Clinical Hepatology : Principles and Practice of Hepatobiliary Diseases: Volume 1

Clinical Hepatology......Page 2 Title Page......Page 3 Copyright Page......Page 4 Dedication......Page 5 Foreword......Page 6 Preface......Page 7 Volume 1......Page 9 Contents of Volume 1......Page 10 Contributors......Page 19 A. Basic Principles......Page 25 Part I: Structure and Function of the Liver......Page 26 Section I: Embryology, Anatomy, and Histology......Page 27 General Development......Page 28 Molecular Control of Liver Development......Page 30 References......Page 31 Descriptive Anatomy......Page 32 Functional Anatomy......Page 34 References......Page 35 Liver Lobule and Liver Acinus......Page 36 Liver Lobule......Page 37 Liver Acinus......Page 39 Intrahepatic Bile Ducts......Page 40 Lymphatics......Page 42 Cell Types......Page 43 Hepatocytes......Page 44 Lateral Membrane......Page 46 Nucleus......Page 47 Golgi Apparatus......Page 48 Lysosomes......Page 50 Mitochondria......Page 51 Cytoskeleton......Page 52 Sinusoidal Endothelial Cells......Page 53 Kupffer Cells......Page 55 Hepatic Stellate Cells......Page 57 Pit Cells (Liver Associated Lymphocytes)......Page 60 Hepatic Stem Cells......Page 61 Collagen Structure......Page 63 Collagen Type V......Page 64 Elastin......Page 65 Proteoglycans......Page 66 Laminin......Page 67 Structural Organization......Page 68 Cell Types......Page 69 Extracellular Matrix......Page 71 Section II: Fundamentals of Hepatic Physiology and Biochemistry......Page 73 Hepatic Blood Flow......Page 74 Regulation of Hepatic Circulation......Page 75 Vasoactive Substances......Page 76 References......Page 78 Basic Principles......Page 79 Transport Proteins......Page 81 Pumps......Page 82 Vesicle Transport......Page 83 Basolateral (sinusoidal) Transporters......Page 84 Apical (canalicular) Transporters......Page 87 Regulation of Intracellular pH......Page 88 Vesicle Transport of Hepatocellular Proteins......Page 89 Receptor Mediated Endocytosis......Page 90 Fate of Ligands and Receptors......Page 91 References......Page 92 Chapter Outline......Page 93 Glycolysis......Page 94 Gluconeogenesis......Page 95 Glycogen Metabolism......Page 97 Liver and Carbohydrate Homeostasis......Page 98 Amino Acids and Proteins......Page 100 Nitrogen Compounds......Page 101 Ammonia......Page 102 Lipids......Page 103 Fatty Acid Degradation......Page 104 Fatty Acid Synthesis......Page 105 Synthesis of Complex Lipids......Page 106 Lipoproteins......Page 107 Copper......Page 110 Heme and Non-Heme Iron......Page 111 Non-Heme Iron......Page 112 Heme Iron......Page 116 Carbohydrates, Amino Acids and Proteins, Lipids, Vitamin A......Page 117 Heme and Non-Heme Iron......Page 118 Bile Composition......Page 121 Bile Formation in the Bile Ducts......Page 122 Bile Lipids and Bilirubin Cholesterol......Page 123 Bile Acid Synthesis......Page 125 Secondary and Tertiary Bile Acids......Page 126 Phospholipids......Page 127 Transport and Uptake into Hepatocytes......Page 128 Glucuronidation......Page 129 Bilirubin in the Intestine......Page 130 Bile Acid Dependent Bile Secretion......Page 131 Bile Acid Independent Bile Secretion......Page 132 Functional Regulation of Bile Secretion......Page 133 Enterohepatic Circulation of Bile Acids......Page 134 Bile Acid Uptake into the Hepatocyte......Page 135 Transcellular Bile Acid Transport......Page 136 Lithogenic Bile......Page 137 Bile Acid Metabolism During Intrahepatic Cholestasis......Page 140 Cholestasis Caused by Toxic Bile Acids......Page 141 References......Page 142 Phase I Reactions......Page 144 Cytochrome P450 System......Page 145 References......Page 147 Parenchymal Cells......Page 148 Glucose Metabolism......Page 150 Detoxification of Ammonium......Page 151 Bile Acid Extraction......Page 152 References......Page 153 Chapter Outline......Page 154 Protein Turnover......Page 155 Carbohydrate Metabolism......Page 156 References......Page 157 Chapter Outline......Page 158 Intrahepatic Lymphocytes......Page 159 AlphaBeta-T Cell Receptor Lymphocytes......Page 160 Natural Killer (NK) Cells......Page 161 Hepatocytes......Page 162 Biliary Epithelial Cells......Page 163 Biliary Immunoglobulins......Page 164 Immunologic Tolerance......Page 165 Intrahepatic Immune Responses......Page 166 Liver and the Acute Phase Reaction......Page 167 References......Page 168 Exogenous Factors......Page 170 References......Page 172 Chapter Outline......Page 174 Morphology of