1.4.2;Technical Investigations;244
8.3.4.1.1.5;Differential Diagnosis;245
8.3.4.1.1.6;Course and Prognosis;245
8.3.4.1.1.7;Therapy;245
8.3.4.1.2;Babesiosis;246
8.3.4.1.3;Cryptosporidiosis;246
8.3.4.1.4;Giardiasis;246
8.3.4.1.5;Leishmaniasis;246
8.3.4.1.6;Malaria;247
8.3.4.1.6.1;Pathology;247
8.3.4.1.6.2;Clinical Manifestations;247
8.3.4.1.7;Toxoplasmosis;247
8.3.4.1.8;References;248
8.3.5;Chapter 67;249
8.3.5.1;Helminthic Infections;249
8.3.5.1.1;Hepatic Schistosomiasis (Bilharziasis);249
8.3.5.1.1.1;Definition;249
8.3.5.1.1.2;Epidemiology;249
8.3.5.1.1.3;Pathogenesis;250
8.3.5.1.1.4;Pathology;250
8.3.5.1.1.5;Diagnosis;250
8.3.5.1.1.5.1;Clinical Manifestations;250
8.3.5.1.1.5.2;Technical Investigations;251
8.3.5.1.1.6;Differential Diagnosis;251
8.3.5.1.1.7;Course and Prognosis;251
8.3.5.1.1.8;Therapy;251
8.3.5.1.2;Fascioliasis;252
8.3.5.1.3;Dicroceliasis;252
8.3.5.1.4;Clonorchiasis and Opisthorchiasis;252
8.3.5.1.5;Echinococcosis (Hydatid Disease);253
8.3.5.1.5.1;Definition;253
8.3.5.1.5.2;Epidemiology;253
8.3.5.1.5.3;Pathogenesis;253
8.3.5.1.5.4;Pathology;253
8.3.5.1.5.5;Diagnosis;254
8.3.5.1.5.5.1;Clinical Manifestations;254
8.3.5.1.5.5.2;Technical Investigations;254
8.3.5.1.5.6;Differential Diagnosis;254
8.3.5.1.5.7;Complications;255
8.3.5.1.5.8;Course and Prognosis;255
8.3.5.1.5.9;Therapy;255
8.3.5.1.6;Ascariasis;256
8.3.5.1.7;Toxocariasis (Visceral Larva Migrans);256
8.3.5.1.8;Capillariasis;257
8.3.5.1.9;Enterobiasis;257
8.3.5.1.10;Strongyloidiasis;257
8.3.5.1.11;References;258
8.3.6;Chapter 68;259
8.3.6.1;Fungal Infections;259
8.3.6.1.1;Candidiasis;259
8.3.6.1.2;Aspergillosis;260
8.3.6.1.3;Mucormycosis (Phycomycosis; Zygomycosis);260
8.3.6.1.4;Cryptococcosis;260
8.3.6.1.5;Histoplasmosis;260
8.3.6.1.6;Coccidioidomycosis, Blastomycosis, and Paracoccidiomycosis;260
8.3.6.1.7;Penicilliosis;261
8.3.6.1.8;References;261
8.4;Section XIII: Hepatobiliary Diseases in HIV-Infected Patients;262
8.4.1;Chapter 69;263
8.4.1.1;Infections;263
8.4.1.1.1;HIV Infection;263
8.4.1.1.2;Viral Hepatitis;263
8.4.1.1.2.1;Hepatitis A;264
8.4.1.1.2.2;Hepatitis B;264
8.4.1.1.2.3;Hepatitis D;265
8.4.1.1.2.4;Hepatitis C;265
8.4.1.1.2.4.1;Effects of HIV Infection on Chronic Hepatitis C;265
8.4.1.1.2.4.2;Effects of Chronic Hepatitis C on HIV Infection;266
8.4.1.1.2.4.3;Diseases of other Systems in HCV/HIV Coinfected Patients;266
8.4.1.1.2.5;Hepatitis E;266
8.4.1.1.2.6;Hepatitis G;266
8.4.1.1.3;Opportunistic Infections;267
8.4.1.1.4;References;267
8.4.2;Chapter 70;270
8.4.2.1;Neoplastic Diseases;270
8.4.2.1.1;Hepatocellular Carcinoma;270
8.4.2.1.2;Kaposi’s Sarcoma;270
8.4.2.1.3;Malignant Lymphomas;271
8.4.2.1.4;References;271
8.4.3;Chapter 71;272
8.4.3.1;Drug-Induced Liver Injury;272
8.4.3.1.1;Nucleosidic Reverse Transcriptase Inhibitors;273
8.4.3.1.2;Non-nucleosidic Reverse Transcriptase Inhibitors;273
8.4.3.1.3;Protease Inhibitors;273
8.4.3.1.4;References;273
8.5;Section XIV: Autoimmune Liver Diseases;275
8.5.1;Chapter 72;277
8.5.1.1;Autoimmune Hepatitis;277
8.5.1.1.1;Definition;277
8.5.1.1.2;Epidemiology;277
8.5.1.1.3;Etiology and Pathogenesis;278
8.5.1.1.3.1;Immunologic Factors;278
8.5.1.1.3.2;Genetic Factors;278
8.5.1.1.4;Pathology;279
8.5.1.1.5;Diagnosis;279
8.5.1.1.5.1;Autoimmune Hepatitis Type I;280
8.5.1.1.5.2;Autoimmune Hepatitis Type II;280
8.5.1.1.5.3;Autoimmune Hepatitis Type III;282
8.5.1.1.5.4;Clinical Presentation;282
8.5.1.1.5.5;Laboratory Findings;283
8.5.1.1.5.6;Imaging Tools;284
8.5.1.1.6;Differential Diagnosis;284
8.5.1.1.7;Course and Prognosis;285
8.5.1.1.8;Therapy;285
8.5.1.1.9;References;288
8.5.2;Chapter 73;291
8.5.2.1;Primary Biliary Cirrhosis;291
8.5.2.1.1;Definition;291
8.5.2.1.2;Epidemiology;291
8.5.2.1.3;Etiology and Pathogenesis;292
8.5.2.1.3.1;Genetic Factors;292
8.5.2.1.3.2;Environmental Factors;292
8.5.2.1.3.3;Immunologic Factors;292
8.5.2.1.4;Pathology;293
8.5.2.1.5;Diagnosis;293
8.5.2.1.5.1;Clinical Presentation;293
8.5.2.1.5.1.1;Extrahepatic Diseases;295
8.5.2.1.5.2;Laboratory Findings;295
8.5.2.1.5.3;Imaging Tools;296
8.5.2.1.6;Differential Diagnosis;296
8.5.2.1.7;Natural Course and Prognosis;296
8.5.2.1.8;Therapy;297
8.5.2.1.9;References;299
8.5.3;Chapter 74;304
8.5.3.1;Autoimmune Cholangitis;304
8.5.3.1.1;References;304
8.5.4;Chapter 75;306
8.5.4.1;Primary Sclerosing Cholangitis;306
8.5.4.1.1;Definition;306
8.5.4.1.2;Epidemiology;306
8.5.4.1.3;Etiology and Pathogenesis;307
8.5.4.1.4;Pathology;307
8.5.4.1.5;Diagnosis;308
8.5.4.1.5.1;Clinical Manifestations;308
8.5.4.1.5.1.1;Extrahepatic Diseases;309
8.5.4.1.5.2;Laboratory Findings;309
8.5.4.1.5.3;Imaging Tools;309
8.5.4.1.6;Differential Diagnosis;310
8.5.4.1.7;Course and Prognosis;311
8.5.4.1.8;Therapy;312
8.5.4.1.9;References;312
8.5.5;Chapter 76;315
8.5.5.1;Biliary Ductopenia (Vanishing Bile Duct Syndrome);315
8.5.5.1.1;Defi nition;315
8.5.5.1.2;Epidemiology;315
8.5.5.1.3;Etiology and Pathogenesis;315
8.5.5.1.4;Pathology;317
8.5.5.1.5;Diagnosis;318
8.5.5.1.5.1;Laboratory Findings;318
8.5.5.1.5.2;Imaging Techniques;318
8.5.5.1.6;Differential Diagnosis;318
8.5.5.1.7;Course and Prognosis;318
8.5.5.1.8;Therapy;318
8.5.5.1.9;References;318
8.5.6;Chapter 77;319
8.5.6.1;Autoimmune Overlap Syndromes;319
8.5.6.1.1;Overlap Between AIH and PBC;320
8.5.6.1.2;Overlap Between AIH and PSC;320
8.5.6.1.3;Overlap Between PBC and PSC;320
8.5.6.1.4;References;321
8.6;Section XV: Acute Liver Failure;322
8.6.1;Chapter 78;323
8.6.1.1;Acute Liver Failure;323
8.6.1.1.1;Defi nition;323
8.6.1.1.2;Epidemiology;323
8.6.1.1.3;Etiology;323
8.6.1.1.4;Prognosis;324
8.6.1.1.5;General Considerations;325
8.6.1.1.6;Special Entities;326
8.6.1.1.6.1;Acetaminophen Intoxication;326
8.6.1.1.6.2;Mushroom Poisoning;326
8.6.1.1.6.3;Drug-Induced Liver Injury;327
8.6.1.1.6.4;Viral Hepatitis;327
8.6.1.1.6.5;Wilson’s Disease;328
8.6.1.1.6.6;Autoimmune Hepatitis;329
8.6.1.1.6.7;Acute Fatty Liver of Pregnancy;329
8.6.1.1.6.8;Budd-Chiari Syndrome;329
8.6.1.1.6.9;Indeterminate Etiology;329
8.6.1.1.7;Therapy;329
8.6.1.1.7.1;General Considerations;329
8.6.1.1.7.2;Encephalopathy and Cerebral Edema;330
8.6.1.1.7.3;Diagnosis of Increased Intracranial Pressure;330
8.6.1.1.7.4;General Therapeutic Measures in Cerebral Hypertension;331