Liver Regeneration......Page 175 Extracellular Matrix......Page 176 Hormones and Neurotransmitters......Page 177 Growth Factors and Cytokines......Page 178 Cell Cycle and Cell Proliferation......Page 180 Molecular Mechanismsof Liver Regeneration......Page 181 References......Page 184 Part II: Pathophysiology and Morphology of Liver Injury......Page 186 Section III: Causes and Mechanisms of Liver Injury......Page 187 Chapter Outline......Page 188 Free Radicals and Reactive Oxygen Species......Page 189 Lipid Peroxidation......Page 190 Oxidative Liver Damage......Page 191 Unfolded Protein Response......Page 192 Vitamin E......Page 193 Enzymes......Page 194 References......Page 195 Hepatocellular Ischemia......Page 196 References......Page 198 Ischemia-Reperfusion Injury......Page 199 References......Page 201 General Principles......Page 202 References......Page 203 Chapter Outline......Page 204 Antiviral Immune Reactions......Page 205 Cytotoxic T Lymphocytes......Page 206 Autoimmune Reactions......Page 207 Primary Biliary Cirrhosis......Page 208 References......Page 209 Endotoxin-Induced Cholestasis......Page 210 References......Page 211 General Principles......Page 212 References......Page 213 Iron and Copper-Induced Liver Injury......Page 214 References......Page 215 General Principles......Page 216 References......Page 217 Section IV: Morphologic Patterns of Liver Injury......Page 218 Hydropic Cell Swelling......Page 219 Cytoplasmic Deposits......Page 220 Necrosis......Page 221 Single Cell Necrosis......Page 222 Necrosis of Cell Groups and Confluent Necrosis......Page 223 Surgical “Necrosis”......Page 224 Apoptosis......Page 225 Molecular Mechanisms......Page 226 Apoptosis in Liver Diseases......Page 229 References......Page 230 Ground-Glass Hepatocytes......Page 231 Megamitochondria......Page 234 Lipids......Page 236 Carbohydrates......Page 237 Proteins......Page 238 Metals......Page 239 Pigments......Page 240 Foreign Bodies......Page 242 Infectious Agents......Page 243 Glycogen......Page 244 Viral Inclusions......Page 245 References......Page 246 Chapter Outline......Page 247 Inflammatory Reactions......Page 248 References......Page 251 Hepatic Histopathology in Biliary Obstruction......Page 252 Ductular Reaction......Page 256 References......Page 257 Immune Granulomas......Page 258 Granulomatous Hepatitis......Page 259 References......Page 260 Etiology......Page 261 Hepatic Stellate Cells (Ito Cells)......Page 262 Transforming Growth Factor-beta1 (TGF-beta1)......Page 263 Endothelin-1......Page 264 Extracellular Matrix......Page 265 Portal and Periportal Fibrosis......Page 266 Perisinusoidal and Pericellular Fibrosis......Page 267 Diagnosis......Page 268 Limitations of Liver Biopsy......Page 269 Cross-Sectional Imaging......Page 271 Serum Biomarkers of Fibrogenesis......Page 272 Therapeutic Approaches......Page 273 References......Page 276 Section V: Scoring Systems in Hepatology......Page 279 Abbreviations......Page 280 Grading and Staging of Chronic Hepatitis......Page 281 Autoimmune Hepatitis......Page 285 Fatty Liver Disease/Steatohepatitis......Page 286 Biliary Diseases......Page 289 Hepatic Iron-Overload......Page 291 Semiquantitative Scoring System for Evaluation of Hepatic Fibrosis......Page 292 Rejection in Liver Transplant Pathology......Page 293 Future Developments......Page 294 References......Page 295 Child-Turcotte-Pugh Score......Page 298 Model for End-Stage Liver Disease......Page 299 Maddrey Index (Discriminant Function) and Lille Model......Page 300 International Autoimmune Hepatitis Group Score......Page 301 References......Page 302 B. Clinical Methods......Page 303 Part III: Evaluation of the Patient with Hepatobiliary Disease......Page 304 Section VI: History and Physical Examination......Page 305 Family History......Page 306 Past and Present Medical History......Page 307 Fatigue......Page 309 Pain......Page 310 References......Page 311 General Appearance of the Patient......Page 312 Examination of the Liver......Page 313 Examination of the Spleen......Page 314 Jaundice (Icterus)......Page 315 Palmar Erythema......Page 316 Lichen Planus......Page 317 Neurologic-Psychiatric Changes......Page 318 References......Page 319 Section VII: Laboratory Testing......Page 320 Enzymes......Page 321 Aminotransferases......Page 322 Glutamate Dehydrogenase......Page 324 Gamma-Glutamyl Transferase......Page 325 Alkaline Phosphatase......Page 326 5'-Nucleotidase......Page 