8.6.1.1.7.5;Drug Therapy of Intracranial Hypertension;331
8.6.1.1.7.6;Supportive Therapy in Intracranial Hypertension;332
8.6.1.1.7.7;Infections in Acute Liver Failure;332
8.6.1.1.7.8;Multi-Organ-Failure in Acute Liver Failure;333
8.6.1.1.7.9;Aspects of Cardiovascular System and Hemodynamics;333
8.6.1.1.7.10;Renal Failure;333
8.6.1.1.7.11;Coagulation Disorders;334
8.6.1.1.7.12;Metabolic Disorders in Acute Liver Failure;334
8.6.1.1.7.13;Basic Aspects of Nutrition in Acute Liver Failure;334
8.6.1.1.7.14;Liver Replacement Therapies;335
8.6.1.1.7.15;Liver Transplantation and Prognosis;335
8.6.1.1.8;References;336
8.7;Section XVI: Liver Cirrhosis and Sequelae;339
8.7.1;Chapter 79;340
8.7.1.1;Liver Cirrhosis;340
8.7.1.1.1;Defi nition;340
8.7.1.1.2;Epidemiology;340
8.7.1.1.3;Etiology and Pathogenesis;341
8.7.1.1.3.1;Cell Death;341
8.7.1.1.3.2;Fibrosis and Circulatory Disturbances;342
8.7.1.1.3.3;Disturbances in Hepatocyte Growth and Proliferation;342
8.7.1.1.4;Pathology;343
8.7.1.1.4.1;Macroscopical Findings;343
8.7.1.1.4.1.1;Micronodular Cirrhosis;343
8.7.1.1.4.1.2;Macronodular Cirrhosis;344
8.7.1.1.4.1.3;Mixed Forms;345
8.7.1.1.4.2;Histological Findings;345
8.7.1.1.5;Diagnosis;346
8.7.1.1.5.1;Clinical Manifestations;346
8.7.1.1.5.2;Laboratory Findings;350
8.7.1.1.5.3;Imaging Techniques;351
8.7.1.1.6;Differential Diagnosis;352
8.7.1.1.7;Course and Prognosis;353
8.7.1.1.8;Therapy;354
8.7.1.1.8.1;References;355
8.7.2;Chapter 80;357
8.7.2.1;Complications of Liver Cirrhosis;357
8.7.2.1.1;80.1 Ascites, Varices, Portal Hypertensive Gastroenteropathy;359
8.7.2.1.1.1;Ascites;359
8.7.2.1.1.1.1;General Measures;360
8.7.2.1.1.1.1.1;Bed Rest;360
8.7.2.1.1.1.1.2;Diet;360
8.7.2.1.1.1.2;Diuretics;360
8.7.2.1.1.1.2.1;Potassium-Sparing Diuretics;361
8.7.2.1.1.1.2.2;Loop Diuretics;361
8.7.2.1.1.1.3;Practical Approach to Diuretic Therapy;362
8.7.2.1.1.1.4;Large-Volume Paracentesis;362
8.7.2.1.1.1.5;Practical Approach to Paracentesis;363
8.7.2.1.1.1.6;Refractory Ascites;363
8.7.2.1.1.1.6.1;Therapy of Refractory Ascites;364
8.7.2.1.1.1.7;Outlook;365
8.7.2.1.1.2;Esophageal Varices;366
8.7.2.1.1.2.1;Defi nition;366
8.7.2.1.1.2.2;Epidemiology;366
8.7.2.1.1.2.3;Anatomy, Etiology and Pathophysiology;366
8.7.2.1.1.2.4;Diagnosis;367
8.7.2.1.1.2.5;Course and Prognosis;368
8.7.2.1.1.2.6;Predictors of Variceal Bleeding;368
8.7.2.1.1.2.7;Prophylaxis and Therapy;369
8.7.2.1.1.2.7.1;“Preprimary” Prophylaxis;369
8.7.2.1.1.2.7.2;Primary Bleeding Prophylaxis;370
8.7.2.1.1.2.7.3;Therapy of Acute Variceal Hemorrhage;372
8.7.2.1.1.2.7.4;Prevention of Recurrent Variceal Hemorrhage;374
8.7.2.1.1.3;Gastric Varices;375
8.7.2.1.1.4;Portal Hypertensive Gastropathy;376
8.7.2.1.1.4.1;Defi nition;376
8.7.2.1.1.4.2;Epidemiology;376
8.7.2.1.1.4.3;Etiology and Pathogenesis;376
8.7.2.1.1.4.4;Pathology;376
8.7.2.1.1.4.5;Clinical Manifestations and Diagnosis;376
8.7.2.1.1.4.6;Differential Diagnosis;377
8.7.2.1.1.4.7;Course and Prognosis;377
8.7.2.1.1.5;Portal Hypertensive Entero-, Colo- and Biliopathy;378
8.7.2.1.1.5.1;References;378
8.7.2.1.1.5.1.1;Ascites;378
8.7.2.1.1.5.1.2;Esophageal Varices;380
8.7.2.1.1.5.1.3;Gastric Varices;383
8.7.2.1.1.5.1.4;Portal Hypertensive Gastropathy;383
8.7.2.1.1.5.1.5;Portal Hypertensive Entero-, Colo- and Biliopathy;383
8.7.2.1.2;80.2 Bacterial Infections;384
8.7.2.1.2.1;Spontaneous Bacterial Peritonitis;384
8.7.2.1.2.1.1;Defi nition;384
8.7.2.1.2.1.2;Epidemiology;384
8.7.2.1.2.1.3;Etiology and Pathogenesis;384
8.7.2.1.2.1.4;Diagnosis;385
8.7.2.1.2.1.4.1;Clinical Manifestations;385
8.7.2.1.2.1.4.2;Laboratory Findings;385
8.7.2.1.2.1.5;Differential Diagnosis;386
8.7.2.1.2.1.6;Course and Prognosis;386
8.7.2.1.2.1.7;Prophylaxis and Therapy;386
8.7.2.1.2.1.7.1;Primary Prophylaxis;386
8.7.2.1.2.1.7.2;Therapy;387
8.7.2.1.2.1.7.3;Secondary Prophylaxis;388
8.7.2.1.2.1.8;References;388
8.7.2.1.3;80.3 Hepatorenal Syndrome;389
8.7.2.1.3.1;Defi nition;389
8.7.2.1.3.2;Epidemiology;389
8.7.2.1.3.3;Etiology and Pathogenesis;389
8.7.2.1.3.4;Clinical Manifestations and Diagnosis;391
8.7.2.1.3.4.1;Type 1 Hepatorenal Syndrome;391
8.7.2.1.3.4.2;Type 2 Hepatorenal Syndrome;391
8.7.2.1.3.5;Differential Diagnosis;392
8.7.2.1.3.6;Course and Prognosis;392
8.7.2.1.3.7;Therapy;393
8.7.2.1.3.7.1;Drug Therapy;393
8.7.2.1.3.7.2;TIPS;393
8.7.2.1.3.7.3;Hemodialysis;393
8.7.2.1.3.7.4;Liver Transplantation;394
8.7.2.1.3.8;References;394
8.7.2.1.4;80.4 Hepatic Encephalopathy;395
8.7.2.1.4.1;Defi nition;395
8.7.2.1.4.2;Epidemiology;395
8.7.2.1.4.3;Etiology and Pathogenesis;395
8.7.2.1.4.3.1;Neurotoxins and Impaired Astrocyte Function;397
8.7.2.1.4.3.2;Amino Acid Imbalance and False Neurotransmitters;399
8.7.2.1.4.4;Pathology;400
8.7.2.1.4.5;Diagnosis;400
8.7.2.1.4.5.1;Clinical Manifestations;400
8.7.2.1.4.5.1.1;Minimal Hepatic Encephalopathy;400
8.7.2.1.4.5.1.2;Overt Hepatic Encephalopathy;400
8.7.2.1.4.5.2;Technical Investigations;401
8.7.2.1.4.6;Differential Diagnosis;402
8.7.2.1.4.7;Course and Prognosis;402
8.7.2.1.4.8;Therapy;402
8.7.2.1.4.8.1;General Measures and Elimination of Precipitating Factors;402
8.7.2.1.4.8.2;Ammonia-Lowering Strategies;404
8.7.2.1.4.8.3;Orthotopic Liver Transplantation;405
8.7.2.1.4.9;References;405
8.7.2.1.5;80.5 Pulmonary Complications;407
8.7.2.1.5.1;Hepatopulmonary Syndrome;407
8.7.2.1.5.1.1;Defi nition;407
8.7.2.1.5.1.2;Epidemiology;407
8.7.2.1.5.1.3;Etiology and Pathogenesis;407
8.7.2.1.5.1.4;Pathology;408
8.7.2.1.5.1.5;Clinical Manifestations and Diagnosis;408
8.7.2.1.5.1.6;Differential Diagnosis;409
8.7.2.1.5.1.7;Course and Prognosis;409
8.7.2.1.5.1.8;Therapy;409
8.7.2.1.5.2;Portopulmonary Hypertension;410
8.7.2.1.5.2.1;Defi nition;410
8.7.2.1.5.2.2;Epidemiology;410
8.7.2.1.5.2.3;Etiology and Pathogenesis;410
8.7.2.1.5.2.4;Pathology;411
8.7.2.1.5.2.5;Clinical Manifestations and Diagnosis;411
8.7.2.1.5.2.6;Differential Diagnosis;411
8.7.2.1.5.2.7;Course and Prognosis;411
8.7.2.1.5.2.8;Therapy;411
8.7.2.1.5.3;Hepatic Hydrothorax;412
8.7.2.1.5.3.1;Defi nition;412
8.7.2.1.5.3.2;Epidemiology;412
8.7.2.1.5.3.3;Pathogenesis;412
8.7.2.1.5.3.4;Clinical Manifestations and Diagnosis;412
8.7.2.1.5.3.5;Course and Prognosis;412
8.7.2.1.5.3.6;Therapy;412
8.7.2.1.5.4;References;412
8.8;Section XVII: Genetic and Metabolic Liver Disorders;422
8.8.1;Chapter 81;423
8.8.1.1;Wilson’s Disease;423
8.8.1.1.1;Defi nition and Epidemiology;423
8.8.1.1.2;Etiology and Pathogenesis;423
8.8.1.1.3;Diagnosis;424
8.8.1.1.3.1;Clinical Presentation;424
8.8.1.1.3.2;Diagnostic Findings;425
8.8.1.1.4;Family Screening;427
8.8.1.1.5;Differential Diagnosis;427
8.8.1.1.6;Prognosis;427
8.8.1.1.7;Treatment;428
8.8.1.1.7.1;Medical Treatment;428
8.8.1.1.7.2;Liver Transplantation;430
8.8.1.1.8;References;430
8.8.2;Chapter 82;432
8.8.2.1;Hereditary Hemochromatosis and Iron Overload;432
8.8.2.1.1;Defi nition and Classifi cation of Iron Overload Diseases;433
8.8.2.1.2;Type 1 HFE Hemochromatosis;434
8.8.2.1.2.1;History;434
8.8.2.1.2.2;Epidemiology;434
8.8.2.1.2.3;Etiology and Pathogenesis;435
8.8.2.1.2.4;Diagnosis;435
8.8.2.1.2.4.1;Laboratory Tests;435
8.8.2.1.2.4.2;Liver Biopsy and Determination of Liver Iron Concentration;436
8.8.2.1.2.4.3;Deferoxamine Testing and Ferrokinetic Measurements;436
8.8.2.1.2.4.4;Computed Tomography (CT), Magnetic Resonance Tomography (MRT) and Biomagnetometry;437
8.8.2.1.2.4.5;Genetic Tests;437
8.8.2.1.2.5;Early Diagnosis and Screening;437
8.8.2.1.2.6;Differential Diagnosis;439
8.8.2.1.3;Complications of Iron Overload;439
8.8.2.1.3.1;Liver cirrhosis;439
8.8.2.1.3.2;Liver Carcinoma;440
8.8.2.1.3.3;Association of Hemochromatosis with other Liver Diseases;440
8.8.2.1.3.4;Diabetes mellitus;441
8.8.2.1.3.5;Heart Disease;442
8.8.2.1.3.6;Arthropathy;442
8.8.2.1.3.7;Endocrine Abnormalities;443
8.8.2.1.3.8;Skin;443
8.8.2.1.3.9;Other Potential Complications;443
8.8.2.1.4;Therapy;443
8.8.2.1.4.1;Phlebotomy;443
8.8.2.1.4.1.1;Iron Removal by Chelators;444
8.8.2.1.4.1.2;Diet;444
8.8.2.1.4.1.3;Liver Transplantation;445
8.8.2.1.5;Prognosis;445
8.8.2.1.6;Juvenile Hereditary Hemochromatosis;445
8.8.2.1.6.1;Prevalence;445
8.8.2.1.6.2;Pathophysiology;445
8.8.2.1.6.3;Natural History;446
8.8.2.1.6.4;Diagnosis;446
8.8.2.1.6.5;Treatment;446
8.8.2.1.7;Transferrin Receptor 2 (TFR2)-Related Type 3 Hemochromatosis;447
8.8.2.1.7.1;Prevalence;447
8.8.2.1.7.2;Pathophysiology;447
8.8.2.1.7.3;Natural History;447
8.8.2.1.7.4;Diagnosis;448
8.8.2.1.7.5;Therapy;448
8.8.2.1.8;Type 4 Hemochromatosis–Ferroportin Disease;448
8.8.2.1.8.1;Prevalence and Pathophysiology;448
8.8.2.1.8.2;Natural History;449
8.8.2.1.8.3;Diagnosis;450
8.8.2.1.8.4;Therapy;450
8.8.2.1.8.5;Pathophysiology;450
8.8.2.1.8.6;Diagnosis;451
8.8.2.1.8.7;Therapy;451
8.8.2.1.9;References;452
8.8.3;Chapter 83;458
8.8.3.1;alpha1-Antitrypsin Defi ciency;458
8.8.3.1.1;Defi nition;459
8.8.3.1.2;Epidemiology;459
8.8.3.1.3;Etiology and Pathogenesis;459
8.8.3.1.4;Pathology;460
8.8.3.1.5;Clinical Manifestations;460
8.8.3.1.6;Laboratory Findings;462
8.8.3.1.7;Therapy;462
8.8.3.1.8;References;463
8.8.4;Chapter 84;464
8.8.4.1;Porphyrias;464
8.8.4.1.1;Abbreviations;464
8.8.4.1.2;Defi nition;464
8.8.4.1.3;Epidemiology;465
8.8.4.1.4;Etiology and Pathogenesis;466
8.8.4.1.4.1;Acute Porphyrias;466
8.8.4.1.4.1.1;Acute Intermittent Porphyria;467
8.8.4.1.4.1.2;Variegate Porphyria;467
8.8.4.1.4.1.3;Hereditary Coproporphyria;467
8.8.4.1.4.1.4;Doss-Porphyria (ALADP) (Delta-Aminolevulinic Acid-Dehydratase-Defect);467
8.8.4.1.4.2;Non-acute Porphyrias;468
8.8.4.1.4.2.1;Porphyria Cutanea Tarda;468
8.8.4.1.4.2.2;Erythropoietic and X-linked Protoporphyria;469
8.8.4.1.4.2.3;Congenital Erythropoietic Porphyria (Guenther’s Disease);469
8.8.4.1.5;Pathology;469
8.8.4.1.5.1;Porphyria Cutanea Tarda;470
8.8.4.1.5.2;Erythropoietic Protoporphyria;471
8.8.4.1.6;Clinical Manifestations;471
8.8.4.1.6.1;Acute Porphyrias;471
8.8.4.1.6.2;Non-acute Porphyrias;473
8.8.4.1.6.2.1;Porphyria Cutanea Tarda;473
8.8.4.1.6.2.2;Hepatoerythropoietic Porphyria;473
8.8.4.1.6.2.3;Erythropoietic Protoporphyria;473
8.8.4.1.6.2.4;Congenital Erythropoietic Porphyria (Günther’s Disease);474
8.8.4.1.7;Diagnosis;474
8.8.4.1.7.1;Acute Porphyrias;474
8.8.4.1.7.2;Non-acute Potphyrias;475
8.8.4.1.7.3;Chronic Hepatic Porphyria and Porphyria Cutanea Tarda;475
8.8.4.1.7.4;Erythropoietic Protoporphyria;475
8.8.4.1.8;Differential Diagnosis;475
8.8.4.1.8.1;Acute Hepatic Porphyrias;475
8.8.4.1.8.2;Chronic Hepatic Porphyria and Porphyria Cutanea Tarda;475
8.8.4.1.9;Therapy and Prognosis;476
8.8.4.1.9.1;Acute Porphyrias;476
8.8.4.1.10;Non-acute Porphyrias;477
8.8.4.1.10.1;Chronic Hepatic Porphyria and Porphyria Cutanea Tarda;477
8.8.4.1.10.2;Erythropoietic Protoporphyria;477
8.8.4.1.10.3;Congenital Erythropoietic Porphyria;478
8.8.4.1.11;References;478
8.8.5;Chapter 85;480
8.8.5.1;Inherited Syndromes of Intrahepatic Cholestasis;480
8.8.5.1.1;Alagille’s Syndrome (Arteriohepatic Dysplasia);480
8.8.5.1.1.1;Liver;480
8.8.5.1.1.2;Cardiovascular System;481
8.8.5.1.1.3;Facies;481
8.8.5.1.1.4;Ocular Changes;481
8.8.5.1.1.5;Skeletal Anomalies;481
8.8.5.1.1.6;Other Changes;481
8.8.5.1.2;Progressive Familial Intrahepatic Cholestasis;481
8.8.5.1.2.1;PFIC 1;484
8.8.5.1.2.1.1;Benign Recurrent Intrahepatic Cholestasis Type 1 (BRIC 1; Summerskill–Tygstrup–Walsh Syndrome);484
8.8.5.1.2.2;PFIC 2 and BRIC 2;485
8.8.5.1.2.3;PFIC 3;485
8.8.5.1.3;References;485
8.8.6;Chapter 86;487
8.8.6.1;Cystic Fibrosis;487
8.8.6.1.1;Defi nition;487
8.8.6.1.2;Epidemiology;487
8.8.6.1.3;Etiology and Pathogenesis;487
8.8.6.1.4;Pathology;488
8.8.6.1.5;Clinical Manifestations;488
8.8.6.1.6;Course and Prognosis;488
8.8.6.1.7;Therapy;489
8.8.6.1.8;References;489
8.8.7;Chapter 87;490
8.8.7.1;Amyloidosis;490
8.8.7.1.1;Defi nition;490
8.8.7.1.2;Classifi cation;491
8.8.7.1.3;Development and Deposition of Amyloid;491
8.8.7.1.3.1;AL-Amyloid;491
8.8.7.1.3.2;AA-Amyloid;491
8.8.7.1.4;Hepatic Amyloidosis;492
8.8.7.1.4.1;Pathology;492
8.8.7.1.4.2;Diagnosis;492
8.8.7.1.4.2.1;Clinical Manifestations;492
8.8.7.1.4.2.2;Laboratory Investigations;493
8.8.7.1.4.3;Differential Diagnosis;493
8.8.7.1.4.4;Course and Prognosis;493
8.8.7.1.4.5;Therapy;493
8.8.7.1.5;References;493
8.8.8;Chapter 88;495
8.8.8.1;Alcoholic Liver Disease;495
8.8.8.1.1;Introduction;496
8.8.8.1.2;Metabolism of Alcohol;498
8.8.8.1.2.1;Gastric First Pass Metabolism of Ethanol;498
8.8.8.1.2.2;Hepatic Alcohol Metabolism;500
8.8.8.1.2.2.1;Alcohol Dehydrogenase;500