327 Cholinesterase......Page 328 Albumin......Page 329 Clotting Factors......Page 330 Ammonia......Page 331 Bilirubin......Page 332 References......Page 333 Chapter Outline......Page 334 Assessment......Page 336 Assessment......Page 337 Principle and Technique......Page 338 Principle and Technique......Page 339 Principle and Technique......Page 340 Confounding Factors......Page 341 Side Effects......Page 342 References......Page 343 Chapter Outline......Page 345 Occurrence and Signifi cance......Page 347 Occurrence and Signifi cance......Page 349 Occurrence and Signifi cance......Page 350 Occurrence and Signifi cance......Page 351 Immunofl uorescence Reactivities......Page 352 Target Antigens......Page 353 References......Page 354 Section VIII: Hepatobiliary Imaging and Manometric Studies......Page 356 Introduction......Page 357 Liver Cirrhosis......Page 358 Acute Viral Hepatitis......Page 359 Primary Biliary Cirrhosis......Page 360 Primary Sclerosing Cholangitis......Page 361 Blood Supply of Hepatic Vessels......Page 362 Vascular (Doppler) Indices......Page 363 Portal Hypertension......Page 364 Doppler Wave Form Changes in Patients with Severe Congestive Heart Failure......Page 365 Portal Vein Thrombosis......Page 366 Which Liver Vein should be examined?......Page 367 Veno-occlusive Disease......Page 368 Focal Liver Lesions......Page 369 Detection and Characterization of Liver Tumors......Page 370 Ultrasound Contrast Agents......Page 371 Differentiation of Benign and Malignant Lesions......Page 372 Hemangioma......Page 374 Focal Nodular Hyperplasia......Page 377 Hepatocellular Adenoma......Page 380 Focal Steatosis......Page 382 Hepatocellular Carcinoma......Page 384 Metastases......Page 385 Abscess......Page 388 Gallbladder Disease......Page 389 Other Congenital Anomalies of the Gallbladder......Page 390 Acute Cholecystitis......Page 391 Miscellaneous Non-tumor Disorders......Page 392 Choledochal Cysts......Page 393 Choledocholithiasis......Page 394 Cholangiocellular Carcinoma......Page 395 Other Extrahepatic Bile Duct Tumors......Page 396 References......Page 397 Chapter Outline......Page 403 Magnetic Resonance Imaging......Page 404 Focal Nodular Hyperplasia......Page 405 Hepatocellular Adenoma......Page 406 Hepatocellular Carcinoma......Page 410 Intrahepatic Cholangiocarcinoma......Page 411 Metastases......Page 412 Bacterial Hepatic Abscesses......Page 415 Amebic Abscesses......Page 416 Hepatic Echinococcal Disease......Page 418 References......Page 419 Principle of Investigation and Pathophysiology......Page 422 Acquisition Technology......Page 423 Acquisition Technology......Page 424 Interpretation......Page 425 Focal Liver Lesions......Page 426 Liver Metastases......Page 427 18F-FDG-PET/ -CT......Page 429 Hepatocellular Carcinoma......Page 430 Liver Metastases......Page 431 References......Page 432 Chapter Outline......Page 434 Indications......Page 435 Technique of Procedure......Page 436 Bile Duct Stones......Page 438 Malignant Biliary Strictures......Page 439 Posttraumatic and Postoperative Lesions......Page 441 Cholangitis......Page 442 References......Page 443 Indications......Page 445 Peroral Cholangioscopy......Page 446 Percutaneous-Transhepatic Cholangioscopy......Page 447 Complications......Page 448 References......Page 449 Indications and Limitations......Page 451 Intraluminal EUS-Miniprobes......Page 452 Normal Anatomy......Page 453 Choledocholithiasis......Page 454 Ampullary Carcinomas......Page 455 References......Page 456 Chapter Outline......Page 458 How to Perform a Percutaneous Liver Biopsy......Page 459 Tru-Cut Versus Aspiration Needle......Page 460 Laparoscopic Liver Biopsy......Page 461 Technique and Risk Assessment......Page 462 The Candidate Patient for Liver Biopsy......Page 464 References......Page 465 Chapter Outline......Page 468 Indications......Page 469 Contraindications......Page 470 Vascular Access......Page 471 Complications and Mortality......Page 472 Bleeding Diatheses......Page 473 Pediatrics......Page 475 Liver Transplant Recipients......Page 476 References......Page 477 A Short History of Diagnostic Laparoscopy in Internal Medicine and Hepatology......Page 480 Preparation and Sedation of the Patient......Page 482 Equipment......Page 484 Insertion of Veres-Needle, Pneumoperitoneum and the “Optimal” Gas......Page 485 Inspection of the Abdomen, Introduction of a Second Trocar and Organ Biopsy......Page 487 Risk Profile of Diagnostic Laparoscopy......Page 488 Laparoscopic Inspection......Page 490 Chronic Liver Disease......Page 491 Diagnostic Accuracy of Laparoscopy Versus Percutaneous Liver Biopsy in the Diagnosis of Liver Cirrhosis......Page 492 Laparoscopy and Laparoscopic Liver Biopsy in Acute and Subacute Liver Failure......Page 494 Hepatocellular Carcinoma......Page 495 Biliary Cancer......Page 496 Colon Cancer......Page 498 Carcinoma of the Distal Esophagus, the Esophago-gastric Junction and the Gastric Cardia......Page 499 Pancreatic Cancer......Page 501 References......Page 502 Chapter Outline......Page 506 Contraindications......Page 507 Results......Page 508 Pathologic Values......Page 509 Complications......Page 510 Clinical Significance......Page 511 References......Page 512 Indications......Page 513 Equipment......Page 514 Normal Findings......Page 515 Complications......Page 516 References......Page 517 Section IX: Approaches to Common Hepatobiliary Problems......Page 518 Origin of Pain......Page 519 Conduction of Pain......Page 520 Referred Pain......Page 521 Clinical Evaluation......Page 522 Biliary Pain......Page 523 References......Page 524 Abbreviations......Page 525 General Considerations......Page 526 Interpretation of Abnormal Liver Enzymes......Page 527 Hepatitic Pattern......Page 528 Cholestatic Pattern......Page 534 General Approach to the Patient with Elevated Liver Enzymes......Page 536 References......Page 538 Determination of Liver Size......Page 540 Clinical Approach......Page 541 Imaging Techniques......Page 543 Diagnostic Approach......Page 544 Differential Diagnosis......Page 545 References......Page 547 Etiology and Classifi cation of Cholestasis......Page 548 Pathogenesis......Page 551 First 3–4 Weeks After Bile Duct Obstruction......Page 557 History......Page 558 Xanthelasmas and Xanthomas......Page 559 Laboratory Findings......Page 560 Imaging Techniques......Page 561 Total Parenteral Nutrition......Page 562 Ischemic Cholangiopathy......Page 563 Sepsis......Page 564 Hemolysis......Page 565 Cholestasis in Graft Versus Host Disease......Page 566 Intrahepatic Cholestasis of Pregnancy......Page 567 Increasing Excretion of Pruritogenic Substances......Page 568 Other Substances and Treatment Modalities......Page 569 Estrogens......Page 570 Etiology and Pathogenesis......Page 571 Diagnosis and Differential Diagnosis......Page 573 Hemolysis......Page 575 Crigler–Najjar Syndrome Type I......Page 576 Gilbert’s Syndrome......Page 577 Rotor Syndrome......Page 578 References......Page 579 Epidemiology......Page 582 Etiology......Page 583 Irreversible Structural Changes......Page 584 Increased Splanchnic and Portal Blood Flow......Page 585 Abdominal Sonography......Page 586 Transient Elastography......Page 587 Invasive Measurement of Portal Pressure......Page 588 Therapy......Page 589 References......Page 591 Etiology......Page 592 Pathogenesis......Page 593 Regulation of Extracellular Fluid Volume......Page 594 Efferent Mechanisms......Page 595 “Underfilling or Peripheral Arterial Vasodilation” Hypothesis......Page 597 Diagnosis......Page 598 Differential Diagnosis......Page 600 Prognosis......Page 601 References......Page 602 Index......Page 604 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. Comprehensive Very readable Practical Up to date Provides a rational basis for the practice of hepatology Your one-step resource for clinical hepatology Fast and easy access to common and rare problems encountered in practice Henryk Dancygier is Chief of the Department of Medicine II at the Klinikum Offenbach, and Professor of Medicine at the Goethe University, Frankfurt/Main. He graduated from the University of Heidelberg and trained in Pathology, Internal Medicine, Gastroenterology and Hepatology. He holds degrees from the University of Heidelberg, Frankfurt and the Technical University of Munich and currently is Adjunct Professor of Medicine at the Mount Sinai School of Medicine, New York. Henryk Dancygier is author of a number of books including (English translations) "AIDS - a Clinical Compendium" (1993), "Memorix - Gastroenterology and Hepatology" (1998), "Endosonography in Gastroeneterology: Principles, Techniques, Findings" (1999), "Liver Diseases - the ABC of Therapy" (2003) and "Clinical Hepatology - Diagnosis and Therapy of Hepatobiliary Disorders" (2003) that have been translated into several languages and he has also contributed numerous chapters in Medical Textbooks.