8.8.8.1.2.2.2;Hepatic Microsomal Ethanol Oxidizing System;501
8.8.8.1.2.2.3;Hepatic Catalase;503
8.8.8.1.2.2.4;Hepatic Acetaldehyde Dehydrogenase;503
8.8.8.1.2.3;Bacterial Metabolism of Ethanol;503
8.8.8.1.3;Alcoholic Liver Disease;504
8.8.8.1.3.1;Epidemiology;504
8.8.8.1.3.2;Natural Course;504
8.8.8.1.3.3;Risk Factors;504
8.8.8.1.4;Alcoholic Fatty Liver;505
8.8.8.1.4.1;Defi nition;505
8.8.8.1.4.2;Epidemiology;506
8.8.8.1.4.3;Pathogenesis;506
8.8.8.1.4.4;Histopathology;507
8.8.8.1.4.5;Clinical and Laboratory Findings;508
8.8.8.1.4.6;Differential Diagnosis;509
8.8.8.1.4.7;Complications;509
8.8.8.1.4.8;Prognosis;510
8.8.8.1.4.9;Therapy;510
8.8.8.1.5;Alcoholic Steatohepatitis;511
8.8.8.1.5.1;Defi nition;511
8.8.8.1.5.2;Pathophysiology;511
8.8.8.1.5.3;Histopathology;518
8.8.8.1.5.4;Clinical Findings;519
8.8.8.1.5.5;Laboratory Findings;520
8.8.8.1.5.6;Imaging;520
8.8.8.1.5.7;Differential Diagnosis;520
8.8.8.1.5.8;Complications;521
8.8.8.1.5.9;Natural Course and Prognosis;521
8.8.8.1.5.10;Therapy;522
8.8.8.1.5.10.1;Drugs;524
8.8.8.1.6;Alcoholic Cirrhosis;526
8.8.8.1.6.1;Defi nition;526
8.8.8.1.6.2;Epidemiology;526
8.8.8.1.6.3;Pathogenesis;526
8.8.8.1.6.4;Histopathology;528
8.8.8.1.6.5;Clinical Findings;528
8.8.8.1.6.6;Differential Diagnosis;529
8.8.8.1.6.7;Natural Course and Prognosis;529
8.8.8.1.6.8;Therapy;529
8.8.8.1.7;Hepatocellular Carcinoma;530
8.8.8.1.7.1;Epidemiology;530
8.8.8.1.7.2;Pathogenesis;531
8.8.8.1.7.3;Clinical Findings, Diagnosis and Therapy;532
8.8.8.1.8;References;532
8.8.9;Chapter 89;536
8.8.9.1;Nonalcoholic Fatty Liver Disease;536
8.8.9.1.1;Defi nition;536
8.8.9.1.2;Epidemiology;536
8.8.9.1.3;Etiology;537
8.8.9.1.4;Pathogenesis;538
8.8.9.1.4.1;NAFLD Associated with Obesity and Metabolic Syndrome;538
8.8.9.1.4.2;The Way to Steatosis;539
8.8.9.1.4.3;From Steatosis to Steatohepatitis;541
8.8.9.1.4.4;NAFLD Associated with Chronic Hepatitis C Virus Infection;543
8.8.9.1.5;Pathology;544
8.8.9.1.6;Diagnosis;546
8.8.9.1.6.1;Clinical Manifestations;546
8.8.9.1.6.2;Laboratory Findings;547
8.8.9.1.6.3;Imaging Techniques;547
8.8.9.1.7;Differential Diagnosis;548
8.8.9.1.8;Natural Course and Prognosis;548
8.8.9.1.9;Therapy;549
8.8.9.1.9.1;Lifestyle Modifi cations;549
8.8.9.1.9.2;Pharmacological Therapy;549
8.8.9.1.9.3;Bariatric Surgery;557
8.8.9.1.10;References;557
8.8.10;Chapter 90;563
8.8.10.1;Other Metabolic Diseases: Tabellary Overview;563
8.8.10.1.1;References;568
8.8.11;Chapter 91;569
8.8.11.1;Malnutrition and Nutrition in Liver Disease;569
8.8.11.1.1;Defi nition;570
8.8.11.1.2;Prevalence;571
8.8.11.1.2.1;Malnutrition;571
8.8.11.1.2.2;Obesity;571
8.8.11.1.3;Pathophysiology of Malnutrition;572
8.8.11.1.3.1;Nutrient Intake;572
8.8.11.1.3.2;Absorption;572
8.8.11.1.3.3;Alcohol;572
8.8.11.1.3.4;Protein Metabolism;572
8.8.11.1.3.5;Energy Metabolism;573
8.8.11.1.3.6;Molecular Mechanisms;574
8.8.11.1.4;Prognosis;575
8.8.11.1.5;Nutritional Assessment;576
8.8.11.1.6;Clinical Features;577
8.8.11.1.6.1;Medical History;577
8.8.11.1.6.2;Physical Examination;577
8.8.11.1.7;Nutritional Requirements;578
8.8.11.1.7.1;Nitrogen;578
8.8.11.1.7.2;Energy;578
8.8.11.1.7.3;Vitamins;579
8.8.11.1.8;Specifi c Alterations in Liver Disease;579
8.8.11.1.8.1;Accelerated Starvation;579
8.8.11.1.8.2;Hepatic Encephalopathy;579
8.8.11.1.8.3;Protein Restriction;580
8.8.11.1.8.4;Non-essential Amino Acids;580
8.8.11.1.8.5;Salt Restriction;580
8.8.11.1.8.6;Alcohol Related Alterations;581
8.8.11.1.8.7;Obesity;581
8.8.11.1.9;Nutritional Management;581
8.8.11.1.9.1;Goals;581
8.8.11.1.9.2;Specifi c Patient Populations;581
8.8.11.1.9.2.1;Cirrhosis;581
8.8.11.1.9.2.2;Alcoholic Hepatitis;581
8.8.11.1.9.2.3;Fulminant Hepatic Failure;581
8.8.11.1.9.3;Weight Loss;583
8.8.11.1.9.4;Liver Transplant;583
8.8.11.1.10;Principles and Practical Implementation;583
8.8.11.1.10.1;Glucose Requirement;583
8.8.11.1.10.2;Route of Nutrient Administration;583
8.8.11.1.10.2.1;Parenteral Nutrition;584
8.8.11.1.10.3;Branched Chain Amino Acids;584
8.8.11.1.10.4;Dietary Supplements;584
8.8.11.1.11;Summary;585
8.8.11.1.12;References;586
8.9;Section XVIII: Drug-Induced and Toxic Liver Disease;590
8.9.1;Chapter 92;591
8.9.1.1;Hepatic Drug Metabolism and Drug Toxicity;591
8.9.1.1.1;Factors Affecting Hepatic Drug Metabolism and Drug Toxicity;591
8.9.1.1.1.1;Gender;592
8.9.1.1.1.2;Genetic Factors;592
8.9.1.1.1.2.1;Gilbert’s Syndrome and Pharmacogenomic Risk;594
8.9.1.1.1.3;Simultaneous Exposure to Several Drugs;597
8.9.1.1.1.4;Biliary Excretion of Drugs;597
8.9.1.1.1.5;Impact of Liver Disease;598
8.9.1.1.2;References;600
8.9.2;Chapter 93;602
8.9.2.1;Drug- and Toxin-Induced Liver Injury;602
8.9.2.1.1;Defi nition;602
8.9.2.1.2;Diagnosis;603
8.9.2.1.2.1;Clinical Manifestations;603
8.9.2.1.2.1.1;Hepatotoxic Liver Injury;603
8.9.2.1.2.1.2;Idiosyncratic Liver Injury;604
8.9.2.1.2.2;Laboratory Findings;604
8.9.2.1.2.3;Histological Patterns of Injury;604
8.9.2.1.2.4;Therapy;607
8.9.2.1.3;References;609
8.10;Section XIX: Granulomatous Liver Diseases;611
8.10.1;Chapter 94;612
8.10.1.1;Hepatic Granulomas;612
8.10.1.1.1;Defi nition;612
8.10.1.1.2;Epidemiology;612
8.10.1.1.3;Etiology;612
8.10.1.1.4;Pathogenesis and Pathology;613
8.10.1.1.5;Diagnosis;614
8.10.1.1.5.1;Clinical Manifestations;614
8.10.1.1.5.2;Laboratory Findings;614
8.10.1.1.5.3;Imaging Techniques;614
8.10.1.1.6;Differential Diagnosis;614
8.10.1.1.7;Course and Prognosis;615
8.10.1.1.8;Therapy;615
8.10.1.1.8.1;References;615
8.10.2;Chapter 95;616
8.10.2.1;Sarcoidosis of the Liver;616
8.10.2.1.1;Defi nition;616
8.10.2.1.2;Epidemiology;616
8.10.2.1.3;Pathogenesis;616
8.10.2.1.4;Pathology;617
8.10.2.1.5;Diagnosis;617
8.10.2.1.5.1;Clinical Manifestations;617
8.10.2.1.5.2;Laboratory Findings;618
8.10.2.1.5.3;Imaging Techniques;618
8.10.2.1.6;Differential Diagnosis;618
8.10.2.1.7;Course and Prognosis;618
8.10.2.1.8;Therapy;619
8.10.2.1.8.1;References;619
8.11;Section XX: Interaction Between the Liver and Other Organ Systems;620
8.11.1;Chapter 96;621
8.11.1.1;Effects of Chronic Liver Disease on Other Organs: Tabellary Overview;621
8.11.1.1.1;References;622
8.11.2;Chapter 97;623
8.11.2.1;Hepatic Involvement in Extrahepatic Disease: Tabellary Overview;623
8.11.2.1.1;References;628
8.12;Section XXI: Pregnancy-Specific Liver Diseases;630
8.12.1;Chapter 98;632
8.12.1.1;Intrahepatic Cholestasis of Pregnancy;632
8.12.1.1.1;Defi nition;632
8.12.1.1.2;Epidemiology;632
8.12.1.1.3;Etiology and Pathogenesis;633
8.12.1.1.4;Pathology;633
8.12.1.1.5;Diagnosis;633
8.12.1.1.5.1;Clinical Manifestations;633
8.12.1.1.5.2;Laboratory Findings;633
8.12.1.1.5.3;Imaging Techniques;634
8.12.1.1.6;Differential Diagnosis;634
8.12.1.1.7;Course and Prognosis;634
8.12.1.1.8;Therapy;635
8.12.1.1.9;References;635
8.12.2;Chapter 99;637
8.12.2.1;Acute Fatty Liver of Pregnancy;637
8.12.2.1.1;Defi nition;637
8.12.2.1.2;Epidemiology;637
8.12.2.1.3;Etiology and Pathogenesis;637
8.12.2.1.4;Pathology;638
8.12.2.1.5;Diagnosis;638
8.12.2.1.5.1;Clinical Manifestations;638
8.12.2.1.5.2;Laboratory Findings;638
8.12.2.1.5.3;Imaging Techniques;638
8.12.2.1.6;Differential Diagnosis;638
8.12.2.1.7;Course and Prognosis;639
8.12.2.1.8;Therapy;639
8.12.2.1.9;References;640
8.12.3;Chapter 100;641
8.12.3.1;The Liver in Toxemia of Pregnancy;641
8.12.3.1.1;Defi nition;641
8.12.3.1.2;Epidemiology;641
8.12.3.1.3;Etiology and Pathogenesis;641
8.12.3.1.4;Pathology;642
8.12.3.1.5;Diagnosis;642
8.12.3.1.5.1;Clinical Manifestations;642
8.12.3.1.5.2;Laboratory Findings;642
8.12.3.1.5.3;Imaging Techniques;642
8.12.3.1.6;Complications;642
8.12.3.1.6.1;HELLP Syndrome;642
8.12.3.1.7;Prognosis and Therapy;643
8.12.3.1.8;References;643
8.13;Section XXII: Primary Tumors of the Liver and Intrahepatic Bile Ducts;644
8.13.1;Chapter 101;645
8.13.1.1;Benign Tumors;645
8.13.1.1.1;Introduction;647
8.13.1.1.1.1;References;647
8.13.1.1.2;101.1 Hepatocellular Adenoma;648
8.13.1.1.2.1;Defi nition;648
8.13.1.1.2.2;Epidemiology;648
8.13.1.1.2.3;Etiology;648
8.13.1.1.2.4;Pathogenesis;648
8.13.1.1.2.5;Pathology;649
8.13.1.1.2.6;Diagnosis;649
8.13.1.1.2.6.1;Clinical Manifestations;649
8.13.1.1.2.6.2;Laboratory Findings;650
8.13.1.1.2.6.3;Imaging Techniques;650
8.13.1.1.2.7;Differential Diagnosis;650
8.13.1.1.2.8;Prognosis and Therapy;651
8.13.1.1.2.8.1;References;651
8.13.1.1.3;101.2 Nodular Regenerative Hyperplasia;652
8.13.1.1.3.1;Defi nition;652
8.13.1.1.3.2;Epidemiology;652
8.13.1.1.3.3;Etiology;652
8.13.1.1.3.4;Pathogenesis;653
8.13.1.1.3.5;Pathology;653
8.13.1.1.3.6;Diagnosis;654
8.13.1.1.3.6.1;Clinical Manifestations;654
8.13.1.1.3.6.2;Laboratory Findings;654
8.13.1.1.3.6.3;Imaging Techniques;654
8.13.1.1.3.7;Differential Diagnosis;654
8.13.1.1.3.8;Natural History and Prognosis;654
8.13.1.1.3.9;Therapy;655
8.13.1.1.3.9.1;References;655
8.13.1.1.4;101.3 Focal Nodular Hyperplasia;656
8.13.1.1.4.1;Defi nition;656
8.13.1.1.4.2;Epidemiology;656
8.13.1.1.4.3;Etiology and Pathogenesis;656
8.13.1.1.4.4;Pathology;656
8.13.1.1.4.5;Diagnosis;657
8.13.1.1.4.5.1;Clinical Manifestations;657
8.13.1.1.4.5.2;Laboratory Findings;658
8.13.1.1.4.5.3;Imaging Techniques;658
8.13.1.1.4.6;Differential Diagnosis;658
8.13.1.1.4.7;Natural History and Prognosis;659
8.13.1.1.4.8;Therapy;659
8.13.1.1.4.8.1;References;659
8.13.1.1.5;101.4 Bile Duct Adenoma;660
8.13.1.1.5.1;Defi nition;660
8.13.1.1.5.2;Histology;660
8.13.1.1.5.3;Clinical Manifestations;660
8.13.1.1.5.4;Differential Diagnosis;660
8.13.1.1.5.5;Therapy;661
8.13.1.1.5.5.1;References;661
8.13.1.1.6;101.5 Biliary Cystadenoma;661
8.13.1.1.6.1;Defi nition and Epidemiology;661
8.13.1.1.6.2;Pathology;661
8.13.1.1.6.3;Diagnosis;661
8.13.1.1.6.3.1;Clinical Manifestations;661
8.13.1.1.6.3.2;Laboratory Findings;662
8.13.1.1.6.3.3;Imaging Techniques;662
8.13.1.1.6.4;Differential Diagnosis;662
8.13.1.1.6.4.1;References;662
8.13.1.1.7;101.6 Biliary Papillomatosis;663
8.13.1.1.7.1;Defi nition;663
8.13.1.1.7.2;Epidemiology, Etiology and Pathogenesis;663
8.13.1.1.7.3;Diagnosis;663
8.13.1.1.7.3.1;Clinical Manifestations;663
8.13.1.1.7.3.2;Laboratory Findings;663
8.13.1.1.7.3.3;Imaging Techniques;663
8.13.1.1.7.4;Differential Diagnosis;663
8.13.1.1.7.5;Prognosis;663
8.13.1.1.7.6;Therapy;663
8.13.1.1.7.6.1;References;664
8.13.1.1.8;101.7 Hemangioma;664
8.13.1.1.8.1;Defi nition;664
8.13.1.1.8.2;Epidemiology;664
8.13.1.1.8.3;Pathology;664
8.13.1.1.8.4;Diagnosis;664
8.13.1.1.8.4.1;Clinical Manifestations;664
8.13.1.1.8.4.2;Laboratory Findings;665
8.13.1.1.8.4.3;Imaging Techniques;665
8.13.1.1.8.5;Differential Diagnosis;666
8.13.1.1.8.6;Natural History and Prognosis;666
8.13.1.1.8.7;Treatment;667
8.13.1.1.8.7.1;References;667
8.13.1.1.9;101.8 Infantile Hemangioendothelioma;667
8.13.1.1.9.1;Defi nition and Epidemiology;667
8.13.1.1.9.2;Pathology;667
8.13.1.1.9.3;Diagnosis;667
8.13.1.1.9.3.1;Clinical Manifestations;667
8.13.1.1.9.3.2;Laboratory Findings;668
8.13.1.1.9.3.3;Imaging Techniques;668
8.13.1.1.9.4;Therapy;668
8.13.1.1.9.4.1;References;668
8.13.1.1.10;101.9 Lymphangioma and Hepatic Lymphangiomatosis;669
8.13.1.1.10.1;References;669
8.13.1.1.11;101.10 Mesenchymal Hamartoma;669
8.13.1.1.11.1;References;669
8.13.1.1.12;101.11 Lipomatous Tumors;670
8.13.1.1.12.1;Lipoma;670
8.13.1.1.12.2;Pseudolipoma;670
8.13.1.1.12.3;Focal Steatosis;670
8.13.1.1.12.4;Angiomyolipoma;670
8.13.1.1.12.4.1;Defi nition and Epidemiology;670
8.13.1.1.12.4.2;Pathology;670
8.13.1.1.12.5;Diagnosis;672
8.13.1.1.12.5.1;Clinical Manifestations;672
8.13.1.1.12.5.2;Laboratory Findings;672
8.13.1.1.12.5.3;Imaging Methods;672
8.13.1.1.12.5.4;References;672
8.13.1.1.13;101.12 Fibrous Tumors;673
8.13.1.1.13.1;Solitary Fibrous Tumor;673
8.13.1.1.13.2;Infl ammatory Pseudotumor;673
8.13.1.1.13.2.1;Defi nition and Epidemiology;673
8.13.1.1.13.2.2;Etiology and Pathogenesis;673
8.13.1.1.13.2.3;Pathology;674
8.13.1.1.13.2.4;Diagnosis;674
8.13.1.1.13.2.4.1;Clinical Manifestations;674
8.13.1.1.13.2.4.2;Laboratory Findings;674
8.13.1.1.13.2.4.3;Imaging Techniques;674
8.13.1.1.13.2.5;Differential Diagnosis;675
8.13.1.1.13.2.6;Therapy and Prognosis;675
8.13.1.1.13.2.6.1;References;675
8.13.1.1.14;101.13 Various Rare Tumors;676
8.13.1.1.14.1;References;676
8.13.2;Chapter 102;677
8.13.2.1;Malignant Tumors;677
8.13.2.1.1;102.1 Hepatocellular Carcinoma;679
8.13.2.1.1.1;Defi nition;679
8.13.2.1.1.2;Epidemiology;679
8.13.2.1.1.3;Etiology;680
8.13.2.1.1.4;Pathogenesis;682
8.13.2.1.1.4.1;Chronic Hepatitis B Virus Infection;683
8.13.2.1.1.4.2;Chronic Hepatitis C Virus Infection;684
8.13.2.1.1.4.3;Liver Cirrhosis;684
8.13.2.1.1.4.4;Alcoholic Liver Disease;684
8.13.2.1.1.4.5;Nonalcoholic Fatty Liver Disease;684
8.13.2.1.1.4.6;Afl atoxin B1 and Other Toxins;684
8.13.2.1.1.5;Pathology;685
8.13.2.1.1.5.1;Macroregenerative Nodules;685
8.13.2.1.1.5.2;Liver Cell Dysplasia;685
8.13.2.1.1.5.2.1;Large Cell Dysplasia;685
8.13.2.1.1.5.2.2;Small Cell Dysplasia;686
8.13.2.1.1.5.3;Dysplastic Foci;686
8.13.2.1.1.5.4;Dysplastic Nodules;686
8.13.2.1.1.5.4.1;Low Grade Dysplastic Nodules;686
8.13.2.1.1.5.4.2;High Grade Dysplastic Nodules;686
8.13.2.1.1.5.5;Progression from Dysplasia to Hepatocellular Carcinoma;686
8.13.2.1.1.5.6;Hepatocellular Carcinoma;687
8.13.2.1.1.5.6.1;Gross Anatomy;687
8.13.2.1.1.5.6.2;Microscopic Anatomy;687
8.13.2.1.1.5.7;Clinical Manifestations;689
8.13.2.1.1.6;Diagnosis;689
8.13.2.1.1.6.1;Laboratory Findings;689
8.13.2.1.1.6.1.1;alpha1-Fetoprotein;690
8.13.2.1.1.6.1.2;Des-gamma-Carboxy-Prothrombin;690
8.13.2.1.1.6.1.3;Novel Markers;690
8.13.2.1.1.6.2;Imaging Techniques;691
8.13.2.1.1.6.3;Approach to a Hepatic Mass in a Cirrhotic Liver;691
8.13.2.1.1.6.3.1;Lesions Less Than 1 cm in Diameter;693
8.13.2.1.1.6.3.2;Lesions 1–2 cm in Diameter;693
8.13.2.1.1.6.3.3;Lesions Larger Than 2 cm in Diameter;693
8.13.2.1.1.7;Differential Diagnosis;693
8.13.2.1.1.8;Prognosis and Staging;693
8.13.2.1.1.8.1;TNM System;695
8.13.2.1.1.8.2;Okuda Staging System;695
8.13.2.1.1.8.3;Cancer of the Liver Italian Program;696
8.13.2.1.1.8.4;Barcelona Clinic Liver Cancer Staging Classifi cation;697
8.13.2.1.1.8.5;Other Scoring Systems;697
8.13.2.1.1.8.6;Comparison of Different Staging Systems;697
8.13.2.1.1.9;Prevention and Screening;698
8.13.2.1.1.10;Therapy;698
8.13.2.1.1.10.1;Surgical Resection;698
8.13.2.1.1.10.2;Liver Transplantation;699
8.13.2.1.1.10.3;Locally Ablative Techniques;699
8.13.2.1.1.10.3.1;Transarterial Chemoembolization;700
8.13.2.1.1.10.3.2;Radiofrequency Ablation;700
8.13.2.1.1.10.3.3;Percutaneous Ethanol Injection;700
8.13.2.1.1.10.4;Medical Therapy;701
8.13.2.1.1.10.4.1;Systemic Chemotherapy;701
8.13.2.1.1.10.4.2;Hormonal Therapy;701
8.13.2.1.1.10.4.3;Molecularly Targeted Therapy;701
8.13.2.1.1.10.5;References;701
8.13.2.1.2;102.2 Fibrolamellar Carcinoma;709
8.13.2.1.2.1;Defi nition;709
8.13.2.1.2.2;Epidemiology;709
8.13.2.1.2.3;Etiology and Pathogenesis;709
8.13.2.1.2.4;Pathology;709
8.13.2.1.2.5;Diagnosis;709
8.13.2.1.2.6;Differential Diagnosis;709
8.13.2.1.2.7;Prognosis and Treatment;710
8.13.2.1.2.7.1;References;710
8.13.2.1.3;102.3 Hepatoblastoma;710
8.13.2.1.3.1;Defi nition;710
8.13.2.1.3.2;Epidemiology;710
8.13.2.1.3.3;Etiology and Pathogenesis;710
8.13.2.1.3.4;Pathology;710
8.13.2.1.3.5;Diagnosis;711
8.13.2.1.3.5.1;Clinical Manifestations;711
8.13.2.1.3.5.2;Technical Investigations;711
8.13.2.1.3.6;Course and Prognosis;711
8.13.2.1.3.7;Therapy;711
8.13.2.1.3.7.1;References;711
8.13.2.1.4;102.4 Angiosarcoma;712
8.13.2.1.4.1;Defi nition;712
8.13.2.1.4.2;Epidemiology;712
8.13.2.1.4.3;Etiology and Pathogenesis;712
8.13.2.1.4.4;Pathology;712
8.13.2.1.4.5;Diagnosis;713
8.13.2.1.4.5.1;Clinical Manifestations;713
8.13.2.1.4.5.2;Laboratory Findings;713
8.13.2.1.4.5.3;Imaging Techniques;713
8.13.2.1.4.6;Differential Diagnosis;713
8.13.2.1.4.7;Course and Prognosis;713
8.13.2.1.4.8;Therapy;714
8.13.2.1.4.8.1;References;714
8.13.2.1.5;102.5 Epithelioid Hemangioendothelioma;715
8.13.2.1.5.1;Defi nition;715
8.13.2.1.5.2;Epidemiology;715
8.13.2.1.5.3;Etiology and Pathogenesis;715
8.13.2.1.5.4;Diagnosis;716
8.13.2.1.5.4.1;Clinical Manifestations;716
8.13.2.1.5.4.2;Laboratory Findings;716
8.13.2.1.5.4.3;Imaging Techniques;716
8.13.2.1.5.5;Differential Diagnosis;716
8.13.2.1.5.6;Course and Prognosis;716
8.13.2.1.5.7;Therapy;716
8.13.2.1.5.7.1;References;717
8.13.2.1.6;102.6 Embryonal Sarcoma;717
8.13.2.1.6.1;Defi nition;717
8.13.2.1.6.2;Epidemiology;717
8.13.2.1.6.3;Etiology and Pathogenesis;717
8.13.2.1.6.4;Pathology;717
8.13.2.1.6.5;Diagnosis;718
8.13.2.1.6.5.1;Clinical Manifestations;718
8.13.2.1.6.5.2;Laboratory Findings;718
8.13.2.1.6.5.3;Imaging Techniques;718
8.13.2.1.6.6;Differential Diagnosis;718
8.13.2.1.6.7;Course and Prognosis;718
8.13.2.1.6.8;Therapy;718
8.13.2.1.6.8.1;References;718
8.13.2.1.7;102.7 Primary Hepatic Malignant Lymphoma;719
8.13.2.1.7.1;Epidemiology;719
8.13.2.1.7.2;Pathogenesis;719
8.13.2.1.7.3;Pathology;719
8.13.2.1.7.4;Diagnosis;719
8.13.2.1.7.4.1;Clinical Manifestations;719
8.13.2.1.7.4.2;Laboratory Findings;719
8.13.2.1.7.4.3;Imaging Techniques;720
8.13.2.1.7.5;Differential Diagnosis;720
8.13.2.1.7.6;Course and Prognosis;720
8.13.2.1.7.7;Therapy;720
8.13.2.1.7.7.1;References;720
8.13.2.1.8;102.8 Various Rare Tumors;721
8.13.2.1.8.1;Malignant Fibrous Histiocytoma;721
8.13.2.1.8.2;Kaposi’s Sarcoma;721
8.13.2.1.8.3;Other Rare Tumors;722
8.13.2.1.8.3.1;References;722
8.14;Section XXIII: Liver Transplantation and Surgery in Liver Disease;723
8.14.1;Chapter 103;724
8.14.1.1;Liver Transplantation: Indications, Preoperative Evaluation and Posttransplantation Management;724
8.14.1.1.1;History;725
8.14.1.1.2;Philosophy;725
8.14.1.1.3;The Evaluation Process;726
8.14.1.1.4;Listing for Transplantation;726
8.14.1.1.5;Scoring Systems and Prioritization on the Waiting List;727
8.14.1.1.6;Impact of MELD on Outcomes and Transplant Benefi t;727
8.14.1.1.7;Indications for Transplantation;728
8.14.1.1.7.1;Minimal Listing Criteria;728
8.14.1.1.7.2;Alcoholic Liver Disease;729
8.14.1.1.7.3;Viral Hepatitis;730
8.14.1.1.7.3.1;Hepatitis C;730
8.14.1.1.7.3.2;Hepatitis B;730
8.14.1.1.7.4;Cholestatic Liver Disease;731
8.14.1.1.7.5;Malignant Diseases of the Liver;731
8.14.1.1.7.5.1;Cholangiocarcinoma;732
8.14.1.1.7.5.2;Hepatocellular Carcinoma;732
8.14.1.1.7.6;Metabolic Liver Disease;735
8.14.1.1.7.7;Vascular Disease of the Liver;735
8.14.1.1.7.8;Fulminant Hepatic Failure;735
8.14.1.1.8;Indications for Liver Transplant in the Child;736
8.14.1.1.8.1;Pediatric Cholestatic Diseases;736
8.14.1.1.8.2;Pediatric Metabolic Liver Diseases;737
8.14.1.1.8.3;Hepatic Malignancy in Children;737
8.14.1.1.8.4;Retransplantation in Children;737
8.14.1.1.9;Evaluation and Listing;737
8.14.1.1.10;Contraindications to Liver Transplant;739
8.14.1.1.10.1;Age;739
8.14.1.1.10.2;Psychosocial Contraindications;740
8.14.1.1.10.3;Cardiopulmonary Contraindications;740
8.14.1.1.10.4;Infectious Contraindications;740
8.14.1.1.10.4.1;HIV Infection;740
8.14.1.1.10.4.2;Other Infectious Contraindications;741
8.14.1.1.11;The Waiting List;741
8.14.1.1.11.1;Management on the Waiting List;741
8.14.1.1.11.2;Timing of Transplantation;741
8.14.1.1.11.3;Delisting Criteria;742
8.14.1.1.12;Living Donor Liver Transplantation;742
8.14.1.1.13;The Posttransplant Period;743
8.14.1.1.13.1;The Perioperative Period;743
8.14.1.1.13.2;Immunosuppression;744
8.14.1.1.13.3;Rejection;746
8.14.1.1.13.4;New Onset Diabetes Mellitus Following Transplant;746
8.14.1.1.13.5;Arterial Hypertension;746
8.14.1.1.13.6;Hyperlipidemia;747
8.14.1.1.13.7;Biliary Complications;747
8.14.1.1.13.8;Vascular Complications;747
8.14.1.1.13.9;Recurrent Disease;747
8.14.1.1.13.10;Infection;748
8.14.1.1.14;Conclusion;749
8.14.1.1.15;References;750
8.14.2;Chapter 104;753
8.14.2.1;Risk of Surgery in Patients with Liver Disease;753
8.14.2.1.1;Effects of Surgery on the Liver;753
8.14.2.1.1.1;Liver Biochemical Tests;753
8.14.2.1.1.2;Hepatic Blood Flow and Hemodynamic Effects;754
8.14.2.1.1.3;Hypoxemia;754
8.14.2.1.1.4;Hepatic Metabolism of Anesthetic Agents;755
8.14.2.1.2;Operative Risk in Patients with Liver Disease;755
8.14.2.1.2.1;Challenges in Estimating Operative Risk;755
8.14.2.1.2.2;Preoperative Screening;756
8.14.2.1.2.3;Conditions for Which Elective Surgery is Contraindicated;757
8.14.2.1.2.3.1;Acute Hepatitis;757
8.14.2.1.2.3.2;Acute Alcoholic Hepatitis;757
8.14.2.1.2.3.3;Acute Liver Failure;758
8.14.2.1.2.4;Surgery in Patients with Chronic Liver Disease;758
8.14.2.1.2.4.1;Chronic Viral Hepatitis;758
8.14.2.1.2.4.2;Nonalcoholic Fatty Liver Disease (NAFLD);759
8.14.2.1.2.4.3;Other Causes of Chronic Liver Disease;759
8.14.2.1.2.4.4;Cirrhosis;760
8.14.2.1.2.4.4.1;Operative Risk Stratifi cation by Child Class;760
8.14.2.1.2.4.4.2;Operative Risk Stratifi cation by MELD Score;761
8.14.2.1.2.4.4.3;Operative Risk Associated with Specifi c Types of Surgery;761
8.14.2.1.2.4.4.4;Endoscopic Procedures;764
8.14.2.1.2.4.5;Ascites;765
8.14.2.1.2.4.6;Renal Dysfunction;765
8.14.2.1.2.4.7;Encephalopathy;765
8.14.2.1.2.4.8;Nutrition;766
8.14.2.1.3;Postoperative Monitoring;766
8.14.2.1.4;References;766
8.15;Section XXIV: Gene Therapy;770
8.15.1;Chapter 105;771
8.15.1.1;A Look to the Future: Gene Therapy in Liver Diseases;771
8.15.1.1.1;Genetic Classifi cation of Diseases;771
8.15.1.1.2;Gene Therapy of Liver Diseases;772
8.15.1.1.2.1;Gene Repair;772
8.15.1.1.2.2;Gene Substitution;772
8.15.1.1.2.3;Hepatocyte Transplantation;773
8.15.1.1.2.4;Block of Gene Expression or Function;773
8.15.1.1.2.4.1;Ribozymes;773
8.15.1.1.2.4.2;Small Interfering RNA;774
8.15.1.1.2.4.3;Antisense Oligonucleotides;774
8.15.1.1.2.4.4;Interfering Peptides or Proteins;774
8.15.1.1.2.5;DNA Vaccination;775
8.15.1.1.2.6;Gene Augmentation;775
8.15.1.1.2.6.1;Suicide Gene Therapy;775
8.15.1.1.2.6.2;Antiangiogenic Gene Therapy;776
8.15.1.1.2.6.3;Oncolytic Viruses;776
8.15.1.1.2.7;Immune Therapy;776
8.15.1.1.2.7.1;AFP-Specifi c Immune Therapy;776
8.15.1.1.2.7.2;Immune Therapy with Antigen Presenting Cells;776
8.15.1.1.2.7.3;Cytokine Gene Therapy;776
8.15.1.1.3;Molecular Prevention of Liver Diseases;776
8.15.1.1.4;Summary and Perspectives;777
8.15.1.1.5;References;777
9;Part V: Diseases of the Gallbladder and Extrahepatic Bile Ducts;781
9.1;Section XXV: Anatomy, Histology and Physiology;782
9.1.1;Chapter 106;783
9.1.1.1;Gross and Microscopic Anatomy;783
9.1.1.1.1;Embryonic Development;783
9.1.1.1.2;Gallbladder;783
9.1.1.1.3;Extrahepatic Bile Ducts;784
9.1.1.1.4;References;787
9.1.2;Chapter 107;788
9.1.2.1;Physiology of the Gallbladder and the Extrahepatic Bile Ducts;788
9.1.2.1.1;Physiology of the Gallbladder;788
9.1.2.1.1.1;Bile Storage and Excretion;788
9.1.2.1.1.2;Gallbladder Motility;789
9.1.2.1.2;Physiology of the Extrahepatic Bile Ducts;789
9.1.2.1.3;Physiology of the Sphincter of Oddi;790
9.1.2.1.4;References;791
9.2;Section XXVI: Developmental Anomalies;792
9.2.1;Chapter 108;793
9.2.1.1;Anomalies of the Gallbladder and the Cystic Duct;793
9.2.1.1.1;Numerical Anomalies of the Gallbladder;793
9.2.1.1.2;Positional Anomalies of the Gallbladder;794
9.2.1.1.3;Internal and External Morphological Anomalies of the Gallbladder;795
9.2.1.1.4;Anomalies of the Cystic Duct;795
9.2.1.1.5;References;796
9.2.2;Chapter 109;797
9.2.2.1;Anomalies of the Extrahepatic Bile Ducts;797
9.2.2.1.1;Anomalies in the Number and/or Course of the Extrahepatic Bile Ducts;797
9.2.2.1.2;Form Anomalies of the Extrahepatic Bile Ducts;798
9.2.2.1.2.1;Congenital Bile Duct Cysts;798
9.2.2.1.2.2;Congenital Biliary Atresia and Stenosis;799
9.2.2.1.2.3;References;800
9.2.3;Chapter 110;801
9.2.3.1;Benign Strictures of the Extrahepatic Bile Ducts;801
9.2.3.1.1;References for Chapters 108, 109, 110;801
9.3;Section XXVII: Motility Disorders;803
9.3.1;Chapter 111;804
9.3.1.1;Motility Disorders of the Bile Ducts and Postcholecystectomy Syndrome;804
9.3.1.1.1;Postcholecystectomy Syndrome;804
9.3.1.1.1.1;Defi nition and Epidemiology;804
9.3.1.1.1.2;Etiology and Pathogenesis;804
9.3.1.1.1.3;Clinical Presentation and Diagnosis;805
9.3.1.1.1.4;Differential Diagnosis;805
9.3.1.1.1.5;Bile Duct Stones;808
9.3.1.1.1.6;Long Cystic Duct with Retained Stone;808
9.3.1.1.1.7;Bile Duct Strictures;808
9.3.1.1.1.8;Stenosis and Tumors of the Papilla;808
9.3.1.1.2;Sphincter of Oddi Dysfunction;808
9.3.1.1.2.1;Diagnostic Procedures;808
9.3.1.1.2.2;Sphincter of Oddi Manometry;810
9.3.1.1.2.3;Pharmacologic Provocation Tests;810
9.3.1.1.2.4;ERCP, MRCP;810
9.3.1.1.2.4.1;Abdominal Ultrasound with Provocation Tests;812
9.3.1.1.2.4.2;Hepatobiliary Scintigraphy;812
9.3.1.1.2.4.3;Probatory Stent Placement;812
9.3.1.1.2.4.4;Botulinum Toxin Injection (BoTox);813
9.3.1.1.3;Sphincter of Oddi Dyskinesia;814
9.3.1.1.3.1;SOD Type I;814
9.3.1.1.3.2;SOD Type II;815
9.3.1.1.3.3;SOD Type III;815
9.3.1.1.3.4;Clinical Course and Prognosis;816
9.3.1.1.3.5;Therapy;816
9.3.1.1.3.5.1;Endoscopic Interventional Therapy;817
9.3.1.1.4;References;817
9.4;Section XXVIII: Gallstones;819
9.4.1;Chapter 112;820
9.4.1.1;Gallbladder Stones;820
9.4.1.1.1;Epidemiology;820
9.4.1.1.2;Etiology and Pathogenesis;822
9.4.1.1.2.1;Cholesterol Stones;822
9.4.1.1.2.2;Black Pigment Stones;825
9.4.1.1.2.3;Brown Pigment Stones;826
9.4.1.1.2.4;Gallbladder Sludge;828
9.4.1.1.3;Diagnosis;828
9.4.1.1.3.1;Ultrasound;828
9.4.1.1.3.2;Radiological Techniques;828
9.4.1.1.3.3;Natural Course;830
9.4.1.1.3.4;Asymptomatic Cholelithiasis;831
9.4.1.1.3.5;Symptomatic Cholelithiasis;831
9.4.1.1.4;Complications of Cholecystolithiasis;832
9.4.1.1.4.1;Acute Bacterial Cholecystitis;832
9.4.1.1.4.1.1;Gallbladder Empyema and Hydrops;834
9.4.1.1.4.1.2;Emphysematous Cholecystitis;834
9.4.1.1.4.1.3;Chronic Cholecystitis;834
9.4.1.1.4.1.4;Chalk Milk Bile, Porcelain Gallbladder;834
9.4.1.1.4.1.5;Mirizzi Syndrome;835
9.4.1.1.4.1.6;Stone Perforation and Stone Ileus;835
9.4.1.1.4.1.7;Gallbladder Carcinoma;835
9.4.1.1.4.1.8;Biliary Pancreatitis;835
9.4.1.1.5;Gallstone Prophylaxis;835
9.4.1.1.6;Therapy;836
9.4.1.1.6.1;Oral Litholysis;836
9.4.1.1.6.2;Extracorporeal Shock Wave Lithotripsy;837
9.4.1.1.6.3;Contact Litholysis;838
9.4.1.1.6.4;Cholecystectomy;838
9.4.1.1.6.4.1;Postcholecystectomy Syndrome;839
9.4.1.1.6.5;Treatment of Cholecystitis and Its Complications;839
9.4.1.1.6.5.1;Cholecystectomy;839
9.4.1.1.6.5.2;Cholecystolithotomy, Cholecystostomy;840
9.4.1.1.7;References;840
9.4.2;Chapter 113;841
9.4.2.1;Bile Duct Stones;841
9.4.2.1.1;Epidemiology;841
9.4.2.1.2;Clinics;842
9.4.2.1.3;Diagnosis;842
9.4.2.1.3.1;Ultrasound;842
9.4.2.1.3.2;Radiologic Techniques;842
9.4.2.1.4;Complications;843
9.4.2.1.4.1;Cholangitis;844
9.4.2.1.4.2;Liver Abscess;844
9.4.2.1.4.3;Biliary Pancreatitis;844
9.4.2.1.4.4;Bile Duct Carcinoma;845
9.4.2.1.5;Treatment of Choledocholithiasis and Cholangitis;845
9.4.2.1.5.1;Endoscopic Stone Extraction;845
9.4.2.1.5.2;Adjuvant Treatment Procedures;846
9.4.2.1.5.3;Treatment of Cholangitis;847
9.4.2.1.5.4;Surgery for Bile Duct Stones;848
9.4.2.1.5.5;Therapy of Hepatolithiasis;848
9.4.2.1.5.6;Cholelitholysis;848
9.4.2.1.6;References;849
9.5;Section XXIX: Infectious Disorders;851
9.5.1;Chapter 114;852
9.5.1.1;Biliary Infections;852
9.5.1.1.1;Bacterial Cholangitis;852
9.5.1.1.1.1;Pathogenesis;852
9.5.1.1.1.2;Bacteria in Bile;852
9.5.1.1.1.3;Bile Duct Obstruction;853
9.5.1.1.1.4;Diagnosis;853
9.5.1.1.1.5;Treatment;854
9.5.1.1.1.6;Antibiotic Treatment;854
9.5.1.1.1.7;Decompression of Bile Ducts;855
9.5.1.1.1.8;Prophylaxis;856
9.5.1.1.2;Fungal Cholangitis;857
9.5.1.1.2.1;Diagnosis;857
9.5.1.1.2.2;Treatment;857
9.5.1.1.3;Parasitic Cholangitis;857
9.5.1.1.3.1;Pathogenesis;857
9.5.1.1.3.2;Diagnosis and Treatment;857
9.5.1.1.3.2.1;Nematode Cholangitis;857
9.5.1.1.3.2.2;Trematode Cholangitis;858
9.5.1.1.4;HIV Cholangiopathy;858
9.5.1.1.5;Pathogenesis;859
9.5.1.1.6;Diagnosis;859
9.5.1.1.7;Treatment;859
9.5.1.1.8;References;860
9.6;Section XXX: Tumors of the Gallbladder and Extrahepatic Bile Ducts;862
9.6.1;Chapter 115;863
9.6.1.1;Benign Tumors;863
9.6.1.2;Benign Tumors of the Gallbladder;863
9.6.1.2.1;Defi nition;863
9.6.1.2.2;Epidemiology;863
9.6.1.2.3;Etiology and Pathogenesis;864
9.6.1.2.4;Pathology;864
9.6.1.2.5;Clinical Manifestations;865
9.6.1.2.6;Diagnosis;865
9.6.1.2.7;Differential Diagnosis;866
9.6.1.2.8;Therapy and Prognosis;866
9.6.1.3;Benign Tumors of the Extrahepatic Bile Ducts;867
9.6.1.3.1;Defi nition;867
9.6.1.3.2;Epidemiology;867
9.6.1.3.3;Etiology and Pathogenesis;867
9.6.1.3.4;Pathology;868
9.6.1.3.5;Clinical Manifestations;868
9.6.1.3.6;Diagnosis;868
9.6.1.3.7;Differential Diagnosis;869
9.6.1.3.8;Therapy and Prognosis;869
9.6.1.4;Benign Tumors of the Ampulla of Vater;870
9.6.1.4.1;Defi nition;870
9.6.1.4.2;Epidemiology;870
9.6.1.4.3;Etiology and Pathogenesis;870
9.6.1.4.4;Pathology;870
9.6.1.4.5;Clinical Manifestations;871
9.6.1.4.6;Diagnosis;871
9.6.1.4.7;Differential Diagnosis;871
9.6.1.4.8;Therapy and Prognosis;872
9.6.1.5;References;873
9.6.2;Chapter 116;876
9.6.2.1;Malignant Tumors;876
9.6.2.1.1;Gallbladder Cancer;876
9.6.2.1.1.1;Defi nition;876
9.6.2.1.1.2;Epidemiology;876
9.6.2.1.1.3;Etiology and Pathogenesis;877
9.6.2.1.1.4;Pathology;878
9.6.2.1.1.5;Clinical Manifestations;878
9.6.2.1.1.6;Diagnosis;878
9.6.2.1.1.7;Differential Diagnosis;879
9.6.2.1.1.8;Therapy and Prognosis;879
9.6.2.1.1.8.1;Curative Treatment;879
9.6.2.1.1.8.2;Palliative Treatment;880
9.6.2.1.1.9;Recurrent Gallbladder Cancer;881
9.6.2.1.2;Extrahepatic Cholangiocarcinoma;881
9.6.2.1.2.1;Defi nition;881
9.6.2.1.2.2;Epidemiology;881
9.6.2.1.2.3;Etiology and Pathogenesis;883
9.6.2.1.2.4;Pathology;884
9.6.2.1.2.5;Differential Diagnosis;886
9.6.2.1.2.6;Therapy and Prognosis;887
9.6.2.1.2.6.1;Curative Treatment for Hilar Cholangiocarcinoma;887
9.6.2.1.2.6.2;Preoperative Biliary Drainage;888
9.6.2.1.2.6.3;Resection of Hilar Cholangiocarcinoma;888
9.6.2.1.2.6.4;Liver Transplantation for Hilar Cholangiocarcinoma;890
9.6.2.1.2.6.5;Resection of Distal Cholangiocarcinoma;890
9.6.2.1.2.6.6;Neoadjuvant and Adjuvant Therapy;891
9.6.2.1.2.6.7;Palliative Management;893
9.6.2.1.2.6.7.1;Stenting for Extrahepatic Cholangiocarcinoma;893
9.6.2.1.2.6.7.2;Photodynamic Therapy (PDT) for Hilar Cholangiocarcinoma;895
9.6.2.1.2.6.7.3;Surgical Palliation;898
9.6.2.1.2.6.7.4;Palliative Chemotherapy;898
9.6.2.1.2.6.7.5;Palliative Radiotherapy;900
9.6.2.1.2.6.7.6;Palliative Chemoradiation;900
9.6.2.1.2.6.8;Future Directions of Palliation;904
9.6.2.1.2.6.8.1;Progress in PDT;904
9.6.2.1.2.6.8.2;Progress in Chemotherapy;905
9.6.2.1.2.6.8.3;Molecular Targeted Therapy;906
9.6.2.1.2.6.9;Recurrent Extrahepatic Cholangiocarcinoma;908
9.6.2.1.2.6.10;Management of Tumor Complications;908
9.6.2.1.2.6.11;Prognostic Indicators in Patients with Hilar Cholangiocarcinoma;908
9.6.2.1.2.7;Malignant Tumors of the Ampulla of Vater;909
9.6.2.1.2.7.1;Defi nition;909
9.6.2.1.2.7.2;Epidemiology;909
9.6.2.1.2.7.3;Etiology and Pathogenesis;909
9.6.2.1.2.7.4;Pathology;909
9.6.2.1.2.7.5;Clinical Manifestations;909
9.6.2.1.2.7.6;Diagnosis;909
9.6.2.1.2.7.7;Differential Diagnosis;910
9.6.2.1.2.7.8;Therapy and Prognosis;911
9.6.2.1.2.8;References;911
10;Index;924
